J. Eugene Lewis

Congenital duodenal obstruction: A 32-year review†

Although survival in infants with congenital intestinal obstruction has improved, duodenal obstruction continues to present unique challenges. One hundred thirty-eight newborns and infants (aged 0 to 30 days) were treated for congenital duodenal obstruction. Sixty-five were boys and 73 were girls. Sixty-one (45%) were premature. Forty-six had an intrinsic defect (atresia, web, stenosis, or duplication), 64 had an extrinsic defect (annular pancreas or malrotation with congenital bands), while 28 had various combinations of these. Presenting signs included vomiting (90%, bilious in 66%), abdominal distention (25%), dehydration (24%), and weight loss (17%). Although plain film abdominal x-ray was diagnostic in 58%, upper and/or lower gastrointestinal contrast studies were obtained in 71% of infants to confirm diagnosis. Thirty-eight percent of patients had associated anomalies, including Downs syndrome (11%), cardiac defects, other atresia, other trisomy syndrome, imperforate anus, and central nervous system anomalies. Fourteen patients (10%) had 3 or more other anomalies, many of which required additional surgical therapy. The operative repair of the various defects included Ladds procedure for malrotation (31%), duodenoduodenostomy (14%), duodenojejunostomy (22%), gastrojejunostomy or gastroduodenostomy (4%), excision of the web and duodenoplasty (3%), or combination of the above (22%). Gastrostomy was placed in 61%. One hundred twenty-eight patients survived (93%). The causes of death were combinations of sepsis, pneumonia, brain hemorrhage, short bowel, and cardiac anomaly. Eight of 10 (80%) who died had other serious anomalies. Twenty patients (14%) required reoperation 5 days to 4 years postoperatively for obstructing lesions (5), wound dehiscence (3), anastomotic leak or dysfunction (6), other atresias (2), choledochal cyst (1), pyloric stenosis (1), and gastroesophageal reflux (2).(ABSTRACT TRUNCATED AT 250 WORDS)

Changing spectrum of cholelithiasis and cholecystitis in infants and children

Cholecystitis and cholelithiasis are infrequent in children and have been historically associated with adolescent pregnancy or hemolytic disorders; however, the incidence and spectrum of cholelithiasis seem to be changing. Between 1970 and 1988, 47 children 17 years of age or less underwent cholecystectomy for cholecystitis or cholelithiasis in our hospital. The patients were divided into chronologic groups: Group 1 encompassed 1970 through 1979 (15 patients) and group 2, 1980 through 1988 (32 patients). The groups were compared for age, sex, pregnancy, blood dyscrasia, family history, obesity, use of total parenteral nutrition (TPN), and incidence of choledocholithiasis with its sequelae. A significant increase in the number of patients with cholelithiasis was found. Infants and young children were affected more frequently in group 2, and many of these young patients had a history of TPN. Choledocholithiasis was also more common in group 2 and presented with life-threatening sequelae. Calculous biliary tract disease should be considered as a possible cause of abdominal pain in children. Timely operative intervention can prevent the increasingly common sequelae of childhood cholelithiasis.

Congenital diaphragmatic hernia beyond infancy

Congenital diaphragmatic hernia (CDH) is a common cause of severe respiratory distress in the newborn. However, the presentation of CDH in older children and adults is rare, and, therefore, little is known concerning its symptoms, operative management, and postoperative complications. Thirteen patients (age range: 2 months to 26 years; 5 males, 8 females) presented with CDH. Four patients had right-sided hernias, eight left-sided hernias, and one bilateral hernias. Symptoms included chronic respiratory tract infections in 6 patients, vomiting in 5, weight loss in 1, severe failure to thrive in 2, and severe respiratory distress in 3; one patient was asymptomatic. Physical signs included the absence of breathing sounds or bowel sounds in the chest in eight patients, hyperresonance in one, and cachexia in two. The diagnosis was confirmed in each patient by chest roentgenogram or gastrointestinal contrast radiograph. All patients underwent immediate repair. After reduction of the viscera, 12 of 13 patients underwent primary diaphragm repair, whereas one patient required a prosthetic diaphragm patch. Twelve of 13 patients (92%) survived. Postoperatively, 7 of the 12 survivors (58%) developed severe gastric atony, and four required further operative therapy. In contrast to newborns, CDH in the older child and adult is frequently seen on the right side, rarely presents with severe respiratory distress, and is occasionally asymptomatic. Postoperative gastric atony is a major cause of morbidity, making transabdominal repair with simultaneous pyloroplasty and/or feeding jejunostomy the preferred operative approach.

Duodenal atresia: A comparison of techniques of repair

Although duodenal atresia is a common cause of congenital intestinal obstruction, the optimal technique of repair remains controversial. In a 10-year period, 41 newborns (20 male, 25 premature) underwent either side-to-side duodenoduodenostomy (SDD, 10 infants), side-to-side duodenojejunostomy (SDJ, 9 infants), or diamond-shaped duodenoduodenostomy (DDD, 22 infants) in a nonrandomized series. One-layer anastomosis was used in each case, and gastrostomy was placed in most patients. Groups were compared for survival, prematurity, associated anomalies, time until feeding onset, total hospitalization time, and complications. The three groups were identical for survival (all 100%), prematurity (60% SDD, 67% SDJ, 59% DDD), and serious associated anomalies (cardiac, gastrointestinal, Downs; 33% SDD, 44% SDJ, 50% DDD). The time until feeding onset was shortest with DDD (4.1 +/- 0.4 days), compared with 8.0 +/- 1.1 days for SDD and 9.6 +/- 1.9 days for SDJ (both P less than .05 v DDD). Total hospitalization was significantly less in DDD (16.2 +/- 2.1 days) v 24.2 +/- 3.1 days for SDD (P less than .05) and 28.3 +/- 4.3 days for SDJ (P less than .01). One complication necessitating reoperation occurred in each group (SDD, adhesions; SDJ, stenotic anastomosis; and DDD, missed second atresia). These data suggest that DDD is superior to SDD and SDJ for repair of duodenal atresia, resulting in earlier feeding and discharge.

Partial duodenal obstruction with incomplete duodenal rotation

Abstract Incomplete rotation of the duodenojejunum with abnormal fixation is a cause of partial obstruction, which may occur even in the presence of a normal colon rotation. The embryology of intestinal rotation is briefly reviewed, and the anatomical features of the obstruction and the surgical treatment are presented.

Newborn duodenal atresia: An improving outlook

From 1973 through 1983, 20 newborns with congenital duodenal atresia were treated. These patients are compared with our previous series and with other published series. There were no fatalities among the 19 patients who underwent operation, an improvement from the 72 percent survival rate in our previous series. Fifty-five percent of the patients had associated congenital anomalies, which frequently complicated their management. The use of a transanastomotic jejunal feeding tube resulted in delayed oral feeding and prolonged hospitalization, and cannot be recommended.

The role of second-look laparotomy in necrotizing enterocolitis

The mortality in newborns with necrotizing entercolitis (NEC) remains unacceptably high. To attempt to improve survival, the most critically ill 14 (of 32 total) newborns with NEC and perforation underwent planned second-look laparotomy 24 to 36 hours after initial exploration to reassess questionably viable bowel and resect if necessary, irrigate purulent material, and search for further perforation. Survival was 10/14 (71%) with, and 12/18 (66%) without second-look laparotomy. Culture-positive pus was found in 14 cases, while in 6 necrotic bowel, and in 2 others a perforation was found at second look. These data suggest that aggressive second-look laparotomy can improve survival in the critically ill neonate with NEC and perforation.

Congenital duodenal atresia

Summary A series of nineteen cases of duodenal atresia seen in a fifteen year period are reviewed. Prematurity, sepsis, severe associated anomalies, and secondary surgery have been important factors in the high mortality. However, during the past three years the survival rate has increased to 72 per cent. Improved supportive care, early diagnosis, and improvements in operative and postoperative management seem to have played a role in this improved survival. The over-all survival rate in the nineteen neonates was 58 per cent.

Pulmonary sequestration with bronchoesophageal fistula

Abstract A 4-month-old white female with total right pulmonary sequestration communicating with the esophagus was successfully treated surgically. The esophogeal communication was suggested by aeration of the opacified lung when the infant cried.

Congenital peritoneal mesothelioma

Only three primary peritoneal mesotheliomas in children have been previously reported. We describe a 6-wk-old girl with a papillary peritoneal mesothelioma of low grade malignancy. This is probably a congenital mesothelioma and is the youngest patient reported to date.