Visceral myopathy of the gastrointestinal and genitourinary tracts in infants.

We describe 4 infants who had chronic intestinal pseudoobstruction caused by visceral myopathy. Three of the 4 were girls. Two were symptomatic at birth and 2 were symptomatic by 3 wk of age. All had abdominal distention and emaciation, 3 of the 4 had severe obstipation and fecal impactions, and 3 had signs of urologic obstruction. All had gaseous distention of the small bowel and colon, and barium studies showed dilated small bowel and colon, with slow transport through the small intestine. Two of 3 had enlarged stomachs and slowed gastric emptying, and 3 had dilated bladders and ureters. The 1 infant studied by esophageal manometry had absence of esophageal contractions. Despite total parenteral nutrition in 3, all died within 10-18 mo. The pathologic features of visceral myopathy were identified in variable sample sites from the esophagus, stomach, small intestine, colon, bladder, and ureter of the 4 infants. Of 170 family members related to 3 of the infants, there was no consanguinity and no one appeared to be clinically affected. Thus, an infantile form of visceral myopathy exists which, pathologically, is identical to the familial and sporadic forms of visceral myopathy previously identified in adolescents and adults.

Presidential address: E pluribus unum.

To promote the best possible overall pediatric surgical care, I think that this may be the time that we should consider making common cause with other surgical specialists who work in the pediatric age range. This will need cooperative organization in our hospitals, medical schools, and on the national level. We now need a combined strong voice to deal with the large organizations through which the policies and the funding are being decided. We must now "hang together, or we (and our patients) may all hang separately".

Biliary Atresia: histopathologic observations and reflections upon its natural history.

Operative liver biopsies and specimens of the extrahepatic ducts and porta hepatis have been studied in 12 cases of biliary atresia. In all cases, the liver showed giant cell transformation and inflammation with mononuclear cells and neutrophils. Most had other features of neonatal hepatitis, including necrosis of hepatocytes. In the intrahepatic bile ducts of all cases but one, the hepatic ducts and glands at the porta hepatis, and in the extrahepatic ducts where epithelium remained, there was degeneration of the epithelium and intramural inflammation. In the ducts at the porta hepatis and in 6 of 8 extrahepatic ducts where epithelium remained, there was extensive mural fibrosis compromising the diameter of the duct lumens. Three cases showed the inflammatory changes distal to sites of closure of the extrahepatic ducts. These findings demonstrate that in biliary atresia, hepatitis, intrahepatic cholangitis, and sclerosing cholangitis of the extrahepatic ducts all interact to produce acquired obstruction to bile flow.

Studies of idiopathic intestinal pseudoobstruction. I. Hereditary hollow visceral myopathy: clinical and pathological studies.

A 15-year-old girl with idiopathic intestinal pseudoobstruction is reported. She presented with a long term history of low grade obstructive symptoms, diarrhea, and poor nutrition culminating in an acute obstructive attack leading to exploratory laparotomy. At surgery, the small bowel and colon were dilated, with no mechanical obstruction found. Further evaluation revealed her to have a diffuse disorder of gastrointestinal smooth muscle function involving esophagus, small bowel, and colon. Because medical management failed to control symptom, a gastrojejunostomy was done to bypass a megaduodenum. A third laparotomy was necessary 2 months later because of intractable obstructive symptoms. At this last laparotomy, the afferent loop was taken down and a duodenoplasty and duodenojejunostomy were performed, resulting in clinical improvement. Light and electron microscopic study of the excised small intestine showed marked thinning and degeneraton of the smooth muscle, with replacement by fibrous tissue. The myenteric plexus was normal. This case demonstrates that a degenerative disease of smooth muscle may be one cause of idiopathic intestinal pseudoobstruction.

The natural history of Meckel's Diverticulum and its relation to incidental removal. A study of 202 cases of diseased Meckel's Diverticulum found in King County, Washington, over a fifteen year period.

To determine the natural history of Meckel's diverticulum, 202 case records of proved disease of Meckel's diverticulum were retrieved, covering a fifteen year period, from all the hospitals of King County, Washington (population, 1,143,800). Using the figure of 2 per cent incidence of Meckel's diverticulum, we calculated that a Meckel's diverticulum has a 4.2 per cent likelihood of causing disease during a lifetime, decreasing to zero with old age. Using previously published mortality and morbidity figures, we calculated that to save one patient's life from the complications of Meckel's diverticulum, it would be necessary to remove approximately 800 asymptomatic Meckel's diverticula. This would be likely to incur a significant amount of postoperative morbidity from postoperative intestinal obstruction and infection. We suggest that the prophylactic removal of Meckel's diverticulum is rarely, if ever, justified.

Position of rectal fistula in relation to the hymen in 46 girls with imperforate anus.

Forty-six records of infant girls with so-called imperforate anus have been reviewed to determine the position of the rectal opening in relation to the hymen. Eight of these children proved to have the cloacal deformity, in which the rectum enters high into a single tube and just behind the opening of a double or septate vagina, with the urethra entering anteriorly at the same level. No normal hymen was visible in these eight children. Seventeen patients had a normal-appearing hymen, and no rectal opening on the perineum. In each of these 17 children the rectal opening was found above the tissue flap overlying the posterior vestibule. In the remaining 21 patients, the hymen was visible and appeared normal, and there was a rectal opening somewhere on the perineum between the vestibule and the normal position for the external sphincters. These findings suggest that in the presence of a normal-appearing hymen, and in the absence of a normal anus, the rectal opening will be either in the posterior part of the vestibule or on the perineum. Where no hymen was visible, we have found in a limited experience that the rectal opening was high in the pelvis in some degree of the so-called cloacal deformity.