Necrotizing enterocolitis in premature twins with twin-to-twin transfusion syndrome.
BACKGROUND
Twin-to-twin transfusion syndrome (TTTS) is a rare but serious clinical constellation that occurs in up to 35 % of monochorionic twin pregnancies. In TTTS, transplacental vascular anastomosis results in a net transfusion from one twin to the other, leading to multiple postnatal complications. We report on two sets of immature twins with TTTS and isolated small bowel perforation with consecutive NEC.


METHODS
The gestational age of the male twins was 25 weeks and the gestational age of the female twins was 27 weeks; the mean birth weight was 560 g in the male and 780 g in the female twins. An assessment of the clinical history, including radiological and intraoperative findings and the postmortem examination is given.


RESULTS
All 4 infants developed NEC with perforation of the distal ileum. Surgical intervention was performed in all 4 children. Three of the four children died due to complications of TTTS with consecutive NEC and septicemia.


CONCLUSIONS
The most common explanation for NEC in TTTS is hypoperfusion of the gastrointestinal tract with consecutive hypoxemic damage, especially in the donor twin. In this report, a combination of immaturity and TTTS contributed to intestinal hypoperfusion and concomitant NEC. The pediatric surgeon should be aware of this complication and we recommend proactive surgical management in cases of TTTS.
Detlefsen
B
B
Kinderkrankenhaus, Kliniken der Stadt Köln gGmbH, Köln, Germany. detlefsenb@kliniken-koeln.de
Boemers
T M
TM
Schimke
C
C
D020345:Enterocolitis, Necrotizing / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D005330:Fetofetal Transfusion / Q000150:complications*; D006801:Humans; D007077:Ileal Diseases / Q000175:diagnosis / Q000209:etiology*; D052577:Infant, Extremely Low Birth Weight; D007231:Infant, Newborn; D007234:Infant, Premature; D007416:Intestinal Perforation / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D011247:Pregnancy; D014427:Twins