Bilateral abdominoscrotal hydrocele: a case report.

A 13-month-old boy, who had been diagnosed in utero as having bilateral hydroceles, was found to have bilateral abdominoscrotal hydroceles. To date, there has been only one previous case of bilateral abdominoscrotal hydroceles in an infant. Magnetic resonance imaging was used to make the diagnosis: bilateral dumbell shaped fluid filled masses were seen in a coronal image. The patient had the abdominoscrotal hydroceles excised through bilateral groin incisions. The postoperative course was uneventful, without complication. The etiology and diagnosis of abdominoscrotal hydroceles are discussed.

Tracheostomy: acute and long-term mortality and morbidity in very low birth weight premature infants.

Thirty-six very low birth weight premature infants (VLBW-PT) born at 24 to 32 weeks gestation and with birth weights 635 to 1,360 g who had tracheostomies performed for acquired subglottic stenosis or for prolonged mechanical ventilation were followed in relation to acute and long-term mortality and morbidity. Mortality due to the tracheostomy occurred in 4 patients (11%); mortality from all other causes was 25%. Death after hospital discharge was associated with the nonuse of prescribed cardiorespiratory monitors. Complications < 1 week postsurgery occurred in 31% of infants and complications > or = 1 week postsurgery occurred in 64% of infants. Fifty percent of infants required tracheostomy for > 2 years and/or extensive reconstructive surgery of the airway. Parents should be counselled that VLBW-PT infants with a tracheostomy may require extended medical and home care. An effective home care program requires parental training in tracheostomy care, the use of ancillary equipment, and infant cardiopulmonary resuscitation.

Intrauterine volvulus without malrotation.

This report describes two cases of intrauterine volvulus without associated intestinal malrotation. Polyhydramnios was present in one neonate; the presentation was very sudden and catastrophic in the other. Neither plain films nor contrast enema indicated the correct preoperative diagnosis in these infants. A "normal" plain film or enema in a suspected case of volvulus may lead to a delay in surgical management resulting in extensive ischemic necrosis of the bowel, often with extremely high mortality. Bile emesis or aspirate in neonates demands a high index of suspicion of intestinal obstruction. Shock, bloody diarrhea, and tense and distended abdomen are ominous, indicating volvulus often with gangrene.

Erosion through the posterior gastric wall by a pancreatic pseudocyst secondary to gastric duplication.

Gastric duplications are the rarest form of enteric duplication. They account for about 20% of all gastrointestinal duplications. Delayed and missed diagnosis leads to prolonged morbidity and mortality. We report a case of a child with a gastric duplication cyst in the body of the pancreas, associated with a pancreatic pseudocyst that ultimately eroded into the posterior wall of the stomach.

Neuronal intestinal dysplasia: quantitative diagnostic criteria and clinical management.

Neuronal intestinal dysplasia (NID) clinically resembles Hirschsprung's disease but is characterized by hyperplasia rather than aganglionosis of the intramural plexus. Surgical intervention is common. We report the 5-year follow-up of an infant with the mixed form of NID managed medically and a method by which NID can be quantified histologically. Hyperganglionosis was determined by counting the number of ganglia per high-power field and the number of ganglion cells per ganglia from at least two biopsy specimens. The patient's biopsies and biopsies from "normal" and "inflamed" patients were compared. Normals contained 0.68 +/- 0.28 (mean +/- SD) ganglia per high-power field and 2.16 +/- 0.31 ganglion cells per ganglion. The inflamed biopsies were similar, 0.69 +/- 0.38 ganglia per high-power field and 2.63 +/- 0.40 ganglion cells per ganglion. The patient's initial rectal biopsy revealed 7.6 ganglia per high-power field and 3.8 ganglion cells per ganglion. Management of the patient included saline colonic irrigations and hyperalimentation with gradual reinstitution of breast-feeding. Clinical improvement was associated with normalization of manometry and biopsy findings, a phenomenon not documented previously in the literature. Irrigations were stopped at age 9 months, and the child is now asymptomatic.

Berry aneurysms, cirrhosis, pulmonary emphysema, and bilateral symmetrical cerebral calcifications: a new syndrome.

Familial idiopathic nonarteriosclerotic cerebral calcification (FINCC) constitutes a rare but pathologically well defined disorder. Thus far, central nervous system symptoms and signs have been the only recorded expression of this disease. Autosomal dominant and autosomal recessive inheritance have both been postulated as cause. We describe three sibs who had symmetrical cerebral calcifications, but three also had cirrhosis and pulmonary emphysema; two had congenital cerebral aneurysms. All were male and of short stature; they also had delayed development and seizures, and two had other neurologic deficits. One sib died at age 3 years of hepatic failure and portal hypertension. Ruptured cerebral aneurysms led to the death of the other two boys at ages 8 and 13 years. The cerebral calcifications symmetrically involved the basal ganglia and thalami, the dentate nucleus, and the cortical and subcortical areas of the cerebrum. The liver was studied by sequential biopsies in two of the children and in all three by autopsy. Fatty degeneration and portal fibrosis preceded a periportal and micronodular cirrhosis. Severe bilateral pulmonary emphysema was present in one sib at age 12 years, whereas all three had bullae and cysts at autopsy. Ruptured left middle cerebral artery aneurysms were demonstrated in two sibs, and one also had aneurysms of the anterior and posterior communicating arteries. We conclude that in this family FINCC is a complex pleiotropic mendelian mutation, either of autosomal or X-linked recessive nature, whose basic pathogenesis remains unknown but may involve a metabolic defect. This form of FINCC may be a previously undescribed syndrome or a form of FINCC in which extraneural manifestations were previously overlooked.

Esophageal perforation secondary to gastrostomy tube replacement.

A 6-week-old child with esophageal perforation secondary to a misplaced gastrostomy Foley balloon is described. She was treated successfully with chest tube drainage, antibiotics, and total parental nutrition. This may be an alternative method of treating this rare complication of gastrostomy.

Hepatitis in children with acquired immune deficiency syndrome. Histopathologic and immunocytologic features.

Hepatic morphology and immunocytology were evaluated in 4 children with clinical and immunologic characteristics of the acquired immune deficiency syndrome or acquired immune deficiency syndrome related complex. All 4 children had hepatomegaly and increased serum alanine and aspartate aminotransferase activity. Both lobular and portal changes were noted. Lymphocytic infiltration, piecemeal necrosis, hepatocellular and bile duct damage, sinusoidal cell hyperplasia, and endothelialitis were prominent. Vesicular rosettes in sinusoidal lymphocytes and tubuloreticular structures in sinusoidal endothelial cells were demonstrated by electron microscopy. The lymphocytic infiltrate in both the lobular and portal spaces was characterized by a relative increase of cytotoxic/suppressor (T8) cells. Hepatitis may be a common feature of pediatric acquired immune deficiency syndrome and acquired immune deficiency syndrome-related complex. Although the histopathologic changes are consistent with chronic active hepatitis, the specific pathogenesis remains to be determined.

Paraplegia in children with malignant teratoma.

Two young children presenting with paraplegia secondary to epidural tumors were found to have unresectable malignant teratomas. Following partial resection and aggressive chemotherapy, both patients have done extremely well with disappearance of all disease and dramatic improvement in neurologic function. It is suggested that children with spinal cord compression might have a much better prognosis for recovery of neurologic function than adults. A treatment approach utilizing chemotherapy as the primary mode of therapy is discussed.

Selective denervation of the S-A node in the treatment of progressive central vagal bradycardia.

The condition of central hypervagism and central vagal bradycardia, up to now, have been largely overlooked. Selective surgical vagal denervation of the SA node for progressive central and local episodic reflex arc bradycardia, via right thoracotomy, is a well-tolerated procedure even in the debilitated child. The bradycardia must, of course, be atropine-responsive. It appears to be a valuable alternative to pacemakers, especially because of the necessity of using the sequential type in this form of heart block. Our follow-ups are from 8 months to 13 years in seven children (five central and two peripheral). Up to now, in an admittedly small series, there has been only one recurrence of bradycardia. This was from crossover left vagal innervation of the SA node. The incidence of this is 10% to 15%. It responded to right cervical vagotomy, following a preliminary diagnostic novocain block. We hope that these findings may lead to more awareness and investigations of conditions due to autonomic dysfunction--both central and peripheral--which may help among other conditions in the elucidation and prevention of sudden infant death syndrome (SIDS) and other related problems, and that this surgical approach will be a definitive means for the control of refractory, persistent, or recurrent vagal bradycardia.

Cardiac effects of esophageal stimulation: possible relationship between gastroesophageal reflux (GER) and sudden infant death syndrome (SIDS).

Twenty-six artificially ventilated newborn pigs were subjected to simulated gastroesophageal reflux; saline (10 cc) of varying pH was flushed through the esophagus from below. At a given pH threshold, reflex bradycardia, which could be blocked by atropine, was elicited. Transecting of the superior laryngeal nerves, the recurrent laryngeal nerves, and the pharyngeal plexus nerves did not block the reflex bradycardia. However, bypassing the regions superior to the esophagus with a shunt prevented the bradycardia. These results indicate that bradycardia caused by gastroesophageal reflux is independent of changes in ventilation and may be an important cause of sudden infant death.

Separation of omphalopagus twins.

The study and successful separation of a pair of omphalopagus twins weighing 1690 g at birth is presented. The test of greatest usefulness was an oral glucose tolerance test that demonstrated an absence of parasitism. Subsequently, it was found that the twins had a joined liver but separate GI and GU tracts. The time for separation was determined by observing the twins' toleration of compression of the bridge. Initially, obliteration of the tunnel-like connection would cause considerable respiratory distress. However, in the month before surgery, the tunnel could be compressed to the point of obliteration without causing tachypnea or other signs of respiratory distress. The change in toleration of compression was related to unequal growth of the twins and their connecting bridge. Though the weight of the twins quintupled in their first 6 mo, the circumference of the bridge remained the same. The surgery was carried out on a single table without frames. The twins were draped using self-adhering plastic. Intravenous fluoroscein was used to demarcate the large liver juncture, thus expediting the separation.