Neonatal survival of prenatally diagnosed exomphalos
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.Get Access
Exomphalos is a midline defect, with a viable sac composed of amnion and peritoneum containing herniated abdominal contents with an incidence of about 1 in 4,000 live births. Associated major abnormalities can be karyotypic, syndromic or structural in up to 70% of cases. The aim of this study is to determine the factors that influence survival of antenatally diagnosed exomphalos.
All antenatally diagnosed and postnatally confirmed exomphalos registered with our fetal medicine unit, during 2002–2007, were reviewed. Both prenatal and postnatal outcomes were analysed.
Of 88 cases identified with exomphalos, 85 were prenatally diagnosed. Fifty-five of them died in utero (45 terminations, 5 spontaneous abortions and 5 still births). There were 33 live births (37.5%), 7 of which were premature (30–35/40 gestation). Five babies died before coming to surgery (all with major exomphalos as well as abnormal karyotype) while 28 were operated upon. Fourteen cases with minor exomphalos, all isolated, were primarily closed and all survived to discharge. Of 14 babies with major exomphalos, 4 were closed primarily. Nine required silo formation and six successfully underwent secondary closure (one of which had a prenatal diagnosis of giant ruptured exomphalos). Three died before closure, two from sepsis and multi-organ failure, and one from an undiagnosed tracheo-oesophalgeal cleft. All three deaths had antenatally diagnosed giant ruptured exomphalos and were less than 34/40 weeks gestation. One baby was managed conservatively with antiseptic solution applied to the sac and left to heal by secondary intention. There were 17 cases of isolated exomphalos (with no other structural abnormalities), all of which survived.
Antenatal diagnosis of exomphalos is 96% sensitive. Severe karyotypic and structural abnormalities were present in all intra-uterine and early postnatal deaths. Overall survival to discharge was 28%. Both minor and isolated exomphalos carried a good prognosis. Isolated exomphalos was a better prognostic factor than severity of the exomphalos itself. Ruptured giant exomphalos were associated with a poorer outcome especially in premature babies.
- Groves, R, Sunderajan, L, Khan, AR, Parikh, D, Brain, J, Samuel, M (2006) Congenital anomalies are commonly associated with exomphalos minor. J Pediatr Surg 41: pp. 358-361 CrossRef
- Lakasing, L, Cicero, S, Davenport, M, Patel, S, Nicolaides, KH (2005) Current outcome of antenatally diagnosed exomphalos: an 11 year review. J Pediatr Surg 40: pp. 516-522 CrossRef
- Hatch, EI, Baxter, R (1987) Surgical options in the management of large omphaloceles. Am J Surg 153: pp. 449-452 CrossRef
- Eijck, FC, Blaauw, I, Bleichrodt, RP, Rieu, PN, Staak, FH, Wijnen, MH, Wijnen, RM (2008) Closure of giant omphaloceles by the abdominal wall component separation technique in infants. J Pediatr Surg 43: pp. 246-250 CrossRef
- Lee, SL, Beyer, TD, Kim, SS, Waldhausen, JH, Healey, PJ, Sawin, RS, Ledbetter, DJ (2006) Initial nonoperative management and delayed closure for treatment of giant omphaloceles. J Pediatr Surg 41: pp. 1846-1849 CrossRef
- Roberts, C (1978) Intrauterine diagnosis of omphalocele. Radiology 127: pp. 762
- Murphy, L, Mazlan, A, Tarheen, A, Corbally, T, Puri, P (2007) Gastroschisis and exomphalos in Ireland 1998–2004. Does antenatal diagnosis impact on outcome?. Pediatr Surg Int 23: pp. 1059-1063 CrossRef
- Congenital Anomaly Register for Oxfordshire, Berkshire and Buckinghamshire (CARROBB) (2008). www.npeu.ox.ac.uk/carobb/. Accessed 9 Dec 2008
- Dykes, EH (1996) Prenatal diagnosis and management of abdominal wall defects. Semin Pediatr Surg 5: pp. 90-94
- Venugopal, S, Zachary, RB, Spitz, L (1976) Exomphalos and gastroschisis: a 10-year review. Br J Surg 63: pp. 523-525 CrossRef
- Fisher, R, Attah, A, Partington, A, Dykes, E (1996) Impact of antenatal diagnosis on incidence and prognosis in abdominal wall defects. J Pediatr Surg 31: pp. 538-541 CrossRef
- Horcher, E, Helmer, F, Gherardini, R, Mohl, W, Rosenkranz, A, Zweymüller, E (1979) [Omphalocele and gastroschisis: clinical differences and surgical considerations. A ten year review (author’s transl)]. Wien Klin Wochenschr 91: pp. 81-84
- Neonatal survival of prenatally diagnosed exomphalos
Pediatric Surgery International
Volume 25, Issue 5 , pp 413-416
- Cover Date
- Print ISSN
- Online ISSN
- Additional Links
- Antenatal diagnosis
- Industry Sectors