Intact omphalocele. Perennial dilemma of operative or "conservative" management

A critical analysis of twenty-seven patients with intact omphalocele is presented. Surgically correctable intra-abdominal anomalies were found in ten patients. Among these ten, three (11 per cent) had associated diaphragmatic hernia, four (14.8 per cent) had atresia of the small bowel, and seven (25.9 per cent) had malrotation with associated congenital duodenal bands.It appears that in patients with intact omphalocele, life-threatening, but surgically correctable, intra-abdominal anomalies occur frequently enough to warrant abdominal exploration and concomitant correction.     

Carcinoma of the head of the pancreas: bypass surgery in unresectable disease

One hundred patients who underwent bypass surgery for unresectable, histologically proven carcinoma of the head of the pancreas were studied to determine: the incidence of a second operation for duodenal obstruction in survivors who had a biliary diversion only; whether the addition of a prophylactic gastroenterostomy is associated with increased mortality. Eighty-six patients (Group A) had a biliary diversion alone while fourteen (Group B) had a biliary diversion and a prophylactic gastroenterostomy. Both groups were comparable regarding sex, age, symptomatology and clinical findings. The postoperative mortality was similar for both groups (Group A, 27.9 per cent; Group B, 14.3 per cent). The mean surgically related hospital stay was 22 +/- 6.5 days (Group A) and 20 +/- 7 days (Group B). Of the 62 patients in Group A who left hospital, 8 (13 per cent) required a second operation for duodenal obstruction (mean interval 5.4 +/- 2.7 months). The in-hospital stay for the second operation was 14.4 +/- 7.7 days. The overall survival for both groups was identical at 6 months (35.5 versus 33.3 per cent) and 12 months (19.3 versus 16.7 per cent). This study suggests that prophylactic gastroenterostomy adds no risk to patients requiring biliary diversion for carcinoma of the head of the pancreas. Its routine use is therefore justified since 13 per cent of the patients who survived the biliary diversion required further surgery for duodenal obstruction before death.     

Congenital duodenal obstruction: the impact of an antenatal diagnosis

In a series of 34 infants with congenital duodenal obstruction, 15 were diagnosed by antenatal ultrasound between the fifth month of gestation and term. Fourteen patients were available for follow-up. In 10 of 14 cases, patients were available for follow-up. In 10 of 14 cases, parents were informed of the diagnosis antenatally and, in 9, were advised of the possibility of surgery in the neonatal period. Parents felt the information helped them prepare for the surgical and medical interventions necessary in the postnatal management of their infants. The mean birth weight and gestational age of these infants were comparable with those diagnosed postnatally. Associated congenital anomalies were more common in the patients with a postnatal diagnosis. In the total group of 34 infants with duodenal obstruction, 32 had surgical repair with a survival rate of 94%. Surgical procedures were comparable between the two groups. Surgical intervention occurred earlier in the neonatal period when an antenatal diagnosis was made. Overall survival in the entire series was 88% with four deaths attributed to severe associated congenital anomalies. We conclude that (1) the antenatal diagnosis of duodenal obstruction influences parents positively in coping with the anomaly, and (2) although surgery was performed sooner, the outcome of infants with duodenal obstruction was not changed by providing an antenatal diagnosis.     

Urogenital anomalies and congenital rubella syndrome

Eight patients with congenital rubella syndrome and associated genitourinary anomalies are reported and the English language literature reviewed. This association appears most prevalent in male patients. Although hypospadias and cryptorchidism are the most common anomalies in the literature, 50 per cent of the patients in this series had vesicoureteral reflux. It is suggested that genitourinary anomalies may be present in 20 per cent of children with the congenital rubella syndrome.     

Choledochal cyst associated with duodenal obstruction

The association between congenital duodenal obstruction and concomitant choledochal cyst has not been reported, although duodenal obstruction is known to be associated with many other anomalies. The authors describe 2 patients with choledochal cyst with duodenal obstruction. In 1 patient, a diverticulum type of choledochal cyst was found within an annular pancreas. Cyst excision, choledochojejunostomy, and side-to-side duodeno-duodenostomy were performed. The other patient showed separated duodenal atresia and other multiple anomalies including imperforate anus. A choledochal cyst was noted at the time of duodeno-duodenostomy and sigmoid colostomy. Cyst-enterostomy was performed at the age of 8 months, but the patient died of multiple anomalies. Intraoperative cholangiography indicated an anomalous pancreatobiliary ductal junction (APBDJ). In both patients the bile in the cyst contained high levels of amylase, suggesting the presence of an APBDJ. An APBDJ is considered to play an etiologic role in the development of the choledochal cysts associated with duodenal obstruction.     

Congenital causes of duodenal ulcers in adults.

In seven cases of congenital anomalies in adults, duodenal obstruction and peptic ulcer disease developed. There were two cases of congenital duodenal web, two of hypertrophic pyloric stenosis, two of annular pancreas, and one of a preduodenal portal vein. The diagnosis is seldom made preoperatively. In the four patients who had preoperative gastric analysis, the acid secretions were increased. Those patients who underwent endoscopy had changes consistent with hypertrophic secretory gastrophy and duodenitis. We believe that treatment should be directed toward relief of the duodenal obstruction and the reduction of basal acid secretion by truncal vagotomy in all such cases.     

Occult intraspinal anomalies and congenital scoliosis

Of 251 patients with congenital scoliosis, occult congenital intraspinal anomalies were diagnosed in forty-six (18.3 per cent). A diastematomyelia was the commonest anomaly (forty-one patients). Other less common anomalies, occurring alone or in association with a diastematomyelia, were: neurenteric, epidermoid, and dermoid cysts; teratoma; lipofibroma; absence of nerve roots; fibrous bands; and a tight filum terminale. Intraspinal anomalies were associated with all types and sites of congenital scoliosis by far the highest incidence (52 per cent) occurred in association with a unilateral unsegmented bar with contralateral hemivertebrae in the lower thoracic or thoracolumbar regions. Thirty of the patients with an intraspinal anomaly had neural abnormalities, which usually affected only one lower extremity, and in twenty-four patients a paralytic foot deformity developed. Neural deterioration occurred in nine of these patients before the age of five years and was halted by excision of the anomaly. An additional twelve patients (4.8 per cent) of the 251 with congenital scoliosis also had a unilateral neural deficit in the lower limb and a paralytic foot deformity, similar to those found in the patients with an intraspinal anomaly, but had no myelographic evidence of a structural anomaly.     

Congenital duodenal obstruction. A review of 65 cases.

We report our experience with 65 patients with congenital duodenal obstruction, 36 with intrinsic and 29 with extrinsic lesions. Seventeen patients had trisomy 21 syndrome. Eight pregnancies were complicated by polyhydramnios. The diagnostic features encountered, the operative procedures used, and the postoperative management regimes used are presented. Thirty-two of the 36 patients with intrinsic lesions and 28 of the 29 patients with extrinsic lesions survived. The data on the five patients who died emphasize the effect of multiple congenital anomalies and prematurity on survival. This review suggests that the surgical procedures available for treating patients with congenital duodenal obstruction are well established and yield predictably good results.     

Endoscopic endoprosthesis insertion following failure of cholecystojejunostomy in pancreatic carcinoma

Sixteen patients in whom malignant biliary obstruction recurred after failure of cholecystojejunostomy had attempted relief of obstruction by endoscopic insertion of an endoprosthesis. This treatment was successful in 13 patients, although one required percutaneous insertion because of duodenal distortion and one required a combined percutaneous-endoscopic approach. Median survival after stenting was 3.5 months (range from 2 weeks to 16 months). Of the three treatment failures, two patients had external drainage only and one succumbed before further treatment could be attempted. There were two complications associated with the procedure (one small bile leak and one episode of cholangitis) but the 30-day mortality rate of 12.5 per cent was related to disease progression rather than the procedure. Endoscopic stent insertion may achieve further worthwhile palliation of jaundice after the failure of surgery to relieve malignant biliary obstruction.     

Analysis of postoperative reoperation for congenital duodenal obstruction

OBJECTIVE: To analyse the risk factors for reoperation after initial surgical repair of congenital duodenal obstruction and demonstrate that they can be decreased with more careful attention and more advanced techniques during surgery. METHODS: The records of newborns and infants (aged 0-2 months) who had surgical therapy for congenital duodenal obstruction in the past 30 years were reviewed and analysed. Of the 298 patients, 132 (44%) were boys and 166 (56%) were girls. All patients who underwent repeat surgery postoperatively were evaluated by the reasons for surgery. The number of patients with various combination lesions of congenital duodenal obstruction was also calculated and the relationship to postoperative reoperation was analysed. RESULTS: Twenty patients (6.7%) had congenital duodenal obstruction with combination lesions including duodenal web, malrotation, annular pancreas and multiple duodenal web. Twelve patients required further operation 5 days to 2 years postoperatively for complications (n = 5) and other duodenal atresias that were not discovered initially (n = 7). CONCLUSION: More than half of reoperated patients (7/12) had multiple lesions of duodenal obstruction that were missed during the primary operation. The postoperative reoperation rate for congenital duodenal obstruction could be decreased with more careful attention to operative details and more preoperative and intraoperative evaluation of the gastrointestinal tract.     

Gastro-oesophageal reflux and oesophagitis before and after vagotomy for duodenal ulcer

Fifty patients undergoing elective vagotomy for the treatment of chronic duodenal ulceration have been investigated pre-operatively and again 3 months postoperatively to determine the extent and severity of associated gastro-oesophageal reflux. Pre-operatively all patients had a normal lower oesophageal sphincter pressure but 50 per cent had symptoms of gastro-oesophageal reflux, 42 per cent had excessive reflux on 24 h pH monitoring and 30 per cent had oesophagitis on endoscopy and/or oesophageal biopsy. Postoperatively, reflux symptoms were present in only 12 per cent of patients but pH studies were still abnormal in 36 per cent and oesophagitis was observed in 32 per cent. Lower oesophageal sphincter pressure was unaffected by vagotomy. Gastro-oesophageal reflux is common in pre-operative duodenal ulcer patients and is not significantly reduced by vagotomy. Careful pre-operative oesophageal assessment is necessary to determine which duodenal ulcer patients require an anti-reflux procedure in addition to vagotomy.     

Duodenal hematoma: Critical analysis of recent treatment technics

Analysis of the fifty-six cases of duodenal hematoma reported since 1966 shows a significant rise in incidence, with uncertainty regarding the most appropriate approaches to therapy. The diagnosis can generally be made by recognizing the “coil spring” sign on upper gastrointestinal series. Thirty-four per cent of the patients reviewed were treated successfully with conservative management. Sixty-two per cent were treated surgically. Fifty-one per cent of the surgical patients were treated by simple evacuation of the hematoma, and 12 per cent of these required reoperation for recurrent obstruction. Forty per cent of the surgical patients were treated by evacuation and gastrojejunostomy, and 21 per cent of these required reoperation for marginal ulceration. A previously unreported case is presented and a new surgical approach utilizing gastroduodenostomy is described, which is believed to offer potentially less risk of subsequent marginal ulceration or recurrent duodenal obstruction.     

Neonatal small bowel atresia, stenosis and segmental dilatation

In 8 years, 44 neonates were treated for small bowel atresia, stenosis or segmental dilatation by one surgical team. There was a single lesion in 30 infants and 14 had two or more sites of intestinal obstruction. Associated gastrointestinal abnormalities were found in 23 (52 per cent) patients. Resection of the atresia(s) and primary anastomosis were performed in 34 infants and staged procedures were carried out in ten patients with complicated lesions. Patients undergoing staged procedures had a higher complication rate than those having primary repair (six out of ten and 11 out of 34 respectively). The overall initial survival rate was 93 per cent, rising to 100 per cent in those without associated anomalies or prenatal complications, but more than a quarter (27 per cent) of survivors have long-term disabilities.     

Association of congenital hypertrophy of the retinal pigment epithelium with familial adenomatous polyposis

Ophthalmological examinations were performed in 49 people (43 patients) from 24 families affected by familial adenomatous polyposis (FAP). No features of congenital hypertrophy of the retinal pigmented epithelium (CHRPE) were noted in six first-degree relatives without colonic polyps. Patients with Gardner's syndrome exhibited CHRPE more frequently than those without exostosis or desmoid tumour (91 per cent versus 45 per cent). In addition the prevalence of CHRPE was slightly higher in the patients with gastric or duodenal polyps (68 per cent compared with 50 per cent) in those who had a normal gastroduodenal examination. There were 11 families in which two or more patients underwent ophthalmological examinations. In five of these 11 families, all 12 patients were negative for CHRPE. In four of the remaining six families, all 11 patients had CHRPE. CHRPE is one of the pleiotropy of FAP and ophthalmological examination is an important screening test for the family of patients with CHRPE associated with FAP.     

Penetrating duodenal injuries. Analysis of 100 consecutive cases.

One hundred consecutive patients with penetrating duodenal injuries were reviewed retrospectively to analyze the results of various methods of treatment. The severity of the abdominal injury was quantified by the Penetrating Abdominal Trauma Index (PATI). The overall mortality was 25%. Sixteen per cent of the deaths were related to extensive associated organ injury, eight per cent to sepsis, and one per cent to concurrent head trauma. Duodenal fistulas occurred in four per cent and were associated with mortality in two per cent. The complications of duodenal fistula, abdominal sepsis, and mortality from sepsis were significantly higher in those patients treated by repair and decompressive enterostomy with or without a serosal patch than in those with repair or resection. The severity of duodenal and associated organ injuries, as well as the clinical status, were similar in both groups. It is concluded that the majority of duodenal injuries from penetrating trauma may be treated effectively by primary repair, and that the use of decompressive enterostomy or serosal patch appears to contribute to an increased morbidity rate.     

Pancreatic cancer: 30 year review (1947 to 1977).

The treatment of pancreatic cancer has been slow to show increased survival, and in the vast majority of patients only short-term palliation can be achieved. However, since there are no 5 year survivors among patients undergoing only palliative procedures, more aggressive surgical therapy may eventually improve the poor survival rate. In our group of 207 patients, 7.2 per cent were considered resectable for cure and had a curative type resection with a resultant 10 per cent 5 year survival rate. Operative mortality was 30 per cent. One hundred one patients were considered unresectable and underwent a palliative procedure. The mean survival of the patients undergoing palliative procedures was 7.7 months for biliary bypass alone, 3.6 months for duodenal bypass alone, and 9.4 months for combined biliary and duodenal bypass. The overall operative mortality for patients undergoing a palliative procedure was 13 per cent (13 of 101 patients). Eleven per cent of our patients undergoing biliary bypass alone required re-operation for duodenal obstruction. Gastrojejunostomy should be performed at the time of initial exploration, particularly for lesions of the head of the pancreas, to avoid reoperation in the short lives of these unfortunate patients.     

Inheritance and spondylolisthesis: a radiographic family survey.

A radiographic suvey has been carried out of 147 first-degree relatives of forty-seven patients treated in Edinburgh for spondylolisthesis of the fifth lumbar vertebra; twelve patients had the dysplastic (congenital) type and thirty-five an isthmic defect. The survey identified 19 per cent of relatives with spondylolysis, and index patients with each type of spondylolisthesis had relatives with the opposite type. Index patients with the dysplastic form had a higher proportion of affected relatives (33 per cent) than had those with the isthmic type (15 per cent), but both figures were significantly in excess of the estimated frequency for the general population of under 1 per cent and 5 per cent respectively. Spina bifida occulta at the fifth lumbar or first sacral level or both, and lumbosacral segmental defects were commoner amongst all individuals with spondylolysis than amongst unaffected relatives (dysplastic form 94 per cent, isthmic type 32 per cent, unaffected relatives 7 per cent). However, there was no single instance of a neural tube defect (anencephaly, spina bifida with or without meningocele, other generalised vertebral anomalies or spinal dysraphism) amongst 826 first-, second- or third-degree relatives. It is concluded that the developmental defects of the vertebrae associated with spondylolysis are not aetiologically related to the neural tube defects. The one in three risk of spondylolysis to near relatives of patients with the dysplastic form of spondylolisthesis is emphasised in order that the deformity in their sibs and children can be recognised at any early age.     

Annular pancreas in children: a recent decade's experience

Purpose

Annular pancreas is a recognized cause of duodenal obstruction in children. The authors sought to characterize the clinical, radiologic, and prognostic findings in this disorder through a 10-year review of all patients with annular pancreas.

Methods

A retrospective review of all annular pancreas cases identified between 1993 and 2002 at 2 tertiary pediatric surgical centers was completed. Factors analyzed included patient demographics, presenting symptoms, associated anomalies, radiologic findings, operation performed, postoperative outcomes, and complications.

Results

Sixteen patients were identified. Four patients (25%) were premature. Twelve patients (75%) presented during the first week of life and the remainder within the first year. All patients presented with vomiting, which was nonbilious in 15 (94%). Five patients (31 %) had chromosomal anomalies, and 6 (38%) had other major congenital malformations. Eleven patients (69%) presented with partial duodenal obstruction. Operations performed were duodenoduodenostomy in 14 (88%), duodenojejunostomy in 1 (6%), and Ladd’s procedure in 1 (6%). Enteral feedings were started, and full enteral feeding was achieved at an average of 8.4 days and 15.9 days after operation, respectively. All patients survived and were discharged after an average hospitalization of 24.0 days.

Conclusions

Patients with annular pancreas have preampullary duodenal obstruction, which is more commonly partial. Duodenoduodenostomy is the appropriate treatment. Prognosis is excellent, despite the frequent association with chromosomal anomalies and major congenital malformations.     

Results of surgical palliation for cancer of the head of the pancreas and periampullary region.

Between 1977 and 1986, 101 patients underwent surgical bypass for periampullary carcinoma. The hospital mortality rate was 18 per cent and the morbidity rate 43 per cent. Mortality was not influenced by the extent of the tumour. Survival rates at 1,2 and 3 years were 28, 9 and 4 per cent, respectively. The median survival time was 17 months for localized tumours, 10 months for those that had invaded surrounding tissues, 6 months in the presence of lymph node involvement and 3 months with distant metastasis. The quality of survival was good for most patients with localized tumours but poor for those with parenchymal metastasis, in whom palliation was transient for 85 per cent and effective for less than half of their survival time for 60 per cent. These results suggest that patients with distant metastasis but without impending duodenal obstruction should undergo palliation by endoscopic or percutaneous routes while those with less advanced disease or with duodenal involvement remain candidates for surgical bypass.     

Immediate results of emergency operation for massive upper gastrointestinal hemorrhage: A Cooperative study by the Connecticut Society of American Board Surgeons

Two hundred ninety-six patients underwent operation to control massive upper gastrointestinal hemorrhage at twenty-eight hospitals in Connecticut during a one year period. Sixty-four per cent of the patients had bleeding from duodenal and/or gastric ulcers. More than half of the sixty-four deaths occurred in the 18 per cent of patients with bleeding from esophageal varices or “stress” ulceration. 3.6 Per cent of the patients less than sixty-five years of age died after emergency operation to control hemorrhage from duodenal and gastric ulcers. Operative mortality was related more closely to advanced age and associated disease than to the type of operation performed.