Phẫu thuật tiết niệu trẻ em

TS NGUYEN THANH LIEM PHfiU THU6T TIET NIECI TRE EM nha xuat ban y hoc PHfkl THU6T TIET NIEU TRE EM TS N G U Y t N THANH U € M PHfitl THUflT TIET NIEU ■ TRE EM nhA x u tfr b An v h o c HA N § l - 2002 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 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 nhQng nam gan day da co rat nhidu tien bo chan doan va dieu tri cac di tat 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 nhieu If khac nhau, cac benh duong tiet nieu van duoc chan doan muon, nhieu benh nhan den vien giai doan suy than Da so benh nhan duoc phau thuat giai doan muon, vi vay ket qua han che ve ca phuong dien chuc nang va tam sinh If Cuon “Phiu 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 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 cuon sach Toi cung xin chan 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 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 cang duoc hoan thien hon TAC GIA MUC LUC • • Trang Than u nude ike ph&n noi be than -nieu quan Than - nieu quan doi 24 Nieu quan lac ch6 42 Phinh to nieu quan tien phat ike 51 Luong trao nguoc bang quang - nieu quan tien phat Tui sa nieu quan hay gian nieu quan 66 94 long bang quang nang Hoi chung bung qua man (Prune belly syndrome) Di dang ton tai ong nieu - ron 119 Cac benh ton tai ong phuc tinh mac 124 10 Bang quang than kinh va 110 138 tao hinh tang dung tfch bang quang 11 Van nieu dao sau 158 12 Di tat 16 tieu lech thap 172 13 H e p b ao q u y d a u 196 14 Gian tinh mach tinh 203 15 Tinh hoan khong xuong biu 213 16 Tinh trang gidi tinh khong xac dinh 235 17 Xo^n tinh hoan 264 18 Dai dam 270 19 Ung thu nguyen bao than (khoi u wilms) 280 THAN N l/O C DO TAC PHAN NO I BE THAN - NIEU QUAN ■ ■ Than u nude ike phan noi be than - nieu quan (TPNBTNQ) gap voi tan suat khoang 1/5000 tre so sinh Benh hay gap d trai nhi6u hon gai (65% a trai), ben trai bi nhi6u hon ben phai vdfi ty le 1, 5/1 Khoang 5% cac trudng hop co ton thuong ca 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 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 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 noi be than vdi nieu quan tan - tan duoc Anderson va Hynes gioi thieu nam 1946 de di£u tri hep nieu quan nieu quan chay sau tinh mach chu nhimg sau 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 dudng tiep can khac (4) I THlfONG TON GIAI PHAU NHU MO THAN Chi6u day cua nhu mo than phu thuoc vao miic tac va muc gian cua dai than Nhirng truong hop tac nang nhu mo than hau nhu khong 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 va thdi gian TPNBTNQ Be than co th6 gian tu vai den hang chuc centimet 19 UNG THU NGUYEN BAO THAN (KHOI U WILMS) Khoi u Wilms la loai u tien trien nhanh va hay di can vao phoi nhirng rat nhay cam vdi hoa chat va quang tuyen Khoi u duoc coi la co the chua khoi va co tien luong tot (1) Ban chat u la u nguyen bao than dupe Max Wilms mo ta lan dau nam 1899 Nam 1936, Priestley bao cao ti le song nam sau cat than la 15% Nam 1941, Ladd va Gross su dung dudng ngang de eat than va bao cao ti le song sau nam la 24% Nam 1950 bang ket hop giua cat than va chay tia, Gross bao cao ket qua song sau nam la 47% Nam 1964 Faber ia ngudi dau tien bao cao ket qua song sau nam la 89% bang ket hop phau thuat, chay tia va hoa chat (2,3) Tu den nhieu phac dieu tri hoa chat da dupe nghien cuu ung dung va ti le song sau nam cang cao I TY LE Khdi u Wilm chiem ty le khoang 6% tong sd cac ung thu d tre em Tai Hoa Ky, ti le mac hang nam la 8/1.000.000 tre dudi 15 tuoi, tuong duong vdi khoang 350-450 benh nhan mdi hang nam Tuoi hay gap nhat la tu 1-5 tuoi Rat ft gap u W ilms d tre so sinh va tre ldn (2,3) Ty le gap d hai gidi bang khong cd sir khac biet ve ty le d than phai va than trai Ty le ca hai than deu bi chiem tu 5-6,5% (1) Cd nhi6u di tat bam sinh ket hop vdi u W ilms Hay gap nhat la di tat dudng sinh due tiet nieu va phi dai nua ngudi mdi loai 280 xuat hien vdi ty le 3% tong so cac benh nhan bi u Wilms Di tat dudng tiet nieu sinh due hay gap nhat la 16 tieu thap va tinh hoan khong xuong biu, xuat hien don doc hoac ket hop vdi ty le 4% Di tat khong co mong mat cung hay gap d cac benh nhan u Wilms Ba hoi chung hay ket hop vdi u Wilms da duoc mo ta la: - H oi chung Beckw ith-W iedem ann bao gom thoat vi ron, ludi to, can nang luc sinh cao va co the khong lo, cac benh nhan co nhieu nguy co bi u Wilms cung nhu bi cac khdi u ac tinh khac d o bung nhu ung thu vo thuong than hoac ung thu nguyen bao gan - Hoi chung Dash bao gom u Wilms, mo ho gidi tinh va benh cau than - Hoi chung Perlman la hoi chirng co tmh chat gia dinh vdi hinh anh noi bat la cac tang khong lo, co the khong lo, da di va mat cd bieu hien bat thudng Yeu to gia dinh va nhung bat thudng ve nhi6m sac the sd 11 va 16 d cac benh nhan u Wilms cung da duoc nhieu tac gia de cap (1,2,3) II GIAI PHAU BENH DAI THE U Wilms thudng la khdi u co the tich ldn, lam thay doi hinl the cua than va thudng nam d mot cuc, nhien cung co the nam d nhieu vi tri The tfch khdi u cd the thay doi tu vai chuc gam den hang kilogam theo phat hien muon hoac sdm Doi nhin thay ranh gidi giua khdi u va nhu md lanh cua than Kh cdt khdi u thay cd nhieu vung khong dong nhat: vung mem nhi to chuc nao, vung nhu to chuc sun xen ke cac vung hoai tir 28 Tinh mach than co the bi huyet khoi gay tac Huyet khoi co the lan theo tinh mach chu dudi den tam nhi phai Cac hach tai ch6 thudng cd kich thudc ldn Di can chu yeu theo dudng mau va hay gap nhat la di can phoi VI THE U Wilms la mot u bao thai, thudng cd ba loai md vdi cac ty le khac nhau: to chuc mam, to chirc ong than va to chuc dem Dira vao to chuc hoc ma u Wilms dupe chia lam hai loai: loai to churc hoc thuan lpi va loai to chuc hoc khong thuan lpi Loai to chirc hoe khong thuan lpi la loai u Wilms khong biet hoa cd tien lupng rat xau mac du chi chiem 10% tong sd u Wilms nhung chiem den 50% sd tir vong ciia loai u Theo quan niem trudc day loai to churc hoc khong thuan lpi bao gom ba hinh thai: loai u khong biet hoa, sarcoma te bao sang va u dang co van ac tmh Tuy nhien hien hai loai sau dupe coi la cac hinh thai rieng biet va khong xep loai vao u W ilms Hinh anh to churc hoc dac trung cua loai khong biet hoa la cac te bao cd nhan rong gap 2-3 lan so vdi cac te bao k