Alexander H. Bill

The natural history of Meckel's diverticulum and its relation to incidental removal

To determine the natural history of Meckels diverticulum, 202 case records of proved disease of Meckels 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 Meckels diverticulum, we calculated that a Meckels 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 patients life from the complications of Meckels diverticulum, it would be necessary to remove approximately 800 asymptomatic Meckels 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 Meckels diverticulum is rarely, if ever, justified.

Anatomy and embryology of congenital intrinsic obstruction of the duodenum

c ONGENITAL DUODENAL OBSTRUCTION has ranked third in our hospital, after imperforate anus and esophageal atresia, as a cause of intrinsic obstruction of the alimentary tract in newborn infants. (Fig. 1.) During these two decades more acceptable survival rates have become apparent, due partly to earlier clinical recognition of the lesion and partly to more effective surgical treatment and approved supportive methods. Less attention seems to have been given to the specific anatomy and related embryology of the lesion. Often the exact location of the obstruction or its relation to the oriiice of the common bile duct has not been reported because the involved segment is usually not opened but is bypassed by an enteroenterostomy. Furthermore, an obstructed proximal segment tends to dilate and elongate, giving the impression that the site of obstruction lies more distally in the duodenum than it really is. Some diaphragms may elongate tremendously in the form of a “wind-sock” [I] or be associated with an intraluminal diverticulum [Z]. Even when autopsy reports are available, detailed descriptions are often lacking or imprecise. The purpose of this report, therefore, is to describe the anatomy of this defect as recorded in autopsy reports and specimens and to relate these findings to the embryology of the area. MATERIAL

The enterocolitis of Hirschsprung's disease

Abstract The clinical syndrome of the enterocolitis of Hirschsprungs disease consists of distention, abdominal pain, explosive watery diarrhea, fever and prostration from acute water and electrolyte loss. The pathogenesis of this enterocolitis is apparently related to partial mechanical intestinal obstruction by the aganglionic segments. Of forty-seven patients with Hirschsprungs disease, twenty-four (50 per cent) were found to have had enterocolitis. There were eight deaths, all from enterocolitis during the neonatal period. Once enterocolitis had occurred further episodes were to be expected. Treatment for the enterocolitis which appears in infancy should be by colostomy. If the enterocolitis appears after resection of the aganglionic segment, anal sphincterotomy and continued dilatations are the treatment of choice. The best treatment for the enterocolitis of Hirschsprungs disease is its prevention by colostomy in infancy as soon as the diagnosis of Hirschsprungs disease is made.

Imperforate anus: A survey from the members of the Surgical Section of the American Academy of Pediatrics+

T ttlS REPORT is based on a survey of the experience of members of the Surgical Section of the American Academy of Pediatrics with imperforate anus for the 5-yr period 1965 through 1969. The authors are the committee appointed to compile d~tta from answers to a questionnaire circulated to 301 members, including honorary overseas members. Many of the members reported as groups from the same institution. The survey represents the experience of pediatric surgeons reporting on 1166 patients from 51 institutions, including childrens hospitals and general hospitals.

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.

Rationale and technic for stabilization of the mesentery in cases of nonrotation of the midgut

Abstract 1. 1. Nonrotation of the midgut is potentially the most dangerous of the various degrees of malrotation because it predisposes the midgut volvulus. 2. 2. The characteristic anatomical feature of these cases is that of a pedicle consisting of the third part of the duodenum, the proximal jejunum, and the ascending colon, overlying and parallel to the superior mesenteric vessels and surrounded by a peritoneal covering. This pedicle constitutes the only attachment of the midgut within the abdomen, thus allowing volvulus. There are other less common anatomical variants of this entity. 3. 3. Nonrotation of the midgut may be found without other anomalies. In addition, it is found in most cases of omphalocele and gastroschisis and also in congenital hernia of the posterolateral diaphragm. 4. 4. Using the conventional operative management for these infants, there were three instances of recurrence of volvulus among 24 cases treated without stabilization. There was one instance of volvulus after diaphragmatic hernia repair in which the nonrotation was not stabilized. 5. 5. When midgut volvulus is encountered, it should be untwisted. After this is done, or when nonrotation of the midgut is found without volvulus, the components of the pedicle of the midgut should be freed by anterior division of the peritoneal bands. Blunt dissection of the duodenum from the proximal transverse colon, fixation of the cecum and its mesentery to the left colon, and suture of the duodenum to the right completes the procedure. 6. 6. A series of 10 operative cases is described.

Immune aspects of neuroblastoma: Current information

Abstract A number of independent studies from Seattle on the immune aspects of neuroblastoma are summarized. It has been demonstrated that most patients with neuroblastoma, cured or otherwise, have lymphocytes which are cytotoxic to neuroblastoma cells in tissue culture. Sixty-eight per cent of twenty-five family members also exhibit the same lymphocyte activity, which was shown in only 4 per cent of control individuals. Further evidence of the importance of the lymphocyte is demonstrated by the fact that both lymphocytic infiltrates in the tumors and higher blood lymphocyte counts at diagnosis correlate with improved survival rates. In a relatively few tests, cytotoxic immune activity has been demonstrated in the serum of some patients and some family members against neuroblastoma cells in tissue culture. The serum of each of six patients with active disease has demonstrated the capacity to prevent the cytotoxic activity of the lymphocytes against neuroblastoma cells in tissue culture by what is known as a “blocking antibody.” This blocking activity was not demonstrated in the serum of four children cured of the disease. An hypothesis of an immune balance in relation to a patients welfare is suggested, in which there are favorable cytotoxic activities by the lymphocytes and sometimes by the serum, and in which there are detrimental effects by the tumor cells themselves and by their protective “blocking antibodies.”

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.

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.

Surgical treatment of esophageal stricture secondary to gastroesophageal reflux

Of 180 patients, 115 evaluated for gastroesophageal reflux (GER) over a 4-yr period were found to be positive. Fourteen patients, ages 3 mo to 15 yr, presented with symptoms of esophageal stricture. Seven patients had a history of previous repair of esophageal atresia (EA). GER was diagnosed variously by barium swallow, radionuclide gastroesophagography, acid reflux test, and endoscopy with biopsy. The stricture may have acted as a barrier and altered the diagnosis of GER higher in the esophagus. Esophageal manometric were performed in 11 of the 14, and were uniformly abnormal. When the diagnosis of persistent esophageal stricture in association with GER was apparent, intraoperative esophageal dilatation and antireflux surgery with intraoperative manometrics were carried out in 13 patients. There was no operative mortality. In 10 of the 13 patients clinical resolution of the stricture was confirmed by barium swallow or esophagoscopy. The remaining 3 patients with stable dense fibrous strictures from long-standing GER, still requie dilatations. Stricture resolution occurred in 9 patients without the need for postoperative dilatations, including 5 with previously repaired EA. There was 1 esophageal perforation and 1 disrupted fundoplication, both of which responded to surgery. Esophageal stricture may be a result of insidious GER. Early diagnosis and surgical correction of GER, aided by esophageal manometrics, will result in healing of esophagitis and rapid resolution of strictures in the majority of patients. Long-standing strictures may require persistent dilatation. Esophageal resection and substitution should be required infrequently.