Judson G. Randolph

Reduction clitoroplasty in females with hypertrophied clitoris

Abstract There is constantly a better understanding of the variety of congenital malformations of the genitalia which comes under the heading of intersexual abnormalities. Prompt recognition of these anomalies can now lead to correct sex assignment shortly after birth, minimizing the anatomical, psychological and social problems for these individuals in later life. For female babies born with an enlarged clitoris, total clitorectomy has become an established procedure. It has been shown that the clitoris is not essential for adequate sexual function, but its preservation would seem to be desirable if this could be achieved while maintaining satisfactory appearance and function. Operations to reduce or cover the enlarged clitoris without removing it have been suggested in the past, 1–3 Certain drawbacks of these procedures have prompted our search for a surgical technique which would preserve the clitoris in its anatomical position, yet permit the reconstruction of normal female contours during the important growing years of childhood. An operation has been devised which we believe meets these requirements. Our experience with this procedure over the past five years and its postoperative evaluation in female patients is reported here.

Tracheal agenesis: Recognition and management

Abstract A case of congenital agenesis of the trachea with esophagobronchial fistula is presented and the literature on the subject reviewed. A therapeutic plan is proposed for the management of this lesion in the immediate postnatal period.

Ectopic umbilical liver in conjunction with biliary atresia: uncommon association.

The case of a neonate with an ectopic liver in the umbilicus in conjunction with biliary atresia in the liver proper and an ectopic pancreas in the jejunum is reported. Following excision of the ectopic umbilical liver and Kasai type 1 hepatic portoenterostomy, bile fistula originating in the anterior inferior area of the right lobe of the liver was a complication, and it was successfully treated by construction of hepaticojejunostomy. No similar case has been reported in English language literature. It is of particular interest that the pathological features of the ectopic liver and the liver proper are quite similar.

Metabolism and Excretion of C14-Labeled Bilirubin in Children with Biliary Atresia

BILIRUBIN metabolism and excretion in children with biliary atresia is complex and poorly understood. Jaundice may not be apparent until the infant is three or four weeks of age. Rarely, as in a case reported by Sterling,1 the jaundice disappears, and for weeks the serum bilirubin levels are normal. Usually, however, the serum bilirubin levels continue to rise for several weeks after birth and thereafter remain relatively stable. Like the normal infant, the child with biliary atresia is continuously producing bile pigment from the breakdown of hemoglobin. Since the serum bilirubin levels do not continue to rise one must assume .xa0.xa0.

Malignant liver tumors in infants and children

At the Children’s Hospital in Washington, D.C., 18 patients have been seen in the past 12 years with primary tumors of the liver. The tumors were divided as follows: 13 hepatoblastoma, 2 hepatocellular carcinoma, 2 rhabdomyosarcoma, and 1 mixed mesenchymal sarcoma. Of the patients with hepatoblastoma, all were seen by 4 years of age, and 8 were under 1 year when first seen. Three had pulmonary metastases when first seen. The 2 patients with hepatocellular carcinoma were 8 and 12 years old, respectively. The patients with embryonal rhabdomyosarcoma were 18 and 30 months of age when seen; 1 had a primary tumor arising in the liver, the other had a tumor in the bile ducts extending into the liver. Ten of the 13 children with hepatoblastoma underwent lobectomy; 5 of these 10 are alive, 1 with known metastases. The other 4 are presumed cured. One of the children with hepatic carcinoma had severe preexisting cirrhosis of the liver and has had 3 local limited excisions. One of the patients with rhabdomyosarcoma is alive and cured after hepatic lobectomy. As yet, there is no known effective chemotherapeutic regimen. Radiation therapy has been useful only in shrinking the tumor, but neither of these modalities has resulted in cure. At the present time, the only known cures are achieved by early detection and successful surgical removal.RésuméAu Children’s Hospital de Washington, D.C., 18 malades ont été traités au cours des 12 dernières années pour tumeur primitive du foie: 13 hépatoblastomes, 2 carcinomes hépatocellulaires, 2 rhabdomyosarcomes, 1 mésenchymome malin. Les patients atteints d’hépatoblastome avaient tous moins de 4 ans lors du premier examen et 8 avaient moins d’un an; 3 présentaient des métastases pulmonaires. Les deux malades atteints de carcinome hépatocellulaire avaient 8 et 12 ans. Les deux enfants porteurs de rhabdomyosarcome embryonnaire avaient 18 et 30 mois; l’un avait une tumeur primitive du parenchyme hépatique, l’autre une tumeur de la voie biliaire infiltrant le foie. Sur les 13 hépatoblastomes, 10 ont eu une lobectomie: 5 sont en vie, dont 1 avec métastases, les 4 autres étant considérés comme guéris. Un des enfants atteint de carcinome hépatocellulaire souffrait d’une cirrhose hépatique pré-existante et avait déjà subi 3 exérèses locales limitées. Un des malades atteints de rhabdomyosarcome est guéri après lobectomie. A l’heure actuelle, il n’existe pas de chimiothérapie efficace. La radiothérapie ne peut que réduire le volume de la tumeur. Aucune de ces 2 modalites thérapeutiques ne peut guérir le malade et, aujourd’hui, les seules guérisons ont été obtenues grâce au diagnostic précoce et à l’exérèse chirurgicale.

Bronchoscopy and bronchial lavage (BBL) in children with cystic fibrosis.

Abstract The morbidity and mortality of cystic fibrosis are consequences of chronic endobronchial obstruction. To retard or reverse the pulmonary complications, we have evaluated bronchoscopy and bronchial lavage with acetylcysteine ∗ ∗Mucomyst, Mead Johnson Laboratories, Evansville, Ind. in the management of these children. Two groups have been treated: (1) a stable group in whom treatments were elective, and (2) those critically ill with advanced pulmonary insufficiency treated on an emergency basis. Our experience with 270 procedures in 100 patients forms the basis of this report.

Surgical correction of bladder-neck obstruction in children.

IN the recent literature considerable attention has been paid to recognition and management of obstructions at the bladder neck in children. This condition is relatively uncommon when compared to the numerous other anomalies of the urinary system, but it is highly important to detect, since it can insidiously lead to extensive destruction of kidney and excretory tracts. It is now known that the institution of appropriate therapy can effectively ward off renal damage and minimize superimposed infection. The entity has been recognized and described for decades, but it has not come into focus until the past few years. In 1915 .xa0.xa0.

Auxiliary liver transplantation in acute liver failure

Abstract Acute hepatic ischemia induced by hepatic artery ligation and mesentericcaval shunt resulted in hepatic failure and death within 72 hr in seven control pigs. Heterotopic hepatic transplantation from unrelated donor shoats was carried out 24 hr subsequent to hepatic ischemia in seven experimental animals. There was one technical fatality. The surviving six animals had gradual return of intrinsic liver function permitting safe removal of the hepatic allograft after 212-5 wk. Sequential 99m technetium scans of the dual-liver system were used to evaluate the relative integrity of the native liver and hepatic allograft, and consequently the timing of transplant excision. The results of this study suggest temporary auxiliary liver transplantation may warrant clinical trial in children with lethal, but potentially reversible, liver failure.

Gastric division: A surgical adjunct in selected problems with esophageal anomalies☆

Abstract Previous reports have suggested the usefulness of gastric division in premature infants or in critically ill infants with esophageal atresia and tracheoesophageal fistula. 1–3 Division of the stomach has also been applied in certain infants with complications of an esophageal anastomosis where it has proved a satisfactory alternative to cervical esophagostomy and abandonment of esophageal function. Although the advent of intravenous hyperalimentation has altered the critical need for early intestinal feeding in these infants, it has not supplanted use of the intact intestinal tract. This report relates our experience with gastric division in caring for infants with specific problems or complications relating to esophageal discontinuity.