OBJECTIVE Wiedemann-Beckwith syndrome is a multisystemic pattern of congenital anomalies with overgrowth. The most characteristic clinical features are macroglossia, high birth weight, omphalocele, visceromegaly and hypoglycemia. PATIENTS AND METHODS We show the clinical and epidemiological characteristics of the 18 cases with Wiedemann-Beckwith syndrome identified in the consecutive series o...

We compared perinatal outcomes between live-born nonisolated and isolated omphaloceles diagnosed during a prenatal ultrasound. Fetuses (n = 86) with omphalocele were identified between 1995 and 2007 at a single institution. Inclusion criteria were an omphalocele >14 weeks' gestation, available fetal and/or neonatal karyotype, and a live-born infant (n = 46). Perinatal outcomes were compared in ...

Introduction Gastroschisis is a congenital anterior abdominal wall defect, adjacent and usually to the right of the umbilical cord insertion. It occurs as a small, full-thickness periumbilical cleft either immediately adjacent to the umbilicus or separated from it by a strip of skin. This results in herniation of the abdominal contents into the amniotic sac, usually just the small intestine, bu...

Giant omphalocele (GO) management is controversial and not easy. Conservative management at birth and delayed surgical closure is usually mandatory. Postponed surgery may be challenging and carry the risk of intensive care treatment. We report on five children who were treated in our department for GO between 2000 and 2010. Initially, the patients were managed conservatively in West Africa. Del...

Deepak Sharma*, MD, DNB; Srinivas Murki, MD, DM; Tejo Pratap, MD, DM Department of Neonatology, Fernandez Hospital, Hyderabad, India Received: Aug 27, 2014; Accepted: Jul 09, 2014; First Online Available: Aug 15, 2014 A late preterm male baby with a birth weight of 2.5 kg was born to primi mother. Baby cried immediately after birth with an Apgar score of 8/8/9. Baby was antenatally diagnosed as...

Abdominal wall defects are associated with other intra-abdominal anomalies. We report two neonates with omphalocele associated with intra-abdominal anomalies. One neonate had multicystic kidney. Other neonate had duplication cyst of ileum which was missed during initial closure in neonatal life.

Short rib-polydactyly syndrome (SRPS) is a rare congenital autosomal recessive inherited disease classified in four subtypes. Prenatal ultrasonography findings and postnatal examinations may help classify one of We present case an infant who presented multiple anomalies the radiography helped to SRPS as type IV (Beemer-Langer). A 23-year-old was referred for prenatal ultrasound which showed cys...