Những tổn thương giải phẫu bệnh ở các cặp sinh đôi dính liền tại Việt Nam và Tunisia

NHUNG TON THUDNG GIAI PHAU BENH 6 CAC CAP SINH 001 DINH U EN

TAI VIET NANI VA TUNISIA

KITOVA TANYA TODOROVA - Khoa Giai phiu, Te bdo hqc v i Thai nhi, Dai hqc Y Plovdiv, Bulgaria

LE QUANG VINH - Benh viqn Phy San Trung uong

BORISLAV DIMITROVE KITOV - Khoa phiu thuit thin kinh, Dai hqc Y Plovdiv, Bulgaria

LUU THj HONG - Dai hqc Y H i N&

t 6 m t At

Nhuvg cdp sinh ddi dinh liin bi dinh lien v&i nhau

mQt phin vd thir&ng cd nhCmg ca quan chung, vd

ludn cd cung mQt gi&i tinh. Nguydn nhdn d in d in cdc

cdp sinh ddi dinh lien v in chira duoc hieu rd, trong

khi dd khd ndng sdng s&t cua chung Id r it thip.

Chung tdi tiin hdnh tim h iiu cdc tdn thirong gidi phau

bdnh & 3 cdp sinh doi dinh liin : cdp sinh ddi dinh

ngu-c (TOP), cdp sinh ddi dinh liin & mdt bdn thdn

(DCP), cdp sinh ddi dinh liin & ngi/c-bung (TAP).

Tir khda: sinh ddi dinh lien, gidi p h iu bdnh,

khuyit tdt.

SUMMARY

PATHOLOGICAL FINDINGS AMONG CONJOINED

TWINS IN VIETNAM AND TUNISIA

The conjoined twins stuck together and often

share some organs, and are almost always the same

sex. The cause of the conjoined twins still poorly

understood, while the probability of their survival is

very low. We proceed to study the pathological

lesions in three conjoined twin pairs: Thoraco￾omphalopagus (TOP), Dicephalic parapagus (DCP),

Thoraco-abdominopagus (TAP).

Keywords: conjoined twins, pathology, defects.

OAT VAN 0 £

Trfen th i gi6i c6 30 tnrdmg hap sinh ddi dinh liin

(conjoined twins) c6n s6ng sau khi sinh di/ac biet

din. Nhung cdp sinh ddi dinh liin bi dinh vdi nhau

mpt phin va thudng c6 nhung cor quan chung. Vdi ti

IS 0,2:10000 trubng hop mang thai, chung ta co the

n6i v l ti 0,005:10 000 cdp dinh liin ducrc sinh ra

Theo Luu Thi Hing dj tat trong song thai dinh nhau

gdp 0,002/10 000 tru’dng hop co thai duoc phat hien

qua sifeu 3m [1],

NhO’ng nguydn nhdn xu it hien cua cac cdp sinh doi

dinh liin v ln chua duoc hiiu ro, trong khi do kha ndng

sing sdt cua chung Id r it th ip - chi 18% [14]. Theo

Tippi [18] khoang 28% so cdp sinh ddi dinh liin chet

trong tip cung, trong khi 54% ch it ngay sau khi sinh.

Cd hai gia thuyit giai thich v i sinh ddi dinh liin.

Gia thuyit thu n hit Id fission theory, theo dd su* phdn

ddi bj gidn doan vd nhuna cd th i da dinh hinh - bi

dinh lai vd’i nhau - phdt trien ridng re [15].

Gia thuyit thu hai Id gia thuyet cua Spencer v l su

nhdn ban cua c iu true true thdng quan su hcrp nhit

thu cip, sau khi hai dla phdi (embryonic disc) phdn

ddi hodn todn - d ii xung quang hpc [17],

Nhung cdp sinh ddi dinh liin ludn ludn cd cung

mdt gidi tinh, vi chung ducrc quy djnh gen. Chung

duac phdt triln tir cung mdt t l bdo tn>ng dd thy tinh,

chung cd mdt nhau thai vd mdt khoang 6i (amniotic

cavity) chung. Chung cd chung tiii di vd chung nhau

thai (monoamniotic-monochorionic) Chiing xu it hifen

nhu Id k it qua cua su phdn ddi khdng hodn todn ciia

dia phdi vdo ngdy thi> 14 sau su thu tinh Thudng

gdp han Id cdc bfe gdi: khoang 70-95% [5] sd cdp cdp

sinh ddi dinh liin cd gidi tinh nu Nguyfen nhdn

thudng gdp ciia cdc bfe gdi vdn chira dirac gidi thlch,

mdc dii khoa hpc cd su phdt triin, nhimg chira tim ra

cdu tra Idi cho v in d l ndy.

Nhung cdp sinh ddi dinh ngire (Thoracopagus

Conjoined Twins) chiim khoang 40% tin g sd cdp sinh

ddi dinh liin, cd sy dinh liin a vung nguc, cd chung

cdc ca quan d khoang ngire vd vi th i tdch bifet chimg

sau khi sinh a h iu h it cdc trirdng hop Id khdng th i

Dang dinh liin thudng gdp khde Id dinh viing rdn

(Omphalopagus Twins), khi cdp ddi dinh vdri nhau tir

xuang ire d in viing rdn. Khoang 33% tin g sd cdc

tnrdmg hap sinh ddi dinh liin dirgrc x ip vdo

Omphalopagus. Sinh ddi dinh liin Thoraco￾omphalopagus (TOP) Id tnrdmg hap d iu tifen trong 3

tru’dng hap duac chiing tdi tiin hdnh nghifen ciru

Tnrdmg hap thu hai Id Dicephalic parapagus

(DCP). T in sd ciia sinh ddi Dicephalic parapagus theo

Gilbert-Bamess 2003 [6] Id 1:14000 a chdu Phi vd

1:6500 [7] a chdu A. Cdp sinh ddi dinh liin a dang ndy

bj dinh vdi nhau a mdt bdn thdn. Dicephalic parapagus

Id tnrdmg hap thir hai trong nghidn ciru ndy

Tnrdmg hap thu ba Id Thoraco-abdominopagus

(TAP). Phin dinh liin or vung ngire ndm phia trudrc,

cdn phin dinh liin a vung bung ndm dr mdt bdn. Nd

la dang k it hap ciia Thoracopagus vd Parapagus

Nghien ciru cdc cdp sinh ddi dinh liin cd y nghTa

trong tim h iiu qud trinh phdt triin cua phdi, trong

chiin luac giai phdu phdn tdch chiing. cung nhu

trong viec hodn thien kiin thirc v l cac dang dinh ddi

khde nhau.

TRUONG HOP BENH

1. Tnrdmg hpT> 1: Cf Hd Ndi, Vidt Nam, sA m i ti>

thi: 02/13.

Bdo thai thu duac tir say thai ty nhidn. Id m iu xdc

irdrp cua Bdnh vifen Phu san Trung uomg D ira c bdo

quan trong dung dich formalin 40% trong thdn gian 10

ndm. Gion tinh thai nhi: NO; trpng lirpmg thai khi sinh:

0,800 kg; tu ii thai: 26 tuin. Khdm nghifem b lng m it

thirdmg: 2 diu, 1 c6, dinh nhau d p hin thdn. a phin

tnrdrc ngire, cd 2 ddi chi trfen - veo ngdn ut, cd 2 ddi

chi dirdi, dj tdt thdnh bgng - thodt vj ron.

48 Y HOC THI/C HANH (902) - SO 1/2014