Omri Z. Lernau

Congenital cricopharyngeal achalasia treatment by dilatations

An infant with cricopharyngeal achalasia, recurrent aspiration pneumonia, and severe failure to thrive was fed for 5 months through a nasogastric tube. She responded promptly to dilatations of the spastic cricopharyngeal muscle. Normal oral feedings followed the treatment with progressive weight gain and no further pulmonary complications over a follow-up period of 1 1/2 years. Dilatations were used in the infant described here, in analogy to the well-known success of treatment by dilatations in infants with achalasia of the lower esophagus.

Bezoar-induced ulceration and perforation of the upper gastrointestinal tract in mentally retarded patients

Symptoms of bezoars are often nonspecific and may mimic those of other gastrointestinal diseases. Therefore, this diagnosis should be kept in mind to avoid severe complications such as bleeding, perforation, or obstruction, especially when treating mentally retarded or psychiatric patients. We describe two young, mentally handicapped patients with perforation of the gut due to bezoars. The pathophysiology of this complication is discussed and the literature reviewed.

Coexisting left diaphragmatic hernia and esophageal atresia

Coexisting left diaphragmatic hernia (DH) and esophageal atresia, a rare phenomenon, is described in two newborns, one with tracheoesophageal fistula (TEF) and the other without. The coexistence of DH and TEF in the one resulted in uncontrolled gastrointestinal distention with severe respiratory distress. Transabdominal division of the TEF was feasible and was performed concurrently with the diaphragmatic repair. Severe hypoplastic lungs and persistent pulmonary hypertension resulted in early postoperative death in both cases.

Nerve mediated responses to drugs and electrical stimulation in aganglionic muscle segments in Hirschsprung's disease

The activity of isolated muscle strips from normal and aganglionic human large bowel was studied in vitro. The intrinsic nerves were stimulated electrically and by nicotinic agonists. The ganglionic preparations displayed a strong inhibitory response due to the release of both norepinephrine and a noncholinergic, nonadrenergic inhibitory neurotransmitter. In the aganglionic strips (obtained from patients with Hirschsprungs disease), nerve activation tended to evoke contraction, apparently due to enhancement in the release of acetylcholine. At the same time, the release of norepinephrine appeared to be less than normal. A particularly interesting finding in the aganglionic muscle strips was the presence of a substantial inhibitory response due to the release of a noncholinergic, nonadrenergic substance. These results provide further evidence for the importance of the innervation of the aganglionic segment in Hirschsprungs disease.

Hindgut duplication. Report of a patient with long-term follow-up

A girl with a rare congenital malformation consisting of duplication of the urinary and genital tracts as well as of the entire colon, terminating in double rectogenital fistulas, is presented. Because of a small-capacity urinary bladder on one side and an atonic neurogenic bladder on the other side, urinary diversion was necessary. A preliminary diverting colostomy of the duplicated transverse colon was followed by anoplasty of one anus. Later on, the duplicated proximal colostomy was anastomosed to the one distal colon leading to the rectum with anoplasty. The treatment of this complex anomaly is described.

Gastric devascularization—An emergency treatment for hemorrhagic gastritis in the neonate

A newborn baby suffering from massive hemorrhage due to erosive gastritis, was treated successfully by devascularization of the stomach which is a rather simple procedure. Experience with adults as well as with this patient justifies the use of this operation when other measures fail to stop massive bleeding in hemorrhagic gastritis.

Sandifer's syndrome reported and reviewed

Sandifers syndrome is a rare manifestation of gastroesophageal reflux in children that occurs in association with abnormal movements and postures of the head, neck, and trunk. Presented are two cases of this syndrome, treated successfully by Nissen fundoplication, and a review of the literature. The major symptoms and signs are described and summarized in tabular form. Theories of etiology and pathophysiology are discussed although these remain unclear. Diagnosis is usually delayed as patients are assumed to be suffering from a neurological disorder. Treatment of this rare condition is by controlling reflux, which when successful, abolishes symptoms in all cases.

Fundoplication and pyloroplasty — A surgical treatment for intractable “cyclic vomiting” in familial dysautonomia

A 12-yr-old boy with familial dysautonomia was successfully treated for intractable, life-threatening vomiting and aspiration pneumonia by fundoplication and pyloroplasty.

The prognosis of low-birth-weight infants undergoing major surgery

The outcome of 158 infants of low birth weight (LBW) undergoing major surgical procedures was studies. Eighty-four patients were operated on for congenital malformations, mainly atresia of the esophagus, duodenum, and small bowel. Laparotomy for necrotizing enterocolitis (NEC) or idiopathic perforation of the intestine and ligation of a patent ductus arteriosus (PDA) were the most common acquired disorders requiring surgery. In the very LBW (less than 1500 g) group, 73% of the patients underwent operations for acquired lesions. The overall mortality was 23% and for the the very LBW infants 32%. The survival rate was better for the congenital disorders (87%) than the acquired lesions (66%) as a whole, but was about the same in the very LBW subgroups. The survival rate correlated with both birth weight and gestational age in the congenital anomalies, but not in the acquired disorders subgroup; here the prognosis was mainly determined by the severity of the underlying disease.

Diffuse intrahepatic bile duct abscesses — Diagnosed by percutaneous transhepatic cholangiography

Multiple intrahepatic abscesses are associated with a very high mortality rate. We believe that percutaneous transhepatic cholangiography (PTC) is the definitive diagnostic procedure in this condition. Two patients with biliary duct obstruction who developed hepatic abscesses are presented. The value of early diagnosis by PTC is stressed.