Distal foregut atresias in consecutive siblings and twins in the same family.

The distal foregut comprises the antrum of the stomach and supra-ampullary region of the duodenum. In this part of the gut, intraluminal mucosal diaphragms, webs, membranes (type I), and rarely solid cord (type II) atresias are occasionally seen in clinical practice. Due to increased awareness, the number of reported cases has increased in recent years. These foregut lesions are known to have a strong genetic association. Both antral (AA) and duodenal atresias (DA) may occur as potentially curable sporadic cases or in combination with lethal malformations such as hereditary multiple gastrointestinal atresias affecting the fore-, mid-, and hindgut. The association of AA and junctional epidermolysis bullosa lethalis is well-known. We describe an unusual occurrence of foregut atresias in two consecutive siblings and a pair of monozygous twins. The involvement of four siblings in a single family is extraordinary and as yet unreported. This cluster of cases not only lends a strong support to a genetic origin, but also the mode of transmission, despite the absence of a history of consanguinity. It indicates that atresia of the gastric antrum and supra-ampullary DA are etiologically closely related. It is also possible that the discordant expression in the twins could be a pleiotropic effect of the same genetic factor. Attempts are made to explain the possible pathogenesis of this unusual atresia and relate it to other associated anomalies.

Multiple gastrointestinal atresias in two consecutive siblings.

Two consecutive female siblings with multiple gastrointestinal atresias are described. The history of consanguinity in the parents and the presence of extensive typical pathological lesions suggest a genetically-induced developmental fault in the alimentary tract during the early embryonic period.

Atresias of the gastrointestinal tract in an inbred, previously unstudied population.

Retrospective analysis of the records of newborns with gastrointestinal (GI) atresias in a 16-year period revealed that more than 25% of patients had genetically-influenced atresias. Among these, an appreciable increase was observed in atresias confined to the antral region and in a syndrome of hereditary multiple GI atresias. In the absence of environmental and teratogenic factors, this increase could be due to the traditional social practice of endogamy in this population. However, this clinical finding remains to be supported by relevant prospective genetic studies.

Junctional epidermolysis bullosa lethalis with pyloric and anorectal obstruction.

The authors present a case of epidermolysis bullosa lethalis (EBL) associated with a double obstruction, one at the pyloric and the other in the anorectal region. Both obstructions could be due to separation of the rectal mucosa during intrauterine life followed by adhesive closure of its wall. Both the gastrointestinal lesions could be part of the generalized denudation process involved in EBL.

Outcome of minimal surgery for hydatid cysts of the liver in children with reference to post-operative biliary leakage.

Analysis of 30 children with hydatid cysts of the liver who were treated by partial pericystectomy and external tube drainage showed that five (21%) of 24 cases in whom clear hydatid fluid was observed during surgery developed biliary leakage. The cysts in the remaining six contained bile-stained fluid, indicating the presence of cystobiliary communications, and five of these children continued to drain bile post-operatively, in spite of appropriate precautions taken during surgery. Histological examination of the pericyst wall confirmed the presence of openings of small bile ducts in the cyst which probably caused the biliary leak. It is possible that some larger openings were overlooked during surgery. It is concluded from this study that biliary leakage during the post-operative period should be expected in a significant number of patients subjected to surgery for hydatid cysts of the liver. However, the leak is likely to cease spontaneously, providing distal biliary duct obstruction is ruled out and external tube drainage is used to prevent accumulation of bile in the pericyst cavity.

Surgical and pathological basis for interval appendicectomy after resolution of appendicular mass in children.

BACKGROUND/PURPOSE: The role of appendicectomy after the resolution of appendicular mass is debatable. A study was conducted to evaluate whether surgical and pathological features of the excised appendices favor the operation in the quiescent period. METHODS: During a 60-month period, 59 patients were admitted in our unit with a diagnosis of appendicular mass and were treated initially with conservative management. Five patients failed to respond to this management and they were operated on immediately. RESULTS: Fifty-four patients recovered fully, and relatives were advised to bring them back to the hospital for appendicectomy on a scheduled date after 6 weeks. Fifteen of 54 (27.7%) patients did not return, but the other 7 came back because they had symptoms of recurrent appendicitis. The remaining 32 patients underwent appendicectomy as scheduled. The surgical findings and pathological features of excised appendices showed various abnormalities. CONCLUSIONS: Considering these features it could be concluded that delayed appendicectomy is unjustified in patients with absent appendix or with its lumen obliterated, whereas the other remaining patients who harbored normal, thickened, fibrotic, transected, stump, and appendix with chronic inflammation or containing fecal casts would benefit from operation. Because we have no method to date to distinguish between these variants "in situ" delayed appendicectomy seems beneficial for all the patients who respond well to the initial management of appendicular mass.

Concordant expression of Hirschsprung's disease in monozygous twins.

A monozygous pair of twins with long segment Hirschsprung's disease born to non consanguineous parents is presented. Mother's history was uneventful. In the absence of prenatal and postnatal illness, the concordant lesions in this pair could be attributed to genetic factors.

Familial occurrence of congenital incomplete prepyloric mucosal diaphragm.

Incomplete prepyloric mucosal diaphragm (IPMD) is an uncommon congenital anomaly that leads to gastric outlet obstruction in infancy and childhood. This report describes the occurrence of IPMD in six children in a closely knit tribal family from a geographically isolated desert town with a small population in the Sahara. Their records showed similarities of clinical, radiological, operative, and histopathological features. These features, as well as its occurrence in brothers, sisters, and cousins, suggest that this unusual anomaly is transmitted as an autosomal recessive trait.

Early versus delayed repair of reduced incarcerated inguinal hernias in the pediatric population.

This study concerns 183 pediatric patients (age range, 14 days to 10 years) who initially presented with incarcerated inguinal hernias. In all of them, initial conservative management to reduce the hernia was successful. This consisted of elevation of the lower half of body and sedation and/or gentle manual pressure. Thereafter, 75 of them had "early" operation (within 72 hours). Early surgery was not possible for the other 108 patients because of various reasons. These patients were discharged from the ward and were scheduled for "delayed" repair within 1 to 3 months. The complication rates were similar for the two groups, but 17 (15.7%) of the 108 patients in the delayed group had repeat incarceration, some of them more than once during the waiting period. Reincarceration occurred as early as 5 days and as late as 120 days after the initial discharge. The authors conclude that the results of delayed repair were unfavorably affected by the recurrent incarceration, and therefore all pediatric patients should have hernia repair within 5 days after the reduction of incarceration.

Limitations of surgery in intraabdominal Burkitt's lymphoma in children.

The role of surgery in intraabdominal Burkitt's lymphoma is a controversial subject, and different views are expressed. In the authors' institution, 41 children (30 boys and 11 girls) have been treated who had intraabdominal Burkitt's lymphoma. A study was undertaken to assess the limitations of surgery in the treatment of intraabdominal Burkitt's lymphoma. An uniform policy was made to resect the tumor if possible or to debulk tumor irrespective of the spread of disease, and if this was not possible to perform only a biopsy. Thirty-nine children had laparotomy and various operative procedures, and in 2 children no laparotomy was performed, but tissue was obtained for histopathologic examination from cervical lymph nodes and jaw. Twelve children underwent complete resection of the intraabdominal tumor. There were no deaths in this group, and 10 of 12 children are long-term survivors. In 15 patients, debulking of the tumor was carried out. In 4, the tumor had perforated the bowel and caused widespread intraperitoneal deposits and sepsis. There were 3 deaths during the immediate postoperative period, 2 caused by peritoneal sepsis and 1 caused by surgical trauma in advanced disease. Three of 15 patients have survived for more than 2 years. Lastly, in 14 children only a biopsy was performed. Notable features in this group were that 6 patients had extraabdominal disease and another 6 presented initially with features of intestinal obstruction. In the latter group, intestinal bypass procedures were performed but no debulking was attempted. Five of 14 patients died after surgery, whereas 3 were long-term survivors. Patients were administered a chemotherapy regimen consisting of cyclophosphamide, vincristine, Adriamycin, and prednisolone.(ABSTRACT TRUNCATED AT 250 WORDS)

Prospective randomized controlled study of excision versus distal splitting of hernial sac and processus vaginalis in the repair of inguinal hernias and communicating hydroceles.

A total of 798 pediatric patients between the ages of 16 days and 10 years underwent a randomized trial of two surgical procedures to assess the superiority of one over the other. In the first group of 231 patients, 236 hernial sacs (HS)/processus vaginalis (PV) were excised completely after transfixation and transection of the sac at the internal ring, whereas in the second group of 567 patients, 595 residual HS/PV were not excised but split longitudinally. The results showed that there was no significant difference in the two groups as far as complications were concerned. Considering the results of these two procedures, it could be concluded that even the less extensive process of distal longitudinal splitting of the residual HS or PV can be preferred in the repair of hernias or communicating hydroceles in male children.

Duodenal atresia: its occurrence in siblings.

The authors describe a case of duodenal atresia occurring in two siblings. It is possible that the third sibling also died after complications of the same malformation. This familial occurrence may support the hypothesis that this anomaly is determined by an autosomal-recessive gene.

Management of appendicular mass in children.

Of 66 children under 12 years of age treated for appendicular mass, seven underwent immediate surgery, and two of them developed post-operative wound infection. The remaining 59 children had conservative management which consisted of bedside observation of vital and abdominal signs, intravenous fluids and triple antibiotic therapy with ampicillin, gentamicin and metronidazole. The majority of the children responded well to this method, and in 54 (91.5%) the mass resolved completely. Two patients did not respond and required drainage of the abscess. Three other children responded initially to conservative management, but returned with recurrence of the mass. After their discharge from hospital, only 14 (24.5%) children returned for interval appendicectomy, which revealed that 13 of the 14 appendices excised still showed patent lumens. This study showed that conservative management of appendicular mass is safe and effective in children. In view of the patent appendicular lumen in those who had complete resolution of the mass, and the attendant risk of re-infection, interval appendicectomy appears to be desirable.

An effective and safe surgical method for the treatment of hydatid cysts of the liver in children.

Between June 1986 and May 1989 35 children (18 boys and 17 girls) below the age of 12 years were surgically treated for hydatid cysts of the liver. Of the children 24 (68.5%) had a single cyst situated in either of the lobes of the liver, while the remaining 11 (31.5%) harboured more than one cyst. A uniform method of enucleation, partial cystectomy and external tube drainage was used in all of them. Small residual cavities were not drained but were kept open on to the surface of liver. Results showed that six patients (17.1%) had transient biliary discharge which resolved within 3-6 days, whereas in two children (5.7%) biliary drainage persisted for four, respectively six weeks following surgery. This also stopped spontaneously. The method we used in our patients was safe and effective.

Hospital observation for right lower quadrant abdominal pain with questionable acute appendicitis in children.

920 children below the age of 12 years were admitted with complaints of pain in the right lower abdomen and a suspected diagnosis of acute appendicitis. In 720 patients, clinical diagnosis was made and immediate operation was performed. In 644 of them (89.5%) an intraabdominal lesion was found but in 76 (10.5%) no disease was encountered. Rest 200 patients were observed in the ward and progression was noted at regular intervals. Eight of these patients did not improve while on observation and they were operated. Five others did not have acute appendicitis but in them definite medical diagnosis was made. However in remaining 187 observed patients abdominal signs gradually resolved and needed no surgery but no definite diagnosis also could be made. They appeared to have non-specific abdominal pain. The conclusion of the study was that inhospital observation of patients with right lower quadrant abdominal pain and questionable appendicitis upto three days was a safe way to reduce the rate of negative appendicectomies and unnecessary surgical exploration.