Atresia of the jejunum and ileum: what is the difference?

BACKGROUND: Atresia of the jejunum and ileum is one of the major causes of neonatal intestinal obstruction. Most affected newborn infants present with bilious emesis and abdominal distention. Traditionally, jejunal and ileal atresia have been grouped together as jejunoileal atresia. OBJECTIVE: To elucidate the difference between jejunal and ileal atresia. MATERIAL AND METHOD: A retrospective analysis of patients diagnosed with jejunal or ileal atresia, who were treated at the Department of Surgery, Queen Sirikit National Institute of Child Health during January 1988 to December 2007, was carried out. RESULTS: There were 74 patients with jejunal atresia and 68 patients with ileal atresia. The mean birth weight and gestational age of patients with jejunal atresia were significantly lower than those with ileal atresia. Antenatal perforation occurred more frequently in ileal atresia. Postoperative course was more prolonged and mortality was higher injejunal atresia. Prolonged ileus and anastomotic dysfunction requiring long-term parenteral nutrition were the major causes of complications leading to death. CONCLUSION: There were many differences between patients with jejunal atresia and those with ileal atresia. The more compliant jejunal wall allows massive dilatation upon obstruction with subsequent loss of peristaltic activity, thus poorer outcome in comparison with ileal obstruction. We suggest that atresia of the jejunum and ileum be considered differently.

Bilateral Morgagni hernias association with left Bochdalek diaphragmatic hernia: a very rare anomaly.

Morgagni hernia association with Bochdalek diaphragmatic hernia is a very rare congenital anomaly. The authors reported a 2-year-and-2-month-old boy with Down syndrome who has a history of recurrent pneumonia over a one-year period. A chest film of the first admission at 6 months of age revealed only minimal pulmonary infiltration and normal findings of both sides of the diaphragm. The last investigations with chest films and CT scan were suggestive of sequestration of the right lung with left Morgagni and left Bochdalek diaphragmatic hernias. An exploratory laparotomy revealed bilateral Morgagni and left Bochdalek hernias with hernial sacs in all of the diaphragmatic defects. All of the hernial sacs were excised and the diaphragmatic defects were closed with 2-0 silk interruptedly. Postoperative course was uneventful and he was doing well during his follow-up at one year.

Successful separation of thoracopagus conjoined twins with a single extra-hepatic biliary system.

A pair of thoracopagus conjoined twins were separated at the age of 3 months at Khon Kaen Regional Hospital, Thailand on November 19, 2004. Pre-operative investigations showed separate hearts, joined duodenum, and fusion of the livers. Separation of the extra-hepatic biliary systems was suspected. Operative findings revealed fusion of the intestines from the second part of the duodenum to the terminal ileum with two normal colons. An intussusception was found at the terminal ileum. Fusion of the livers with only one extra-hepatic biliary system was noted In one of the twins, the gastrointestinal tract was anastomosed with Roux-en- Y enteric loop to one area of good bile drainage at the cut surface of liver Post operative course was hectic but both twins recovered satisfactorily. Both are doing well at present, two years after the separation. This was the first reported case of thoracopagus conjoined twins with complex biliary tract anomalies in Thailand. From the literature, pre-operative investigations in most cases of these conjoined twins failed to define the precise anatomy of the biliary system and may be misleading as in the presented case. The mortality rate remains high. Meticulous pre-operative planning, decision-making in the operative field and postoperative management as well as a multidisciplinary team are very important for a successful separation.

Epigastric heteropagus twins: a report of four cases.

Epigastric heteropagus twins (EHT) is a rare form of conjoined twins. It refers to unequal and asymmetric conjoined twins in which the dependent part (parasite) is attached to the epigastrium of the dominant part (autosite). The authors herein report four cases of EHT. Omphalocele was present in 3 patients. Surgical excision of the parasite and repair of the abdominal wall defect were successful in three cases. Three infants had associated cardiac anomalies and one of them died during surgery from cardiopulmonary failure.

Technology assessment for management of congenital diaphragmatic hernia: immediate versus delayed surgery.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is one of the high-risk diseases in pediatric surgery, especially in neonates with symptom presentation within 6 hours after birth. Opinion regarding the time of surgery has gradually shifted from immediate repair to a policy of stabilization and delayed repair. Whether delayed surgery is beneficial remains controversial. OBJECTIVE: To evaluate the outcomes regarding whether delayed surgical repair improves survival in CDH neonates, who are symptomatic immediately after delivery, is more beneficial than immediate surgery. MATERIAL AND METHOD: Data were obtained by searching MEDLINE (1966-2002) and the Cochrane Database, Issue 2, 2003 using the term "congenital diaphragmatic hernia" and "surgery". Inclusion criteria were randomized controlled trial (RCT), prospective trial (PT), retrospective analysis (RA) and meta-analysis (MA). Information from the literature was analyzed by the computer program of Epi Info Version 3. Statistical significance was reliable at the level of p < 0.05. RESULTS: Twenty-five studies were obtained and RCT:RA was 2:23. Analysis of results of CDH management revealed that pre-operative stabilization and delayed surgery improved the survival rate in 14 of 25 in the literature (p < 0.05), while the remaining 11 articles showed no statistical difference of survival between immediate and delayed surgery (p > 0.05). CONCLUSION: From the evidence-based analysis, the results of CDH management between immediate versus delayed surgery were unclear. From the reviewer's experience at the Queen Sirikit National Institute of Child Health, the strategy of pre-operative stabilization and delayed surgery had better improved survival of CDH than immediate surgery.

Conjoined twins: surgical separation in 11 cases.

Eleven pairs of symmetrically conjoined twins underwent surgical separation at the Queen Sirikit National Institute of Child Health. Six were omphalopagus, 4 were thoracopagus and 1 was pygopagus. Eight were female and 3 were male. Three pairs were separated on emergency or semi-emergency bases, and the remaining 8 pairs were separated electively at an older age. Of the 3 pairs who had early emergency separations, one pair, whose combined birth weight was only 2,500 g, underwent emergency separation at the age of 44 days after the death of one twin. The second twin also expired one hour after the separation. In the remaining 2 pairs, early separation was done because of the deterioration of one twin due to complex cardiac anomalies. In both cases, the infants with cardiac anomalies expired but the others survived the separation satisfactorily. In one pair of thoracopagus conjoined twins, one twin had cyanotic cardiac anomalies. They were electively separated at the age of 2 years and 9 months. The twin with cardiac anomalies expired 2 hours after surgery, but the other survived the separation satisfactorily. In the remaining 7 pairs who underwent elective separations, both twins of each pair survived the separation satisfactorily. However, one twin expired unexpectedly 10 days after the separation.

Tubularized, incised plate urethroplasty in hypospadias repair: experience at Queen Sirikit National Institute of Child Health.

BACKGROUND: Hypospadias is the most common penile anomaly. Many techniques have evolved during the past 150 years to address chordee and construct a neourethra. The current concept of understanding of chordee and emphasis upon preserving the urethral plate have brought hypospadias surgery over the last decade near its ultimate goal of a reconstructed penis that is functional and cosmetically normal. Warren T Snodgrass primarily reported successful urethral plate preservation urethroplasty in 1994. The urethral plate can be primarily tubularized without an additional skin flap after a dorsal midline relaxing incision is made known as tubularized incised plate urethroplasty. It is currently used worldwide by pediatric urologists due to its advantages. The authors also performed this technique and initial experience at Queen Sirikit National Institute of Child Health, Bangkok, Thailand is reported. MATERIAL AND METHOD: 88 of 180 hypospadias boys were treated with tubularized incised plate urethroplasty for primary repair of hypospadias over a 24 month period. All had moderate to severe chordee. Urethral plate preservation is the principle concept of this technique and the entire length of the urethral plate was incised along the midline and the neourethra was tubulized over a 6 or 8 Fr. catheter. The chordee usually disappeared after the penis was degloved. Dorsal plication was performed in mild residual chordee. A subdartos flap was created to cover the neourethra and pressure dressing applied with bactracin gauze for 7 days. The urethral stent was removed on the 7th post-operative day. RESULTS: The tubularized, incised plate urethroplasty was performed in 88 boys, age range from 6 months to 12 years. The operative time was 60 to 100 minutes. Follow-up was up to 18 months. There were 13 distal penile, 60 midshaft and 15 penoscrotal types. All penes had excellent cosmetic appearance, even in complicated cases. The pinhole and large fistulas were noticed in 10 and 3 penes respectively (14%). All fistulas were repaired successfully at a later date. Twenty cases had a mild degree of meatal stenosis of which 18 had good response to self meatal dilatation by their parents and 2 underwent a meatotomy procedure. No urethral diverticulum or stricture was noticed during follow-up. CONCLUSION: Even though high percentages of fistula complications were noticed in the authors' experience, they seemed to be acceptable and easy to correct. This technique seems to be suitable for both distal and proximal hypospadias. The advantages of this technique include its simplicity, low complication rate, very good appearance of the glans penis and normal meatus in most boys. Tubularized incised plate urethroplasty is now the procedure of choice for distal and proximal hypospadias repair.

Splenic lymphangioma and presentation with frequent urination: a case report.

Lymphangioma of the spleen is a rare disorder with a clinical manifestation ranging from incidental findings of an abdominal mass to symptomatic abdominal pain. The authors reported a case of splenic lymphangioma with the presenting symptom of frequent urination in a 9-year-old boy. A large firm mass was found at the left upper quadrant, 12 cm in diameter. The diagnosis was confirmed by ultrasonography and computerized tomographic (CT) scan. The symptoms of frequent urination disappeared after operative splenectomy. No evidence of recurrence occurred over a 3-year follow-up.