William K. Sieber

Alimentary tract duplications in children.

This report reviews the experience with 78 alimentary tract duplications found in 64 patients over a 40 year period at the Childrens Hospital of Pittsburgh. Ten patients had the duplication discovered at autopsy. Multiple duplications were found in 15% of patients. The symptoms and physical findings of a duplication varied with location, size and mucosal pattern. About one-filth of the duplications contained ectopic mucosa, usually gastric. Two-thirds of the patients were diagnosed prior to one year of age. Vertebral anomalies, as a clue to the presence of the lesion, were present in 15% of the patients. The most common indications for surgery included a mediastinal or abdominal mass, intestinal obstruction, and gastrointestinal bleeding. The results of surgery were favorable, with a mortality of 20%. Surgical complications accounted for six deaths, while four children died of severe associated anomalies. Three others died without surgical treatment, but with symptoms from the duplication.

Hypertrophic pyloric stenosis at the children's hospital of pittsburgh from 1912 to 1967 A critical review of current problems and complications

Abstract Review of 1215 patients treated for hypertrophic pyloric stenosis during the period from 1912 to 1967 inclusive, demonstrates that the mortality has decreased to 0.5 per cent and the rate of complications to 9.5 per cent. Infections, usually pneumonia, have been the principle cause of death in the 27 infants who did not recover following pyloromyotomy. Postoperative complications now most commonly observed are incisional infections and gastroenteritis. Roentgen examination was used to aid in the diagnosis of pyloric stenosis in 254 infants. In 154 instances, it was probably unnecessary. When pyloric stenosis is present in an infant with a cleft lip and palate, diagnosis may be difficult and delayed. Such infants require special care. Nine infants in this series required a second pyloromyotomy. Only 35 per cent of the patients in this series never vomited postoperatively. Postoperative vomiting of varied degree occurs frequently after successful pyloromyotomy and probably represents variations in the return of gastric peristalsis.

IMPERFORATE ANUS. REVIEW OF A SIXTEEN YEAR EXPERIENCE WITH 146 PATIENTS.

Abstract A critical analysis of a series of 146 cases of imperforate anus is recorded and compared with other recent large series appearing in the literature.

Traumatic Pseudodiverticulums of the Pharynx in Newborn Infants

Abstract Perforation of the posterior pharyngeal wall during or immediately after delivery produced symptoms of esophageal atresia and traumatic diverticulums of the pharynx in two newborn infants....

Reconstruction of penile agenesis by a posterior sagittal approach

Penile agenesis is a rare condition requiring gender reassignment and staged perineal reconstruction. This report describes two children reconstructed by taking advantage of the posterior sagittal approach. This approach allows a precise anatomic dissection, construction of a neovagina and accurate positioning of all perineal orifices. We think that this is the preferred approach for this rare condition.

Sacrococcygeal teratomas in children

Abstract Forty-four patients with sacrococcygeal teratomas are reported, and their surgical management discussed. Early complete excision with removal of the coccyx is recommended and was practiced in 33 cases. Such management eliminates recurrence, with its increased chance of neoplastic change. The mortality in this series from all causes was 27%.

Meconium ileus: A critical review of treatment and eventual prognosis

Abstract Forty-six patients with meconium ileus were treated from 1951 to 1969, of whom 23 (50%) patients died in the immediate postoperative period. Fourteen of the remaining are now alive and are 2 to 14 years of age. Long-term survival does not differ from other patients with cystic fibrosis. One-half of the patients survived to leave the hospital, one-half of the remainder succumbed to cystic fibrosis, leaving one-quarter of the total to live a reasonably normal life but under the constant regimen to which all children with cystic fibrosis must submit. The majority of these patients (24) were treated by enterotomy and hydrogen peroxide or acetylcystine irrigation.

Does Zonal Aganglionosis Really Exist? Report of a Rare Variety of Hirschsprung's Disease and Review of the Literature

The aganglionic segment of intestine in Hirschsprungs disease begins at the anus and extends proximally for a distance that varies from case to case. Occasional reports describe patients in whom the aganglionosis is segmental, with normal distal innervation or a skip area of normal innervation within an area of aganglionosis. This paper describes 4 patients with Hirschsprungs disease wherein a segment of normally innervated colon was found in an otherwise aganglionic colon. Two of these patients were siblings with different fathers. Problems encountered in the management of these patients are detailed. In a critical review of the literature, 2 additional male patients with well-documented zonal aganglionosis were identified. Although variations from the usual morphology or Hirschsprungs disease do exist, they are so rare that they merit clinical consideration only when the anatomic record and the clinical course are in obvious disagreement. Rectal biopsy remains the best method for the diagnosis of Hirschsprungs disease.

Fertility in true hermaphroditism

Abstract Fertility is documented in a patient with true hermaphroditism who presented at birth with ambiguous genitalia. Gender assignment was initially as a male, then appropriately changed to female. Appropriate reconstruction of the external genitalia and removal of the right ovatestes was done in infancy. The uterus, ovary on the left, and left fallopian tube were left intact. Puberty and menstruation occurred normally. Further genital and vaginal surgery was done during late teenage years. Conception and pregnancy were uneventful.

Double choledochus with ectopic drainage into the stomach—A rare congenital anomaly of the biliary ductal system†

A patient with an accessory bile duct originating from the left common hepatic duct and emptying into the stomach along the lesser curvature presented with periodic symptomatic bile gastritis. Reimplantation of the accessory duct into the duodenum relieved his symptoms.