Phẫu thuật tiết niệu trẻ em

Phan tren cua nieu quan se co bop de day nude tieu xuong dudi trong khi doan noi be than - nieu quan dong lai de ngan chan nude tieu trao nguoc.. Luc dau khi ap luc tang len thi luu luon

TS NG UYEN THANH LIEM

PHfiU THU6T TIET NIECI

TRE EM

PHfkl THU6T TIET NIEU TRE EM

LCfl N O I D A U

Benh hoc tiet nieu ma chu yeu la cac di tat ducmg tiet nieu la mot nhom benh If quan trong trong benh hoc ngoai khoa tre em Cac di tat duong tiet nieu co bieu hien lam sang rat da dang va

co tien lircmg tot neu duoc chan doan va dieu tri sdm Tren the giai trong nhQng nam gan day da co rat nhidu tien bo trong chan doan va dieu tri cac di tat nay Cac phucrng phap chan doan hinh anh hien dai dac biet la sieu am truoc sinh da giup chan doan duoc cac dj tat tiet nieu o giai doan rat som va vi vay co the chu dong hoach dinh ke hoach dieu tri Nhieu phuong phap phau thuat moi lien tuc duoc nghien cuu va ap dung da gop phan ciru song va cai thien dang ke chat luong song cua nhieu benh nhan

O niroc ta hien nay do nhieu If do khac nhau, cac benh duong tiet nieu van con duoc chan doan muon, nhieu benh nhan den vien trong giai doan suy than Da so benh nhan duoc phau thuat trong giai doan muon, vi vay ket qua han che ve ca phuong

dien chuc nang va tam sinh If Cuon “P hiu thuat tiet nieu tre e m ”

duoc bien soan voi mong muon giup cac ban dong nghiep, nhat

la cac dong nghiep d cac tuyen trudc co the phat hien duoc cac benh ducmg tiet nieu ngay mot sdm hon

Chung toi xin cam on gia dinh va cac dong nghiep da tao cho toi moi dieu kien thuan loi de hoan thanh cuon sach nay Toi cung xin chan thanh cam on cac benh nhan ma chung toi co dip phau thuat da cho phep chung toi tich luy nhieu kinh nghiem bo ich v6 chuyen mon Chac chan cuon sach nay con co nhung thieu sot nhat dinh, vi vay rat mong nhan duoc cac y kien dong gop cua ban be dong nghiep xa gan d6 cuon sach ngay cang duoc hoan thien hon

TAC GIA

MUC LUC• •

Trang

1 Than u nude do ike ph&n noi be than - nieu quan 7

5 Luong trao nguoc bang quang - nieu quan tien phat 66

trong long bang quang thanh nang

7 Hoi chung bung qua man (Prune belly syndrome) 110

tao hinh tang dung tfch bang quang

THAN 0 N l/O C D O TAC PHAN N O I

BE THAN - NIEU Q UAN■ ■

1

Than u nude do ike phan noi be than - nieu quan (TPNBTNQ)

gap voi tan suat khoang 1/5000 tre so sinh Benh hay gap d con

trai nhi6u hon con gai (65% a con trai), ben trai bi nhi6u hon ben

phai vdfi ty le 1, 5/1 Khoang 5% cac trudng hop co ton thuong ca

2 ben (1)

H in h 1.1 Tdc phan noi be: than - nieu quan

Tai Vien Nhi quoc gia, m6i nam co khoang 15 benh nhan duoc dieu tri (2).

Truoc the ky XIX, phuong phap dieu tri chu yeu la cat than Trendelenburg duoc coi la ngudi dau tien da thuc hien ky thuat tao hinh de dieu tri tac phan noi be than - nieu quan 6 nhirng nam cuoi cua the ky XIX Tru6c nam 1891, phau thuat tao hinh duoc tien hanh theo kieu Heineken - Mikulicz Nam 1891, Kuster lan dau tien da dieu tri thanh cong TPNBTNQ bang that phan cuoi be than va noi be than voi nieu quan ben - ben Ky thuat tao hinh phan noi be than - nieu quan theo kieu chu Y duoc Foley giai thieu nam 1936 Ky thuat cat pM n cuoi be than va phan tren nieu quan, sau do noi be than vdi nieu quan tan - tan duoc Anderson va Hynes gioi thieu nam 1946 de di£u tri hep nieu quan do nieu quan chay sau tinh mach chu nhimg sau do da duoc su dung rong rai de di6u tri TPNBTNQ (3) Cac phuong phap dieu tri bang ky thuat dieu tri noi soi duoc phat trien tir dau thap ky 80 vdi nhieu con dudng tiep can khac nhau (4)

I TH lfO N G TON GIAI PHAU

NHU MO THAN

Chi6u day cua nhu mo than phu thuoc vao miic do tac va muc do gian cua dai than Nhirng truong hop tac nang nhu mo than hau nhu khong con ton tai

DAl THAN VA BE THAN

Dai than va be than co the gian nhe hoac gian r£t rong phu thuoc vao mire do va thdi gian TPNBTNQ Be than co th6 gian tu vai den hang chuc centimet

8

Phan noi be than - nieu quan: nho hep, co the co nga'n lorn

o phfa ngoai Nieu quan co the noi vdi be than o vi tri blnh

thuong hoac cao Trong hau het cac truong hop co the cho duoc mot que tham do di qua doan noi be than - nieu quan

NIEU QUAN

Long nieu quan thucmg nho hon blnh thuong

Mach mau bat thuong 6 cuc dudi than Khoang 1/3 so trucmg hop TPNBTNQ duoc bao cao la co cac mach mau bat thucmg o cuc duoi than (3), tuy nhien Stephens cho rang mach mau bat thucmg o cuc duoi co lien quan den qua trinh quay cua than Neu mach mau cua cuc duoi duoc hinh thanh sdm hon va di vao ron than truoc khi than quay het se gay chen vao be than va gay tac (5)CAC DI TAT PHOI HOP

Luong trao nguoc bang quang - nieu quan phoi hop gap tir

10 - 15% cac truong hop TPNBTNQ (6) Cac di tat khac nhu tac phan noi nieu quan - bang quang, than mong ngua, than nieu quan doi gap voi ti le thap hon

II CO CHfe B $N H SINH

Tac phan noi be than - nieu quan co the do cac nguyen nhan

tu ben ngoai hoac ben trong

Bat thuong ve cau true to chuc hoc cua doan noi be than - nieu quan da duoc mot so tac gia bao cao: cac soi co vong bi thay the boi cac soi co doc, co qua nhieu soi keo giua cac bo co, cac te bao co bi mat lien tuc Cac bat thucmg nay lam cho doan noi be than - nieu quan khong co nhu dong va khong co kha nang day nude tieu xuong nieu quan, vi vay doan noi be than -

nieu quan duoc goi la doan mat churc nang hoac doan khong co dong hoc (7, 8, 9).

Trong than u nude do tac ph<in noi be than - nieu quan, phan tren nieu quan thuong hep mac du co the van cho duoc ong thong di qua Phan hep nay duoc cho la do co bat thudng trong qua trinh phat trien cua nieu quan

Doan noi be than - nieu quan duoc hinh thanh khoang vao tuan thir 5 cua thdi ky bao thai, ve mat to chuc hoc bao gom 3 lop: ldp trong la niem mac, ldp giua la co doc va ldp ngoai la co vong.Trong thdi ky bao thai, nieu quan luc dau la mot ong dac nhung sau do trd thanh mot ong r6ng Khi qua trinh nay khong xay ra hoan toan se lam cho nieu quan bi hep va tac (10)

Tac phan noi be than - nieu quan cung co the do nep cua nieu quan trong thoi ky bao thai con ton tai Cac nep gap nieu quan xuat hien trong thdi ky bao thai do co va niem mac cua thanh nieu quan bi loi vao trong Trong qua trinh phat trien, cac nep gap nay se bien mat nhung khi con ton tai se hoat dong giong nhu mot van day kin long nieu quan (1)

Tac phan noi be than - nieu quan do polyp rat hiem gap Cau true cua polyp la mot khoi to chuc lien ket xo - m ach mau duoc phu bdi bi£u mo binh thucmg (11)

Nguyen nhan gay tac phan noi be than - nieu quan tir ben ngoai hay gap nhat la dong mach than phu hoac bat thucmg Cac mach mau nay chay phia truoc be than va nieu quan gay chen ep vao phan noi be than - nieu quan va gay tac (12) M ac du vay, nhieu tac gia cho rang trong cac truong hop nay nguyen nhan tien phat co the do doan be than - nieu quan khong co churc nang lam cho be than bi gian qua mire va bi xoay, do do nguori ta co cam tudng rang doan noi be than - nieu quan bi chen ep bdi mdt mach mau bat thudng (9)

10

Nieu quan bam vao b i than d vi tri cao cung co the gay tac

phan noi be than - nieu quan, thuong ket hop vdi dinh phan noi

be than - nieu quan va lam mat hinh thai cua be than, lam cho phan noi be than - nieu quan bi gap goc va nieu quan bi ep vao phan be than gian (1,9)

III SINH L f VA SINH L f B$N H

Nude tieu duoc san xuat tir cau than chay vao be than qua cac dai than, voi toe do san xuat nude tieu sinh ly, be than se duoc lap day dan Ap lire trong be than tang len cho den luc cao hon d nieu quan Cac dai than va co be than co bop day nude tieu xuong phan tren nieu quan (13) Phan tren cua nieu quan se co bop de day nude tieu xuong dudi trong khi doan noi be than - nieu quan dong lai de ngan chan nude tieu trao nguoc Long cua doan noi be than - nieu quan thong va nhu dong blnh thudng cua vung nay la cac ye'u to can thiet de cho nude tieu duoc day tir be than xuong nieu quan

Tac phan noi be than - nieu quan co the la do cac bat thudng

ve mat giai phau hoac do nhu dong kern

Tac do nguyen nhan ben trong va ben ngoai se gay nen hai kieu tac voi hai kieu dan luu nude tieu hoan toan khac nhau (9).Tac do cac nguyen nhan ben trong nhu hep nieu quan, doan noi khong co chuc nang han che dong nude tieu thoat qua be than tao nen kieu tac phu thuoc vao ap lire Luu luong nude tieu thoat qua va ap lire d be than phu thuoc vao ap lire va co moi tuong quan tuyen tinh voi ap lire Khi the tfch va ap luc trong be than tang len, luu luong nude tieu thoat qua cung tang len bdi vi mure

do can trd nude tieu d phan noi be than - nieu quan co dinh

Nguoc lai, tac do cac nguyen nhan co hoc tu ben ngoai nhu mach mau bat thudng, nieu quan bam cao tao nen kieu tac phu thuoc vao the tfch va khong co tuong quan tuyen tinh giua luu

luong nude tieu thoat qua va ap luc Luc dau khi ap luc tang len thi luu luong nuoc tieu thoat qua cung tang len, nhung sau do luu luong dot ngot giam xuong vi khi the tfch b£ than tang qua muc se lam tang goc gap giua be than va nieu quan gay can trd nude tieu thoat qua.

Tac phan noi be than - nieu quan co the thucmg xuyen, timg dot, hoan toan hoac khong hoan toan, phu thuoc luong nude ma benh nhan tiep nhan vao va tuong quan giai phau giua be than va phan noi be than - nieu quan

Neu phan noi bi hep nang, ap luc b6 than phai tang len de day nude tieu qua se gay u nude va thuong ton than nang dan, nhung neu phan noi hep nhe thi be than van co kha nang tong duoc nude tieu ma ap lire khong tang len dang ke Trong truong hop sau tang ap lire chi xay ra trong khi bai nieu, khi do nguy co

u nude va thuong ton than tien trien phu thuoc vao nhieu yeu to bao gom tan suat va khoang each bai nieu, the tfch va su thfch ung cua be than, mifc do can trd dong nude tieu

Tac do nguyen nhan ben ngoai co the don doc hoac ket hop vdi nguyen nhan ben trong Tac phan noi be than - nieu quan va

u nude than, be than co the xuat hien timg dot do tac dong cua the tfch len giai phau cua doan noi va su thong thoat cua be than Trong khi bai nieu, tinh trang hep phfa trong se ngan can thoat nude tieu va lam be than gian, khi the tfch be than dat den muc

do ldn, thi co che tac ngoai lai duoc tac dong Ket qua gay gian

be than qua mire va tang cao ap lire trong be than Tidm nang u nude va thuong-ton than phu thuoc vao tan sua't va thdi gian xuat hien bai nieu U nude than co the xuat hien tung dot, nhi6u truong hop the hien khong ro neu the tfch be than thap (14).Cac nghien curu cho thay hau het cac chuc nang cua than deu

bi ton thuong tru chuc nang pha loang nude tieu Sau phau thuat mac du u- nude be than va luu thong nude tieu duoc cai thien nhung chuc nang than hoi phuc khong dang ke (3, 15)

12

IV LAM SANG

Bieu hien lam sang cua tac phan noi be than - nieu quan khac nhau tuy thuoc vao tuoi cua benh nhan Dau bung, dai mau hoac nhidm khuan tiet nieu la cac bieu hien hay gap d tre ldn, trong khi do u bung la bieu hien chu yeu cua tre nho (3, 15).Dau bung hay gap nhung khong dac hieu, dau don doc hoac

co the kern theo cac trieu chung tieu hoa khac nhu non, buon non Dau bung co the xuat hien timg dot sau m6i lan uong nhieu nude lam be than gian dot ngot ket hop vdi bai nieu tang Hien tucmg nay thudng xay ra vdi cac truong hop tac do nguyen nhan tir ben ngoai nhu mach mau bat thudng

Dai mau hay xay ra sau khi dau bung hoac cha'n thuong bung nhe vdi cac muc do khac nhau Ty le benh nhan co nhiSm khuan tiet nieu tuy theo timg nghien curu: Cendron va Valayer gap d 40% benh nhan, trong khi do Kelalis thay hiem gap va neu

co thi la do luong trao nguoc bang quang - nieu quan phoi hop

U bung so thay d viing ho than hoac co the chiem gan het mot nua ben o bung: u mat nhan, deu, kh6ng dau, kfch thudc co the khong doi, thudng nho lai tham chi khong sd thay sau khi benh nhan di tieu rat nhieu trong mot hoac hai ngay

Tac phan noi be than • nieu quan co the thudng xuyen, timg dot, hoan toan hoac khong hoan toan, phu thuoc luong nude ma benh nhan tie'p nhan vao

V CAC X E T N G H IE M CAN LAM SANG

l s i e u Am

Sieu am hien nay duoc sir dung khong chi la mot phuong tien chan doan ma con duoc dung de theo doi tien trien cua than

ur nude do tac phan noi be than - nieu quan Hinh anh sieu am la mot cau true kieu nang 6 trung tarn thong vdi cac nang nho d ngoai vi do cac dai than bi gian tao thanh Nieu quan co dudng kfnh blnh thudng hoac hoi nho, hinh anh nay khac vdi than loan san nhifeu nang bao gom cac nang khong thdng nhau, co cau true Ion xon va khong co nhu mo than (16) Sieu am cung co th£ phan biet duoc tac phan noi be than - nieu quan vdi gian b£ than

do trao nguoc bang quang - nieu quan hoac do tac phan noi bang quang - nieu quan Trong cac benh ly nay, gian be than thudng kem theo gian nieu quan Sieu am cung co the danh gia duoc do day cua nhu mo than cung nhu phat hien duoc cac benh khac nhu loan san than

Sieu am gan day da duoc su dung de ch£n doan cac trudng hop nude be than trudc sinh Cac trudng hop co be than gian tren 10 cm sau khi thai duoc 24 tuan thudng co bieu hien lam sang cua gian b£ than sau de (17) Do nhung kho khan trong viec

do ludng chinh xac kich thudc be than nen Hiep hoi sieu am thai nhi da sir dung moi tuong quan giua kich thudc dai than va nhu

mo than nhu la mot yeu to' tien luong trong TPNBTNQ (18)

2 UIV (C hup dudng tiet nieu can q uang d u dng tin h m ach)

Chup UIV hien van la xet nghiem pho bien de ehcin doan tac phan noi be than - nieu quan Tuy nhien doi vdi cac trudng hop gian be than duoc phat hien trudc de bang sieu am, nen tien hanh chup UIV vao thdi diem nao sau khi de thi hien van con chua duoc thdng nhat

Trudc khi sinh, rau dam nhan churc nang loc cua thai nhi, trong khi than dang duoc hinh thanh Sau khi sinh, than la co quan duy nhat dam nhiem chuc nang dieu hoa nude va dich Ngay sau de, do sure can cua mach mau than tang len lam cho dong mau den than va chuc nang loc cua cau than giam, do do

ap lire be than thudng xuyen thap San xuat nude tieu giam

14

xuong dot ngot sau khi de, sau do tang dan va on dinh d muc toe

do 1 - 2 ml/kg/gid Do so luong nude tieu giam xuong sau de nen chup UIV sdm co the khong thay be than bj gian mac du tac

se bieu hien tren lam sang v6 sau Nhieu y kien cho rang chup UIV chi co the nhin ro duoc than sau khi de 2 - 4 tuan, vi vay chi nen chup UIV sau thang dau (1)

Hinh anh tac phan noi be’ than - nieu quan tren UIV giai doan sdm bao gom gian be than, dai than va khong nhin thay nieu quan cung ben (Hinh 1.1) Cac phim chup muon rat quan trong bdi vi khi be than ngam day thudc se lam cho chan doan d6 hon, dong thdi cung cho phep xac dinh thudc can quang co thoat xuong nieu quan trong giai doan muon khong Phim chup UIV se cho phep xac dinh chfnh xac duoc vi tri tac

Benh nhan bi mat chuc nang hoac suy than co the lam cho hinh anh gian be: than va dai than khong the hien ro 0 tre ldn, khi nghi ngd co tac phan noi be than - nieu quan thi nen chup UIV trong con dau Benh nhan phai duoc uong nhieu nude, co the dung furosemid 0, 5 - 1 mg/kg de tang bai nieu lam cho tac the hien ro Dau xuat hien khi dung thudc loi tieu cung la dau hieu giup cho chain doan

Chup UIV mac du co nhieu uu diem, tuy nhien can phai than trong khi phan tfch ket qua Kinh nghiem cho thay mot than hoan toan khong ngam thudc tren phim UIV khong phai bao gid cung la mot than mat hoan toan churc nang Nhieu trudng hop chuc nang than da trd lai sau khi dan luu than tarn thdi

3 CHUP THAN BANG DONG VI PHONG XA

Chup than bang Tc99m DTPA hoac MAG 3 la phuong phap tot de chan doan than u nude do tac phan noi be than - nieu quan, dong thdi danh gia chfnh xac chuc nang cua than bi benh Chup than bang dong vi phong xa cung la phuong phap d6 phan biet gian be than do tac phan noi b6 than - nieu quan va gian be

than khong do tac Than ur nude duo: coi la do gian b i than

nhung khong co tac khi churc nang than con tir 30 - 50% toan bo churc nang hai than va thdi gian ban thai chat phong xa (T l/2 ) tir

he thdng ong gop dudi 15 phut, gian be than do tac phan noi be than - nieu quan duoc chan doan khi T l/2 >15 phut (19)

4 CHUP BANG QUANG - NIEU DAO TRONG KHI DAl

Chup bang quang nieu dao co gia tri xac dinh cac trudng hop tac phan noi be than - nieu quan co kem theo trao nguoc bang quang - nieu quan

5 CHUP NIEU QUAN NGUOC DONG

Cho phep loai tru tac phan cuoi nieu quan phoi hop, mac du vay chi duoc mot so tac gia thuc hien tren ban mo trudc khi tien hanh phau thuat

VI THAN IT Nl/CfC VA LUONG TRAO NGUOC BANG QUANG - NIEU QUAN PHOI HOP

Luong trao nguoc bang quang - nieu quan phoi hop dat ra nhung kho khan ca trong chan doan va didu tri bdi vi can phai xac dinh xem luong trao nguoc bang quang - nieu quan la thuong ton phoi hop hoac la nguyen nhan gay gian be than va phai mo d phan nao cua nieu quan trudc

Trao nguoc bang quang - nieu quan cung vdi than U nude co the xay ra dudi 3 hinh thai:

- Hinh thai thur nhat la cac benh nhan co than u nude nang do tac nhung cung co trao nguoc bang quang - nieu quan nhe phoi hop Trong tinh huong nay, tac phan noi be than - nieu quan tien phat ton tai va bieu hien ro, trong khi do trao nguoc bang quang - nieu quan lai co bieu hien khong dang ke.16

Ph^u thuat duoc tien hanh d phan noi be than - nieu quan, luong trao nguoc bang quang - nieu quan duoc tiep tuc theo doi va xu ly sau.

- Hinh thai thur hai bao gom nhung benh nhan co luong trao nguoc bang quang - nieu quan nang, nieu quan gian to dong thdi co than ur nude va hep tuong doi d phan noi be than

- nieu quan Cac phim chup bang quang cham se khong thay nude tieu u dong d be than va toan bo he thdng tiet nieu hau nhu khong con dong chat can quang, dieu nay chung to trao nguoc la van de tien phat Nhung trudng hop nay can dieu tri luong trao nguoc bang quang - nieu quan trudc

- Hinh thai thu ba bao gom nhung benh nhan co trao nguoc trung binh ket hop vdi than u nude dang ke va co bieu hien tac phan noi be: than - nieu quan Be than van con day sau khi nieu quan da rdng Trong cac trudng hop nay the tich nude tiSu tir ngoai trao nguoc vao be than da lam mat can bang giua nude tie:u den va nude tieu thoat khoi be than lam cho be than

bi gian cang qua muc De chan doan can phai xac dinh lieu phan noi be than - nieu quan co thich ung vdi dong nude tieu thoat qua khi khong co trao nguoc khong Dieu nay co the duoc thuc hien bang chup than do vdi dong vi phong xa trong khi dat sonde bang quang de dan luu nude tidu lien tuc, bang cach nay co the’ loai trir duoc trao nguoc va xac dinh duoc co tac phan noi b6 than - nieu quan khong, neu khong co tac phai chu y den phan noi nieu quan - bang quang thay vi can thiep vao phan noi be than - nieu quan

VII DI^U TRI

Cac ky thuat di6u tri than ut nude do tac phan noi b t than -

nieu quan bao gom:

- Ky thuat tao hinh be than theo Anderson - Hynes

- Ky thuat tao hinh ki£u chu Y cua Foley

- Ky thuat tao hinh vat thang va vat xodn

- Cac ky thuat noi soi

D udng mo: nhieu dudng mo duoc su dung de tao hinh

be than nhu dudng trang giua, trang ben, dudng ngang phia bung that lung va dudng phia sau lung tuy nhien hien nay 2 con dudng duoc su dung nhidu nhat la dudng ngang phia bung

be than - nieu quan Dung mot ong thong nho cho vao nieu quan day sau ve phia bang quang va bom nude de kiem tra su luu thong M d doc nieu quan dai khoang 1 cm Cat bo bdt mot phan

be than theo chieu tren dudi de lam hep bdt be than (trir trudng hop be than gian nhe) Khau lai be than theo chieu tu tren xuong dudi va tru lai khoang 1 cm phan thap nhat Dat mot ong thong nho qua nhu mo than vao be than va sau do xuong nieu quan Khi nhu mo than con day, nen dat dan luu true tiep qua be than Noi dau tren nieu quan vdi phan tha'p cua be than bang chi vicryl hoac PDS 6/0 (hinh 1 2)

18

H in h 1.2 Ky thuat Anderson - Hynes.

A.cdt bdt mot phan be than va cat nieu quan theo dudng cheo B

Khau lai phan tren be? than C Noi phan dudi bi than \'di nieu quan

2 DI/CJNG SAU LUNG

Khong chi dinh cho than lac ch6, quay be than hoac than mong ngua, m6 lai nieu quan doi, doan hep dai Sir dung cho tre nho, than u nude d muc do trung binh Benh nhan nam sap don d bung Sd nan bd ben cua co cung gai song de1 lam moc, rach da theo dudng ngang dudi xuong sudn 12 tu bd ngoai cua co dai khoang 4 - 5 cm, mo can co cung gai song theo chieu doc Keo co cung gai song va co vuong that lung vao trong de boc lo be than Cac thi con lai tiep tuc tien hanh nhu ky thuat mo bang dudng bung

3 cAc k y t h u A t N 0 I SOI

Tao hinh b6 than bang noi soi ducmg tiet nieu hoac bang ky thuat m6 noi soi o bung duoc ap dung nhieu d ngudi Ion, tuy nhien chua duoc ap dung nhieu d tre em

Ky thuat Anderson - Hynes co ket qua rat cao Ti le thanh cong dat tren 95% Cac bien chung nhu ro mieng noi, hep mieng noi gap vdi ti le tir 2 - 3% (16)

VIII THAN ITNUCIC TRONG THCU KY THAI NHI

Thai nhi bat dau san xuat nude tieu vao khoang tir tuan thur 5 den tuan thur 9 cua thdi ky bao thai Nude tieu thai nhi noi chung

la nhuoe truong (14), khoi luong nude tieu tang dan va dat den dinh cao d cuoi thdi ky thai nghen, nhung lai dot ngot giam xuong sau khi de tir vai ngay den vai tuan va sau do tang dan len

on dinh d toe do 1 - 2 ml/kg/h

DiSu nay giai thfch tai sao trong mot so trudng hop cac xet nghiem tham do tien hanh sdm sau de co the khong thay be than gian nhung lai co cac bidu hien rd rang ve sau (1)

Hien nay nhung tien bo trong ky thuat sieu am da cho phep nghien cuu duoc hinh thai cua dudng tiet nieu trudc de O thai nhi binh thudng, bang quang co the quan sat thay luc 14 tuan than cung bat dau nhin thay luc 14 tuan nhung the hien ro luc 18 tuan (14) Theo doi lien tuc bang sieu am da cho phep hieiu ro hon di6n bien cac trudng hop than u nude duoc phat hien trong thdi ky thai nhi Than ur nude trong thdi ky thai nhi co the tiep tuc phat trien nang hon sau khi de nhung cung co th6 on dinh hoac tu het Grignon theo doi 92 trudng hop than u nude duoc phat hien trong thdi ky thai nhi da thay 50% cac trudng hop co bieu hien binh thudng sau khi de (20) Dejter da quan sat tha'y cac than ur nude trong thdi ky thai nhi mac du co bieu hien binh20

thuong tren sieu am sau de nhung 50% trong so nay lai co b6 than gian va cac benh ly tiet nieu ro rang khi sieu am lai sau 2 thang (21) Ransley bao cao 142 trudng hop than ur nude duoc chain doan trudc de khi benh nhan duoc kidm tra sau de 1 thang

da thay 9 trudng hop chuc nang than giam, trong do 6 trudng hop khong con chuc nang (22) Nhung nghien curu ve di£n bien

tu nhien cua than u nude duoc chain doan trudc sinh da cho ohep rut ra chi dinh theo doi va dieu tri nhu sau:

Tat ca benh nhan chain doan trudc luc sinh can phai sieu am lai sdm sau de, neu sieu am binh thudng phai tiep tuc theo doi va lam lai vai lan trong nam dau trudc khi loai trir duoc tac phan noi be! than - nieu quan, neu sieu am thay be: than gian can lam UIV, than do chup bang quang Neu tac duoc xac dinh can mo sdm khi chuc nang than giam hoac than ur nude nang Nhung trudng hop co tac nhung chuc nang than binh thudng nen mo tir 2 - 4 thang sau de

TAI LIEU THAM KHAO

PP, King LR Belma AB, eds Clinical Pediatric Urology Philadelphia: WB Saunder company; 1992: 693 - 725

Chain doan va ket qua dieu tri than ur nude do tac phan noi

be than - nieu quan Nhi khoa so 2, 1998: 11 - 114

In: Gonzales ET, Bauer SB Pediatric Urology Pratice Philadelphia: Lippincott Williams&Wilkins: 181 - 204

Eur Urol 9: 122 - 124, 1983

5 STEPHENS FD Ureterovascular hydronephrosis and the

aberrant renal vessels J Urol 1982, 128: 984 - 987

existing UPJ obstruction and vesicouretral reflux: diagnosis and therapeutic complications J urol 1989 142: 490 - 493

structure and ultrastructure Part II: Congenital ureteropelvic junction obstruction and primary obstructive megaureter J Urol 1976, 116: 725-727

the pelvi - ureteric junction Br J Urol 1968, 40: 37 - 39

9 KOFF SA Anomalies of the renal collecting system In:

O ’Donelle B, Koff SA, eds Pediatric Urology Oxford:Butterworth;1997: 371 - 379

10 RUANO GIL D, COCA PAYERAS A, TEJEDO

-M ATEN A Obstruction and normal recanalization of the

ureter in the human embryo: its relation to congenital ureteric obstruction Eur Urol 1975, 1: 287 - 289

11 MACKSOOD MJ, ROTH DR, CHANGCH, ET AL Benign

fibroepithelial polyps as a cause of intermittent ureteropelvic junction obstruction in a child: a case report and review of the litarature J Urol 1985, 134: 951 - 952

12 JOHNSTON JH, EVAN JP, GLASSBERG Kl, E T AL

Pelvic hydronephrosis in children Br J Urol 1969, 41:

724 - 726

13 GRIFFITHS DJ, NO TCHAELE C The mechanics of the

urine transport in the upper urinary tract Neurourol urodynamic 1983, 2: 155 - 157

22

14 ELDER JS, KOFF SA The pathophysiology and biological

potential of hydronephrosis in the fetus and neonate In:

O ’Donelle B, Koff Sa, eds Pediatric Urology Oxford: Butterworth;1997: 381 - 395

15 GILLENWATER J HYDRONEPHROSIS In: Gillcnwater

J, Grajhack J, Howard S, et al Adult and pediatric urology 3rd ed St Louis: Mosby, 1996: 973 - 998

16 ROWE MI, O "NEILL JR, GROSFELD Jl.t ET AL, eds

Essential pediatric Surgery St Louis: Mosby; 1995: 693 - 702

17 ANDERSON N, CLAUTICE - ENGLE T, ALLAN R, ET

AL Detection of obstructive uropathy in the fetus:

predictive value of sonographic measurements of renal pelvic diameter at various gestational ages A J Ar 1995, 164: 719 - 7120

18 FERN BACK SK, MAIZELS M, CONWAY JJ Ultrasound

grading of hydronephrosis: introduction to the system used by the Society for fetal urology Pediatr Radiol

1 993,23:478 - 479

19 KASS EJ, FINK - BENETT D Comtemporary techniques

for the radioisotop evaluation of a dilated urinary tract

Urol Clin North Am 1990,17: 2 - 5.

20 GRIGNON A, F1LON R, FILIATRAULT D, ET AL

Urinay tract dilatation in utero: classification and clinical applications Radiology 1986, 160: 645 - 647

21 D E JIE R SU' GIBBONS MD The fate of infant kidney

with fetal hysronephrosis but initially normal ultrasound

J urol 1989, 192: 661 -663

22 RANSLEY PG, DLULLON HK, GORDON I, ET AL The

postnatal management of hydronephrosis diagnosed by prenatal ultrasound J Urol 1990, 144: 584 - 587

THAN - NIEU Q U A N O O I■ ■

Than - nieu quan doi la di tat hay gap nhat cua duong tiet nieu tren vdi ti le 0,7% tren md tu thi va tu 2-4% tren tong so phim chup dudng tiet nieu (1,2) Ty le di tat tim thay trong cong dong la 0,5% (1) O nhieu benh nhan, di tat duoc phat hien tinh

cd, khong co trieu chung lam sang va khong can dieu tri Di tat gap d con gai nhieu hon con trai gap 3,4 lan, ti le gap d ben phai

va ben trai khong khac nhau, than - nieu quan doi ca hai ben chiem ti le 15% (3) Tien sir gia dinh kha ro net, tan suat benh nhan co anh em ruot hoac bd me cung bi di tat la 1/8 (1)

1 CAC HINH THAI CUA THAN - NIEU QUAN DOI

Than - nieu quan doi co the hoan toan hoac khong hoan toan (1,

2, 4, 5) Cac hinh thai than - nieu quan doi co the gap la (hinh 2.1):

- Nieu quan doi hoan toan: hai nieu quan chay tir hai b i

than khac nhau, hai Id nieu quan do vao cac vi tri khac nhau

- Nieu quan doi khong hoan toan: hai nieu quan dan luu tir hai be than khac nhung chi chay rieng nhau mot doan roi sau do

dd chung vao mot phan tan ciing, doan chung co the dai tao thanh hinh chu Y hoac ngan sat bang quang tao thanh hinh chu V

Nieu quan che doi khong hoan toan nguoc chi6u: meu quan d tren tach thanh 2 nieu quan rieng nhau d phia dudi

- Nieu quan doi hoan toan hoac khong hoan toan vdi mot nhanh tan cung thanh tui ciing tit trudc khi den be than

- Cac trudng hop 3 hoac 4 nieu quan do co nhieu mam nieu quan rat hiem gap (2,5)

24

H in h 2.1 Cac hinh thai cua than - nieu quan doi.

A Nieu quan doi khong hoan toan

B Nieu quan doi hoan toan

II SINH BENH H O C

Nieu quan doi la hau qua cua nhung bat thudng trong qua trinh phat trien bao thai

Nieu quan duoc hinh thanh tu ong Wolf Cac ong Wolf co nguon goc tu trung mo duoc hinh thanh trong moi lien quan vdi cac ong ti6n than va trung than nam d thanh lung cua xoang co the.Cac ong trung than phat tridn ve phia duoi cua bao thai va sau do quay ngoat ve phia trudc de gap o nhdp va do vao do khoang vao tuan 16 thu 5 (thai 4mm) Nieu quan duoc hinh thanh tir mot mam nieu quan, phat sinh tir ong W olf (ong trung than), noi ong nay uon cong trudc khi den o nhdp (thai 5-6mm) Mam nieu quan dai ra tien ve va xuyen vao mam hau than trong khi hau than dang di chuyen tir viing xuong cung len vung that lung Tir mam nieu quan se hinh thanh nen he thdng ong trong than va dai than, trong khi do cac nephron lai duoc hinh thanh tir to chuc hau than

Trong giai doan phat trien tiep theo ong W olf gian ra va hoa vao trong xoang tiet nieu sinh due Cac nieu quan tach rdi khoi ong trung than, cac 16 nieu quan do vao phan thap cua xoang tiet nieu sinh due de tao thanh trigon

0 con trai, ong W olf di chuyein vao giua va xuong dudi de hinh thanh ong dan tinh, tui tinh, ong phong tinh va do vao nieu dao tien liet tuyen, d con gai ong W olf teo di hoac ton tai nhu mot di tich nam d mat trudc ben thanh am dao, tir cung va day chang rong (1, 2, 4, 5, 6)

Than - nieu quan doi hoan toan la do co hai mam nieu quan phat sinh tir ong W olf rieng re nhau Mot mam d phia tren va mot mam d phia dudi Moi mam nieu quan tien rieng ve phia hau than Nieu quan tren hinh thanh dai than tren va dan luu be! than tren Nieu quan dudi hinh thanh dai than giua va dudi, dan luu26

phan giua va duoi cua than Ve phia xoang tiet nieu sinh due, sau mot qua trinh tien trien phuc tap ong Wolf do vao phia dudi va phia trong so vdi vi tri ban dau va khong eon nam trong bang quang ma d phan day cua xoang tiet nieu sinh due noi ma cu Muller se hinh thanh u nui d nam gidi hoac tien dinh d nu gidi Trong qua trinh di chuyen nay, ong W olf loi keo mam nieu quan tren lam cho no di vao phia trong va sau do di ra phia sau nieu quan dudi roi do vao bang quang d vi tri tha'p hon (quy luat Weigert-Meyer) Nieu quan doi khong hoan toan la do mam nieu quan phan doi sdm trudc khi tien den mam than (1 ,2 , 5).

III THUONG TON GIAI PHAU

1 THAN - NIEU QUAN DOI HOAN TOAN

1.1 Than

Thudng chi co mot khoi nhu mo than duoc bao quanh bdi mot vo than Than co hinh dang cua than binh thudng, tuy nhien kfch thudc nho hon va dai hon Be than tren nho, be than tren thuc ra chi co mot dai than tuong irng vdi khoang 1/3 nhu mo, be than dudi to hon Giua hai be than doi khi co mot ngan nho phan cach Khi co viem than-be than (phan tu than tren) hoac u nude (phan tu than dudi), gidi han giua hai viing than va nieu quan trd nen ro hon

Doi khi giua hai be than co the co mot khoi mo cau than tuong ung vdi mam hinh thanh 2 cue than va du phat trien de co the hinh thanh mot khoi tan sinh trong than (6)

Thuc ra thudng khong co binh dien boc tach ro rang giua hai be

than, nen khi can phai md b i than cua don vi benh ly de: lay bo no

ma khong lam rach be than con lai hoac lam ton thuong mach mau

Co the; co hai cuong than nhung thudng chi co 1 cuong than chung, tuy nhien co mot hoac hai mach mau di vao circ tren tir ngoai ron than, vi vay cat phan tir than tren thudng d i

Nieu quan tren chay sau cac mach mau cua than roi chay vao phia trong so voi be than duoi, sau do hai nieu quan co the chay song song va khong bat cheo nhau nhung da so trudng hop hai nieu quan bat cheo nhau hai lan Nieu quan tren luc dau chay

tu trong ra ngoai roi sau do chay tu ngoai vao trong, thudng d phia trudc nieu quan dudi Co the tach hai nieu quan xa nhau ma khong so lam thuong ton mach mau cua m6i nieu quan trir doan sat bang quang

Khi den sat bang quang, hai nieu quan co the dinh chat vao nhau va nam trong mot bao chung cung vdi he thdng mach mau chung Khi tach rdi hai nieu quan, co the lam mat nuoi dudng mach mau con lai

Hai nieu quan chui vao bang quang cung mot khe chung neu khong co nieu quan nao do lac ch6 ra ngoai bang quang Sir sap xep cua chung tuan theo quy luat W eigert-M ayer (nieu quan tren

do xuong dudi thap, trong khi do nieu quan dudi lai do cao d phia tren) (1, 2, 7)

L6 nieu quan dudi thudng d vi tri binh thudng trong tarn giac Trigone nhung co the lac ch6 sang ben va len cao, do do dudng ham dudi niem mac ngan de gay trao nguoc bang quang-nieu quan

Nieu quan tren chay qua 16 nieu quan d phia trong nieu quan dudi, roi chay ra phia sau no do vao trong va xuong thap hon vi vay co dudng ham dudi niem mac dai hon va co lien quan bat thudng vdi co d vung Trigone nen d i bi u dong nude ti6u va tac.Tuong quan giua cac nieu quan khong theo quy luat Weigert-M ayer hiem gap L6 nieu quan tren nam canh va phia trong 16 nieu quan dudi, tham chi co the do len phia tren 16 nieu quan dudi (8)

1.2 Nieu quan

28

2 THAN NIEU QUAN DOI KHONG HOAN TOAN

IV CAC HINH THAI BENH L t GAP TRONG N I$U

q u A n DOI

Ben canh 16 nieu quan do lac ch6 ra ngoai bang quang va gian doan cuoi nieu quan trong long bang quang se duoc trinh bay d cac phan sau, di tat nieu quan doi con co the dan den cac bien chung sau:

1 TRAO NGUOC BANG QUANG - NIEU QUAN

Luong trao nguoc tu bang quang vao nieu quan dudi ratthudng gap, Fehrenhaker gap 75% cac trudng hop nieu quan doi

co bieu hien nhiim trung tiet nieu (9) Lu6ng trao nguoc thudng

d muc do nang Mollard thay 58% cac trudng hop be than dudi

bj bien dang vdi cac dai than bi co keo (2)

Luong trao nguoc thudng ket hop vdi viem than-be thannguoc dong Luong trao nguoc vao nieu quan tren hoac vao ca hai nieu quan co th i xav ra nhung hiem gap, nguyen nhan la do

16 nieu quan cua nieu quan tren md rong hoac cung do ngang muc vdi nieu quan dudi (6)

2 PHINH TO NIEU QUAN

La bien chung dac trung thur hai cua nieu quan doi hoan toan Nieu quan tren bi phinh to do doan chay trong dudng ham duoi niem mac bang quang qua dai va co tuong quan bat thudng vdi co d Trigone hoac co bang quang 10% cac trudng hop nieu quan doi co nieu quan tren phinh to (2) Thuong ton do phinh to nieu quan thudng nang ne, 90% cac trudng hop be than tren bi pha huy, gian cuc dai kern theo viem than-be than nang Phan tu than tren thudng khong ngam thudc tren phim chup UIV, nhung day dai be than dudi xuong dudi va ra ngoai

Phinh to nieu quan tren ket h(^) vdi luong trao nguoc vao nieu quan tren va dudi rat hiem gap Mollard gap 5 trudng hop phinh to nieu quan tren va trao nguoc vao nieu quan dudi (2)

3 TRAO NGUOC NIEU QUAN-NIEU QUAN VA IT DONG 6 VUNG HOP LUU

Trao nguoc nieu quan-nieu quan la hien tuong trao nguoc nude tieu tir nhanh nieu quan nay vao nhanh nieu quan kia trong cac trudng hop nieu quan doi khong hoan toan Luong trao nguoc la do cac co bop d hai nhanh cua nieu quan khong dong

bo va do ap luc trong nhanh nieu quan luc nghi thap horn so vdi vung hop luu va vung nieu quan chung Luong trao nguoc khong gap khi hop luu nam thap trong thanh bang quang vi luc do cac song nhu dong se lam cho doan nieu quan tan cung chi co cac dai co doc d i dang mo ra va nude tieu duoc tong xuong bang quang (10, 11)

4 TAC PHAN NOI BE THAN-NIEU QUAN

Tac phan noi be than-nieu quan thudng xay ra d be than va nieu quan dudi Khi be than dudi khong ngam thudc, hinh anh30

tren phim chup UIV d i nham vai u Wilms vi mat hinh cac dai than giua va dudi.

V T R IE U CH U N G VA CHAN DOAN

N hiim khuan tiet nieu, dai kho, dau vung tren xuong mu hoac that lung cung vdi cham tang trudng la cac bieu hien thudng gap

Cac benh nhan co nieu quan tren lac ch6 ngoai bang quang thudng co bieu hien son nude tieu lien tuc dong thdi van co cac lan tieu tien binh thudng 0 con trai neu nieu quan tren lac ch6 vao ong dan tinh, tui tinh, thudng co bieu hien viem tinh hoan hoac mao tinh hoan cung ben Cac benh nhan den muon co gian nieu quan va u mu be than, co the co bieu hien noi bat la mot khoi u bung cong vdi da'u hieu nhiim khuan (13) Nghien curu 40 trudng hop bi than nieu quan doi, chung toi thay cac dau hieu lam sang chu yeu nhu sau (3):

B A N G 2.1 C A C DAU HIEU LAM SANG CUA THAN NIEU

Chup UIV: la phuong phap chan doan co gia tri nhat Chup

UIV co the: thay hinh anh true tiep cua nieu quan doi (hai nhom dai be than, hai nieu quan) khi ca hai dcm vi than con chuc nang

va ngam thuoc tot (hinh 2.2)

Khi don vi than khong con chuc nang, chan doan than nieu quan doi co the? dua vao cac dau hieu gian tiep sau:

Bong than dai hon binh thudng

Be than va dai than dudi bi day xa cot song, xuong dudi, nam ngang hon so vdi binh thudng (hinh anh bong hoa ru) (hinh 2.3)

- Nieu quan dudi dai va uon khuc, co khi bi day lech sang phia doi dien

Sieu am: thay duoc hai khoi nhu mo than va co th6 thay

duoc be than cung vdi nieu quan tuong irng bi gian

Chup bang quang: phat hien trao nguoc bang quang - nieu

quan hoac tui sa nieu quan ket hop

VI DIEU TRI

Than nieu quan doi chi co chi dinh dieu tri khi co nieu quan

do lac ch6 ra ngoai bang quang hoac khi co cac bien chung

D iiu tri cac trudng hop nieu quan doi co nieu quan tren lac cho co th6 la cat nieu quan cung vdi don vi th an tren hoac d iiu tri bao ton nhu noi nieu q u an -n ieu quan hoac trong lai ca hai nieu quan tuy thuoc vao churc nang th an

1 CAT BO PHAN TU TH A N TREN CUNG VCti NIEU QUAN TUONG UNG KHI THAN TREN KHONG CON CHUC NANG

Ky thuat tien hanh nhu sau:

Benh nhan nam ngua co don sau lung Rach da theo dudng ngang sau ben, ngay dudi xuong sudn XII Sau khi cat co, day32

phuc mac vao trong, tim nieu quan Nieu quan tren thucmg gian to hon nieu quan dudi neu khong co trao nguoc tu bang quang vao nieu quan duoi Phau tfch nieu quan tren khoi nieu quan duoi Cat doi nieu quan tren Tiep tuc boc tach nieu quan tren khoi nieu quan dudi len den sat be than Luon nieu quan phia dudi cuong mach cua than keo nguoc len phia tren Cap cat va buoc cac mach mau di vao phan than tren Sau khi cap cat mach mau co the nhin thay r5 ranh gidi giua vung than tren va than dudi dua vao mau sac cua nhu mo Cat phan than tren bang cach di sat theo bd ngoai be than tren KM u cam mau mom cat.

Tiep tuc tach nieu quan tren khoi nieu quan dudi cho den sat thanh bang quang Chu y khong lam ton thuong mach mau nuoi nieu quan dudi Cat bo nieu quan tren sat thanh bang quang M d rong mom cung, co the dot niem mac cua phan tan cung va de ho mom cat (hinh 2.4)

Co the cat than-nieu quan tren bang cat than trudc Sau khi boc lo than, tim , cap cat va buoc cac m ach m au cua cuc tren than Cat than tren khoi than dudi Tach be than tren khoi be than dudi va tach nieu quan tren khoi nieu quan dudi theo chieu tir tren xuong dudi de cat bo nieu quan dudi

d phan thap nhat

Tu nam 2000, chung toi da bat dau cat than - nieu quan tren bang ky thuat noi soi Benh nhan nam ngua hoi nghieng sang ben doi dien Vao o bung bang 3 cua so: cua so qua ron cho ong soi, mot cua so dudi ron va mot cua so tren ron cho dung cu Cac chi tiet ky thuat con lai tien hanh nhu trong phau thuat quy udc

H inh 2.2 Phoi thai hoc cua nieu quan doi.

A Hai mam nieu quan phat sinh tir ong Wolffian

B Mam nieu quan duoi do vao phia tren xoang tiet nieu sinh due khi ong Wolffian bi cuon vao thanh bang quang

C Mam nieu quan tren do vao phan thap cua xoang tiet nieu sinh due

D Hinh anh cuoi cung cua nieu quan doi sau khi ong Wolffian

do vao nieu dao nhu ong xuat tinh

H in h 2.3 A Hinh anh true tiep cua than nieu quan doi.

B Hinh anh gian tiep cua than nieu quan doi phai kern tui sa hieu qua

H in h 2.4 Cat nieu quan va don vi than tren.

A Cat doi nieu quan

B Luon lieu quan phia sau cuong mach len tren

C Cap cat cac mach mau vao don vi than tren va cat dai dan vi than tren cung vai nieu quan

D Khau cam mau mom cat than

36

H in h 2.5 Noi nieu quan

tren vao bS than duoi

Hinh 2.6 Noi nieu quan tren vao nieu quan duoi sat thanh bang quang

2 CAC KY THUAT BAO TON

Khi benh duoc phat hien sdm, dan vi than tren co churc nang tot co the tien hanh noi nieu quan tren vao be than duoi (hinh 2.5) hoac noi nieu quan tren vao nieu quan dudi sat thanh bang quang (hinh 2.6) hoac trong lai ca hai nieu quan vao bang quang (hinh 2.7)

Ky thuat duoc chung toi hay dung hien nay la noi nieu quan vao nieu quan sat thanh bang quang Ky thuat duoc tien hanhnhu sau:

Rach da theo dudng Pfanenstiel Boc lo bang quang Boc tach thanh ben ngoai bang quang Tim day chang tron, cat day chang tron Dung day chang tron lam moc tim nieu quan Tach rdi hai nieu quan d vi tri sat bang quang Nieu quan tren thudng

to hon nieu quan dudi neu khong co trao nguoc vao nieu quan dudi Cat rdi nieu quan tren sat thanh bang quang Noi nieu quan tren vdi nieu quan dudi tan ben bang chi tieu 6/0

Cac nguyen tac dieu tri luong trao nguoc bang quang-nieu quan trong nieu quan doi giong nhu ludng trao nguoc bang quang-nieu quan don

Tuy nhien kha nang trao nguoc tu het bang khang sinh du phong d cac trudng hop than-nieu quan doi it hon trong cac trudng hop nieu quan don (6, 12, 14)

Trong cac trudng hop co chi dinh trong lai nieu quan thi nen trong lai ca hai nieu quan tren va dudi cung vdi nhau

K y thuat nhirsau:

Rach da theo dudng Pfannenstiel Boc lo va md bang quang theo chieu doc Tim cac lo nieu quan Dat sonde vao cac nieu quan de lam moc Khau hai soi chi d hai lo nieu quan can trong lai Rach vong quanh ca hai 16 nieu quan Boc tach ca hai nieu

38