Jay L. Grosfeld

Papillary epithelial neoplasm of pancreas in a child. Report of a case with electron microscopy

An asymptomatic tumor of the head of the pancreas observed in a 12‐year‐old girl was removed by radical excision. the cell of origin of the tumor is demonstrated ultrastructurally to be other than islet or exocrine, and probably to have been derived from ductular epithelium. the malignant or benign nature of this tumor is discussed and requires longer follow‐up for clarification.

Combined therapy in childhood rhabdomyosarcoma: An analysis of 42 cases

Abstract Forty-two cases of rhabdomyosarcoma in infants and children are presented. The relation of survival to age, site, cell type, stage of disease and therapeutic management is evaluated. The majority of survivors are between one and 3 years, have localized disease of embryonal or alveolar cell type and have been treated with combined surgery, radiation and chemotherapy. Postoperative survival of 2 years without evidence of recurrence relates well to a cure. In instances of rhabdomyosarcoma in infants and children, radical operation, postoperative regional radiation in combination with simultaneous and prolonged repeated courses of Actinomycin D and Vincristine appear justified and are recommended.

Hemodynamic and manometric observations in experimental air-block syndrome

Abstract In experimental air block, interstitial emphysema is characterized by early tachypnea, arterial hypertension, and hypoxemia. Pneumomediastinum causes a significant rise in central venous pressure and an initial respiratory and subsequent metabolic acidosis. This is a result of a reduction in venous return, cardiac output, and tissue perfusion. Pneumopericardium and coronary and systemic arterial air emboli were observed when vigorous insufflation pressures were used. Animal deterioration was characterized by severe hypoxia, metabolic and respiratory acidosis, arterial hypotension, and cardiac arrhythmias leading to cardiac arrest. These observations point out the potential dangers of vigorous resuscitation in small infants and suggest that vena caval and esophageal manometrics, arterial blood pressure, pH, and blood gas tension determinations indicate significant changes in pulmonary and mediastinal hemodynamics. Monitoring these factors in addition to pulse, respiration, and the EKG would permit early detection of critical changes and allow for prompt respiratory support and, hopefully, improved survival rates in infants with air-block syndrome.

Primary Mediastinal Neoplasms in Infants and Children

Abstract Primary mediastinal tumors were observed in 62 infants and children. Thirty-nine of the tumors were located posteriorly, 16 anteriorly, and 7 in the middle mediastinum. Neurogenic tumors occurred in 36 patients, with neuroblastoma accounting for 25. Lymphoma was noted in 20 patients, teratoma in 5 patients, and a rare lipoma in 1. Overall, 47 patients had malignant tumors, and 16 survived. All 15 patients with benign tumors survived. Because of the high incidence of primary malignancy in this age group, all childhood mediastinal lesions should be explored. Although dismal results were obtained with lymphoma (1 patient is alive, a survival of 5%) a 56% survival (14 patients) was achieved with mediastinal neuroblastoma. Patients under 1 year of age with neuroblastoma had an 88% survival rate (8 of 9 patients). Those diagnosed after 1 year had a 37.5% survival rate (6 of 16). Results were more favorable with mediastinal lesions (56% survival) than with retroperitoneal neuroblastoma (19% survival), regardless of the patients age. Complete or partial extirpation and radiation therapy yielded the most success, while chemotherapy offered significant palliation.

Duplication of pylorus in the newborn: A rare cause of gastric outlet obstruction

Abstract Complete pyloric obstruction caused by a duplication was relieved by excision without resection of the contiguous pylorus. Histologically all of the normal layers of the pylorus were present. In addition a few foci of pancreatic tissue showing acute inflammation were found.

Vascularized Appendiceal Transplants in Biliary and Urinary Tract Replacement

Abstract Vascularized appendiceal transplants were prepared in 20 dogs. In ten dogs completely obstructed by bile-duct ligation, the neck of the appendix was anastomosed to the duodenum. One week later, the blind end was anastomosed to the gallbladder or obstructed common bile duct in five dogs each. All dogs survived and rapidly recovered both clinically and chemically from biliary obstruction. Intravenous cholangiograms and contrast barium swallow showed neither obstruction nor reflux. At sacrifice the transplants were viable and patent and showed no evidence of stricture, bile, stasis, or stone formation. In ten dogs the right ureter was divided and anastomosed to the tip of the prepared appendix. The open appendiceal base was brought out as a urinary stoma. The left ureter was kept intact for control. Vascular injury aborted one procedure. Prompt urinary function was observed in nine dogs. Intravenous pyelogram showed prompt function without dilatation in three. One animal showed poor function on pyelogram, but no obstructions were observed on loop-o-gram. Significant infection occurred in only one case due to stomal stricture. At sacrifice (up to 10 mo postoperatively) functional anastomoses, no stones, and normal renal architecture were observed. The use of the small and relatively short appendix for urinary diversion provides a minimal reservoir for urinary statis and electrolyte absorption, and avoids the necessity of bowel anastomosis. This study suggests that autogenous vascularized appendiceal transplants are suitable biliary and urinary tract conduits in the experimental animal. Further investigation of their use in bile-duct replacement and urinary diversion is clearly indicated.

The role of splenectomy in neonatal idiopathic thrombocytopenic purpura

Abstract Although the clinical course of most infants with neonatal idiopathic thrombocytopenic purpura is benign and self-limited, extracutaneous bleeding is relatively common and potentially dangerous. Intracranial bleeding is the most common complication of this disease, and its occurrence demands prompt control. Emergency splenectomy has been most effective in accomplishing such control in this series. Splenectomy should be avoided as an elective procedure in early childhood. However, in the life-endangering situation of central nervous system bleeding with this disease, the operation has been dramatically successful and thoroughly justified.