Angelo J. Eraklis

Primary bronchial tumors in childhood: A clinicopathologic study of six cases

A primary bronchial tumor was diagnosed in six children younger than age 12 years. These cases include four bronchial carcinoids, a low‐grade mucoepidermoid carcinoma, and a granular cell tumor (“myoblastoma”). Lobar or segmental resection was performed in each case and remains the treatment of choice. The most common bronchial tumors in childhood are carcinoids with 17 other evaluable cases in the English Literature. This series includes the youngest child to be reported with bilateral choroidal metastases leading to blindness; in addition, there were clinical manifestations of gigantism and acromegaly possibly related to ectopic hormone production. Low‐grade mucoepidermoid carcinomas rank second in frequency to carcinoids and have an excellent prognosis. Judging from the childhood bronchial tumors reported to date, proved examples of bronchogenic carcinoma are vanishingly rare.

Achalasia in childhood: A 20-year experience

The incidence of achalasia in children is lower than that in adults, but the disturbances in growth and development, and the severity of pulmonary symptoms are more profound. We have treated 20 children with the disease over the past 20 yr. All children were first treated with dilatation of the cardioesophageal junction either with filoform and followers, or in older children, pneumatic dilatation under fluoroscopic control. Five children, all older than 9 yr of age and all female, had prolonged relief of symptoms following two dilatations and never required a surgical procedure. Fifteen children responded unsatisfactorily to repeated dilatation and 12 of these accepted a surgical procedure. A modifier Heller procedure was performed in 11 children and one patient was treated with a transthoracic cardioplasty. The operations resulted in dramatic relief of symptoms, satisfactory weight gain, and the disappearance of pulmonary symptoms in every case. We recommend that all children under 9 yr of age should have a modified Heller procedure as primary therapy. In children older than 9 yr of age, dilatation is an appropriate initial therapy but should not be repeated unless significant improvement results.

Adenocarcinoma at the site of ureterosigmoidostomies for exstrophy of the bladder

Adenocarcinoma of the colon is a rare complications of ureterosigmoidostomy done for exstrophy of the bladder. Only 13 cases have been previously reported, occurring from 10 to 46 yr after the procedure. In a series of 150 patients who have had ureterosigmoidostomies carried out at the Childrens Hospital Medical Center, Boston, over the past 40 yr, 4 patients have been treated at our institution for this complication. The tumor was noted 20 to 32 yr after the initial procedure. Two patients are alive and well 1 and 4 yr after resection. The increasing incidence of this complication with advancing age will require careful observation and will undoubtedly influence the indications for the procedure. An additional question of great importance is how to evaluate and follow the thousands of patients who have had this procedure done at one time in their lives. It is suggested that an annual intravenous pyelogram is indicated in those patients with functioning ureterostomies and that a colonoscopy to the level of the anastomoses be carried out at 6-mo intervals in all patients. In those patients in whom the ureterosigmoidostomies have been taken down, a sleeve resection of that area of the colon should be strongly considered.

A procedure for lengthening the phallus in boys with exstrophy of the bladder

Abstract A surgical procedure is described in which the two corpora and their attachments are brought to the midline with preservation of nerve and blood supply. Early clinical experience in three patients has indicated that a very satisfactory increase in the visible length of the penis can be accomplished without loss of sensation or erectile ability.

Bilateral Neonatal Torsion

Extravaginal neonatal torsion of the spermatic cord, although rare, is being recognized with increasing frequency. Bilateral neonatal torsion is even more uncommon. Only 1 previous case of asynchronous neonatal torsion has been reported. Controversy exists concerning the urgency of exploration as well as the need for contralateral orchiopexy, since some reports suggest that the contralateral testicle is not at risk for torsion. We present 4 consecutive cases of bilateral neonatal torsion seen between 1966 and 1986. Two cases were asynchronous, 1 of which with a previously normal-appearing contralateral gonad was rendered anorchid. In the other patient a testicle was salvaged by prompt intervention. We have adopted a policy of prompt exploration with contralateral orchiopexy in all cases of unilateral neonatal torsion. Treatment of the ipsilateral testicle is determined by operative findings.

Pentagastrin stimulation of technetium-99m uptake by ectopic gastric muscosa in a Meckel's diverticulum

A case is described in which the preoperative diagnosis of Meckels diverticulum was made by scintigraphy after the administration of pentagastrin. Pentagastrin premedication may be helpful in demonstrating ectopic gastric mucosa in patients with gastrointestinal bleeding in whom initial radiopertechnetate scintigraphy is equivocal or normal.

Circular esophagomyotomy of upper pouch in primary repair of long-segment esophageal atresia.

A method of performing circular esophagomyotomy of the upper pouch is described as an effective technique for use in repair of esophageal atresia where the ends cannot be brought together.

Intravenous dextrose-amino-acid feeding: The metabolic response in the surgical neonate

Abstract Extensive metabolic balance studies were undertaken in three neonates whose nutrition was maintained parenterally with a 20 per cent dextrose, five per cent fibrin hydrolysate mixture during staged repair of complicated omphalocele. All three showed, besides a satisfactory weight gain, positive nitrogen balance in the complete absence of oral feedings. The dextrose administered was adequately utilized; and significant glycosuria, osmotic diuresis or other adverse side effects were not detected.

Polyalveolar lobe: anatomic and physiologic parameters and their relationship to congenital lobar emphysema.

Polyalveolar lobe is one of the recently described pathological entities that can give rise to congenital lobar emphysema (CLE). In polyalveolar lobe, the total alveolar number as determined by microscopic point-counting of randomly taken lung sections is increased three to fivefold. The airways and arteries are normal for age in number, size and structure. Follow-up over 10-20 yr of patients operated upon for congenital lobar emphysema, including those of polyalveolar lobe etiology shows that these patients do well clinically. All patients have some pulmonary function abnormalities, specifically a decrease in airway conductance, and forced expiratory volume. It is not possible to distinguish patients with polyalveolar lobes from other CLE patients on the basis of pulmonary function data. All patients had normal lung volumes and vital capacities, despite losing 8%-20% of their pulmonary parenchyma. Based on compensatory lung growth, it may be preferable to operate earlier, rather than later in these cases.

Pancreatitis and intravenous fat: An association in patients with inflammatory bowel disease

Since recently adding Intralipid (IL) to our standard total parenteral nutrition (TPN) regimen for children with inflammatory bowel (IBD) disease, two children undergoing major intestinal resections have developed acute pancreatitis in the early postoperative period. Herein, we present the clinical summaries of these two patients, discuss the multifactoral etiologies of acute pancreatitis, and review the role of emulsified fat solutions in TPN in children with IBD.