Kenneth Kenigsberg

Xiphopagus conjoined twins: A 300-year review of the obstetric, morphopathologic, neonatal, and surgical parameters☆

Obstetric, morphopathologic, neonatal, and surgical features of 36 reported cases of xiphopagus conjoined twins were correlated and reveal: (1) a 6% incidence of prenatal diagnosis; (2) a 36% rate of dystocia; (3) a 19% incidence of stillbirths; (4) variation in the extent of fusion; (5) a high rate of occurrence of concurrent congenital malformations, not limited to a conjoined area, especially of the heart (25%) and gastrointestinal tract (33%); (6) the importance of diagnostic tests to elucidate conjoined or abnormal structures because of the high rate of concurrent malformations; (7) opisthotonos as the body alignment position of choice; (8) a 69% female sex bias; (9) surgical separation usually in the first year of life; and (10) a 53% survival rate. This paper correlates the world literature for xiphopagus conjoined twins for the past 300 years.

Neuronal intestinal dysplasia : quantitative diagnostic criteria and clinical management

Neuronal intestinal dysplasia (NID) clinically resembles Hirschsprungs 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 historically. 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 patients biopsies and biopsies from “normal” and “inflamed” patients were compared. Normals contained 0.68 ± 0.28 (X ± 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 patients 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.

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.

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.

Hepatitis in children with acquired immune deficiency syndrome

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.

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.

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.

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.

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.

Unusual injury caused by a pencil

A case involving a 3-year-old boy who sustained a penetrating injury to the perineum is presented.