Samuel H. Kim

Laryngotracheoesophageal cleft (type IV): Management and repair of lesions beyond the carina☆

Since the first successful repair of a complete laryngotracheoesophageal cleft (LTEC) to the carina in 1982, three newborn infants were observed with a particularly difficult variant in which the cleft extends beyond the carina into the mainstem bronchi. This type IV LTEC creates a long common tracheoesophagus, whose successful separation requires meticulous preoperative, operative, and postsurgical care. Three infants had complete surgical repair at our institution at 29, 49, and 225 days old and survived a minimum of 8 months. Recurrent tracheoesophageal fistulae at the thoracic inlet occurred in two infants, but was not observed in one patient when sternocleidomastoid muscle was interposed between the trachea and esophagus in the neck. Microgastria is an associated finding in each infant with the tracheoesophageal cleft extending beyond the carina. The small stomach is problematic as it is anatomically inadequate for any antireflux procedure and has not grown well, even with prolonged feeding. Early cleft repair is essential to prevent the development of chronic lung disease secondary to recurrent aspiration. The techniques to make the diagnosis, the preferred treatment to initially protect the airway, a single-stage operation performed simultaneously through the chest and neck to definitively repair the cleft, and finally the intraoperative and postoperative management critical for an optimal outcome are described.

Familial superior mesenteric artery syndrome

Five members of a family of eight, including the father and four daughters, presented with symptoms previously attributed to the superior mesenteric artery syndrome. The four daughters also had radiographic studies supporting that diagnosis. This familial clustering raises the possibility of a genetic predisposition to this symptom and radiographic complex.

Sexual precocity in a male due to thoracic polyembryoma

A 7-year-old boy with sexual precocity of recent onset was found to have elevated levels of chorionic gonadotropin, alpha fetoprotein, and testosterone. Removal of a retropleural, posterior mediastinal mass with an intraspinous epidural extension was followed by prompt declines in the plasma concentrations of the tumor markers and cessation of adolescent development. The neoplasm proved to be a polyembryoma, unusual in the multiplicity of its embryoid bodies and extra gonadal location. Following local irradiation and two years of systemic chemotherapy, the patient, now 13 years of age, has done well except for evidence of testicular tubular insufficiency.

Surgically correctable hypertension of renal origin in childhood

Since 1960, 22 children were treated for surgically correctable hypertension or renal origin. The series included two children with tumors, two with hydronephrosis from ureteropelvic junction obstruction, nine in whom one kidney was atrophic, and nine with renal artery narrowing from fibromuscular dysplasia (with bilateral involvement in two). Hypertension was cured in the cases with tumors and ureteropelvic junction obstruction. It was also cured in four of the nine patients with an atrophic kidney and in five of the nine with a narrow renal artery. In those not cured hypertension was more easily controlled by medication. One patient died from brain hypoxia during surgery. About 10 percent of the children investigated for hypertension at the Massachusetts General Hospital proved to have a surgically correctable cause of renal origin.

Ectopic class II major histocompatibility antigens in Hirschsprung's disease and neuronal intestinal dysplasia

Although the etiology of Hirschsprungs disease and neuronal intestinal dysplasia remains obscure, both have histological abnormalities involving ganglion cells and neuronal elements. Searching for a common pathway that may inhibit normal maturation of neurogenic precursors, we examined the possible role of an immune mechanism in the maldevelopment of the enteric neural network. Six patients with Hirschsprungs disease were studied by comparing biopsy specimens from diseased colon with ones taken from proximal ganglionic colon in the same patients. These were similarly compared with colonic biopsy specimens from patients studied with chronic constipation or bowel removed at the time of operation for other disorders. Biopsies were taken from four other patients with neuronal intestinal dysplasia. Each was examined by hematoxylin & eosin staining, acetylcholinesterase histochemistry, and immunohistochemistry of major histocompatibility complex (MHC) class I and class II antigens. All rectal samples from Hirschsprungs disease patients exhibited elevated acetylcholinesterase histochemistry and absent ganglia to confirm the diagnosis. These findings were correlated with marked elevation of class II MHC in the aganglionic area, whereas the proximal normal ganglionic segments showed no elevation. Rectal biopsy specimens from patients with chronic constipation exhibited no such elevation. A similar elevation of class II MHC was detected in the mucosa and submucosa of all four patients with the rare neuronal intestinal dysplasia disorder whose diagnosis was confirmed by giant ganglia in Auerbachs plexuses, aberrant Meissners ganglia in the lamina propria mucosa, and giant neurofibrils in the mucosa and submucosa. The correlation of elevated class II MHC in these two neuronal dysfunction disorders may indicate an underlying autoimmune mechanism as is seen in thyroiditis and insulin dependent diabetes mellitus.(ABSTRACT TRUNCATED AT 250 WORDS)

Repair of mild hypospadias.

A one stage repair is presented for mild hypospadias with or without chordee, entailing a local flap of shaft skin rotated forward to the tip of the glans and tubularized. The two halves of the glans penis are brought together to enclose the neourethra. This procedure results in a normal appearing penis. In 50 cases complications were limited to two meatal stenoses. There were no fistulas. This procedure is not used in cases with greater than minimal deformity.

The inferior epigastric vein as an alternate site for central venous hyperalimentation

The inferior epigastric vein is a good access site when central venous nutrition cannot be delivered through the usual routes. The babies can remain mobile without angulating the catheter and, by tunneling above the diaper line, contamination can be avoided.

Pediatric splenic trauma: Predicting the success of nonoperative therapy

The charts of all pediatric patients discharged from the Massachusetts General Hospital with a diagnosis of a ruptured spleen were reviewed over a six-year period to determine if any factors could be used to predict which children could be managed safely without operation. Seventy-five percent of these injuries were so managed. The patients who required surgical intervention were older (mean age, 17 years), had multiple injuries (mean ISS, 41), presented with more blood loss (mean hematocrit, 23.5%), and suffered their injuries as a result of a motor vehicle accident. The patients who were successfully managed nonoperatively were younger (mean age, 12 years), had fewer associated injuries (mean ISS, 18), required fewer blood transfusions, and suffered their traumas secondary to falls, sporting events, or altercations. We propose that the injury severity score, the number of units of blood transfused, patient age, as well as the type of trauma suffered be used to predict which patients can be safely managed nonoperatively.

Gastroesophageal Reflux and Hiatus Hernia in Children: Experience With 70 Cases

Seventy cases of gastroesophageal reflux with or without hiatus hernia were operated upon since 1959. The technique described herein was used in the majority of cases with satisfactory results.

Infantile intestinal leiomyosarcoma: Surgical resection (without adjuvant therapy) for cure

A 7-week-old boy presented with a 6-week history of failure to thrive, acute intestinal obstruction, and an apparently irreducible intussusception (noted on contrast enema). He underwent abdominal exploration, during which a cecal mass was identified and resected. The mass proved to be a leiomyosarcoma. Histologically, it was an intermediate-grade malignancy with a predicted 5-year survival rate of 16% to 23% based on data from the adult experience. Three years after resection and without having received adjuvant therapy, he is healthy and free of disease. A review of the literature showed that in infants these tumors are predominantly colonic, compared with the predilection for small intestinal lesions found in the older pediatric and adult populations. Infantile intestinal leiomyosarcomata are rare malignancies that do well if complete surgical excision of the disease can be accomplished. The histological prognostic indicators proposed for intestinal leiomyosarcomas in the adult population cannot be extrapolated to infants because when they occur in infants, they appear to be less aggressive, and these patients do well without adjuvant therapy.