Ebook Surgical treatment of colorectal problems in children: Part 2

Part 2 book “Surgical treatment of colorectal problems in children” has contents: Rectal atresia, rectovestibular fistula, cloaca, posterior cloaca and absent penis spectrum, postoperative evaluation, operations for the administration of antegrade enemas, urologic problems in anorectal malformations,… and other contents.

A Peña, A Bischoff, Surgical Treatment of Colorectal Problems in Children,

DOI 10.1007/978-3-319-14989-9_14, © Springer International Publishing Switzerland 2015

Rectal atresia is a very unique malformation that

deserves a special description It happens in our

experience, in about 1 % of all cases of

anorec-tal malformations In this defect, the anus seems

to be completely normal, including the quality of

the sphincter and the location of the anal orifi ce

However, deep inside the anus, just at the junction of

the anal canal with the rectum, there is an atresia or

narrowing (stenosis) (Fig 14.1 ) Occasionally, we

see atresias or stenosis located at a different level

The space that separates the dilated blind rectum,

from the anal canal, is represented by a septum that

sometimes is extremely thin and can be perforated,

and other times it is very thick In some unusual

cases, there is a signifi cant separation between the

blind upper rectum and the lower anal canal

Interestingly, the sphincter mechanism is lent in most cases There is one particular malfor-mation similar to this one that is represented by a stricture or by atresia of the rectum, associated to a presacral mass and a sacral defect (see Chap 8 , Sect 8.2 ), which is a completely different type of defect The only thing they have in common is the fact that the rectum is narrow or atretic

excel-We believe that rectal atresia with normal sacrum and no presacral mass is unique, because the sphincter mechanism is normal and also because these patients do not have the typical association with all the defects that we see in other anorectal malformations As a conse-quence, the prognosis for these patients is excel-lent, in terms of bowel control They have a

signifi cant tendency to suffer from severe

consti-pation because they are born with a blind, very

dilated rectum These malformations have been

previously described in the literature [ 1 5 ]

Rectal atresia has been traditionally described

in the old textbooks The baby is born with a

normal- looking anus, and the nurse or the

pedia-trician tries to pass a thermometer through the

anus and fi nds an obstruction In fact, part of a

routine examination of every “normal” newborn

is to check the patency of the anus, unless the

baby is already passing meconium

14.1 Treatment

If one could think in an ideal indication for a

pos-terior sagittal approach, this would be the

malfor-mation which seems to be more indicated The

defect is easily repaired through a posterior

sagit-tal incision In our initial cases, we simply remove

the septum that separates the upper rectum from

the anal canal and created an end-to-end

anasto-mosis (Figs 14.2 and 14.3 ) Subsequently, we

found some cases in which the size discrepancy

between the upper blind rectum and the small

anal canal was very severe, and in order to expand

the size of the anal canal, we introduced a

techni-cal modifi cation maneuver [ 5 ] (Fig 14.4 ) Most

of the patients that we operated on came to us already with a colostomy in place Since the patient has a colostomy, one can perform a distal colostogram and simultaneously introduce a metallic dilator in the anal canal to have a lateral image of the atresia and estimate the distance between the upper pouch and the anal canal If

we could make the diagnosis early in an wise healthy newborn baby, we would recom-mend to do the operation without a colostomy

at the same location as the atresia (Fig 14.2a ) Unfortunately, we still see some of these patients, previously operated in whom the sur-geon considered that the little anal canal was use-less and therefore decided to resect it and pulled down the dilated piece of rectum That is rather

Fig 14.2 Repair of a Rectal Atresia ( a ) Incision, exposed defect, open upper rectum, and anal canal ( b ) Anastomosis

of the upper rectum to anal canal

regrettable, because the anal canal, as we know,

represents the area of sensation that will provide

bowel control to these patients It is, therefore,

very important to preserve that little anal canal

Sometimes the size of the anal canal is too small

For that, we introduced a technical modifi cation

[ 5 ], consisting in mobilizing the posterior rectal

wall, down to the skin of the anus (Fig 14.4 ),

enlarging the circumference of the anus We

real-ize that by doing that, the posterior aspect of the

anus will no longer be a real anal canal, but rather

a rectal wall However we manage to preserve

most of the circumference of the original anal

canal, which will provide enough sensation to

have bowel control We must keep in mind that

after we fi nish this procedure, the anastomosis

that we created between the upper dilated rectum

and the anal canal is going to be permanently lapsed by the effect of the sphincter mechanism that keeps the anal canal closed all the time, except during defecation; therefore, these babies must be subjected to the same protocol of anal dilatations that we already described

Some surgeons [ 4 ] went as far as to perform a

“laparoscopic transanal approach” to repair this malformation To demonstrate that something can be done does not mean that it must be done

We cannot justify to change a limited, painless, bloodless, quick, minimally invasive, non- laparoscopic procedure for a laparoscopic inva-sive operation that includes an unnecessary total rectal dissection

Our experience includes 11 cases and has been previously published [ 5 ]

Fig 14.3 Diagram showing the repair of rectal atresia ( a ) Rectum repaired, ( b ) Sagittal view of the fi nished operation

References

1 Dias RG, Santiago Ade P, Ferreira MC (1982) Rectal

atresia: treatment through a single sacral approach

J Pediatr Surg 17(4):424–425

2 Upadhyaya P (1990) Rectal atresia: transanal, end-to- end,

rectorectal anastomosis: a simplifi ed, rational approach to

management J Pediatr Surg 25(5):535–537

3 Kisra M, Alkadi H, Zerhoni H, Ettayebi F,

Benhammou M (2005) Rectal atresia J Paediatr

Child Health 41(12):691–693 doi: 1754.2005.00763.x

4 Nguyen TL, Pham DH (2007) Laparoscopic and transanal approach for rectal atresia: a novel alterna- tive J Pediatr Surg 42(11):E25–E27 doi: 10.1016/j jpedsurg.2007.08.049

5 Hamrick M, Eradi B, Bischoff A, Louden E, Pena A, Levitt MA (2012) Rectal atresia and stenosis: unique anorectal malformations J Pediatr Surg 47(6):1280–

1284 doi: 10.1016/j.jpedsurg.2012.03.036

Fig 14.4 Technical variant to expand the size of a very small anal canal ( a ) Incision ( b ) Open rectum Arrows show

the portion of the rectum to be mobilized ( c ) Sutures on one side of anal canal and rectum ( d ) Sutures tied down ( e ) Same maneuver, opposite side ( f ) Finished operation

A Peña, A Bischoff, Surgical Treatment of Colorectal Problems in Children,

DOI 10.1007/978-3-319-14989-9_15, © Springer International Publishing Switzerland 2015

15.1 Defi nition/Frequency

Rectovestibular fi stula is the most important

anorectal malformation in females This is due

to the fact that, by far, it is the most common

defect seen in females Two hundred and ninety

of our 1,123 female patients were born with this

malformation Two hundred and seventeen were

operated primarily by us, and 73 were

reopera-tions due to a previous failed attempted repair

Interestingly, our series include 531 patients

with a cloaca However, we are convinced that

this high number of cloacas in our series is

because ours is a referral center We believe that

vestibular fi stula is much more common in the

general population Most of the vestibular fi

s-tula cases are operated at the place where the

babies are born, whereas many cloacas are

referred to us due to the complexity of the repair

When this malformation is repaired with a

meticulous surgical technique, the recovery of

the patients is excellent, and the functional

results are also very good Unfortunately,

another characteristic of this malformation is

the fact that it is frequently mismanaged We

compared the results obtained in a group of

patients that were repaired primarily by us with

those of the group that had a secondary

proce-dure due to the fact that the patient underwent a

previous failed attempted repair The difference

in terms of bowel control is signifi cantly ent Therefore, we can repeat what other pediat-ric surgeons through history have said, and that

differ-is that “these patients have a single opportunity

to have a good repair.”

In the old literature [ 1 ] one can fi nd that this malformation received different names, includ-ing “anovestibular fi stula.” The authors believed that this was a more benign variant of defect and that these patients had a very short fi stula and a very low-lying rectum They also believed that malformation owed to be distinguished from a

“rectovestibular fi stula,” which has a long, row fi stula and a rectum located higher in the pel-vis, and, therefore, they believed that the prognosis was not as good as the one observed in cases of “anovestibular fi stula” [ 1 , 2 ] We also found the term “vestibular anus”; the authors believed that some patients were born with an otherwise normal anus located in the vestibule of the female genitalia [ 2 ] We have never seen this type of defect We do not use those three terms mentioned here, because we found that all our patients with an anal opening located in the vesti-bule can be repaired with the same surgical pro-cedure and have the same functional prognosis; therefore, we consider the old terminology impractical and misleading It is true that some patients have a longer fi stula than others; those cases may require more dissection to bring the rectum down However, our results are uniformly good, regardless the type of fi stula

15

Electronic supplementary material Supplementary

material is available in the online version of this chapter at

10.1007/978-3-319-14989-9_15

Rectovestibular Fistula

Rectovestibular fi stula is a defect in which

the rectum opens in the vestibule of the female

genitalia This should not be confused with a

rectovaginal fi stula In order for us to call a

mal-formation “rectovaginal fi stula,” one must see the

anal opening located inside the vagina, deeper

to the hymen Vestibular fi stula patients have a

normal hymen, and the anal orifi ce is located

posterior to the hymen (Figs 15.1 and 15.2 ) The

anal opening is visible most of the time, provided

the clinician separates the labia of the baby’s

genitalia The newborn female frequently has a

signifi cant degree of edema and swelling of that

area, considered to be a consequence of the effect

of maternal hormones Therefore, in the

new-born baby, it may be a little bit more diffi cult to

see the precise location of the vestibular fi stula

(Fig 15.3 )

Some cases of vestibular fi stula have the anal

opening located rather deep and are almost

impossible to see it without general anesthesia In

fact, some patients have a rather small-looking

genitalia (vulva) similar to what we see in cases

of cloaca The anal orifi ce is located very deep in

the vestibule, and the urethra is also located

deeper than normal, which is what urologists call

“female hypospadias.” This particular variant, we

call “cloaca type I,” one could also use the term

“deep vestibular fi stula with a female

Fig 15.1 Diagram of vestibular fi stula ( a ) Sagittal view ( b ) Perineum

Fig 15.2 Picture of a vestibular fi stula

dias” (Fig 15.4 ) We consider this particular type

of malformation a transition in between a cloaca

and a vestibular fi stula

Some patients are born with the anal orifi ce

located just in between the perineal body (skin

lined) and the vestibule, wet tissue (Fig 15.5 )

This type of defect is considered intermediate

between the vestibular fi stula and perineal fi stula

The management of these patients is not different

from any other type of vestibular fi stula This

defect is also known as “fourchette fi stula.”

15.2 Associated Defects

A retrospective review of 290 patients with

ves-tibular fi stulas operated by us (217 primary and

73 secondary) showed a signifi cant number of

associated defects Since vestibular fi stula is

con-sidered a malformation representative of the

“good side” of the spectrum of anorectal defects

Fig 15.3 Vestibular fi stula in a newborn baby Arrow

shows the fi stula site

Fig 15.4 Deep rectovestibular fi stula with female spadias – observe small vulva

Fig 15.5 Fourchette fi stula

in general, the frequency of association of all the

defects is rather low Yet, it is signifi cant enough

to be searched for

15.2.1 Sacral

We were able to measure the sacral ratio in 113

of our cases and found that the average AP ratio

was 0.57 and lateral was 0.7 Six percent of these

cases had a ratio lower than 0.4 This is

consis-tent with the fact that we consider this

malforma-tion a “benign” one, with good funcmalforma-tional

prognosis Fourteen cases had a hemisacrum and

a presacral mass, and as previously mentioned,

presacral masses occur more frequently in lower

defects

15.2.2 Spinal

Approximately, 9 % of our patients had some

form of spinal defect, mainly hemivertebra

15.2.3 Urologic

Ten percent of vestibular fi stula cases had a

sin-gle kidney, which as we know is the most

com-mon anatomic abnormality associated to all

anorectal malformations, and 13 % of patients

had vesicoureteral refl ux, which is consistent

with the fact that this disorder is the most

com-mon functional urologic abnormality seen in

ano-rectal malformation cases Hydronephrosis was

present in 6 % of the cases

15.2.4 Gynecologic

There are not many reports in the literature,

related to this very important assoc [ 3 4 ] A

ret-rospective review of our patients with vestibular

fi stula showed that 17 % of them had associated

genital anomalies [ 5 ] Eight percent had absent

vaginas or vaginal atresia Figure 15.6 shows the

different types of absent vaginas or vaginal

atre-sias encountered

Figure 15.6a shows the perineum of one of these patients, and there is no vaginal opening Figure 15.6b shows a diagram of a sagittal view and the type of repair that we used, consisting in leaving the rectum attached to the urethra, to function as a neovagina and pulling the upper rectum down to the perineum

Eighty percent of the patients with vestibular fi tula and absent vagina are born with agenesis of the internal genitalia (uterus and fallopian tubes) In such cases the vagina is replaced with a piece of colon; this is done only for the patient to have sexual function Twenty percent of the patients have a uterus and a blind ending of vagina, usually located very high in the pelvis (Fig 15.6c ) In that type of case, the lower vagina is replaced with a piece of colon with dual purpose (sexual and reproductive) Some cases of vestibular fi stula with absent vagina can be repaired without vaginal replacement, but rather pulling down their native vagina That can only be done in cases with a large blind vagina Five percent suffered from some sort of septa-tion disorder of the Müllerian structures These included a vaginal septum, always associated with the presence of two hemicervices and two hemiuteri (Fig 15.7 ) Three patients had a unilat-eral streak ovary; the rest had two normal ovaries Two patients had a perineal lipoma, and one patient had a labial hemangioma

We were able to see patients born with a tibular fi stula that came to us as adolescents; they had a repair in the past, but the surgeons missed the diagnosis of a vaginal septum These vaginal septa can only be detected when the surgeon sus-pects their existence Based on these fi ndings, it

ves-is our routine and our recommendation to perform

a vaginoscopy with a pediatric cystoscope in all patients with vestibular fi stula The presence of a vaginal septum may, in some cases, interfere with tampon placement and sexual intercourse when the patient grows up But more important than that is the fact that the presence of a vaginal sep-tum means, by defi nition, that the patient has two hemiuteri, representing a partial or total septation disorder Hemiuteri have important gynecologic and obstetric implications We know that patients with hemiuteri may have a higher degree of infer-tility, and those patients who become pregnant

have a higher incidence of miscarriages and

pre-mature labor Therefore, it is extremely important

to make the diagnosis as early as possible in order

to provide these patients with special

gyneco-logic and obstetric care later in life

It is our routine to do a vaginoscopy in every

case of vestibular fi stula We perform that study

with a baby cystoscope, during the same

anesthe-sia given for the repair Figure 15.8 shows the

aspect of a normal infant cervix

15.2.5 Gastrointestinal

Six percent of our patients with vestibular fi stula

had an associated esophageal atresia, one patient

without a fi stula, and all the others with a

tra-cheoesophageal fi stula; 1 % had a form of

duode-nal obstruction (atresia or stenosis)

15.2.6 Tethered Cord

Fifty-seven patients were evaluated with an ultrasound (fi rst 3 months of life) or with an MRI, looking for spinal cord anomalies; twenty

of them had tethered cord (35 %) These fi gures are higher than the average of all anorectal mal-formations, and we believe that this is explained

by the fact that the incidence of presacral masses

is also high in this malformation Tethered cord

is very common in cases with presacral mass

15.2.7 Cardiovascular

Twenty-seven patients (9 %) had an atrial septal defect Twenty-two (8 %) had a ventricular sep-tum defect Fourteen (5 %) had a patent ductus arteriosus, and four (1 %) suffered from tetralogy

Fig 15.6 Vestibular

fi stula with absent or

partially absent vagina

( a ) Photograph

( b ) Diagram of a sagittal

view and one type of

repair, using the rectum to

replace the vagina

( c ) Diagram showing the

internal genitalia of a

patient with a high vaginal

atresia, with a piece of

colon ( d ) Diagram

showing an absent vagina

as well as the uterus, the

vagina totally replaced

with colon ( e ) Two types

of vaginal atresia

Prepuberty and

postpuberty

e

c

Fig 15.6 (continued)

of Fallot Most of these defects (approx 80 %)

did not require treatment, since the patients were

hemodynamically stable

15.3 Diagnosis

The diagnosis of vestibular fi stula is a simple one It only requires a meticulous inspection of the genitalia of the baby Yet, amazingly, many patients are not diagnosed or are misdiagnosed as having “rectal vaginal fi stula.” From our series of 1,123 female patients, we have only seen seven cases of documented real rectovaginal fi stula During the same period of time (over 30 years),

we have operated on 290 cases of vestibular fi tula Fifteen of them come to our center with a previous diagnosis of “rectovaginal fi stula.” Actually, they were born with a vestibular fi stula

s-as evidenced by the presence of a little pocket where the vestibular fi stula used to be located (Fig 15.9 ) Forty-fi ve female patients also came

to us after a failed attempted repair of a malformation diagnosed as “rectovaginal fi stula.”

A careful examination revealed that those patients actually had a persistent urogenital sinus, which means that they were actually born with a cloaca and the surgeons only repaired the rectal compo-nent of the malformation, because they were thinking that the patient only had a rectovaginal

fi stula (see Chap 16 , Sect 16.1.4 )

Prior to 1980, the literature [ 2 6 13 ] reported

an elevated number of cases of “rectovaginal fi

Fig 15.7 Pocket of the original vestibular fi stula in a

patient previously operated with the erroneous diagnosis

of a “rectovaginal fi stula.” ( R ) rectum, V original fi stula

Fig 15.9 Aspect of a normal cervix in a baby with a tibular fi stula

Fig 15.8 Vestibular fi stula with a vaginal septum Arrow

shows the fi stula site

tula” in female patients In contrast, those authors

reported very few vestibular fi stula cases and

very rare cloaca cases A few publications after

1980 persist reporting “rectovaginal fi stula

cases.” Interestingly, looking at the diagrams of

most of those publications, they actually show

vestibular fi stulas, although they call them

“vagi-nal fi stula.” The term “vestibular fi stula” has been

used correctly by some authors with large

experi-ence in the management of these defects

[ 14 – 21 ]

We believe that this is not a simple semantic

problem, but rather has important clinical

implications [ 22 ] We have seen patients born

with vestibular fi stula that were previously

misdiagnosed as “vaginal fi stula” and

under-went a type of repair designed to repair “high”

malformations, namely, a contraindicated

abdominoperineal (open or laparoscopic)

pro-cedure that resulted in fecal incontinence We

also have seen that at least 30 patients born

with a cloaca received the wrong diagnosis of

“rectovaginal fi stula” and underwent a repair

only of the rectal component of the

malformation, leaving the patient with a

uro-genital sinus [ 22 ]

15.4 Treatment

15.4.1 Colostomy or No Colostomy

This is a frequently debated subject Many

sur-geons claim that they routinely repair vestibular

fi stulas without a colostomy and they have “good

results” [ 12 , 23 – 27 ] Many others prefer to open

a colostomy in all cases with a vestibular fi stula

In the meantime, we see many patients that

underwent a repair of a vestibular fi stula without

a colostomy and suffered from serious

complica-tions, including dehiscence and retraction of the

rectum as well as reopening of the fi stula

However, this recurrent fi stula is frequently an

acquired rectovaginal fi stula, due to the fact that

during the attempted repair, the posterior wall of

the vagina was damaged

As discussed in the Chap 5 , we believe that

the answer for this question of colostomy or no

colostomy is different for every surgeon and his/her different surrounding circumstances It very much depends on the experience of the surgeon, the clinical condition of the patient, and the infra-structure of the hospital where the patient is treated

In general, at our institution, if a baby is born with a vestibular fi stula, we operate on her within the fi rst 5 days of life without a colostomy, pro-vided the baby is in good clinical condition, is full term, and does not have severe associated defects

Consider the case of a premature baby with a cardiac condition and vestibular fi stula Under those circumstances, dilatations of the fi stula may prove to be useful for the patient to be able

to pass stool, eat, and grow That would allow the surgeon to postpone the decision of colostomy or primary repair On the other hand, a full-term baby in good clinical condition without associ-ated defects in an institution with a good infra-structure and a pediatric surgeon with experience

in the management of this defect, the patient can

be operated before starting her feedings, at a time when the patient is still passing meconium, because when it is done in that way, the patient actually does not need any kind of bowel preparation

Most of our patients come to us after the born period and with a colostomy already opened

new-at another institution, sometimes in another try Many other patients come to us after several months of passing stool with diffi culty through the non-operated vestibule, with severe constipa-tion and megacolon Those patients are also treated without a colostomy at our institution, but our routine includes the admission of the patient

coun-1 or 2 days prior to the main operation, insertion

of a nasogastric feeding tube, and administration

of GoLYTELY 1 at a rate of 25 mL/kg/h until the colon is completely clean The patient receives a PICC line and parenteral nutrition for a period of 7–10 days postoperatively

When the patient has a colostomy, the tion can be done without following this routine,

opera-1 GoLYTELY… (Polyethylene glycol/electrolytes.) Braintree Laboratories, Braintree, MA, USA

but rather irrigating only the distal stoma of the

colostomy the day before surgery In that case,

the baby can eat the same day of the operation;

she will stay for 48 h in the hospital receiving

intravenous antibiotics Our experience is that the

pain that these patients experience

postopera-tively is rather minimal We have operated on

pri-marily without a colostomy in approximately

50 % of our cases

We use a posterior sagittal approach to repair

these malformations Other approaches do exist,

and the most traditional and popular was

described by Dr Potts and is called a fi stula

trans-plant [ 28 – 32 ] Some surgeons describe an

opera-tion called “anterior sagittal approach” [ 33 – 37 ]

We found that the word “anterior” in those

publi-cations was actually not referring to the incision,

but rather to the position of the patient; in other

words, the patient is positioned in lithotomy

posi-tion, rather than prone, but the incision is always

posterior to the fi stula, because there is no way to

make an incision anterior to the fi stula site In

other words, the so-called “anterior sagittal

approach” is actually a posterior sagittal approach

performed in lithotomy position

Interestingly, Professor Francesco Rizzoli

from Bologna, Italy, published in 1869 [ 38 ] the

technique now referred as “anterior sagittal

approach”; his publication includes magnifi cent

illustrations

The essential components of the posterior

sag-ittal approach described below avoid the fl aws

observed in those other techniques, and the most

common problems seen in our reoperations were

retraction of the anoplasty and an inadequate

perineal body (anteriorly located anal orifi ce)

15.5 Main Repair (Animation 15.1 )

The patient is brought to the operating room, and

we start the procedure with the patient in the

lithotomy position in order to perform a

vaginoscopy using a baby cystoscope We do this

with the specifi c purpose to rule out vaginal

malformations Although a vaginal septum can

be simply seen by separating the labia without

the use of a cystoscope, we prefer to use a

cysto-scope, because sometimes one can see a vaginal septum that is only present in the lower part of the vagina, and the upper part has a single cervix (Fig 15.8 ) Most of the times, however, the sep-tum is complete, and one can see two cervices at the end of the vagina

The patient is then turned into the prone tion with the pelvis elevated, and the perineum,

posi-as well posi-as the genitalia and perianal area, is washed, prepped, and draped in the usual man-ner Most of the times, one can see the anal ori-

fi ce in the vestibule, and in such case multiple 5-0 silk stitches are placed at the mucocutane-ous junction of the anal opening (Fig 15.10 ) These stitches serve the purpose of applying uniform traction to facilitate the separation of the rectum from the vagina Occasionally, the

fi stula is located so deep that it is impossible to

do this; in such case, we fi rst make the incision and go deep enough to be able to see the edges

of the fi stula and apply multiple 5-0 silk stitches (Fig 15.11 ) We use the electrical stimulation to determine the limits of the sphincter and to guide ourselves to try to stay as much as possi-ble exactly in the midline, dividing the entire sphincter mechanism leaving equal portions of the sphincter in both sides of the midline For this, we use a needle-tip cautery, changing from cutting to coagulation The size of the incision usually is shorter than the regular posterior sag-ittal anorectoplasty The incision usually runs from the lowest part of the sacrum and coccyx down to the fi stula orifi ce, passing through the sphincter mechanism We divide the entire sphincter, including the parasagittal fi bers, the muscle complex, and the levator mechanism Deeper to the levator mechanism, one can iden-tify the characteristic white fascia that covers the posterior wall of the rectum (Fig 15.12 )

Figure 15.13 shows the aspect of the rectum with traction sutures Traction creates the plane The white fascia is removed from the posterior rectal wall, including the extrinsic blood supply of the rectum We do this to identify the real rectal wall completely clean The dissection is then extended to the lateral walls of the rectum (Fig 15.13 ) The next step consists of extending the dissection of the lateral walls of the rectum all

the way down to the skin It is important to

remember that at the level of the skin, there is no

real plane of dissection between the rectal wall

and the surrounding tissues Whereas

approxi-mately 1 cm proximal in the rectum, one can clearly identify the plane that separates the rectum from the surrounding tissues, and therefore the recommendation is to follow the steps mentioned

in this description, meaning to identify the

poste-rior rectal wall; continue the dissection to the

lat-eral walls of the rectum and then from there,

applying uniform traction on the multiple 5-0 silk

sutures; and continue the dissection from the

lat-eral walls of the rectum down to the skin One

must expect to fi nd important vessels that provide

the blood supply of the lower rectum while

dissecting the lateral walls of the rectum

A Weitlaner retractor is used to achieve adequate,

optimal exposure At this point, we are ready to

initiate the most important part of the operation,

which is the separation of the rectum from the

vagina

One must keep in mind that the rectum and

vagina share a common wall with a variable

length from 1 to 3 cm and that there is no real plane of dissection between both structures In other words, one must make two walls out of one, and very often, this common wall is extremely thin This happens to be the most important ana-tomic feature of this malformation Surgeons must keep in mind that the main challenge in the repair of these defects is the separation of the rec-tum from the vagina and should take it as a per-sonal challenge The separation of the rectum from the vagina requires a very meticulous, deli-cate surgical technique It cannot be done by blunt dissection We like to perform this separa-tion using the needle-tip cautery while applying traction on the rectal wall and checking with a lacrimal probe the thickness of the anterior rectal wall and the posterior vaginal wall very fre-quently, to be sure that we are not getting too close to one or the other (Fig 15.14) As we progress in this meticulous dissection, the wall of the rectum, as well as the wall of the vagina,

Fig 15.12 “White fascia” after dividing the entire

sphincter mechanism, the rectum is identifi ed covered by

the white fascia

Fig 15.13 Diagram showing the dissection of rectum

starts getting thicker, which indicates that we are

getting close to the point where both are expected

to be completely separated and have a full

thick-ness At this time, the surgeon should not be

overconfi dent, because in that point he could

injure either the rectum or the vagina (Fig 15.14 )

The dissection must continue until the rectum has

been completely separated from the vagina

(Fig 15.14 )

It is extremely common for surgeons to ask

what happens and what to do in the event of

acci-dentally opening either the vagina or the rectum

Our routine answer is as follows: if it happens that

we opened the vaginal wall, but maintained intact

the rectal wall, one can actually leave the vaginal

orifi ce of the injury open, provided the rectum is

intact, and the anoplasty is not under tension, and

the patient is going to do alright Something

simi-lar can be said when the orifi ce is created in the

rectum, but the vaginal wall is intact What is

con-sidered nonacceptable is to have an injury of the

rectal wall in front of an injury to the vaginal wall,

leaving sutures in front of sutures, since that is

considered an obvious predisposing factor for the

formation of a rectovaginal fi stula Under such

circumstances (vaginal injury and a rectal injury),

one must continue the dissection of the rectum

until we can leave a normal rectal wall in front of the vaginal orifi ce or suture

We must always remember that in dealing with anorectal malformations, the real challenge in the surgical repair is represented by the separation of the structures, namely, the rectum from vagina, the rectum from urethra, and the vagina from ure-thra, because all those structures share a common wall without a plane of dissection Most of the complications that we have seen in patients who underwent failed attempted repairs of anorectal malformations occur during the separation of these structures

Sometimes when the rectum has been fully separated from the vagina, we fi nd that we have enough rectal length to do an anoplasty without tension and with good blood supply However, many other times, the rectum needs further mobi-lization To do this, one must continue applying uniform traction on the multiple silk stitches By doing this, it becomes evident that there are some bands and vessels holding the rectum up in the pelvis These must be separated from the rectum, independently burned and divided in a circumfer-ential manner, continuing until we have enough rectal length to create an anastomosis without tension

Fig 15.14 Different stages of the separation of the rectum from the vagina Posterior vaginal wall and anterior rectal wall intact

The incision required to repair

rectovestibu-lar fi stulas includes the opening of the muscle

complex and part of the levator mechanism

Sometimes, it is not necessary to open

com-pletely the levator mechanism, and therefore we

call this a limited posterior sagittal

anorecto-plasty However, we are convinced that the size

of incision does not affect, in any way, the future

functional prognosis, provided all of the other

important surgical steps are done correctly

Once the rectum has been separated from the

vagina and mobilized, in preparation for the

reconstruction, the limits of the sphincter are

elec-trically determined and marked with temporary

silk stitches The goal at this stage is to bring

together the anterior limits of the sphincter and by

doing that to reconstruct the perineal body of the

patient (Fig 15.15 ) This is the space that

sepa-rates the vagina from the rectum It is extremely

important to use strong sutures (5-0 or 4-0

long-term absorbable sutures depending on the patient’s

age) to approximate both sides of the perineal

body There, we usually fi nd a fi brous tissue that

surrounded the original vestibular fi stula We use

this tissue to anchor our stitches These deep

stitches must relieve most of the tension of the perineal body to be sure that the skin edges in the perineal body come together with no tension We close the skin of the perineal body with 6-0 Vicryl sutures, only to be sure that the edges of the skin have come together, but those sutures hold no ten-sion Figure 15.15 shows the repaired perineal body The rectum then is located within the limits

of the sphincter immediately behind the perineal body The posterior edges of the muscle complex and levator are sutured together in the midline using 5-0 long-term absorbable sutures, including

a bite to the posterior rectal wall to anchor it in normal location (Fig 15.16 ) These stitches are aimed to avoid retraction and prolapse The ischiorectal fossa, as well as the subcutaneous tis-sue, is obliterated using 5-0 long-term absorbable sutures, and the skin is closed either with subcu-ticular 5-0 monofi lament, absorbable, or inter-rupted 6-0 long- term absorbable sutures

The anoplasty is done as previously described, using two layers of interrupted 5-0 or 6-0 long- term absorbable sutures (Fig 15.17 ) We try to trim off as little as possible rectal tissue, but we

do not hesitate to remove all of the tissue that is

Fig 15.15 Perineal body reconstructed ( a ) Diagram ( b ) Intraoperative diagram

considered damaged, to be sure that we have healthy rectal tissue with good blood supply to create a healthy anoplasty (Fig 15.18 )

Patients operated without a colostomy are kept on parenteral nutrition with nothing by mouth for a period not shorter than 7 days After 7 days, we look into the external aspect of the perineum, and if it looks that it has healed nicely, we allow the patient to eat and discharge her Anal dilatations start 2 weeks after surgery following our protocol (see Chap 18 ) Occasionally, when one examines the patient’s perineum 1 week after the operation, one may

fi nd that there is an area of partial dehiscence of the perineal body or the posterior sagittal inci-sion, and this is a good opportunity to take the patient to the operating room and resuture that, taking advantage of the fact that the patient has been with nothing by mouth Under those cir-cumstances we keep the patient 2 or 3 more days fasting, receiving parenteral nutrition

At the time of colostomy closure (in those patients with a colostomy) 2 or 3 months after the operation, the external aspect of the anus and the vagina, as well as the perineum in these patients,

is remarkably normal looking (Fig 15.19 )

Fig 15.16 Diagram showing sutures taking the posterior

edges of the muscle complex, including a bite to the

pos-terior rectal wall to anchor it

Fig 15.17 Anoplasty and wound closed ( a ) Diagram ( b ) Intraoperative picture

s-15.7 Functional Results

Ninety percent of our patients have voluntary bowel movements by the age of three This is, provided they have a normal sacrum, not tethered cord, and they had received a good operation Over 50 % of the patients suffer from signifi cant constipation that deserves special attention Not taking good care of the constipation will provoke chronic fecal impaction and overfl ow

Fig 15.18 Photograph of a fi nished operation

Fig 15.19 External

appearance 2–3 months

postoperatively

pseudoincontinence We usually have a long

con-versation with the parents and explain in detail the

importance of taking care of the constipation We

emphasize the fact that the constipation that these

patients suffer from is much more severe than the

common idiopathic constipation of the general

pediatric population The amount of laxatives that

these patients need sometimes is two, three, four,

or fi ve times higher than in other types of patients

We try to make the parents paranoid against the

problem of constipation We also emphasize the

fact that the amount of laxatives that these patients

require to empty the colon every day cannot be

predicted We determine the amount of laxatives

by trial and error over a period of several days,

taking abdominal x-ray fi lms to be sure that the

patient empties the colon If the patients are

receiving breast feedings at the time of our

opera-tion, most likely they will not need laxatives until

they start decreasing the amount of breast milk

and receiving another type of formula

Sexual life in these patients is normal, and as

we have seen, many of our patients are becoming

adults and are getting married They also can

deliver babies vaginally, since we did not actually

injure the vagina which preserves a normal

elas-ticity in most of its circumference, since we only

dissected the posterior vaginal wall

In the past, some surgeons [ 2 ] claimed that

these patients could have a normal life without an

operation or simply doing a “cutback” type of

procedure to enlarge the anal opening We have

seen that this is not true First of all, the bowel

control under those circumstances is rather poor

In addition, when these patients grow up, they

feel very unhappy about the fact that they have

the anus located immediately behind the vagina

with no perineal body This gives them insecurity

and psychological problems, and in addition, a

vaginal delivery is contraindicated, because it

will produce severe rectal damage

15.8 Reoperations in Patients

with Vestibular Fistula

From all anorectal defects treated by us, it is the

vestibular fi stula type of case that most frequently

came to us after a failed attempted repair at

another institution In fact, from our total series

of 290 patients with vestibular fi stula, 73 of them are reoperations We believe that this is a refl ec-tion of the fact that surgeons in general probably underestimate the complexity of the repair of this defect As previously mentioned, vestibular fi s-tula is by far the most common anorectal malfor-mation seen in females The functional prognosis

in girls when they are born with a good sacrum, have no tethered cord, and receive a good opera-tion is excellent Unfortunately, patients who underwent a failed attempted repair followed by

a reoperation do not have the same good tional prognosis Eighty percent of them have voluntary bowel movements as compared to

func-90 % for those operated primarily

Probably, the surgeons fi nd it relatively easy to imagine that the orifi ce of the rectum located in the vestibule could easily be moved back to the normal location of the anus In reality, the repair

of this malformation is a delicate and technically demanding procedure

The most common scenario in dealing with reoperations for vestibular fi stulas is a patient that was operated without a protective colostomy and soon after suffered from dehiscence and retraction of the rectum, followed by opening of the rectum into the posterior vaginal wall In other words, the original malformation was a ves-tibular fi stula, but the patient comes with a real acquired rectovaginal fi stula secondary to a poor initial operation During the re-exploration, our most common fi nding in this specifi c type of problem has been an intact common wall between the rectum and vagina In other words, the sur-geons try to repair the malformation but failed to separate the rectum completely from the vaginal wall They still tried to pull the rectum down which was left, we think, under tension, because

it was still attached to the vaginal wall As a sequence, the rectum retracted We assume that during the attempt to separate the rectum from the vagina, the lower part of the vaginal wall was injured, and therefore when the rectum retracted,

con-it reopened into the posterior vaginal wall ing an acquired vaginal fi stula

Another common scenario in reoperations for vestibular fi stula is a group of patients that underwent a previous operation called cutback

procedure at another institution [ 39 , 40 ]; these

consisted in making a posterior slit in the

poste-rior edge of the anal opening in the vestibule

and suturing it horizontally like a

Heineke-Mikulicz type of procedure That procedure

only enlarges the anal opening and leaves the

rectum attached to the vaginal wall with no

peri-neal body (Fig 15.20 ) We believe that, perhaps,

in cases of perineal fi stula, the cutback

proce-dure could be considered an acceptable

thera-peutic alternative, but we strongly believe that

this type of operation is contraindicated in

patients with vestibular fi stula There was an old belief that went from generation to generation that by leaving the rectum attached to the vagina,

as time went by, the perineal body would grow, which is defi nitely not true

Another fi nding that is interesting to mention

is the fact that in some of these patients, we found that they had two hemivaginas, and such malfor-mation was never mentioned in the operative reports of the previous surgeons (Fig 15.21 ) Again, we like to say that “our eyes see only what our mind suspects.”

Fig 15.20 Pictures of

two patients born with a

vestibular fi stula and

underwent a cutback

procedure prior to coming

to our center

Fig 15.21 External

aspect of perinea of two

patients born with a

vestibular fi stula and two

hemivaginas They

underwent a poor

attempted repair and were

left with no perineal body

and two hemivaginas

15.9 Surgical Technique

Reoperations for recurrent or dehiscent,

retracted vestibular fi stulas are currently done by

us without a protective colostomy Figure 15.22

shows examples of cases that came to us after

a failed attempted repair of their malformation

However, we follow the precautions already

mentioned in the chapter related to bowel

prepa-ration We take the baby to the operating room

with the bowel completely clean As part of

our routine, we perform vaginoscopy and

cys-toscopy to rule out the presence of associated

defects (mainly vaginal septum) We place the

patient in prone position with the pelvis elevated

and make a posterior sagittal incision following

the specifi cations already described Multiple

5-0 silk stitches are placed at the

mucocutane-ous junction of the rectovaginal fi stula or the

rectal opening in order to apply uniform

trac-tion Through the posterior sagittal incision, all

structures are divided in the midline until the

posterior rectal wall is identifi ed and then the

dissection of the rectum proceeds, fi rst on the

lateral walls and eventually in the common wall

between the rectum and the vagina As

previ-ously mentioned, we have been impressed by

the fact that most of these patients have an intact common wall between the rectum and vagina, which refl ects the fact that the surgeons did an incomplete mobilization of the rectum We go ahead and make two walls out of one In other words, we separate the rectum from the vagina

as previously described in the primary dure We must suture the defect of the posterior vaginal wall Once the rectum has been com-pletely separated, we then mobilize the rectum enough to guarantee that an intact anterior rectal wall is left in front of the vaginal sutures We are convinced that the vaginal defect can even

proce-be left unsutured, and it will heal normally vided the rectal wall left behind is intact We dissect the rectum enough to guarantee that the rectal wall in front of the vagina is completely normal and also to be sure that the anastomo-sis between the rectum and the skin of the anal dimple is performed without tension Before we

pro-do the anoplasty, we repair the posterior vaginal wall with long-term absorbable sutures, deter-mine the limits of the sphincter, and continue the operation as described for primary cases, reconstructing the perineal body and doing the anoplasty The patients remain 10 days fasting and receiving parenteral nutrition

Fig 15.22 External appearance of the perineum of different patients referred to us, after failed attempted repairs

We were impressed by the fact that many

patients had a failed operation early in their life,

remained incontinent during childhood, and

searched for help only when they became

teen-agers and had decided to become sexually

active We believe that they had become aware

of their defective anatomy and felt very upset

about the fact that their rectum and vagina were

located one next to the other, with no perineal

body In other words, they felt embarrassed at

considering sexual life with that kind of

anatomy

15.10 Rectovestibular Fistula

with Normal Anus

See Chap 27 , Sect 27.2

References

1 Cigarroa FG, Kim SH, Donahoe PK (1988)

Imperforate anus with long but apparent low fi stula in

females J Pediatr Surg 23(1 Pt 2):42–44

2 Stephens D, Smith D (1971) Chapter 4: Individual

deformities in the female In: Anorectal

malforma-tions in children, vol 4 Year Book Medical Publisher,

Chicago, pp 81–117

3 Digray NC, Mengi Y, Goswamy HL, Singh N, Atri

MR, Sharma R, Thappa DR (2001) Complete vaginal

prolapse: an unusual presentation of anovestibular fi

s-tula Pediatr Surg Int 17(2–3):226–227

4 Banu T, Hannan MJ, Aziz MA, Hoque M, Laila K

(2006) Rectovestibular fi stula with vaginal

malforma-tions Pediatr Surg Int 22(3):263–266

5 Levitt MA, Bischoff A, Breech L, Peña A (2009)

Rectovestibular fi stula–rarely recognized associated

gynecologic anomalies J Pediatr Surg 44(6):1261–

1267 doi: 10.1016/j.jpedsurg.2009.02.046

6 Hanley PH, Hines MO, Stephens JE (1954) Anal

sphincter-preserving operation for congenital low

rectovaginal or rectoperineal fi stula Am J Surg

88(5):737–745

7 Stone HB (1936) Imperforate anus with rectovaginal

cloaca Ann Surg 104(4):651–661

8 David VC (1937) The treatment of congenital

open-ings of the rectum into the vagina—atresia ani

vagina-lis Surgery 1(2):163–168

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anus Ann Surg 147(2):203–213

10 Rosenblatt MS, Gustavson RG (1958) The treatment

of congenital malformations of the anus and rectum

13 Simmang CL, Paquette E, Tapper D, Holland R (1997) Posterior sagittal anorectoplasty: primary repair of a rectovaginal fi stula in an adult: report of a case Dis Colon Rectum 40(9):1119–1123

14 Duhamel B (1960) Le Traitement des anus vulvaires Ann Chir Infant 1:53–70

15 Bill AH, Hall DG, Johnson RJ (1975) Position of rectal fi stula in relation to the hymen in 46 girls with imperforate anus J Pediatr Surg 10(3):361–365

16 Salamov KN, Dultsev YV, Protsenko VM (1987) Surgical treatment of the vestibular ectopia ani in adults Acta Chir Plast 29(4):209–215

17 Heinen DFL, Bailez M, Solana J (1992) Malformaciones anorectales I Fístula vestibular Area Cirugía Hospital de Pediatría J.P Garrahan, Buenos Aires, pp 148–154

18 Sawicka E (1995) Results of surgical treatment of girls with ano-vestibular fi stula Surg Childh Intern 3(2):94–98

19 Heinen FL (1997) The surgical treatment of low anal defects and vestibular fi stulas Semin Pediatr Surg 6(4):204–216

20 Javid PJ, Barnhart DC, Hirschl RB, Coran AG, Harmon CM (1998) Immediate and long-term results

of surgical management of low imperforate anus in girls J Pediatr Surg 33(2):198–203

21 Martín RS, Molina E, Cerdá J, Estellés C, Casillas MAG, Romero R, Vázquez J (2002) Manejo del ano vestibular en niñas mayors Cir Pediatr 15:140–144

22 Rosen NG, Hong AR, Soffer SZ, Rodriguez G, Peña A (2002) Rectovaginal fi stula: a common diagnostic error with signifi cant consequences in girls with anorectal malformations J Pediatr Surg 37(7):961–965

23 Demirbilek S, Atayurt HF (1999) Anal transposition without colostomy: functional results and complica- tions Pediatr Surg Int 15(3–4):221–223

24 Upadhyaya VD, Gopal SC, Gupta DK, Gangopadhyaya AN, Sharma SP, Kumar V (2007) Single stage repair of anovestibular fi stula in neonate Pediatr Surg Int 23(8):737–740

25 Kumar B, Kandpal DK, Sharma SB, Agrawal LD, Jhamariya VN (2008) Single-stage repair of ves- tibular and perineal fi stulae without colostomy

J Pediatr Surg 43(10):1848–1852 doi: 10.1016/j jpedsurg.2008.03.047

26 Menon P, Rao KL (2007) Primary anorectoplasty in females with common anorectal malformations with- out colostomy J Pediatr Surg 42(6):1103–1106

27 Upadhyaya VD, Gangopadhyay AN, Pandey A, Kumar

V, Sharma SP, Gopal SC, Gupta DK, Upadhyaya A (2008) Single-stage repair for rectovestibular fi s- tula without opening the fourchette J Pediatr Surg 43(4):775–779 doi: 10.1016/j.jpedsurg.2007.11.038

28 Potts WJ, Riker WL, Deboer A (1954) Imperforate

anus with recto-vesical, -urethral-vaginal and

-peri-neal fi stula Ann Surg 140(3):381–395

29 Carter RF, Lyall D (1940) Congenital

rectovagi-nal defects; operative repair Surg Gynecol Obstet

71:89–93

30 Keighley MR (1986) Re-routing procedures for

ecto-pic anus in the adult Br J Surg 73(12):974–977

31 Živkovi ć SM, Krstić ZD, Vukanić DV (1991)

Vestibular fi stula: the operative dilemma –cutback,

fi stula transplantation or posterior sagittal

anorecto-plasty? Pediatr Surg Int 6:111–113

32 Grant HW, Moore SW, Millar AJW, Rode H, Cywes S

(1994) Is posterior anal transfer a good treatment for

vestibular anus? Pediatr Surg Int 9(1–2):12–16

33 Ando S, Yamaguchi S, Sakasaki Y (1987) Anterior

perineal anorectoplasty for intermediate and high

imperforate anus Jpn J Surg 17(3):213–216

34 Okada A, Kamata S, Imura K, Fukuzawa M, Kubota

A, Yagi M, Azuma T, Tsuji H (1992) Anterior sagittal

anorectoplasty for rectovestibular and anovestibular

fi stula J Pediatr Surg 27(1):85–88

35 Kulshrestha S, Kulshrestha M, Singh B, Sarkar B, Chandra M, Gangopadhyay AN (2007) Anterior sag- ittal anorectoplasty for anovestibular fi stula Pediatr Surg Int 23(12):1191–1197

36 Wakhlu A, Kureel SN, Tandon RK, Wakhlu AK (2009) Long-term results of anterior sagittal ano- rectoplasty for the treatment of vestibular fi stula

J Pediatr Surg 44(10):1913–1919 doi: 10.1016/j jpedsurg.2009.02.072

37 Shehata SM (2009) Prospective long-term functional and cosmetic results of ASARP versus PASRP in treatment of intermediate anorectal malformations in girls Pediatr Surg Int 25(10):863–868 doi: 10.1007/ s00383-009-2434-7

38 Rizzoli F (1869) Atresia Congenita Collezione delle memorie Chirurgiche ed Ostetriche 2:321–357

39 Mariño Espuelas JM, Martinez Utrilla MJ, Gonzalez Utrilla Y (1988) Consideraciones A La Fistula Vestibular Cir Pediátr Hosp Infat 1(2):88–90

40 Matley PJ, Cywes S, Berg A, Ferreira M (1990) A 20-year follow-up study of children born with vestibular anus Pediatr Surg Int 5(1):37–40 doi: 10.1007/BF00179636

A Peña, A Bischoff, Surgical Treatment of Colorectal Problems in Children,

DOI 10.1007/978-3-319-14989-9_16, © Springer International Publishing Switzerland 2015

16.1 Cloaca

16.1.1 Defi nition and Management

A cloaca is a malformation that affects the

rec-tum and urogenital tract in females These girls

are born with a single perineal orifi ce The vagina,

urethra, and rectum are fused together inside the

pelvis, creating a single common channel that

opens into a single orifi ce in the location where

the urethra normally opens (Fig 16.1 ) The

length of the common channel varies from case

to case, from 1 to about 10 cm with an average of

approximately 3 cm

Thirty percent of these patients suffer in

addi-tion from a very dilated vagina full of fl uid and/or

mucus, called hydrocolpos [ 1 ] (Fig 16.2 ) The

reason why these very dilated vaginas retain fl uid

remains a mystery, since they are never really

atretic We speculate that there must be some sort

of valve mechanism that interferes with the

emp-tying of the fl uid Most of the patients with

hydrocolpos, in addition, have duplicate

Müllerian systems (Fig 16.3 )

The hydrocolpos may produce two important

complications:

(a) The fi rst is the possibility of compressing the trigone of the bladder, producing an extrinsic ureterovesical obstruction, megaureter, and hydronephrosis

(b) The second possibility is that the pos, left undrained, may become infected; creating a pyocolpos that eventually may perforate, which is a catastrophic event with risk of death In addition, the resulting infl ammation may scar the vagina and impact the future reconstruction

Approximately 60 % of the patients with acas also have a double Müllerian system con-sisting of the presence of two hemiuteri and two hemivaginas [ 1 ] This septation disorder may be partial or total In addition, it can be symmetric or asymmetric In the asymmetric types, the double Müllerian system phenomenon is frequently associated with a unilateral atresia of the Müllerian structure When this goes unrecog-nized, it may produce an accumulation of men-strual blood at the age of puberty, as well as retrograde menstruation into the peritoneal cavity (Fig 16.4 ) which produces rather dramatic signs

clo-of an acute abdomen and requires an emergency laparotomy The presence of double Müllerian systems also has important potential obstetric implications that will be discussed later in this chapter

Cloacas represent a very wide spectrum of defects, but the common denominator is the pres-ence of a single perineal orifi ce On the very bad side of the spectrum, one may fi nd patients with a

16

Electronic supplementary material Supplementary

material is available in the online version of this chapter at

10.1007/978-3-319-14989-9_16

Cloaca, Posterior Cloaca and Absent Penis Spectrum

common channel as long as 10 cm; in such cases,

usually two little hemivaginas, as well as the

rec-tum, connect to the urinary tract at the bladder neck

or above the bladder neck (at the trigone) (Fig 16.5 )

We are far from knowing the genetic causes of

this condition [ 2 ] Yet, we have never seen two

cases of cloaca in the same family

The knowledge of the intrinsic anatomic

characteristics of this malformation is relatively

new We were able to detect and read old tions that we think described patients suffering from cloacas, although were not recognized as such [ 3 6 ]

We were also very impressed by the fact that most publications prior to 1982 reported high numbers of rectovaginal fi stula cases and very few cloacas [ 7 ] In retrospect, we are convinced that the authors were reporting patients suffering

Fig 16.1 Diagram of a cloaca ( a ) Short common channel ( b ) Long common channel

Fig 16.2 Diagram of a cloaca with hydrocolpos Fig 16.3 hemivaginas Diagram showing a cloaca with two

from cloacas as “vaginal fi stulas.” We believe

that because of the large number of cases of

clo-acas that we have seen, coming with a history of

suffering from a “rectovaginal fi stula,” yet, when

we examined them, we found an untouched

persistent urogenital sinus and a pulled-down rectum; in the medical records of those cases, the word cloaca is not present

Most publications prior to 1982 reported very few cases of cloacas; many of them were

showing a cloaca with a

very long common channel

autopsy fi ndings The cases that underwent an

attempted repair suffered from a high mortality

The treatments used include a colostomy at

birth, followed by a rectal pull-through, leaving

the patient with a urogenital sinus to be repaired

“later” [ 8 23 ]

The terminology used in those years was also

confusing The authors frequently published

series that included cloacas and other different

conditions such as “adrenal hyperplasia,”

“vagi-nal atresia,” and “high anorectal malformation.”

One particular publication from 1973 [ 17 ] is the

most prominent one because it proposes the full

repair of the vagina However, looking at the

dia-grams, it becomes clear that the technique used

by Dr Raffensperger, the author, may be

appli-cable only in cases with a relatively large vagina,

located very low

All pediatric surgeons as well as hundreds of

patients are in debt with Dr Hardy Hendren for

his contributions in the fi eld of pediatric surgery

and pediatric urology His seminal work on the

surgical management of cloacas is the most

important one that we found in our literature

review [ 24 – 32] In his initial publications, Dr

Hendren referred to this malformation as

“uro-genital sinus and anorectal malformation” [ 25 ]

Dr Hendren’s contribution was particularly

important in dealing with complex reoperations

and repairing the challenging urologic-associated

defects of these patients

Some authors refer to cloacas with a clear

embryologic bias, and therefore, they used rather

confusing terms such as “urorectal septum

mal-formation sequence” [ 33] or “urorectal septal

defects” [ 34 ] including variants such as adrenal

hyperplasia, as well as male cases [ 35 ] Others

include the cloacas as part of “Müllerian duct

anomalies” [ 36 ]

16.1.1.1 Associated Defects

Twenty percent of those patients with a common

channel longer than 3 cm had an absent kidney

When the common channel was shorter than

3 cm, 17 % of the patients suffered from this

anomaly Hydronephrosis occurred in 45 and

22 %, respectively, in patients with a common

channel longer or shorter than 3 cm

It is important to notice that in all other rectal malformations, absent kidney is the most common anatomic-associated anomaly The high incidence of hydronephrosis in this malformation

ano-is consano-istent with the fact that the most serious problems that these patients will suffer from (including death) are urologic

Vesicoureteral refl ux occurs in 40 and 21 %, respectively, in patients with common channel longer and shorter than 3 cm

Most patients with hydronephrosis suffered from vesicoureteral refl ux At birth, however, some patients with hydronephrosis and megaure-ter seemed to suffer from a ureterovesical obstruction In reality, the obstruction was an extrinsic one, caused by a tense hydrocolpos When the hydrocolpos was drained, the vesico-ureteral refl ux becomes obvious

Hemivertebra occurs in 13 % of cases bar, thoracic, cervical, and sacral) The average sacral ratio in patients with cloacas is 0.52 AP and 0.64 lateral Table 16.1 shows the correlation between sacral ratio and the length of the com-mon channel

Cardiovascular anomalies occur in 20 % of cases in cloacas Patent ductus arteriosus occurs

in 8 % of cases, atrial septum defect in 19 % of cases, ventricular septum defect in 5 % of cases, and tetralogy of Fallot in 2 % of cases Tethered cord occurs in 36 % of the patients with cloacas Esophageal atresia was present in 11 % of our cloacas and duodenal atresia in 3 %

16.1.1.2 Goals of Treatment

The treatment of cloacas represents a signifi cant technical challenge The fi nal goals of treatment must result in a patient with urinary control, bowel control, sexual function, and capacity to procreate These goals, of course, are sometimes achieved, sometimes partially achieved, and

Table 16.1 Correlation between sacral ratio and length common channel

Length of common channel

Sacral ratio Lateral fi lm

Anterior/

posterior fi lm Average Less than 3 cm 0.68 0.55 0.615 More than 3 cm 0.6 0.53 0.56

sometimes not achieved at all For the worst

sce-nario, we are fi rm in our philosophy that all

patients with anorectal and urogenital

malforma-tions should be clean of stool and dry of urine in

the underwear after the age of three, either

because they were born with a benign

malforma-tion that was adequately reconstructed or because

even when they were born with a malformation

with bad functional prognosis, the patient is

maintained artifi cially clean of stool (subjected to

a successful bowel management program) [see

Chap 20 ] and dry of urine (subjected to

intermit-tent catheterization) through the native urethra or

through a neourethra (continent diversion)

Since we are dealing with a spectrum of

defects, one should expect a spectrum of results

after the treatment

16.1.1.3 Neonatal Management

The diagnosis of a cloaca is a clinical one It is

enough to look at the patient’s perineum and

make the correct diagnosis (Fig 16.6 ) These

patients have a single orifi ce, yet, the perineum

has other important characteristics that help to

predict the internal anatomy and the fi nal

func-tional prognosis A “good-looking” perineum

consists of the presence of a well-formed midline

groove and a well-located and clear anal dimple,

indicating that the patient has a good sphincter

(Fig 16.7 ) On the other hand, a “bad-looking”

perineum includes a single perineal orifi ce but, in addition, a completely “fl at bottom” with no traces of sphincter mechanism (Fig 16.8 ) and most likely, a poor functional prognosis In between those extremes of the spectrum, one can

fi nd a variety of external appearances

Occasionally, one can fi nd a very large single perineal orifi ce, leaking urine, and with evi-dence of a mild separation of the pubic bones (Fig 16.9 ) Those external signs correspond to

a patient who has separated pubic bones and a malformation called covered cloacal exstrophy [ 37 ] These patients have no bladder neck; their bladder is very small because it has never been

Fig 16.6 Perineum of a patient with a cloaca ( a ) Without separating the labia ( b ) Separating the labia

Fig 16.7 “Good-looking perineum.” Obvious midline groove and prominent anal dimple

full Eventually, these girls will need a total

uri-nary reconstruction In addition, in these

patients (covered exstrophies), it is very

com-mon to fi nd inside the abdomen the same kind

of anatomic abnormalities seen in cloacal

exstrophies, mainly the presence of a very short colon with a very abnormal blood supply (Fig 16.10 ) Yet, the abdominal wall is intact, which makes a difference with cloacal exstro-phies (Fig 16.11 )

Fig 16.8 “Bad-looking perineum.” “Flat bottom,”

absence of midline groove, no evidence of anal dimple

Fig 16.9 Perineum of a patient with a large single

peri-neal orifi ce The pubic bones are separated

Fig 16.10 Grotesque abnormal colon frequently seen in cases of cloacal exstrophies or in covered cloacal exstrophies

Fig 16.11 External appearance of a patient with covered cloacal exstrophy The umbilicus is frequently located lower than normal Observe low implantation of the umbi- licus and hemangiomas

It is not unusual to fi nd hypertrophic folds of

skin in the area of the single perineal orifi ce,

which gives a false impression of a phallus

(Fig 16.12 ) [ 38 ], and that is why 65 cases in our

series came to our institution with the

misdiagno-sis of intersex made at other hospitals In our

experience of over 531 cases, we only had one

case of gonadal dysplasia associated with a

ves-tibular fi stula, but never with a cloaca

The patients that came to our institution with

a cloaca and with a history of a suspected

diag-nosis of “intersex” described the unpleasant

experience of being told that their baby had an

undetermined gender It usually took a couple

of weeks, with consultation to urology,

genet-ics, and many laboratory tests, to conclude that

the patients actually were females suffering

from a cloaca

The key for the diagnosis of those cases with

a pseudophallus resides in the palpation of that

structure One can feel that it is actually folded

skin with no palpable corpora Retrospectively, we

found fi ve publications referring to this condition

as “pseudohermaphroditism” [ 39 ] Some authors

used the term “caudal anomalies” [ 40 ],

“ambigu-ous genitalia with VATER” [ 41], or “caudal

developmental fi eld defect with female

pseudo-hermaphroditism and VACTERL anomalies” [ 42 ]

and fi nally “penis-like clitorises with thra in non-virilized female fetus” [ 43 ] Looking

megaloure-at the pictures of all those cases presented, it was obvious that all those patients suffered from cloacas with normal female gonads and chromosomes

Figure 16.13 shows a single, very small, very narrow perineal orifi ce located in the tip of a pseudophallus, which usually means that the patient has other important associated urologic malformations and a long common channel

Fig 16.12 Pictures of patients with a cloaca and a pseudophallus Palpation of this structure allows to feel only folded skin and no real corpora

Fig 16.13 Picture of a cloaca The single perineal orifi ce

is very small and is located at the tip of a pseudophallus This kind of external anatomy is usually associated with severe urologic defects and a long common channel

Figure 16.14 shows the perineum of patients

with cloaca and lipomas Lipomas are relatively

common in the perineum of patients with cloacas

and do not necessarily mean that they require a

more complicated type of treatment At the time

of the main repair, the lipoma can be easily

excised (Fig 16.15 )

As previously shown, a patient with a cloaca

has a very high likelihood of suffering from a

urologic condition The fi rst 24 h of life, like in

all other babies with an anorectal malformation,

should be used to rule out the presence of associated

problems that may represent a risk for life The most important one, in patients with a cloaca, of course, is urinary tract obstruction The baby must have a kidney ultrasound to rule out the presence of hydronephrosis and also a pelvic ultrasound to rule out the presence of hydrocol-pos and megaureter (Fig 16.16 ) A plain abdomi-nal fi lm in a baby with a single perineal orifi ce may show an image of a pelvic mass, as shown in Fig 16.17 This represents, most likely, hydro-colpos that must be drained soon An ultrasound that shows hydronephrosis, megaureter, and a

Fig 16.14 Lipomas in the perineum of patients with cloaca

Fig 16.15 Preoperative and postoperative appearance after a cloaca repair, including the resection of lipomas

pelvic cystic mass most likely represents a

hydro-colpos that is compressing the trigone and is the

cause of the bilateral megaureters and the

hydronephrosis

Hydrocolpos as a cause of megaureters and

hydronephrosis has been poorly recognized in

the cases that we have received from other

institutions Many patients with hydronephrosis and megaureters were subjected to unnecessary, non- indicated ureterostomies, vesicostomies, and/or nephrostomies (Fig 16.18 ) Most of the time, the simple drainage of the hydrocolpos takes care of the problem of megaureter and hydronephrosis, except in those patients who have, in addition, vesicoureteral refl ux and diffi -culty emptying their bladder

Vesicostomies are occasionally indicated in these babies when we demonstrate that the common channel is too narrow and interferes with the empty-ing of the bladder Also, we have seen an indication

in babies with massive refl ux and megaureters However, if the patient has a hydrocolpos, the fi rst step should be the drainage of the hydrocolpos, prior to making decisions concerning other proce-dures Almost always, drainage of the hydrocolpos

is all that is needed to decompress the ureters Also, the baby must have an echocardiogram

to rule out cardiac conditions Esophageal atresia must be ruled out in the usual manner A spinal ultrasound is indicated to evaluate for the pres-ence of tethered cord, and an x-ray fi lm of the abdomen will show the characteristics of the lumbar and thoracic spine, as well as the sacrum, and a sacral ratio can be calculated

Between 18 and 24 h after the baby is born, a decision must be made concerning the surgical treatment These babies need a colostomy The primary treatment of a cloaca without a colos-tomy has not been attempted, as far as we know

In Chap 5 in this book, we recommended the opening of a descending colostomy with a mucous fi stula, completely separated from the proximal stoma and reduced in size to avoid pro-lapse, since we only need that orifi ce to perform irrigations and diagnostic tests (high-pressure distal colostogram) In patients with cloaca, we must put more emphasis in being sure that the patient is left with a piece of colon, distal to the mucous fi stula, long enough to guarantee that the pull-through will be possible in the future

If the patient has evidence of hydrocolpos, the surgeon must be prepared to drain the hydrocolpos

at the same time We specifi cally recommend a midline subumbilical incision that will give the surgeons access to the entire lower abdomen

Fig 16.16 Pelvic ultrasound of a patient with bilateral

hydrocolpos

Fig 16.17 Abdominal fi lm of a newborn baby with a

cloaca and a large hydrocolpos

Through this incision, the surgeon can identify the

junction between the descending and sigmoid

colon There, the colon is divided and the stomas

are created, separated enough to be able to use a

stoma bag without including the mucous fi stula

The proximal stoma is created in the left fl ank and

the mucous fi stula in the left lower quadrant At

the same time, through this incision, the surgeon

will be able to drain the hydrocolpos We prefer to

drain it with a tube We have used different types

of catheters for this drainage We specifi cally

rec-ommend the use of a pigtail catheter that can be

exteriorized through one of the lower quadrants

We like a curled catheter because it is less likely to

fall out during the initial several months of life as

the infl ammation recedes and the vagina moves

away from the abdominal wall Since most of the

patients with hydrocolpos have both hemivaginas

distended, what we have done in such cases is to

create a window in the septum between both

hemi-vaginas in order to use a single tube to drain both

hydrocolpos Figures 16.19 and 16.20 show the aspect of the abdomen of one of these babies with

a cloaca after the colostomy has been opened and the hydrocolpos has been drained

Figure 16.21 shows the kidney ultrasound of a baby with a cloaca born with hydronephrosis and hydrocolpos: (a) before the drainage of the hydrocolpos and (b) shows the same patient’s ultrasound after the hydrocolpos has been drained We cannot overemphasize the impor-tance of draining the hydrocolpos We have received a series of patients that had a colostomy, vesicostomy, nephrostomy, or ureterostomy, but not drainage of the hydrocolpos Those patients had multiple problems, including vesicostomy prolapse One of the patients had a pyocolpos, and another one had a perforation of the infected vagina with severe peritonitis Several presented with failure to thrive, acidosis, and urinary tract infections, all of which resolved once the hydro-colpos was drained

If the bladder cannot empty due to the presence

of a quasi-atresia of the common channel, then a

vesicostomy would be indicated Also, in the event

of a patient who has a drained hydrocolpos,

well-decompressed urinary tract but severe refl ux,

megaureter as demonstrated on a cystogram, and

urinary tract infections, a vesicostomy could be

indicated, with a plan for urologic reconstruction

in the future Early ureteral reimplantation of

megaureters in a little baby with a bladder that

most likely will have some degree of malfunction

and kidneys with signifi cant congenital damage is

not recommended We therefore prefer the

open-ing of a temporary vesicostomy, which represents

the best way to protect the kidneys

Some of the hydrocolpos are giant and may

even interfere with the respiratory function Also,

some babies with cloacas are extremely sick at

birth; they have ascites, hydronephrosis, high

creatinine, and severe renal damage

The presence of calcifi ed meconium in the

abdominal fi lm of a newborn baby with a cloaca

[ 44 ] may represent a serious sign Sometimes the

meconium passes through the fallopian tubes into

the peritoneal cavity producing severe peritonitis

Once the colostomy and urogenital tract

decom-pression has been done, the surgical emergency has

been solved Patients usually recover very well

from this operation, and they start eating, growing,

and developing normally The exceptions, of

course, are patients that are born with severe kidney

damage and renal failure, requiring hemodialysis

and consideration for a kidney transplant

A female baby born with a cloaca that has a colostomy and is not doing well postoperatively usually has an undrained obstructed urinary tract with or without hydrocolpos Therefore, the fi rst study in such patients should be an ultrasound to rule out the presence of hydronephrosis, megaureter, large bladder, or hydrocolpos and act accordingly

Fig 16.19 Intraoperative appearance of a case with two large vaginas (bilateral hydrocolpos) ( a ) Before drainage ( b )

Creation of a “window” in the septum between both hemivaginas

Fig 16.20 Abdomen of a patient with a cloaca showing the separated stomas and the vaginostomy tube

Another reason why these patients have sepsis

and do not grow well sometimes is because the

colostomy is inadequate We are strongly opposed

to the opening of loop colostomies in these

babies, because that type of stoma frequently

allows the passing of stool into distal bowel with

direct fecal contamination of the urinary tract

When the patients are well treated, their

colos-tomy is adequate, and their urinary tract and

hydrocolpos are well drained, they usually recover

very rapidly and can go home Within several

months, they will be ready for the main repair

16.1.1.4 Main Repair

In June 1982, for the fi rst time, we had the

oppor-tunity to use the posterior sagittal approach to

repair a cloaca under direct vision Fortunately,

that fi rst cloaca was what we now consider a

“benign type” of malformation, meaning that the

common channel was relatively short (less than

3 cm), and therefore, we were able to repair the

malformation successfully That particular

patient today has urinary control, bowel control,

sexual function, and already has successfully

delivered a baby by cesarean section

During the fi rst few years after 1982, our

approach for the repair of cloacas consisted of

separating the rectum from the urogenital tract

like in all other malformations, followed by the

separation of the vagina from the urethra and bladder, reconstruction of what used to be the common channel as a neourethra, mobilization and dissection of the vagina to be able to pull it down to be placed posterior to the urethra, and performing a pull-through of the rectum to be placed within the limits of the sphincter [ 45 ] (Fig 16.22 ) Soon enough, we learned that that approach was highly successful in a certain type

of malformations that now we call “benign,” but was not successful in other more complex types The main lesson learned during the last 32 years

is that we are dealing with a wide spectrum of defects [ 1 46 ] It has been an eye-opening, con-stant learning experience The more experience

we develop, the more we understand that the spectrum seems to be wider and wider As will be shown in this chapter, the learning process allowed us to design surgical maneuvers appli-cable to different anatomic variants of these defects The posterior sagittal anorectovagino-urethroplasty is the name that we gave to the repair when it was done in the way that was already described, meaning separation and mobi-lization of the three structures (rectum, vagina, and urethra), done posterior sagittally

In many patients, the posterior approach was not enough to repair the malformation, and it was nec-essary to open the abdomen to complete the repair

a b

e

Fig 16.22 Diagrams and pictures showing the technique

originally used by us, before the advent of the total urogenital

mobilization ( a ) Opening ( b ) Diagram showing the

separa-tion of the rectum from the vagina ( c ) Diagram showing the

vagina being separated from the urethra The old common

channel is reconstructed as a neourethra ( d ) Intraoperative picture showing rectum and vagina separated ( e ) Diagram

showing the reconstruction being completed

In 1996, for the fi rst time, we used an innovative

surgical maneuver that we called “total urogenital

mobilization,” which allows us to reduce the

opera-tive time about 70 %, signifi cantly reduces the

blood loss, makes the operation more reproducible,

and renders better cosmetic and functional results

in the management of cloacas [ 47 ] (Fig 16.23 )

(Animations 16.1 and 16.2 ) Subsequently, we

found that the total urogenital mobilization was not

enough to repair more complex types of defects,

and, therefore, we designed the “transabdominal

extended total urogenital mobilization,” which

allows us to repair cloacas with common channels

between 3 and 5 cm Yet, even with the use of an

“extended transabdominal” approach, some acas required further technically demanding maneuvers, including the complete separation of bladder and urethra from the genital tract (Animation 16.3 ) In order to do that, we had to open the bladder and pass feeding tubes through the ureters to avoid their injury In addition, in some patients, we perform a maneuver called “carving the pubic cartilage,” in order to create a shorter tra-jectory for the urethra and vagina to be pulled down behind the pubis and to be sutured next to the clito-ris In some specifi c type of cases, we apply a

clo-a

b

Fig 16.23 Total urogenital mobilization (diagrams showing the basic concept) ( a ) Separation of the rectum ( b ) Total

urogenital mobilization

maneuver called “vaginal switch” that will be

described below [ 48 ] In another group of cases, we

have to replace the vagina totally or partially, and

we perform that with the rectum, colon, or small

bowel Finally, there is a group of cloacas with an

extremely long common channel (more than 5 cm),

in which we leave intact the common channel to be

used eventually as a conduit for intermittent

cathe-terization, and we go directly through the abdomen

to separate the vagina(s), and the rectum, from the

trigone or the bladder neck

As we learned more about the complexity of

cloacal malformations, we developed a serious

concern about the reproducibility of some of the

techniques used to repair complex cloacas

Fortunately, more than 50 % of the cloacas have a

common channel shorter than 3 cm This means

that they can be repaired posterior sagittally,

with-out opening the abdomen and using the maneuver

called “total urogenital mobilization.” We believe

that the total urogenital mobilization is highly reproducible, and we believe that most pediatric surgeons can learn to do it well On the other hand,

we believe that those cases of cloacas with a mon channel longer than 3 cm must be repaired by those surgeons specially dedicated and experi-enced in dealing with these malformations Figure 16.24 shows the different steps of the decision-making algorithm in the repair of clo-acas We will describe each one of them

Cloacas with a Common Channel

of Less Than 1 cm

Figure 16.25 shows a cloaca with a short mon channel In these cases, we recommend a relatively simple procedure that we call a posterior sagittal anorectovaginoplasty The ure-thra is left untouched Basically, what we do in these cases is to separate the rectum from the vagina the same way that we do in cases of

com-(3–5 cm) PSARVUP Total Urogenital Mobilization

Does not reach:

open abdomen extended total urogenital mobilization

-Does not reach:

Separation of vaginas from urinary tract

(catheters on ureters)

Reaches perineum

>5 cm laparotomy

Does not reach:

Big vagina → vaginal switch

Does not reach:

Small vagina → Vaginal replacement

Carve pubic bone

vestibular fi stulas (see Chap 15 ) Next to that,

rather than separating the vagina from the urinary

tract or performing a total urogenital

mobiliza-tion, we mobilize only the lateral and posterior

walls of the vagina, enough as to be able to suture

the edges of the vagina to the skin of the neolabia

(Fig 16.25b ) By doing that, we do not disturb

the urethra or the common wall between the

vagina and urethra, which is a high-morbidity

type of maneuver The cosmetic effect of this

operation is excellent The patients look and

behave basically like a patient operated on for a

rectovestibular fi stula We call this type of cloaca

“cloaca type 1.” The results in terms of bowel and

urinary control are not different from those of

patients with rectovestibular fi stulas when they

have a normal sacrum Figure 16.25c shows the

fi nal result after one of these introitoplasties

These patients may have mild female

hypospa-dias, which is irrelevant because they do not need

intermittent catheterization and because the

ure-thral meatus is readily visible

Cloacas with a 1–3 cm Common Channel

Fortunately, 66 % of our patients with cloacas

belong to this type These patients have, in

gen-eral, a good prognosis Twenty-eight percent of

them will require intermittent catheterization

after the reconstructive operation, and the bowel

function depends very much on the quality of the sacrum and spine [ 1 , 46 ]

The procedure to repair these malformations was performed by us any time from 1 to

12 months of age If a baby happens to be born in our institution and is growing and developing normally, we do it between 1 and 3 months of life Most of our patients, however, come from other institutions, and, therefore, we have experi-ence doing this procedure at different ages

We start the operation by performing copy and cystoscopy We strongly recommend for the general pediatric surgeon to do the vaginoscopy and cystoscopy as a separate setting By doing that,

vaginos-he or svaginos-he will be able to measure tvaginos-he length of tvaginos-he common channel and based on that to:

• Determine whether or not he is capable of doing that operation or if the patient should rather be referred to another center

• Determine whether or not it will be necessary

to open the abdomen for the reconstruction This represents important information for the anesthesiologist as well as the entire operating team It helps with equipment needs, predict-ing operating time, etc

• Determine the fi nal functional prognosis

• Determine whether or not the patient needs a total bowel preparation, in case some form of vaginal replacement with bowel is necessary

Fig 16.25 Intraoperative picture of a cloaca with 1 cm

common channel ( a ) Exposure – multiple silk sutures in

the rectum Observe the vaginal septum ( b ) Sutures

placed in lateral vaginal walls The rectum has been

already separated, and the vaginal septum has been

resected ( c ) Repaired introitus – the lateral walls of the

vagina are sutured to the labia The introitus has been enlarged