Madan Samuel

Necrotizing fasciitis: A serious complication of omphalitis in neonates

Necrotizing fasciitis occurs when the inflammation (cellulitis) spreads beyond the umbilicus to include the subcutaneous tissue and underlying fascia. Presently, omphalitis is relatively uncommon because of aseptic delivery techniques and antimicrobial therapy. One hundred three neonates aged 7 to 28 days, with varying degrees of omphalitis, were treated on an outpatient or inpatient basis between 1989 and mid-1993. The neonates were full-term and weighed at least 2.5 kg. Patients with necrotizing fasciitis initially appear deceptively well, which results in less-than-optimum treatment at the outset, followed by a rapid and fulminating downhill course, in turn resulting in death within 24 to 72 hours. Early recognition of the condition, with aggressive resuscitation, appropriate antibiotics, and early surgery are necessary to salvage this high-risk group. The risk factors that may predict the development of necrotizing fasciitis and its early detection are discussed.

Congenital alveolar capillary dysplasia and associated gastrointestinal anomalies.

Congenital alveolar capillary dysplasia is a rare cause of irreversible pulmonary hypertension with 100% mortality. We present three cases of congenital alveolar capillary dysplasia with associated gastrointestinal abnormalities. Three full-term neonates presented with pulmonary hypertension needing ventilatory support by oscillation. Of the three, two neonates subsequently needed extracorporeal membrane oxygenation. Abdominal distension associated with bilious aspirates was the gastrointestinal manifestation. One child had duodenal atresia and anorectal anomaly, one with intestinal malrotation and the other with a rare combination of intestinal malrotaion and total colonic Hirschsprungs disease. All three infants succumbed to pulmonary hypertension at mean age 34 days. The etiopathogenesis and pathology of this condition are discussed with a comprehensive review of the literature.

Lingual Thyroglossal Duct Cyst Presenting in Infancy

Lingual thyroglossal duct cysts are a rare form of thyroglossal cysts. We present two infants, 10 and 12 weeks of age, with midline intraoral cystic swellings stretching from the base of the tongue to the thyroid cartilage. Complete excision of the cysts by Sistrunks procedure were carried out. Both infants are well on follow-up, at 6 months postoperatively. Their unique presentation with regard to age, location, and symptomatology is discussed. Lingual thyroglossal duct cysts large enough to cause dysphagia, stridor, respiratory distress, and failure to thrive in infants have not been previously reported in the literature.

Primary modified Duhamel operation for Hirschsprung's disease in infants

A primary Duhamel pull-through procedure without a covering colostomy was performed for Hirschsprungs disease in 22 infants from 1987 to 1992. The age range at operation was 14 to 90 days (mean 43.7 days). The average weight at surgery was 3.69 kg (2.28–6.20 kg). The most common level of aganglionosis was rectosigmoid (50%). A one-stage Duhamel procedure using a Proximate (Ethicon, Somerville, USA) linear anastomosing instrument (PLC50) was performed in all cases. Postoperative morbidity occurred in 4 patients (18%). There were no associated deaths. Primary corrective treatment without an enterostomy and the feasibility of utilizing a stapling device for the colorectal anastomosis in infants under 3 months of age using the Duhamel retrorectal, transanal pull-through for Hirschsprungs disease is discussed.

Herniation through the foramen of Winslow presenting as obstructive jaundice

Herniation through the foramen of Winslow is a rare variety of paraduodenal hernia. We report a 19-month-old child with obstructive jaundice due to midgut herniation through the foramen of Winslow with associated volvulus. The herniation and volvulus were precipitated by intestinal malrotation. The patient underwent extraamniotic silo repair of exomphalos major in the neonatal period. Investigation for malrotation is recommended after extraamniotic closure of exomphalos.

Multiple gastrointestinal atresias

Three neonates with numerous mucosal septal atresias that occluded the intestinal lumen, commencing at the prepyloric region and ending at the rectum, are reported. The clinical and pathological features are presented and an attempt is made to offer a concept for the pathogenesis of multiple gastrointestinal atresias.

The appendix ― a junkyard

Appendicitis remains the most common condition leading to emergency abdominal operations in children and adolescents. We present a case of foreign bodies in the appendix causing obstruction and perforation of the appendicular tip by a nail.

Low imperforate anus associated with seminal vesicle cyst, posterior urethral valve, and lung hypoplasia

This report describes the association of a low imperforate anus with a seminal vesicle cyst, posterior urethral valve, and lung hypoplasia complicated by necrotizing enterocolitis and unilateral vesicoureteral reflux. The embryological basis, diagnosis, and management of these anomalies is reviewed.