Robert J. Touloukian

The pathogenesis of ischemic gastroenterocolitis of the neonate: Selective gut mucosal ischemia in asphyxiated neonatal piglets

Abstract Necrotizing enterocolitis and intestinal perforation, occurring without distal obstruction of the gastrointestinal tract, are serious lesions of the neonatal period. The onset of gastrointestinal findings is often preceded by severe postnatal stress, usually of cardiorespiratory origin, producing hypoxemia, hypercarbia, and systemic acidosis. Resuscitated infants, usually premature, remain clinically well during a latent period until they develop characteristic signs of enterocolitis, including gastric retention, bile vomitus, abdominal distension and blood streaked, diarrheal stools. Hemorrhagic and necrotic lesions, with or without perforation, are most commonly found in the stomach, ileum, or colon at operation and/or autopsy. The pattern of asphyxia, resuscitation, and a latent period followed by gastrointestinal findings has been the subject of much speculation about the causal relationship of cardiorespiratory distress in infants with necrotizing enterocolitis. The temporal relationship of perinatal asphyxia to the acute onset of gastroenterocolitis is summarized on Fig. 1. An experimental model was designed that simulates conditions of postnatal respiratory distress and allows measurement of intramural and mucosal perfusion to the gut of asphyxiated and resuscitated neonatal piglets, as well as histologic studies of the gut. We suggest that the term “ischemic gastroenterocolitis of the neonate” be adopted to best describe this condition.

Surgical experience with necrotizing enterocolitis in the infant

Summario in interlingua Enterocolitis necrotisante es un morbo de neonatos que es altemente mortal. Illo se vide principalmente in prematuros e es characterisate per un tableau clinic de retention gastric, vomito biliari, distention abdominal, e feces a striation sanguinee o—a vices—diarrheic. Iste tableau resimila a vices, super toto initialmente, illo de obstruction intestinal. Perforation es un occurrentia commun. A base de un studio de 25 casos, le conclusion es formulate que perforation intestinal-manifeste in pneumoperitoneo roentgenographic—es le sol clar indication pro un intervention chirurgic.

Early presentation of metastatic medullary carcinoma in multiple endocrine neoplasia, type IIA : Implications for therapy

A girl 5 years 11 months of age, belonging to an extensive kindred with multiple endocrine neoplasia, type IIA (MEN IIA), was found to have multifocal medullary thyroid carcinoma with metastasis in one paraglandular lymph node after positive findings on a calcium-pentagastrin stimulation test. Her sister, 3 years 8 months of age, also had an elevated calcitonin level, and thyroidectomy revealed C-cell hyperplasia and a focus of medullary thyroid carcinoma. These two cases underscore the need for prophylactic thyroidectomies in MEN IIA patients as young as 5 years of age and strict yearly provocative screening beginning at age 1 year.

Evaluation of Nissen Fundoplication in Neurologically Impaired Children

The value of performing Nissen fundoplication in neurologically impaired children is a controversial issue. To evaluate the benefit of fundoplication in these children, hospital records were reviewed for 77 children who underwent fundoplication for gastroesophageal reflux (GER). Fifty-two children were neurologically impaired; 25 children had no neurological impairment. Impaired children had significantly fewer hospital admissions (1.8 v 0.7; P less than .005) and total days of hospitalization (36 v 14; P less than .005) during the first postoperative 6 months, compared with the immediate preoperative 6-month period. Normal children had fewer hospital admissions and days postoperatively, but the difference was not significant. Impaired children with preoperative failure to thrive (FTT had significantly increased average monthly weight gain over the first 6-month period postoperatively, compared with preoperative growth rate (3.0% v 0.9% of total body weight; P less than .05). Average monthly weight gain at 1 and 2 years postoperatively was not significantly different from preoperative values for impaired children. Growth rate of normal children with FTT did not change significantly postoperatively. Symptomatic relief was comparable in the normal and impaired children. Perioperative mortality was 0% in the normal children and 6% in the impaired children. This study demonstrates that Nissen fundoplication in neurologically impaired children with GER can be performed safely, reduces the frequency of hospitalization, and improves short-term weight gain.

Neonatal Necrotizing Enterocolitis: An Update on Etiology, Diagnosis, and Treatment

Necrotizing enterocolitis is a unique surgical disease of the neonate characterized by diffuse or patchy intestinal necrosis, accompanied by sepsis, and often followed by perforation, peritonitis, and death. It is an acquired condition that can be successfully treated without operation in most cases.

Benign clinical behavior of immature mediastinal teratoma in infancy and childhood: Report of two cases and review of the literature

Germ cell tumors of the mediastinum can be divided into three categories: 1) mature teratomas which have all elements at a mature level; 2) immature teratomas which are similar to the mature teratomas, but also contain immature epithelial or mesenchymal elements or blastema; and 3) embryonal tumors which contain elements which are recognized as dysgerminoma, embryonal carcinoma with or without yolk sac elements, and choriocarcinoma. Immature teratomas are the rarest type, accounting for only about 1% of mediastinal teratomas. Two cases of immature teratoma of the mediastinum occurring in infants are reported. One tumor was completely excised. The child is well without evidence of disease two years later. The other immature teratoma was unresectable. Biopsy showed it to be similar to the teratoma that was excised. No postoperative treatment was given. The tumor has not changed appreciably in size, but the child has grown normally for six years so that the tumor mass, which initially filled his chest, is now evidenced as mediastinal widening. A review of the reported cases of immature teratomas in the mediastinum shows that the prognostic value of the histologic appearance of these tumors has not been developed to the same degree as it has for teratomas in the ovary or the sacrococcygeal region. The immature teratomas that occur in infants behave as mass lesions as do the mature teratomas. Immature teratomas in the mediastinum of children in their late teens and in young adults behave as highly malignant tumors similar to the embryonal carcinomas.

Protocol for the nonoperative treatment of obstructing intramural duodenal hematoma during childhood

A prospective plan for the nonoperative treatment of obstructing intramural duodenal hematoma was developed to (1) promptly establish the diagnosis and rule out transmural leaks by obtaining a contrast roentgenogram on admission and to subsequently study evolution of the obstructing intramural duodenal hematoma with sequential roentgenograms at 5 to 7 day intervals; (2) identify associated pancreatic injury with ultrasonography and serum amylase and lipase determinations; and (3) to determine effectiveness of nasogastric suction and total parenteral nutrition. Twelve children, who ranged in age from 2 to 15 years, with obstructing intramural duodenal hematoma following blunt injury were admitted over the past 9 years. The two youngest were battered children and two others had subsequently diagnosed clotting disorders (idiopathic thrombocytopenic purpura and von Willebrands disease). Significant resolution of the obstruction allowed resumption of oral intake by the end of the first week of treatment in eight patients, whereas the remaining four required 13, 14, 22, and 38 days of nasogastric suction and total parenteral nutrition. Residual deformity seen on roentgenograms did not interfere with achieving adequate oral nutrition. Excellent results in this series substantiate the conclusion that a management plan that assesses the evolution of an obstructing intramural duodenal hematoma and provides adequate nutrition is a successful alternative to surgical treatment. The presence of underlying hematologic disorders and child abuse must be suspected.

Cesarean section does not improve outcome in gastroschisis

Elective cesarean section (CS) following prenatal diagnosis of gastroschisis has been advocated to decrease morbidity and mortality. To examine this hypothesis, we reviewed the records of 28 consecutive patients with gastroschisis treated between 1975 and 1987. Fourteen infants were delivered vaginally (V) and fourteen by CS, of which seven were elective, five were for fetal distress, and two were for breech presentation. Prenatal diagnosis was made in nine infants in the CS group and none in the vaginal group. The two groups were comparable in gestational age (V = 37.6 weeks, CS = 35.8 weeks, P = .05), birth weight (V = 2,508 g, CS = 2,444 g, P = NS), and five-minute Apgar score (V = 7.8, CS = 6.8, P = NS). Outcome was similar as measured by hospital mortality (V = 0/14, CS = 1/14, P = NS), complications (V = 4/14, CS = 5/14, P = NS), days to enteral feeding (V = 14, CS = 19, P = NS), and days in the hospital (V = 27, CS = 34, P = NS). The only complication related to mode of delivery was preventable; an infant delivered vaginally had avulsion of a short segment of mesentery requiring bowel resection. Infants born by CS were slightly more likely to have primary closure (5/14) than babies delivered vaginally (3/14, P = NS), but this may reflect independent trends in the last 5 years. Elective CS following prenatal diagnosis in seven patients did not improve outcome; primary closure was achieved in only one infant, and three had a complication. Since these data show no significant difference in morbidity and mortality between vaginal and CS delivery, we suggest that CS should not be recommended simply because a prenatal diagnosis of gastroschisis is made.

Antibiotic Treatment of Renal Carbuncle

Renal carbuncles in seven young males were successfully treated with long-term administration of penicillinase-resistant antibiotics. Selective renal arteriography provided an accurate means of diagnosis and permitted a trial of medical therapy. All patients experienced a prompt and sustained clincial remission; surigical exploration was thus obviated in all but one instance, in which post-treatment radiographic changes persisted.

Partial decapsulation of splenic epithelial cysts: Studies on etiology and outcome

PURPOSE The presence of squamous epithelium in the cyst wall of children with large splenic cysts has generally been thought to require complete resection of the cyst lining to prevent recurrence. The risks of major bleeding or possible total splenectomy necessitates a simplified procedure to preserve splenic function without cyst recurrence. METHODS Six patients treated for splenic cyst over a 10-year period underwent partial splenic decapsulation retaining the hilar portion of the spleen. The patient group, including five girls, ranged in age from 6 to 18 years (mean, 13 years). Presenting findings were splenomegaly (n = 4), progressive cyst enlargement (n = 4), left upper quadrant pain (n = 2), ultrasound for unrelated condition (n = 2), and prior history of trauma (n = 1). Cyst location varied from peripheral to deep cortical and ranged in size from 5 to 20 cm containing from 200 to 1,700 mL of sterile straw-colored to dark brown-colored liquid. RESULTS Palpable splenomegaly resolved in all patients with progressive decrease in size of the splenic remnant, and return of vascularity to normal was confirmed by Doppler ultrasound. Each patient remains asymptomatic without recurrence of the splenic cyst from 1 to 10 years after surgery. Squamous epithelial lining cells found in sheets and clusters were seen within the cyst lining of all patients. Immunohistochemistry of the paraffin-embedded tissue showed that these cells expressed keratin, epithelial membrane antigen (EMA), carcino-embryonic antigen (CEA), but were negative for BerEP4 (conventionally positive in cells of epithelial origin). CONCLUSION The authors conclude that splenic decapsulation is an effective treatment for splenic epithelial cysts, which both preserves splenic function and prevents recurrence despite retention of hilar cyst lining. Epithelial metaplasia of the mesothelial-mesodermal undifferentiated cells from exposure to an unidentified irritant may explain the histological presence of squamous epithelium within the cyst wall.