Cleft Lip and Palate - part 4 ppsx

aThe facial morphology in a 2-month-old unoperated infant with unilateral complete cleft lip and palate UCCLP.. So far, we have analyzed infant craniofacial morphology and early craniofa

growth of the cranial vault, the maxilla, and the

mid-dle face and the increase in length and height of the

mandible (Fig 8.1b) Gradual improvement in the

fa-cial profile was recorded by the changes in the fafa-cial

angle and in the angle of convexity The facial angle is

a measure of the degree of protrusion or recession of

the chin In this instance, the facial angle increased

from 64°, at 3 months of age, to 70°, at the age of 3

1/2 years, indicating a reduction by 6° in the

recessive-ness of the chin While the mandible is still in a

retru-sive relation to the rest of the face, the potential for

further improvement with continued growth still

ex-ists

The changes in the angle of convexity were more

interesting This measurement relates the maxilla to

the total facial profile At 3 months of age, the angle ofconvexity was 140°, and at 3 1/2 years it measured at154° The integrated growth of the several areas of theface was such as to improve the overall configuration

of the facial profile Serial photographs at 2 months, at

13 months, and at 3 years of age further testify to thechanges in this child’s face In the last photograph, thepatient is posed beside her older sibling

The changes in the position of the hyoid bone are

of particular interest, insofar as they reflect a change

in the relative position of the tongue The tongue iscomposed of several individual muscles originatingfrom the base of the skull, the mandible, the hyoidbone, and the walls of the pharynx Changes in the po-sition of any of its bony or fibrous attachments would

Fig 8.2. Pierre Robin sequence A series of tracings of the

lat-eral film from 3 months, 1 day of age (0-3-1) to 3 years, 5 months,

12 days (3-5-12) The pharyngeal airway is filled in (black), and

the stippled area denotes the border of the tongue A

tracheoto-my tube is visible in the first two films Soon after birth (3 and

4 months of age) the dorsum of the tongue is visible at a level

above the palatal plane within the palatal cleft space

Posterior-ly, the tongue just above the epiglottis impinged on the airway.

At this level, the airway was almost completely occluded

Com-parison with the remaining three figures, ages 0-8-10, 1-1-0 and 3-5-12, reveals the configuration of these structures under nor-

mal circumstances after closure of the cleft The airway in its posteroanterior dimensions is fairly wide, and the tongue occu- pies a more protrusive relationship to the mandible (Reprinted with permission from [1])

3-5-12 3-5-12

3-5-12

tend to reflect on the position of the tongue

Con-versely, changes in the posture of the tongue would

re-flect on the spatial relations of the mandible and

hy-oid bone Therefore, to study the position of the hyhy-oid

bone is, in a sense, to study the position of the tongue

With growth there occurs a forward and downward

migration of the hyoid bone from the base of the skull

The pattern of changes in the posture of the hyoid

bone observed in this patient sheds further light on

the favorable adjustments consequent to growth

Dur-ing the first 5 months of our studies, the hyoid bone

migrated downward and forward This resulted in an

increase in the angle S-N-H But, from 8 months

on-ward, this angle became fairly stable and the hyoid

bone began to descend principally in a downward

di-rection

Comment: This case was selected to typify the

find-ings in several similar cases, one of which has been

followed to the age of 7 years Not all cases of Pierre

Robin syndrome present such acute histories When

clinical evaluation suggests that there will be no

im-provement or that possibly death may ensue,

tra-cheotomy should be undertaken without hesitation to

prevent further aggravation of the symptoms Once an

adequate respiratory exchange was made possible,

improvement in oxygenation and feeding followed In

such instances, we have recorded rapid growth and

favorable changes in the facial appearance

J.G, a white girl, was referred to the outpatient clinic of

the Cleft Palate Center at the age of 2 months with a

diagnosis of cleft palate and mandibular

micro-gnathia Following an uneventful pregnancy, the

de-livery was normal and at full term The birth weight

was 6 lb 11 oz (3,030 gm) The infant had some

diffi-culty in breathing, but this was relieved by placing her

in a prone position Tube feeding was employed for

the first few days after which she was given bottle

feedings At 6 days of age, the infant was discharged

from the hospital There was no family history of cleft

palate The mother suffered no illness during her

pregnancy

Oral examination revealed an unusually small

tongue closely attached to the floor of the mouth In

the course of our first examination under sedation,

the infant became cyanotic and failed to initiate

mandibular movements sufficient to permit the

pas-sage of air This was relieved immediately by

main-taining forward traction on the tongue and mandible

After about 5 min, the infant recovered control of

mandibular movements, and respiration normally

Aside from this isolated episode, which occurred

un-der sedation, the parents did not report any similardifficulties The child has continued to grow and de-velop at a satisfactory rate

Growth Studies: The casts disclose symmetrical cleft

of the hard and soft palate, extending distally from theregion of the nasopalatine foramen Additional castsobtained at regular intervals revealed that the clefthad narrowed, so that it now presents a narrow V-shaped defect (Figs 8.3, 8.4)

The earliest lateral head palate, at 2 months 10 days

of age, displayed a small mandible and small tongue.The latter was positioned high and above the floor ofthe nose, but relatively remote from the posterior wall

of the pharynx The airway appeared sufficient to tain respiration without any undue effort on the part

sus-of the infant Progressive growth changes recorded

up to the age of 3 years, 4 months, 13 days disclosedmandibular growth and generalized growth in allareas of the face and cranial vault Mandibular growthwas continuous and progressively downward and for-ward During the period studied, from 2 to 40 months

of age, the facial angle increased from 61.5, becomingmore obtuse The angle of convexity increased from147° to 155° Altogether, the changes were in a direc-tion tending to minimize the recessiveness of the chin

in relation to the rest of the face

Comment: Micrognathia by itself is not sufficient toproduce glossoptosis and respiratory embarrassment

If the tongue is large or even normal in size, the smallrecessive mandible will tend to displace the tonguedistally and superiorly It is this displacement thatproduces the respiratory obstruction both into thehypopharynx and into the posterior choanae On theother hand, if the tongue is small, there will be noobstruction of the airway even in the presence of amicrognathic mandible In this instance, the simulta-neous occurrence of micrognathia and microglossiaaverted the respiratory difficulties commonly experi-enced in such instances

The tendency to lose reflex control of the muscles

of respiration and deglutition under anesthesia or dation renders such procedures unusually hazardous

se-in these patients because of the limited reserve It is,therefore, important that such procedures be under-taken with full knowledge and anticipation of possi-ble respiratory obstruction, in order that adequateemergency provisions for the establishment of an air-way be available

8.1.3 Case 3

E.C., a white boy aged 5 weeks, was referred to the

out-patient clinic of the Cleft Palate Center for

longitudi-nal growth studies The delivery had been normal

and at full term His birth weight was 7 lb., 8 oz

(3,400 gm.) There was no history of cleft on either

side of the family No difficulty in breathing was

en-countered, and the infant was discharged from the

hospital on the sixth day After a brief adjustment riod, the infant was readily fed by a combination of ahard nipple and by means of a premature baby bottlenipple

pe-Some snoring sounds were heard, especially as theinfant was placed on its back and the head elevatedwith slight ventroflexion on the chest The infant pre-ferred to sleep on either side, and in these positionsthe snoring sounds were at a minimum This baby

Fig 8.3 a, b. Palatal growth changes in a child with a Pierre

Robin sequence This sequence is characterized by glossoptosis,

micrognathia, and isolated cleft palate In many cases the cleft

plate which is initially wide at birth can spontaneously narrow

with palatal growth.aComputer-generated tracings of the

iso-lated cleft of the hard palate from 2 months and 10 days (0-2-10)

to 6 years, 1 month, and 1 day (6-1-1) The palate was closed at

4 years, 2 months.bSuperimposed tracings of each cast [on the

baseline created by connecting postgingivale points (the

poste-rior limits of the hard palate) and registered at the bisector of

the line] show that the length of the cleft increases with palatal

growth and narrows due to spontaneous growth at the medial

border of the palatal processes Obturators which interfere with

tongue posturing within a relatively small intraoral space are

contraindicated [16]

a

b

showed progressive improvement, and at the age of

5 months he weighed 15 lb., 8 oz (7,030 gm.)

Growth Studies: Two sets of records are available in

this case The first was obtained at the age of 1 month

7 days, and the second at 3 months 25 days of age

The first cast of maxilla revealed a wide parabolic

cleft extending distally from the nasopalatine

fora-men The widest portion of the cleft, at the level of the

maxillary tuberosities, measured 16 mm Although

the second cast exhibited an increase in the length and

width of the palate, there was a decrease of 1.5 mm in

the width of the cleft at its widest portion During the

first examination, the tongue was observed to occupy

at rest the opening into the nasal chambers provided

by the cleft in the palate This was further confirmed

by examining the frontal and lateral views of the head

plates The second series of films indicated that the

tongue was now postured in a more inferior position

and no longer occupied the nasal cavity to the same

extent previously noted This new position of the

tongue could be explained by the downward and

for-ward growth of the mandible that had occurred in the

interim

In the first lateral film, the recessive chin, the

distal-ly and superiordistal-ly malposed tongue, and relativedistal-ly

restricted airway were clearly observed Two and

one-half months later, considerable growth in the

mandible had occurred to improve the facial profile,

alter the posture of the tongue, and increase the

an-teroposterior diameter of the airway The tongue was

no longer in close apposition to the posterior geal wall, and its superior margin did not extend intothe nasal cavity to the degree previously observed Co-incidentally, the mother reported a diminution of thestertorous breathing that had been present

pharyn-Superimposition of the tracings of the bony tures revealed the rapid growth characteristic of thisearly period in life In 2 1/2 months, that cranial vaultand all parts of the face exhibited proportionate in-creases Particularly encouraging was the amount anddirection of growth displayed by the lower jaw.Mandibular growth was responsible not only for re-ducing the glossoptosis and increasing the airway, butfor the improvement in the appearance of this baby’sface

struc-Comment: The problem presented by this baby wasunique and different from the two previous cases ofthe partial obstruction of the airway Diligent nursingcare to determine the most comfortable position forbreathing and feeding may be sufficient to tide suchcases through their critical period In some instancesthe prone positioning and orthostatic feeding sug-gested in the literature are most successful Again, one

is impressed by the remarkable potential for prolificgrowth during this period of life; a potential that isshared by the small mandible It follows then, thatevery effort must be made to permit the realization ofthe baby’s potential for growth by providing an ade-quate airway, which, in turn, facilitates feeding Theclinical course to be followed is varied and depends on

Fig 8.4. A series of cast of the maxillary arch from 2 months 10 days of age to 6 years

1 month Note the progressive narrowing in the lateral dimension of the cleft (Reprinted with permission from [1])

the severity of the symptoms and principally upon the

degree of obstruction of the airway

8.2 Comment

The representative sampling of cases presented

pro-vides an answer to the questions which the study was

designed to solve It is observed from the data

present-ed that the mandible possesses remarkable

potential-ities for growth in patients with the Pierre Robin

syn-drome Thus, all efforts should be directed toward

sustaining life in a metabolically favorable climate in

order that a more physiologic airway may be

estab-lished as growth proceeds With growth, the

glossop-tosis is minimized and spontaneous resolution of the

respiratory and feeding problems occurs It is our

opinion that tracheotomy should be resorted to

promptly if respiratory embarrassment is significant,

in order to achieve a sufficient airway to provide

ade-quate oxygenation This is undoubtedly a life-saving

procedure in some patients

On the basis of our longitudinal growth studies,

certain prognostications concerning the future

growth of the micrognathic mandible are permissible

In most instances, the increment in mandibular

growth, as related to total facial growth, is sufficient to

overcome the extreme recessiveness of the chin that is

observed at birth Since mandibular growth continues

until late adolescence, it is possible to hope for an

es-thetically pleasing profile in adulthood The

manage-ment of the cleft palate has been in keeping with the

established criteria for the treatment of palatal

de-fects

The lateral cephalometric film served as a valuable

diagnostic tool in estimating the degree of

obstruc-tion of the airway as a result of the glossoptosis In our

experience, there was a high positive correlation

be-tween the degree of obstruction revealed in the x-ray

film and the incidence and severity of the respiratory

difficulties When obstruction of the air passage was

complete and the tongue was practically in contact

with the posterior wall of the pharynx, tracheotomy

was recommended as a life saving procedure If the

obstruction was incomplete, more conservative

meas-ures were employed Care was taken to ascertain the

most comfortable postures for breathing and feeding

for the individual case, and the nurse or parent was

carefully instructed in the care of the infant

Appro-priate nipples were selected to minimize the energy

expended by the infant in the feeding process

In the course of these studies, we were aware of an

obvious objection to placing so much reliance on

these roentgenograms Since many of these films were

obtained under mild sedation, was it not possible that

the posture of the mandible or of the tongue might

have been altered by the sedative? Secondly, the filmdepicted a static view of the airway and representedonly two dimensions Did this view properly reflectthe kinetic ability of the infant to manipulate thetongue and jaw hence the consistent correlation be-tween the findings in our films and the clinical state?Moreover, when the films were repeated in the sameinfant without sedation, similar postures were record-

ed for the structures under analysis It was importantthat the postures of the head in relation to the neck bekept constant Dorsiflexion or ventroflexion of thehead varied to posture of the mandible and tongueand produced changes in the configuration of the air-way To indicate alterations in the posture of the head

to the neck, our tracings purposely included at leastthe first two cervical vertebrae

We recognize that few institutions possess metric roentgenographic equipment Therefore, weshould like to point out that an ordinary lateral filmobtained by carefully positioning the infant can pro-vide useful diagnostic data To minimize enlargement,

cephalo-a tcephalo-arget-object distcephalo-ance of cephalo-at lecephalo-ast 3 ft (90 cm) is ommended For the sake of definition and to furtherdecrease enlargement, the object film distance should

rec-be kept at a minimum Sjölin [15] has published esting films to describe his experiences with a case ofmicrognathia Although his films did not permitquantification of the growth changes, they were ade-quate for diagnostic purposes

inter-A number of papers in the literature claim to ulate” the growth of the mandible by a variety of me-chanical devices or surgical procedures For example,

“stim-a speci“stim-al nursing bottle w“stim-as designed to force the fant to protrude his jaw in order to obtain nourish-ment and, by this protrusion, to stimulate mandibulargrowth [4] From our data, we would conclude that thenursing care enabled the infant to survive untilmandibular growth was sufficient to provide a moreadequate airway

in-In another report, continuous traction on themandible was maintained by circumferential wiringaround the symphysis The authors claimed growth-stimulating properties for this procedure [10] Fromthe findings in our series, it would seem that mandi-bular growth probably occurred spontaneously andnot because of the stimulus provided by surgical trac-tion

The important and prime objective in the care ofthese children is to provide an airway If possible, thisshould be accomplished with a minimum of trauma.Secondly, the infant’s total needs should be assessed toprovide optimal conditions for somatic growth As the potential for growth is permitted to express itself,the chin grows downward and forward away from thebase of the skull With this pattern of growth, ade-quate space for the tongue is provided, the airway

enlarges, and there follows a spontaneous resolution

of the symptoms Also, there are progressive

improve-ments in the facial appearance

There is another dimension to the abnormal

pos-ture of the tongue, as observed in these patients, that

merits discussion Not only does the tongue block the

pharyngeal processes and hence prevent their fusion

The high incidence of micrognathia in the population

of clefts involving only the hard and soft palate lends

support to this theory Mandibular micrognathia is a

physiological finding in early intrauterine life If for

some reason the micrognathia persists and fails to

carry the tongue down and out of the nasal cavity, a

cleft in the palate might result

In early postnatal life, the tongue acts to keep the

cleft palatal processes apart As the tongue descends

with mandibular growth and no longer forcefully

in-trudes itself into the nasal cavity, the palatal processes

tend to approximate in the midline Fusion of the

palatal processes cannot occur, but the narrowing in

the clefts is recorded fact

8.3 Summary and Conclusions

The development of the accurate techniques for

cephalometric roentgenography of infants has made

possible a longitudinal study of the growth of the

micrognathic mandible As a result of these studies,

useful diagnostic and prognostic information has

been obtained to provide a rationale for the

manage-ment of individual cases

The lateral cephalometric roentgenogram is a

valu-able diagnostic aid in assessing the severity of the

glossoptosis and its obstruction of the airway A

defi-nite correlation exists between the degree of

constric-tion of the airway and the severity of the clinical state

On the basis of these findings, it is possible to

recom-mend either conservative management or

tracheoto-my in extreme situations, or distraction osteogenesis

Three cases, out of a larger series of similar cases, were

presented to indicate the spectrum of variations to be

encountered

In all instances, it was found that where an

ade-quate metabolic situation was provided and the infant

gained weight, mandibular growth during the first few

months was sufficient to provide for a natural

resolu-tion of the symptoms attending the glossoptosis

Longitudinal records have indicated that

mandibu-lar growth is proportionally adequate to reduce the

retrognathic profile and provide an esthetically monious facial appearance

har-Based on investigations performed during thetenure of Special Research Fellowship from the Na-tional Institute of Dental Research Institutes of Health(Dr Pruzansky, Senior Assistant Dental Surgeon [R],United States Public Health Service, National Institute

of Dental Research, Department of Health, Educationand Welfare)

(microg-5 Davis AD, Dunn R Micrognathis: a surgical treatment for correction in early infancy Am J Dis Child 1933; 45:799– 806.

6 Callister AC Hypoplasia of the mandible (micrognathy) with cleft palate: treatment in early infancy with skeletal traction Am J Dis Child 1937; 53:1057–1064.

7 Lleweyllyn JS, Biggs AD Hypoplasia of the mandible: report

of case, with resume of literature and suggestions for ified form treatment Am J Dis Child 1943; 65:440.

mod-8 Douglas B The treatment of micrognathia associated with obstruction by plastic procedure Plast Reconstruct Surg 1946; 1:300.

9 Nisenson A Receding chin and glossoptosis: cause of ratory difficulty in infant J Pediat 1948; 32:397–401.

respi-10 Longmire WP Jr, Sandford MC Stimulation of mandibular growth in congenital micrognathia by traction Am J Dis Child 1949; 78:750–755.

11 May H, Chun LT Congenial ankyloglossia (tongue-tie) sociated with glossoptosis ( retruded mandible) and pala- tum fissum (clift palate) Pediatrics 1948; 2:685–687.

as-12 Brodie AG On the growth pattern of the human head from the 3rd month to the 8th year of life Am J Anat 1941; 68: 209–262.

13 Brodie AG Behavior of normal and abnormal facial growth patterns Am J Orthod 1941; 27:633–655.

14 Pruzansky S Description, classification and analysis of operated clefts of the lip and palate 1953; Am J Orthod 39:590.

un-15 Sjolin S Hypoplasia of the mandible as a cause of

respirato-ry difficulties in the infant Acta Paediat 1950; 39:255– 261.

16 Berkowitz S Cleft lip and palate-perspectives in ment 1st ed Little, Brown; 1996.

manage-9.1 Introduction

Congenital clefts of the lip and/or palate can arise in

isolation or together with other malformations

(syn-dromes) [28] This chapter deals solely with

“nonsyn-dromic” clefts

Both individuals with unoperated and operated

clefts have a face which differs from those of

unaffect-ed individuals Since the introduction of

roentgen-cephalometry more than 70 years ago [8] hundreds of

cephalometric studies, including both unoperated

and operated cleft individuals have suggested that

some deviations are directly caused by the primary

anomaly, while others are caused by the surgical

inter-ventions and the following dysplastic and

compensa-tory growth of the facial bones [e.g., 1, 3, 4–10, 12, 15,

18–27, 29, 30, 48, 50, 51, 53–55, 57, 59–61] However, the

relative importance of the intrinsic factors, the

iatro-genic factors, and the functional or adaptive factors

for the facial development is still unclear There are

probably several reasons for this Firstly,

comprehen-sive knowledge of craniofacial morphogenesis in cleft

newborns or infants before surgery, based on large,

consecutive, well-controlled samples, is very scarce

This situation is not surprising since, in developed

countries, the cleft of the lip is surgically treated

with-in the first couple of months after birth Thus, the

pos-sible period of examining the unoperated state is

short and several methodological problems are

in-volved Secondly, the cephalometric analyses are most

often limited to the lateral projection using simplistic

cephalometric analyses, typically based on 15–20

reference points, and almost invariably measuring

maxillary prognathism as the S-N-A angle or similar

measurements to the premaxilla, and the use of infant

cephalometry has been very limited These authors

are of the opinion that incomplete knowledge about

the intrinsic factors related to the cleft anomaly has

automatically lead to excessive emphasis on the

im-portance of iatrogenic and adaptive factors in facial

development of cleft children

9.2 The Danish Experience

In the middle of the 1970s we decided to take tage of the very favorable sampling conditions in Den-mark in an effort to contribute to the question of thecharacteristics of facial growth and development inchildren born with clefts [42] In Denmark, for morethan 65 years, all newborns with facial clefts have beenrecorded at the Institutes for Speech Disorders inCopenhagen and Århus Repeated follow-up examina-tions have shown that the registration of clefts in Denmark is highly reliable and nearly complete Thepopulation is homogeneous and stable, and only veryfew children are lost to follow-up Furthermore, allprimary cleft surgery is performed in one hospital

advan-by one surgeon

Inspired by [52] we constructed a three-projectioninfant cephalometer, which can obtain truly orthogo-nal lateral, frontal, and axial cephalograms [43] Acomprehensive cephalometric analysis system wasdeveloped including all craniofacial regions (calvaria,cranial base, orbits, maxilla, mandible, airway, cervicalspine, and soft-tissue profile) [44, 32, 36], and themethod was validated [36] Furthermore, new meth-ods of visualization of differences in craniofacial mor-phology and growth between different groups weredeveloped using mean plots [36, 44], color-codedvectorgrams [36], and color-coded surfaces on a 3DCT-model [16]

During the 6 years from 1976 to 1981, there were359,027 live births in Denmark A total of 678 new-borns of Northern European ancestry with cleft lip,cleft palate, or both were registered in the period.Twenty-four infants died before 22 months of age, andfor practical reasons material uptake had to be omit-ted in some patients with isolated cleft palate Onlynonsyndromic clefts were included in the study, but

602 of the 678 children (about 90%) were examined

by us [42] and nearly all at both 2 months of age(before any surgical or orthopedic treatment) and

at 22 months of age (before closure of the posterior

Characteristics of Facial Morphology

and Growth in Infants with Clefts

Sven Kreiborg, Nuno V Hermann, Tron A Darvann

9

palate in the children with clefts of the secondary

palate) All children were treated by the same surgeon

(Dr Poul Fogh-Andersen), and in the children with

cleft of the primary palate the cleft lip was, in all

cas-es, closed using a Tennison procedure One third of

the children had isolated cleft lip (CL), about 40% hadcombined cleft lip and palate (CLP), and about 27%had isolated cleft palate (CP) The clefts were subclas-sified according to the method of Jensen et al [42]

Table 9.1. Summary and comparison of the most important findings in the primary anomaly in children with RS, ICP, BCCLP, and UCCLP*

Maxilla

Decreased length measured to premaxilla (sp-pm)a + 1 + – 2 –

Retrognathia measured to premaxilla (s-n-ss) b + + – 3 –

Retrognathia measured to base of jaw (s-n-ci) d + + + +

* RS, Robin Sequence; ICP, Isolated Cleft Palate; BCCLP, Bilateral Complete Cleft Lip and Palate; UCCLP; Unilateral Complete

Cleft Lip and Palate.

1 The deviation from the norm is shown as + or –, meaning, e.g., that decreased total length of the maxilla was observed in the ICP and RS groups but not in the UCCLP group, and that the length of the mandible is decreased in the UCCLP group, very decreased in the ICP and BCCLP groups, and severely decreased in the RS group.

2 The total length was significantly increased.

3 The prognathism was increased measured to the premaxilla.

a sp-pm: Anterior nasal spine to point pterygomaxillare.

b s-n-ss: S-N-A.

c ci-pm: Point crista infrazygomatica to point pterygomaxillare.

d s-n-ci: Maxillary prognathism measured to the infrazygomatic crest.

Fig 9.1 aThe facial morphology in a 2-month-old unoperated infant with unilateral complete cleft lip and palate (UCCLP).

bThe facial morphology in a 2-month-old unoperated infant with bilateral complete cleft lip and palate (BCCLP)

In the 602 children included in the study,

cephalo-grams were obtained in the lateral, frontal, and axial

projections by three experienced orthodontists (Dr

Birgit Leth Jensen, Dr Erik Dahl, and Dr Sven

Krei-borg) In addition, impressions were made of the

maxilla, and anthropometric registrations (body

height, body length, and head circumference) were

carried out The results of the cephalometric analyses

have been presented in a number of publications

[13, 14, 17, 33–41, 45–47] So far, we have analyzed

infant craniofacial morphology and early craniofacial

growth in detail in three dimensions in the following

groups: incomplete unilateral cleft lip (UICL), isolated

cleft palate (ICP), Robin sequence (RS), unilateral

complete cleft lip and palate (UCCLP) (Fig 9.1a), and

bilateral complete cleft lip and palate (BCCLP)

(Fig 9.1b) In the following, we shall summarize our

findings, with emphasis on the unoperated infant to

shed light on the intrinsic factors related to the cleft

condition (see Fig 9.2 and Table 9.1), and compare

them to data in the literature on unoperated

adoles-cents and adults with clefts

9.2.1 Cleft Lip (CL)

Isolated CL involves only structures of the embryonic

primary palate The craniofacial morphology in CL

subjects has been shown to be fairly normal except for

the small region of the cleft including the premaxilla

and the incisors In unoperated bilateral complete CL

the premaxilla may, however, protrude markedly In

unilateral, complete CL the protrusion is less

pro-nounced but asymmetric In subjects with

unoperat-ed, unilateral, incomplete cleft lip UICL the protrusion

of the premaxilla is negligible [33] The interorbital

distance in CL subjects seems to be slightly increased

compared to the norm (11) The basal part of the

maxilla has a normal prognathism in relation to the

anterior cranial base, and the mandible is of normal

size, shape, and inclination [12; 33] Following lip

sur-gery, the premaxilla is molded into a normal position,

and maxillary prognathism measured to the point A

or ss (subspinale) is normal [12, 31, 33–35] In

conclu-sion, subjects with UICL have a very close to normal

craniofacial morphology from infancy to adult age,

and consequently, we have used our group of infants

with UICL as a control group in the study of

devia-tions in craniofacial morphology and growth of

infants and young children with ICP, RS, UCCLP, and

BCCLP, since no actual normative cephalometric data

for Danish infants and young children are available

9.2.2 Cleft Palate (CP)

Isolated cleft palate (ICP) involves only structures ofthe embryonic secondary palate In Fig 9.2a, themean facial diagrams of the ICP group is superim-posed on the mean facial diagram of a group of age-matched infants with UICL (control group) The ma-jor deviations in the ICP group were: reduced lengthand posterior height of the maxilla; maxillary retro-gnathia; increased width of the maxilla and the nasalcavity; and reduced length of the mandible withmandibular retrognathia Thus, the ICP group re-

vealed bimaxillary retrognathia The sagittal jaw

rela-tionship was, however, normal In addition, in the ICPgroup the upper airway dimensions were reduced

Bimaxillary retrognathia and a short mandiblewere previously documented in unoperated olderchildren [60] and adults with ICP [12, 2]

Robin sequence (RS) is defined as a triad of toms: isolated cleft palate, micrognathia, and glossop-tosis [28] RS may be part of several syndromes, e.g.,Treacher-Collins syndrome [11, 46] In this chapter,only nonsyndromic cases of RS will be discussed Weconsider this group as a subgroup of the ICP group[39] In Fig 9.2b, the mean facial diagram of the RSgroup at 2 months of age is superimposed on the meanfacial diagram of the control group The major devia-tions in the RS group were: decreased length and pos-terior height of the maxilla; maxillary retrognathia;increased width of the maxilla and nasal cavity; veryshort mandible with marked mandibular retro-

symp-gnathia Thus, the RS group revealed bimaxillary retrognathia; the retrognathia was, however, most

marked for the mandible and the sagittal jaw relationwas increased In addition, the RS group had a signi-

ficantly smaller cranial base angle (n-s-ba) resulting

in a smaller depth of the bony nasopharynx than thecontrols, and the upper airway dimensions weremarkedly reduced The degree of maxillary retro-gnathia was similar in the RS and the ICP group How-ever, the mandibular retrognathia in the RS group waseven more marked than in the ICP subjects It wouldseem that RS subjects probably represent the extremepart of the ICP population in terms of mandibularretrognathia and upper airway constriction As men-tioned above, we consider the RS group as a specialsubgroup of the ICP group Accordingly, we believethe bimaxillary retrognathia to be intrinsically associ-ated with the cleft of the secondary palate

c

b

a

9.2.4 Cleft Lip and Palate (CLP)

Combined clefts of the lip, alveolus, and palate involve

structures of both the embryonic primary palate and

secondary palate In Fig 9.2c the mean craniofacial

morphology in 2-month-old unoperated infants with

unilateral complete cleft lip and palate (UCCLP) was

compared to the control group [33] The major

devia-tions in the UCCLP group were: decreased posterior

length and height of the maxilla; retrognathia of the

basal part of the maxilla with relative protrusion of

the premaxilla; the width of the maxilla and nasal

cavity was markedly increased and the premaxilla

deviated to the noncleft side; the mandible was short

and retrognathic Thus, the UCCLP group revealed

bimaxillary retrognathia combined with a relative

protrusion of the premaxilla, which deviated to the

noncleft side In addition, in the UCCLP group the

upper airway dimensions were reduced

Increased width of the midface and nasal cavity

was previously reported in unoperated UCCLP infants

[31] and in unoperated adults with UCCLP [49]

Rela-tive protrusion and asymmetry of the premaxilla have

also been reported in unoperated UCCLP children,

adolescents, and adults [5–7, 9, 50, 51] The relative

protrusion and deviation is probably due to

over-growth in the premaxillary-vomerine complex [53,

22, 24], due to the lack of structural integrity of the

maxilla on one side This relative protrusion of the

premaxilla explains why we found the measurements

s-n-ans (S-N-ANS) and s-n-ss (S-N-A) in the infant

UCCLP group to be comparable to the values in the

control group, despite the fact that the UCCLP group

showed significant maxillary retrognathia measured

to the basal part of the maxilla

Dahl et al [13] and Hermann et al [39, 40] analyzed

facial morphology in 2-month-old infants with

unop-erated bilateral complete cleft lip and palate from our

sample Fig 9.2d illustrates the mean facial diagram

of the BCCLP group superimposed on the mean facial

diagram of the control group The most obvious

fea-tures in the BCCLP group were: protrusion of the

pre-maxilla both in relation to the anterior cranial base

and in relation to the basal part of the maxilla; the

length of the basal part of the maxilla and posteriormaxillary height were decreased; retrognathia of thebasal part of the maxilla; markedly increased width ofthe maxilla and nasal cavity; a short and retrognathic

mandible Thus, the BCCLP group revealed lary retrognathia with a truly protruding premaxilla.

bimaxil-In other words, the protruding premaxilla was

situat-ed in a totally retrognathic face with a fairly normalsagittal jaw relationship In addition, the upper airwaydimensions were reduced

The extreme protrusion of the premaxilla is bably the result of marked overgrowth in the pre-maxillary-vomerine complex secondary to total lack

pro-of structural integrity in the region

For comparison, Mars and Houston [48] and

da Silva Filho et al [58] described groups of adult unoperated patients with BCCLP and found extremeprotrusion of the premaxilla and a very convex profilemeasured as the ANB-angle No measurements wereperformed to describe the position of the body ofthe maxilla Da Silva-Filho et al [56, 58] also found the mandible to be short and retrognathic and discussed whether this finding was related to the primary anomaly or if it was caused by secondaryfunctional adaptations

The retrognathia of the basal part of the maxilla,and the short and retrognathic mandible found in oursample are, in our opinion, variations intrinsically associated with the cleft of the secondary palate asdiscussed above

9.3 Discussion and Conclusions

The Danish study of craniofacial morphology in treated cleft infants is the hitherto most comprehen-sive and well-controlled, since it covers a whole popu-lation, which is homogeneous and in which centralregistration of clefts has been carried out for morethan 65 years; a registration which has been shown to

un-be highly reliable and nearly complete Furthermore,all cleft infants are surgically treated at one hospital byone surgeon using the same techniques All infantswere examined with state-of-the-art three-projectioncephalometry using the hitherto most comprehensivecephalometric analysis covering all craniofacial re-gions and the methods were validated The study in-cluded more than 600 children, and even after break-down into subgroups, the sample sizes were adequatefor statistical testing (except maybe for the RS group).Based on these facts, the findings related to the infantcraniofacial morphology at 2 months of age, prior toany surgical or orthopedic treatment, must be consid-ered to represent the “true” malformation, primarilycaused by intrinsic factors

Fig 9.2 a–d. Mean plots in three projections (lateral, frontal,

and axial) of the four different cleft groups superimposed on

the control group with UICL The lateral mean plots are aligned

on the n-s line and registered at s The frontal mean plots are

aligned on the latero-orbital line and registered at the center

point of that line The axial mean plots are aligned on a line

between the two tuber points and registered at the center point

of that line Superimposition of the mean plots for the

2-month-old aICP and UICL groups,bRS and UICL groups,cUCCLP

and UICL groups, and dBCCLP and UICL groups

䊴

In Table 9.1 the most important findings in the

pri-mary anomaly in the Danish infants with RS, ICP,

BCCLP, and UCCLP are given, revealing a rather clear

pattern The findings support the suggestion of Dahl

[12] and others, that facial clefts should be classified

based on the embryonic facial development, i.e., into

clefts involving the primary palate only (CL), clefts

involving the secondary palate only (CP), and clefts

involving structures of both the primary and the

sec-ondary palate (CLP) The postnatal facial morphology

in these groups differs greatly Infants with cleft of the

secondary palate, with or without cleft of the primary

palate, shared a number of characteristic

morpholo-gical traits when compared to the norm: decreased

posterior length of the maxilla; maxillary

retro-gnathia; decreased posterior height of the maxilla;

increased width of the maxilla and the nasal cavity;

decreased length of the mandible; mandibular

retro-gnathia; and reduced size of the pharyngeal airway

As seen from Table 9.1 and Fig 9.3, the mandibular

involvement was most pronounced in the RS group

followed by the ICP and BCCLP groups, and, finally,

the UCCLP group A similar pattern was observed for

the reduced size of the pharyngeal airway As for the

maxilla, the increased width of the maxilla and the

nasal cavity was most pronounced in the groups with

clefts of both the secondary and the primary palate,

i.e., BCCLP and UCCLP None of these groups showed

decreased total length of the maxilla or retrognathia

of the maxilla when measured to the premaxilla; the

reason for this being a true and relative protrusion of

the premaxilla, respectively

In conclusion, a short and retrognathic mandible

was a constant finding in infants with cleft of the

sec-ondary palate The reduction in size of the pharyngeal

airway in infants with cleft of the secondary palate

was clearly related to the short and retrognathic

mandible, being most severe in the RS group, which

had the added effect of a reduction in the cranial baseangle But, in principle, all four groups had restrictedupper airways as part of the primary anomaly The in-creased width of the maxilla and nasal cavity wasmost pronounced in the groups which also had cleft ofthe primary palate (UCCLP and BCCLP) The UCCLPgroup was also characterized by relative protrusion ofthe premaxilla which was positioned asymmetrically,deviating to the noncleft side, whereas in the BCCLPgroup the premaxilla showed true protrusion both inrelation to the basal part of the maxilla (the lateralsegments) and to the anterior cranial base On aver-age, the premaxilla was found to be positioned in themidline in this group, although most of the individualcases showed some degree of asymmetry The protru-sion of the premaxilla is suggested to be secondary tothe primary anomaly of clefting, allowing for over-growth in the premaxillary-vomerine complex, due

to partial or total lack of anatomical integrity in theregion

It has been the aim of this chapter to summarizeour findings about the intrinsic variations in facialmorphology associated with the different types ofcleft malformations to form a basis for valid estima-tions of the amount of surgical iatrogenesis, especial-

ly to the maxillary development, introduced by ent surgical procedures and regimes, including thetiming of treatment In Fig 9.4, the growth changes ofthe craniofacial skeleton from 2 to 22 months of age inthe UCCLP group has been compared to the UICLgroup (control group) using color-coded surfaces on a3D CT-model In both groups the cleft lip was surgi-cally closed just after the examination at 2 months ofage using a Tennison procedure In the UCCLP group,the anterior part of the palate was closed with a vomerflap at the same time The method of producing theillustrations will be given below

differ-9.3.1 Intuitive Visualization of the Location

of Growth Differences

Cephalometric measurements in three projectionsprovided growth vectors at each of the 279 (230 skele-tal and 49 soft tissue) anatomical landmarks Thegrowth vectors, computed as the vector difference between corresponding landmark locations at theages 2 and 22 months, respectively, after alignment to

a common coordinate system [35], have been used

to form average growth patterns previously shown

in Hermann et al [34, 33] (UICL, UCCLP) and mann et al [41] (UICL, BCCLP) Results of compar-isons of growth between the UCCLP and the BCCLPgroups, respectively, and the control group (UICL)have been shown as color-coded average growth patterns in Hermann et al [33, 34] (UCCLP vs UICL)

Her-Fig 9.3. Mean plots of the mandible in the RS, ICP, BCCLP,

UCCLP, and UICL groups Superimposition was made on the

mandibular line (ML) registered at pogonion (pg)

Fig 9.4 a–d. 3D visualization of locations of growth

differ-ences Locations where UCCLP growth differs significantly

(p <0.01) from UICL growth (2–22 months of age) are colored

red (UCCLP <UICL) or blue (UCCLP >UICL) The surface

reconstruction shown is of a noncleft subject of comparable

age, and is used solely for illustration Cleft side is on patient’s left in the figures Locations of differences in the amagnitude of growth,bsagittal,cvertical and dtransverse growth compo- nents are shown

d

c

b

a

and Hermann et al [41] (BCCLP vs UICL) These

color-coded growth diagrams disclosed the locations

of significantly different growth (1%, 5%, and 10%

levels) in the study group when compared to a

refer-ence group, and the diagrams were shown separately

for each of the 3 projections (lateral, frontal and

axi-al), as well as for the growth magnitude and the two

growth directions (x and y in each of the projections,

respectively) In order to facilitate the effective

com-prehension of these diagrams, the locations of

signif-icant difference are color-coded onto the surface of a

skull reconstructed from a CT scan of a single

(non-cleft) infant As an example, Fig 9.4 shows such

color-coded surfaces for the comparison of the UCCLP with

the UICL (control group) The color-coded surfaceswere created by landmarking the 3D CT scan of thesingle noncleft infant at locations corresponding tothe 230 skeletal cephalometric landmarks and colorcoding the surface in the vicinity of each landmark

by a color corresponding to the significance of thegrowth difference The landmark locations are shown

in Fig 9.5 A color table was chosen such that colorssignify Student’s t-test p values smaller than 0.01 Bluecolors correspond to locations where the study groupexhibits larger growth than the control group, whilethe opposite is the case at locations colored red.Regions without any significant differences betweenthe two groups remained gray In the UICL and UCCLP

Fig 9.5. 3D landmark locations corresponding to the skeletal landmarks used in the three-projection cephalometric analysis as well as for creating the color-coded surfaces in Fig 9.4

groups the frontal and axial projection data were

mirrored in order to have all clefts on the left side

Accordingly, the cleft is on the patient’s left side in

Fig 9.4 The spatial extent of colored surface area in

the vicinity of a landmark was governed by the

dis-tance to its closest landmark, and a maximum extent

(spherically from landmark position) was chosen as

40 mm Color-coded skulls are shown for differences

in growth magnitude, as well as for each of the three

growth directions (sagittal, vertical and transverse)

The colors for sagittal growth differences were

com-puted from the x-component of the growth vectors in

the lateral cephalometric projection and the

y-com-ponent of the growth vectors in the axial projection

The colors for vertical growth differences were

com-puted from the y-component of the growth vectors in

the lateral projection and the y-component of the

growth vectors in the frontal projection The colors

for transverse growth differences were computed

from the x-component of the growth vectors in the

frontal projection and the x-component of the growth

vectors in the axial projection The method of color

coding has previously been described and applied

for visualization of the growth differences between

UCCLP and UICL in Darvann et al [16]

Secondary to surgical closure of the lip at 2 months

of age in the UCCLP group we found that the

pre-maxilla was molded into place, demasking the

in-trinsic maxillary retrognathia and leading to a normal

sagittal jaw relationship at 22 months of age Maxillary

growth was, besides the premaxillary molding,

characterized by smaller vertical growth on the cleft

side and reduced transverse development, which

could probably be related to the effects of surgery

The amount of mandibular growth was similar in the

two groups However, the direction of growth was

slightly more vertical in the UCCLP group This

growth pattern was probably related to the intrinsic

pattern of mandibular development Otherwise,

cra-niofacial growth seemed to be very similar in the two

groups

We found that surgery to the lip and anterior

part of the hard palate at 2 months of age in UCCLP

subjects seemed to influence the development of the

maxillary complex, as observed at 22 months of age, in

a number of beneficial ways: the premaxilla was no

longer relatively protruding, and it was less

asymme-tric; the nasal septum deviated less toward the noncleft

side; the width of the nasal cavity and the posterior

part of the maxilla became relatively more normal;

and the transverse position of the lateral maxillary

segment on the noncleft side was closer to normal

The posterior height of the maxilla was, however, still

reduced to the same degree; the mandible was still

short and retrognathic to the same degree; and

bimaxillary retrognathia was still present The only

iatrogenic effect observed was that the lateral lary segment on the cleft side had become displacedtoward the midsagittal plane anteriorly, resulting in amuch too narrow dental arch at the level of the deci-duous canine [35]

maxil-It is noteworthy that several studies of older, operated UCCLP children and adults find the maxil-

un-lary prognathism to be within normal limits or evenincreased when compared to normative data [9, 48, 50,51].All these studies, however, only measure maxillaryprognathism to the A-point or to the point ANS, bothlocated in the relatively protruding premaxilla Ortiz-Monasterio et al [50] concluded based on their find-ings in unoperated adults with UCCLP, that: “The em-bryonic factor responsible for the facial cleft does notinterfere with maxillary growth This evidence leads

us to believe that growth defects of the middle third ofthe face so frequently seen are caused by early or re-peated and aggressive surgery.” We disagree some-what with this conclusion Based on our studies ofinfants with UCCLP, it would seem that maxillaryretrognathia in this group is part of the intrinsic vari-ations associated with the cleft malformation of thesecondary palate In the unoperated infant and theunoperated adult the maxillary retrognathia is, how-ever, partly masked by relative protrusion of the pre-maxilla, secondary to overgrowth in the premaxillary-vomerine suture Surgical closure of the lip at

2 months of age molds the premaxilla back into place,demasking the maxillary retrognathia Thus in the 22-month-old lip-operated UCCLP group, it is our opin-ion that the bimaxillary retrognathia illustrates thefacial type characteristic of the group rather than aniatrogenic effect of cleft surgery [34, 35] Thus, we donot consider the maxillary retrognathia observed at

22 months of age as the result of surgical iatrogenesis,rather we believe it represents a normalization of the

“intrinsic facial type” characteristic of subjects withUCCLP; and at 22 months of age the face is still har-monious with a normal sagittal jaw relationship Wehave, at this point in time, not re-examined the sample

at older ages, and can, therefore, not comment onfacial growth and signs

In conclusion, we are not arguing that cleft surgerydoes not lead to disturbed maxillary developmentduring the growth period But we are suggesting thatsubjects with cleft of the secondary palate have a spe-cial “intrinsic” facial type, primarily characterized bybimaxillary retrognathia and increased maxillarywidth.We are speculating that this facial type could be

a “liability factor” increasing the probability of CP orCLP [33, 34] Finally, we suggest that when outcome ofcleft surgery in CLP subjects is evaluated at adoles-cence or adulthood, comparisons should not be made

to normal standards, but rather to the adolescent andadult morphology seen in CP subjects

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Studies of subjects with unoperated clefts of the lip

and palate have been undertaken for the past 80 years

[1–29]

Adults with unoperated clefts of the lip and palate

provide the ideal control group for investigators

studying the natural history of facial growth and

mor-phology in these subjects The absence of surgical

intervention provides an opportunity to study the

outcome of facial growth, morphology, and speech,

using an absolute comparative baseline.An evaluation

of the intrinsic versus the potential iatrogenic

influ-ences can be separately analyzed, when this group is

compared to conventionally operated subjects They

also highlight the discrepancies in health care

pro-vision between the wealthy western world and the

developing/least developed world

Withdrawal of surgery for research purposes

would be unethical where such facilities are available

Likewise, assignment to surgical or nonsurgical

man-agement programs on a prospective random

alloca-tion basis could not be permitted For these reasons,

studies on the unoperated subject have been made on

individuals from the developing world, where surgery

may not be readily available This has led to many of

the limitations listed below being evident in such

studies Additional limitations have been imposed by

the authors themselves, using inappropriate

manage-ment of the material collected

The difficulties experienced by research workers in

the field of cleft lip and palate are seen in the

limita-tions of many studies These may be summarized as

follows:

1 Small sample size

2 Wide age distribution

3 A narrow age distribution of very young subjects

4 Mixtures of unoperated, partially operated,

late operated and early operated subjects

5 Mixtures of subjects from different cleft types

6 Males and females grouped together

7 No controls from the normal population

8 Controls of treated patients from the same tion seldom available (applicable to studies ofunoperated versus operated subjects)

popula-9 Few postoperative follow-up studies where latesurgery has been performed

Unquestionably, the major problem is that of smallsample size, which has been responsible for the inap-propriate handling of the available data

The general consensus in the literature relating tothe unoperated UCLP subject suggests that they havethe potential for near normal facial growth However,serious limitations are obvious in the analysis of thedata, especially by attempts to increase the sample size

by pooling nonhomogeneous groups Despite tions of all previous studies into facial growth in thetotally unoperated cleft lip and palate subject, the re-sults suggest that facial growth proceeds reasonablywell Midface depth, the parameter of most concern inCLP, is not compromised in unoperated subjects

limita-10.1 Sri Lankan Cleft Lip and Palate Project

The Sri Lankan Cleft Lip and Palate Project has oped as the largest multidisciplinary surgical and re-search program concerned with the unoperated cleftlip and palate subject [30] Since 1984, extensiverecords have been collected on over 1,000 subjects, onwhom 820 operations have been performed Therehave been 13 visits from 1984 to 2002 The aims have always been threefold: treatment, teaching,and research based on a multidisciplinary team Over

devel-50 professionals have been involved, many havingattended on over 10 visits

The unresolved conflicts of opinion regarding theaetiology of facial growth distortion in repaired cleftlip and palate subjects were the main reasons for the establishment of this Project The possibility of

in the Unoperated Cleft Lip and Palate Subject:

The Sri Lanka Study

Michael Mars

10

addressing these questions by studying “nature’s

ex-periment” on hundreds of unoperated and

late-oper-ated subjects of all ages from birth to old age was

unique Such an opportunity is rare Investigators

usually have to resort to animal experiments in order

to provide sufficient numbers in a controlled manner

[31, 32]

This chapter, which deals with totally unoperated

subjects from Sri Lanka presents a descriptive account

of facial morphology, a cephalometric analysis (UCLP,

BCLP, and ICP groups), a GOSLON yardstick analysis

(UCLP group), and a reflex microscopic analysis of

dental study models (UCLP and ICP groups) The

material comprises 55 UCLP, 23 BCLP, and 41 ICP

sub-jects (Table 10.1), and 119 healthy control Sri Lankan

subjects (Table 10.2) All unoperated subjects

de-scribed in this chapter were over 13 years of age when

they first presented

Because the onset of puberty is some two to three

years later in Sri Lanka than in the West, it was

neces-sary to separate pre- and postpubertal groups [33]

Further, there are significant differences in the timing

of the onset and termination of puberty between

males and females This, and the fact that there are

some significant differences in facial morphology

be-tween the sexes, has necessitated their separate

analy-sis

10.1.1 Controls

One hundred nineteen healthy adult Sri Lankan cleft subjects provided Control data These comprised

non-40 male medical students and 79 female hospital

nurs-es and medical students The Controls were aged tween 20 and 30 years of age (Figs 10.1, 10.2)

be-10.1.2 Records Collected for Study

Lateral skull radiographs and dental study models ofthe unoperated subjects comprise the material forcephalometric and Goslon Yardstick analyses

10.1.3 Radiographs

The lateral skull radiographs were taken in a stat sited in a private sector hospital in Galle Thesame design of cephalostat was used in Kandy andGalle on all subsequent visits

cephalo-In 1984, the protocol for setting up the

radiograph-ic apparatus was established and supervised for eachexposure by the author In subsequent visits, all ortho-dontic members of the Team, after training, tookturns in placing the subjects in the cephalostat andsupervising all exposures

Great care was taken at each radiographic session

to ensure that the anode to midsagittal distance wasprecisely 152.5 cm (the Imperial measurement of 5 ft)and the midsagittal plane to film distance was 16 cm.The central ray was arranged at right angles to thesagittal plane This was determined by an electric lightsource within the anode housing, casting a superim-posed shadow of both ear rods on a sheet of whitepaper which was attached to the x-ray film cassette.These measures ensured reproducible skull radi-ographs on all occasions with consistent magnifica-tion error The patients were posed with the teethlightly occluded in maximal intercuspation and theFrankfort plane parallel to the ground

Table 10.1. Unoperated groups

Table 10.2. Control group

Temperatures were always around 30 °C, and the

humidity was often near 100%, but this did not

ad-versely affect the quality of the films, which were

im-mediately processed in wet tanks They were dried

and suspended from wire “washing lines” strungacross the main x-ray room Patients were detaineduntil after their x-ray had been examined, in case arepeat x-ray was required (Fig 10.3, Table 3)

e d

Fig 10.1 a–e. Lateral skull x-ray and dental study models of a healthy male control subject

c

Table 10.3. Mature unoperated subjects cephalometric results (UCLP, BCLP, and ICP)

Ba_N Cranial Base 112.5±2 108±2 110±2 110±4 102.3±7 100.2±8 108.4±5 100.3±5

SNA Maxillary protrusion 85.4±3 85±3 85±2 86±4 90.3±10 90.6±8 83.2±6 83.4±4

SNANS Basal maxillary protrusion 87.1±3 87.1±3 84±2 86±4 94.6±10 94.5±8 84.7±6 85.3±5

Ans-Ptm Palatal Length 57.8±5 53.1±4 54±2 51±2 61.6±6 61.3±6 50.3±6 49.2±5

SNB Mandible protrusion 82±4 83.4±3 80±2 81±3 81.6±5 81.7±6 81.3±5 81.3±4

AR-TGO Ramus height 57.5 50.0 51.3 48.9 48.5±4 47.8±41 48.6 47.1

S_N_ANS Upper face height 54.5 51.5 52.6 47.5 46.6±3 45.5±2 49.2 45.6

N-S-Ptm Upper post face height 44.0 40.3 38.3 37.0 33.5±4 31.2±3 39.7 33.4

a b

c

e d

Fig 10.2 a–e. Lateral skull x-ray and dental study models of a healthy female control subject

Fig 10.3. Digitized points used for cephalometric analysis

10.2 Unoperated Unilateral Cleft Lip

and Palate

The most striking feature in the unoperated

unilater-al cleft lip and punilater-alate subject is the protrusion of the

upper labial segment Subjects present with large

overjets, proclined upper incisors, eversion of the

ma-jor segment and a mild contraction of the lesser

seg-ment in the anterior region Buccal cross-bites are

rare Cephalometric analysis displays normal cranial

base measurements and protrusion of the maxilla

rel-ative to the mandible No cases presented with

maxil-lary retrusion (a common feature in the operated

case) Figures 10.4 and 10.5 illustrate typical examples

of the facial appearance and dental study models of

unoperated unilateral cleft lip and palate cases

10.2.1 Dental Study Models

Impressions were taken in alginate material with thepatients sitting upright on a wooden chair Bite regis-tration was made using conventional denture wax In

1984, the author and accompanying oral surgeon castthe impressions in white dental plaster in the hotelbathroom after the day’s work This process wasfraught with difficulty because dental plaster setsextremely rapidly in the tropics

On all subsequent visits, a local Sri Lankan cian was employed Impressions were cast in dentalstone on the day that they were taken, incorporatingthe tooth bearing and important anatomical areas.Care was taken to include all anatomically visiblestructures This necessitated modifying aluminium

techni-Fig 10.4 a–j. Adult female subject with complete unilateral cleft of the lip and palate

a

c

b

d

Fig 10.4 a–j.(continued)

h g

trays with white wax rims Huge volumes of alginate

were used on some of the adults with wide-open

clefts, and included impressions of the middle and

in-ferior turbinate bones, the entrance to eustachian

tubes, and the posterior wall of the pharynx Some

impressions utilized 13 scoops of alginate powder

None of the patients vomited Figure 10.6 shows

algi-nate impressions with extension beyond the inferiorturbinates, the entrance to the eustachian tubes, andthe posterior wall of the pharynx

Final orthodontic trimming and basing of themodels was performed at the laboratory in the Max-illofacial and Dental Department in the Hospital forSick Children, Great Ormond Street, London

Fig 10.5 a–j. Adult male subject with complete unilateral cleft of the lip

a

c

b

d

Fig 10.5 a–j.(continued)

h

j i

g

Fig 10.6 a, b. Upper arch alginate impressions, maximally extended beyond the inferior turbinates, the entrance to the eustachian tube and the posterior wall of the pharynx

10.2.2 The GOSLON Yardstick

The GOSLON Yardstick [34] a London/Oslo consensus

of 5 groups of increasing deformity in dental arch

re-lationships was applied to 49 unoperated cases for

whom cephalometric analysis had been performed

Two additional cases where study models, but not

lat-eral skull radiographs, were available have been

in-cluded

“UNOPS” in GOSLON STUDY(UCLP) [51]

The ranking was performed by the author Previous

reports have demonstrated the reproducibility of the

author’s assessments, and the robustness of the

Yard-stick in the discrimination between differences in

samples from different centres [35, 36]

The GOSLON yardstick results showed 98% of the

cases in group 1 or 2 (excellent or very good arch

rela-tionships) and no cases in groups 4 or 5 (Fig 10.7)

Because the yardstick is not a direct measure of

skeletal morphology or growth, it is possible to pool

males and females over 13 years in a single group

These results are in marked contrast to those of anycentre worldwide examining operated patients, whereonly a very small minority of patients are found ingroup 1 (Fig 10.8)

10.2.3 Unilateral Cleft Lip and Palate Study Models Analysis

by Reflex Microscope (Fig 10.9)

There were significant differences in tooth size tween the cleft group and the controls: the teeth of thecleft group were consistently smaller and the largestdifferences were in the central incisors No significantdifferences were found in comparisons of the cleft andnoncleft sides, sexes, or side to side within each group

be-Fig 10.7. GOSLON Grouping Sri Lankan

Unoperated UCLP n = 51; 98% in Excellent

(1) or Good (2) arch relationships

Fig 10.8. UK CSAG Study 1998 GOSLON

CSAG (Clinical Standards Advisory Group)

GOSLON results of the whole of the UK

10.2.3.3 Chord Lengths

No significant differences were found in chord lengths

There was a higher prevalence of crossbites in the cleft

subjects (8 cases) than in the control group where

there were none

The overjet was much larger in the cleft group The

mean overjets in the cleft group was 8.2 mm

com-pared to 3.7 mm in the controls

The percentage of missing teeth was higher in the cleftgroup; the most commonly missing teeth in the cleftgroup were the maxillary lateral incisors

There was no crowding in the buccal segments ofeither the control group or the cleft group In fact, alldental arches examined were very well aligned andsome spacing was generally evident

Fig 10.9. The digitized points, chord lengths, and arch widths used in the reflex microscopic analysis of study model

10.2.4 Summary of Reflex Microscope

Findings on Study Models: UCLP [15]

Tooth size and arch widths that were found to be

smaller in the unilateral cleft lip and palate subjects

than in the control group would suggest that there is

some degree of primary hypoplasia in this group

However, these differences are small and would not

account for the gross maxillary retrusion frequently

reported in surgically repaired unilateral cleft

pa-tients Even with this small primary hypoplasia,

over-jets in the cleft group are larger than the controls and

the prevalence of crossbites is surprisingly low These

results concur with the cephalometric findings of a

smaller retrusive mandible in the unilateral cleft and

palate subject

These features demonstrate the intrinsic potential

for these subjects to grow relatively normally with

mi-nor distortions around the cleft site itself where the

dentition is unrestrained because of the disrupted

musculature There are, however, some intrinsic

growth deficiencies, particularly a shortened ramus

height, and reduced upper anterior midface heightand upper posterior face height

10.3 Unoperated Bilateral Cleft Lip and Palate

Unoperated bilateral cleft lip and palate cases strate massive protrusion of the premaxilla with grossproclination of the upper incisors Interestingly, theyshow significantly smaller cranial base lengths Thereare intrinsic growth deficiencies; a shortened ramusheight, and reduced upper anterior midface heightand upper posterior face height The initial character-istics of the newborn bilateral cleft lip and palate sub-ject persist during growth The prominent premaxilla,wide alar bases, often laterally deviated premaxillarysegment, short columella, and rudimentary prolabi-

demon-um with no muscle attachment are characteristic tures Figures 10.10–10.12 illustrate the typical exam-ples of the facial appearance and dental study models

fea-of unoperated bilateral cleft lip and palate cases [37]

Fig 10.10 a–d. Mature male BCLP, note lateral segments have moved to the midline excluding the premaxilla anteriorly

d

c

Fig 10.11 a–f. Female unoperated BCLP unrestrained forward and downward growth of the premaxilla

10.4 Unoperated Isolated Clefts

of the Palate

Unoperated isolated clefts of the palate present with

relatively normal appearance However, this group

demonstrates more intrinsic deficiencies than clefts

of the lip and palate They have normal upper and

lower dental arch relationships but with bimaxillary

retrusion They present with short maxillary length,

small mandibles, and reduced upper anterior and

pos-terior face heights Figures 10.13–10.15 illustrate

typi-cal examples of the facial appearance and dental study

models of unoperated isolated clefts of the palate

10.4.1 Isolated Cleft Palate Study Models

Analysis by Reflex Microscope [38]

10.4.1.1 Tooth Sizes

The tooth sizes in clefts of the secondary palate were

found in general to be smaller than in the normal

group, although these differences were not large and

were clinically unimportant The greatest differences

were found in the incisor region No differences were

found between the sexes of the same group

10.4.1.2 Chord Lengths

and Perpendicular Distances

Chord lengths from the first molar to canine regionwere not different between the cleft and the controlgroup but were different in the canine to central inci-sors distance This reflects the consistently smallersize of the teeth in the patients with clefts of the sec-ondary palate In addition, perpendicular distanceswere generally smaller

The arch widths of the clefts of the secondary palatewere narrower than the controls This may be anadaptation of the dentition to the smaller and narrow-

er mandible in this group

The overjets in the cleft group were slightly increasedcompared to the control despite the fact that the chordlengths and the perpendicular distances were shorter.This again may be a reflection of small mandibularsize

Fig 10.12. Male lateral skull x-ray and

study models of unoperated BCLP Note

gross unrestrained forward and downwards

growth of the premaxilla

Fig 10.13 a–f. Adult male subject with isolated cleft of the hard and soft palate

d

f c

e

10.4.2 Factors Influencing Interpretation

of Results from the Sri Lankan Cleft

Lip and Palate Project

10.4.2.1 Malnutrition and Growth

It should be recognized that the above studies are

de-rived from subjects in the developing world Although

Sri Lanka is a relatively advanced developing country,

there is nevertheless significant malnutrition and

en-demic infections, for example, malaria

The failure of infants with clefts to gain weight

ad-equately has been documented by several authors [39,

40] There is increasing evidence to suggest that poor

nutrition in early life may be an important factor in

growth disturbances seen in later life [41]

However, if there is failure to thrive or no catch-up

growth by the age of 2 or in some papers by 5 years

then perhaps attainment of normal limits for height,

weight, or body mass index can never be expected

Malnutrition, particularly at a period of especiallyrapid growth such as in utero, has long-lasting effects.Subsequent influences of undernutrition in the firstand second years of life or later in childhood leave along-lasting, complex growth problem During child-hood, stature is determined by the size that an infanthas reached by the end of the first year of life, which ispartly determined by genetic circumstances and in-fluenced greatly by nutrition and the subsequent rate

at which the child grows

Nutritional status has a profound effect on growthhormone secretion Malnutrition is a well-recognizedform of reversible growth hormone resistance, whichcan be normalized with nutritional supplements Amalnourished mother is likely to give birth to a babywith low birth weight, while children with protein-en-ergy malnutrition do not grow as well as others, ac-cording to a recent report [42] This kind of malnutri-tion is an underlying cause of almost one third of thedeaths among children under 5 years in Sri Lanka.Malnutrition is still a serious problem in Sri Lanka[43] Food insecurity is one of the major reasons formalnutrition in Sri Lanka according to the Depart-ment of Census and Statistics Poor financial andphysical access to food is responsible for the malnutri-tion and food insecurity Drastic price increases of es-sential food commodities and stagnating or deterio-rating incomes created poor financial access to food.The civil war from 1984–2002 in Sri Lanka has exacer-bated the essential food and financial problems

A recent survey of 16,000 Sri Lankan childrenfound that only one quarter were properly nourished[44] More than one third were suffering from thirddegree malnutrition, the level beyond which childrenexhibit distended stomachs and skinny frames Sup-porting evidence from the National Peace Council in-dicated that only 4,863 children under 5 years out of arandom sample of 16,767 were within normal nutri-tional limits Six thousand three hundred and seventyone children had third degree malnutrition, 3,186with second degree malnutrition, and 2,347 with firstdegree malnutrition [45] According to this report,diseases such as malaria, which is still prevalent in Sri Lanka are a primary cause of malnutrition Worminfestations are the second most common cause Lack

of food itself is the third cause

In another supporting paper it was found that Sri Lankans require a calculated average of 2,260 calo-ries per day Availability of protein has gradually in-creased; nevertheless a high incidence of malnutritionexists with 60% of children under 5 suffering frommalnutrition [46, 47] Poor growth of preschool children, high rates of low birth weight babies, poormaternal nutritional status, and micronutrient defi-ciencies are common nutritional problems in SriLanka

Fig 10.14. Mature female with U-shaped ICP

Fig 10.15. Mature female with V-shaped ICP

To measure quality of life in a nation, the United

Nations Development Program [48] started figuring a

Human Development Index (HDI) A nation’s HDI is

composed of life expectancy, adult literacy, and gross

national product per capita

There are vast differences when comparing or

studying a different ethnic culture The HDI for the

UK is ranked as number 13 out of 130 nations Sri

Lan-ka is ranked at 79 – much lower than the UK The

com-parisons between the two countries are shown in the

table below

UK and Sri Lanka Human Development Index

UK Sri LankaLife expectancy (years) 77.7 72.1

Total population (millions) 59.4 18.9

Annual population (growth rate) 0.1% 0.8%

Population under age 15 19% 26.3%

Children underweight for age 0 33%

Children underheight for age 0 17%

Infants with low birth weight 8% 17%

Malaria cases (per 100,000 people) 0 1,111

Nutrition or subsequent malnutrition is only one

en-vironmental factor that can leave a long-lasting

com-plex growth problem Emotional deprivation also has

a profound influence on the growth process and may

interact with the provision of food [41] A well-loved

child is fed and nurtured, whereas a child with no

prospect of a job or marriage or who is a burden may

not be Many of the subjects in this study were social

outcasts, who dropped out of school Females in

par-ticular were hidden away in their houses and only one

female in the unoperated population married

Chil-dren need a good emotional climate to thrive The

mechanism of the effects of emotional deprivation on

growth is not well documented but is linked to duced growth hormone secretion and its associatedgrowth failure

While facial growth in the unoperated subject ents without maxillary retrusion, unlike many operat-

pres-ed patients, the speech outcomes for the same series

of patients demonstrate almost unintelligible speechfor the whole sample This is illustrated in Fig 10.16,which compares and contrasts the GOSLON resultswith the speech articulation outcomes for a group ofUCLP subjects who had both dental study model andspeech recordings

Research on the Sri Lankan Cleft Lip and Palatearchive has demonstrated that surgery, when delayedbeyond 8 years of age and even earlier, results in per-manent irremediable speech disorders [17, 49]

The Sri Lankan Cleft Lip and Palate project was ful to use healthy control subjects from the Sri Lankanpopulation Sri Lankans present with bimaxillary pro-trusion, females on a skeletal 2 dental base and males

care-on a mild skeletal 3 dental base These are reflectedwithin the cleft population Interestingly, only onesubject presented with a class II/2 dental arch rela-tionship on a skeletal 2 dental base (Fig 10.17)

In a recent (2003) project in Gujarat and Rajasthan

in northern India, the vast majority of patients senting had class II/2 dental arch relationships onskeletal 2 dental bases This further emphasizes theneed to have control subjects from the same racialgrouping (Fig 10.18)

pre-Fig 10.16. Outcome for unoperated subjects beyond 13 years of age (n = 42).

In both GOSLON and Articulation groupings 1 and 2 are excellent or good, while groups 4 and 5 are bad or awful

Fig 10.17 a–f. Adult male subject with unoperated unilateral

cleft of the lip and palate This is a rare example of class II/2

arch relationship demonstrating retroclined upper incisors.

Very broad upper arch and very low FM angle The lower lip controls the upper labial segment causing retroclination

d

f e

c

10.4.2.4 Surgical Implications

It should not be inferred that because unoperated cleft

lip and palate subjects grow relatively normally,

surgi-cal regimens delaying palatal surgery are indicated

This study does not examine the effects of surgery or

the timing of that surgery upon facial growth

out-comes

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42 Fernando R Prevention of non-communicable diseases – challenges in the next century Daily News, Associated Newspapers of Ceylon; 1998.

43 Rajapaksha U, Siriwardena J Sri Lanka A hard time tional Reports; 2002.

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45 National Peace Council of Sri Lanka Sri Lanka: Health sis in the Vanni Districts.Asian Human Rights Commission 1998; 8.

cri-46 Rajapaksha U, Siriwardena J Successes and lapses UN velopment Programme Human Development Report 2000.

De-47 Fernando SD, Paranaritane SR, Rajakaruna J, Weerasinghe

S, Silva D, Wickremasinghe AR The health and nutritional status of school children in two rural communities in Sri Lanka Trop Med Int Health 2000; 5:450–452.

48 United Nations Development Program Human ment Index United Nations Development Program 2002; 200249.

Develop-49 Sell DA Speech in Sri Lankan cleft palate subjects with layed palatoplasty Leicester Polytechnic, redesignated De Montfort University 1991; PhD Thesis.

de-A review of the psychological issues for children with

cleft lip and/or palate (CLP) must take into account

the social, emotional, behavioral, and cognitive

func-tioning of the children There is a complex interaction

among genetically determined traits such as

tempera-ment and intelligence and environtempera-mental factors such

as parenting styles and social milieu These factors

in-terrelate to affect a child’s self-concept, social

accept-ance, behavioral adjustment, and school success

These factors also affect a child’s ability to cope with

the stress of growing up with CLP This chapter will

provide a brief overview of these complex issues as

they relate to (1) social and emotional functioning

and (2) cognitive functioning and school achievement

in individuals with CLP

11A.1 Social and Emotional Adjustment

The social and emotional adjustment of a child with

CLP must be viewed as a developmental process

rather than a static state Factors that have the

poten-tial of affecting this process will be connected to

parental functioning, child functioning, and societal

acceptance

When a child is born with a birth defect such as

CLP, parents experience multiple emotions that

dis-rupt the equilibrium of the family Shock, sadness,

fear, grief, guilt, and anger are some of the emotional

reactions that parents report [11, 54] The first months

of life can be extremely difficult for parents of a child

with CLP as they handle their feelings, learn to feed

their baby, and work to integrate their baby into the

family While most families are able to cope with this

crisis and reorganize to meet the child’s needs, some

struggle [10] Parental success during these first

months of life will depend in part on the individual

mental health of each parent, their coping skills, and

the strength of the marital relationship Parents who

report high levels of stress during infancy that persists

into toddlerhood also report higher levels of ment problems for their children [39] However, re-search also suggests that mothers who believe thatthey are able to meet their child’s needs, who take sat-isfaction in their role as parents, and who are able toopenly acknowledge the stress of the current situationare best able to nurture their infants in a manner thatpromotes healthy attachment during the early child-hood, better emotional self-regulation during the pre-school years, and fewer behavior problems duringearly elementary school [9, 52]

adjust-As the child grows, the role of the parent also ops Parents serve as the child’s first model of how tohandle the difficult social situations that individualswith facial differences encounter Parents also provideemotional support, impart positive discipline, andteach their child how to effectively relate to peers andadults Children whose mothers reported less stressand greater parenting confidence during the pre-school years were rated as more socially skilled, self-confident, and well adjusted when they entered theprimary grades [24] Similarly, Pope and Ward [40]found that parents who were anxious worriers abouttheir children’s social acceptance, but didn’t act pro-ductively to assist their child, were more likely to havechildren who were having social difficulties while par-ents who actively encouraged their children’s efforts

devel-to engage with peers had children who displayedhigher levels of social competence

Although parents have an important role in achild’s long-term adjustment, child characteristicsalso have an effect on social and emotional function-ing that is independent of the parent’s behavior Childdevelopment research has defined differences in tem-perament that influence personality types and are re-lated to psychological adjustment While there are anumber of temperament systems, Hart, et al [13] de-scribe three broad personality types that provide ageneral guide to differences among children Thesetypes are: (1) the resilient child who is socially compe-

A Brief Overview of Psychological Issues

in Cleft Lip and Palate

Kathleen A Kapp-Simon

11A

tent, gets along with both adults and peers, tends to be

gregarious, and exhibits positive emotions; (2) the

overcontrolled child who typically exhibits extreme

shyness, is quite compliant, and highly dependent on

others; and (3) the undercontrolled child who is

gen-erally uncooperative, has difficulty with social

rela-tionships, is noncompliant, and more likely to exhibit

negative emotions These temperamental

characteris-tics exist on a continuum and may be influenced by

environmental factors

Children respond to the stresses of growing up

with CLP differentially based on their inborn

tem-peramental characteristics A resilient child tends to

be more even tempered, tolerates surgeries with less

distress, and is less likely to be bothered by questions

about the scaring from his or her cleft Because of

their easy-going positive nature, these children are

also less likely to be teased by their peers If teasing

does occur, they are more likely to view the teasing as

the other person’s problem In contrast, a child who is

overcontrolled and inhibited may demonstrate

in-creased anxiety in social situations or when faced

with surgery This same child is more likely to feel

stigmatized by his or her facial differences, believing

that teasing from peers is an indication of a personal

flaw The child who is undercontrolled has difficulty

complying with adult demands and may antagonize

peers due to his or her inability to play cooperatively

or comply with group mores Thus differences in

per-sonality type may influence the way in which a child

reacts to situations that are commonly encountered

when growing up with CLP (see [16] for further

dis-cussion of how temperament affects coping)

Societal reactions also play an important role in the

emotional adjustment of individuals with CLP

Multi-ple studies exist that demonstrate a relationship

be-tween facial configuration, attractiveness and the

re-actions and judgments that observers make about an

individual, with more attractive individuals receiving

positive attributions and less attractive individuals

being judged more harshly [1, 7, 20, 25, 30, 32, 56]

Lan-glois et al [26] completed a meta-analysis of studies

on attractiveness and concluded that: “Beauty is more

than just in the eye of the beholder; people do judge

and treat others with whom they interact based on

at-tractiveness; and perhaps most surprisingly, beauty is

more than skin deep.”

Thus it is not surprising that parents frequently

at-tribute difficulties with peer teasing, exclusion from

the peer group, or choosing to play with younger

(safer) children to facial differences or

accommoda-tions of their children to those facial differences [17,

27, 38] Objective ratings of facial difference have been

associated with increased behavioral inhibition [47],

which may also be related to quality of social

interac-tion [19] Focus group interviews with adolescents

who have facial differences concluded that these teensattribute their perceived lack of welcome by peers totheir facial appearance [8] A natural response to aperceived lack of welcome may be to limit social con-tacts to one or two peers with whom a child feels safe

or the child may choose solitary activities Eitherstrategy may serve to decrease anxiety [14, 29, 43]

11A.2 Cognitive Development and School Achievement

The majority of children with CLP demonstrate lectual development that places them within thebroad range of normal [6, 12, 31, 50] although meanscores tend to be 3–5 points lower than the populationmean of 100, particularly in the area of verbal skills[45] A recent controlled study examining intellectualfunctioning of adult males with CLP compared to anage-, gender-, and SES-matched comparison groupreplicated the findings of intelligence research onchildren[37] The mean full scale intelligence quotient(FSIQ) for male adults with CLP was within the nor-mal range (FSIQ = 96.96, SD = 13.2) but 12.5 points be-low the control group (FSIQ = 109.5, SD = 9.27).Verbalintelligence was not more impaired than nonverbalintelligence for the adult subjects; however, verbal flu-ency was significantly less well developed for the sub-jects with CLP even when FSIQ was controlled.The discrepancy between verbal and nonverbal in-telligence has been researched in some detail by Rich-man and his colleagues [42, 44, 46] The hypothesis ofthis body of research has been that children with CLPdemonstrate two broad types of language-relateddeficit One type involves a mild verbal, expressivedeficit that has been associated with developmentalreading problems and the other is a more pervasivelanguage disability that affects language comprehen-sion and associative reasoning abilities

intel-The incidence of learning disabilities, particularlyreading disabilities, in children with CLP is signifi-cantly higher than the rate found in the general popu-lation, with an estimated rate of approximately30%–40% [5, 46] Recent research on the etiology ofreading disabilities in children with CLP has identi-fied several areas of interest Continued research byRichman has identified deficits in rapid naming andautomatic verbal memory, similar to the dysnomiamodel of dyslexia, as a primary deficit for childrenwith CLP who have a reading disability [48] Furtherinvestigation of this memory deficit suggests thatchildren with CLP do not automatically provide a ver-bal label to information that is presented in a visualform even though their short-term memory for thesame information presented orally is not impaired[49] According to Richman, an important prelimi-

nary recommendation from this line of research is

that children with CLP and reading disabilities would

benefit from training in verbal labeling and oral

phonics using a program similar to that

recommend-ed by the NICHD branch on dyslexia [28] Despite

ar-ticulation problems secondary to cleft palate, children

with CLP should be taught using a phonetic approach

rather than a whole word or sight reading approach

Reading and language disorders in children

with-out CLP have been associated with changes in

neu-roanatomy and function [21, 22, 41, 51] Nopoulos and

her colleagues have postulated a similar relationship

between CLP and brain structures and function [37]

The focus of Nopoulos’ research to date has been adult

males with CLP She has found changes in brain

struc-ture including enlargement of the anterior regions of

the cerebrum, with a corollary decrease in size of the

lateral and posterior regions of the cerebrum and a

decrease in size of the cerebellum that have been

asso-ciated with cognitive functioning; the greater the

change in brain structure, the lower the FSIQ [34–36]

A significant number of children with CLP are

con-sidered developmentally “at risk” during infancy and

the preschool years [18, 23, 33, 54, 55, 57] An “at risk”

classification generally indicates that the child is not

progressing at the expected rate; however, the

long-term implications of that delay are not always clear In

the case of children with CLP, developmental delays

may be considered secondary to known biological

risk in the form of early disruption of cranial cell

mi-gration and, therefore, indicators of possible learning

difficulties during the school years (see Aylward’s

work for a discussion of neuropsychological risk in

infancy [2, 3]) Since early intervention for

develop-mental reading disabilities has been demonstrated to

remediate the problems for a significant number of

children, it is critical that Cleft Teams provide the

nec-essary screening for cognitive deficits with

recom-mendations to the families for early intervention

serv-ices [4]

11A.3 Summary

Psychological factors including parental adjustment,

child temperament, social and emotional adjustment,

intellectual development, and school achievement for

children with CLP were discussed in this chapter The

emotional, behavioral, and social adjustment of

chil-dren with CLP is dependent on multiple factors Some

of these have been described in this short overview It

is clear that parents play a vital role in supporting

their children with CLP and parental confidence and

skill can facilitate good adjustment [9, 15, 39]

Nonetheless, characteristics intrinsic to the child

including temperament and intelligence will also

make critical contributions to the child’s overall being While the interplay between intrinsic charac-teristics and environmental factors is not unique tochildren with CLP, recognition of these factors bymembers of the Cleft Team will assist them in under-standing the needs of both children with CLP andtheir parents

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