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International Abstracts 449 umbilical incision. The authors compared aspects of bowel recovery between an extracorporal (ECP) and an intracorporal pyloromyotomy (ICP) via supraumbilical incision. Between 1994 and 1997, 29 patients were treated with extracorporal pyloromyotomy, whereas 75 patients had an intracorporal myotomy between 1995 and 2002. The technique was chosen by the surgeon. Medical charts were retrospectively reviewed for alkalosis, muscle thickness, operating time, time to return to full feeding without vomiting, any complications and outcome. Diagnosis was made by ultrasonography. After supraumbilical skin incision, the abdomen was entered via a longitudinal incision of the linea alba. Pylorus was delivered manually for extracorporal myotomy or exposed with three pairs of traction sutures for the intracorporal approach. Postoperative feeding began with 20 ml of regular formula the next morning and was increased by 20 ml at every other feeding as tolerated. Patients received an iv cephalosporin for 3 days postoperatively. Preoperative conditions were similar. Average operation time in the ICP group was significantly longer (56.4 F 13.6 min vs. 48.7 F 16.3 min), but the average time to return to full feeding without vomiting (2.1 F 1.6 days vs. 2.9 F 1.9 days) was significantly shorter. Extension of skin and/or fascia was necessary in 3% in ICP compared with 38% of ECP. One ICP patient had a mucosal perforation. Wound infections occurred in 5.3% of ICP and 6.9% of ECP (not significant). The intracorporal pyloromyotomy was initially described for a large un- deliverable pylorus. The authors recommend this technique to further reduce skin incision, to minimize the force to deliver the pylorus, to improve the cosmetic outcome and to shorten recovery in all pyloromytomies regardless of the size of the pylorus. Economic standpoints make this procedure prefe- rable to laparoscopy. The increase in operation time seems to be negligible. No explanation was given for the prophylactic use of antibiotics. Peter Schmittenbecher Diaphragmatic duodenal atresia: Laparoscopic repair Steyaert H, Valla JS, Van Hoorde E. Eur J Pediatr Surg 2003 (December);13:414-416. Stenosis due to a diaphragm is a type of intrinsic duodenal obstruction in newborns and even childhood when obstruction is partial. The author present a case of a 13-month-old girl with diaphragmatic stenosis associated with dilatation of the prestenotic duodenum. Surgical management consisted of partial excision of the diaphragm after vertical incision of the anterior part of the duodenum, followed by transverse suture. This diamond-shaped anastomosis was successfully carried out laparoscopically. No tapering of the duodenum was performed as is suggested by some authors in cases of megaduodenum. The rapid resumption of peristalsis and fewer adhesions after such a minimally invasive procedure could render more invasive approaches unnecessary. —Thomas A. Angerpointner Familial syndromic duodenal atresia: Feingold syndrome Holder-Espinasse M, Ahmad Z, Hamill J, et al. Eur J Pediatr Surg 2004 (April);14:112-116. Familial duodenal atresia appears as part of Feingold syndrome. Other features of this variable autosomal dominant condition include tracheo- esophageal fistula and esophageal atresia, microcephaly, hand and foot anomalies, facial dysmorphism, and developmental delay. The authors report a father and two sons with Feingold syndrome. One has bi- lateral dysplastic kidneys, which have not been reported previously. — Thomas A. Angerpointner Duodenal atresia and gastric antral web. A significant lesion to learn Ferguson C, Morabito A, Bianchi A. Eur J Pediatr Surg 2004 (April);14:120-122. The authors report a case of a child who had a duodenal atresia and also a gastric-antral web. The two conditions were not known to be associated. The child had a duodenoduodenostomy for the duodenal atresia that had been diagnosed as bdouble-bubbleQ appearance on antenatal ultrasound. She eventually had a second laparotomy due to persistent gastric outlet obstruction where a pyloroplasty was performed and an antral web excised. Post-operative course was uneventful. This case confirms, in the context of congenital anomalies, the absolute necessity to consider other possible lesions, and particularly those lying proximal to a bowel anastomosis. — Thomas A. Angerpointner TAR syndrome with annular pancreas and anal atresia — A case report Karaman I, Karajan A, O ¨ zalevli SS, et al. Eur J Pediatr Surg 2004 (April);14:123-125. TAR syndrome (thrombocytopenia with absent radii) is manifested by the presence of hypomegakaryocytic thrombocytopenia and bilateral absence of the radii. An 8-day-old female newborn was admitted with diagnosis of TAR syndrome and symptoms of intestinal obstruction. She also had anal atresia with a rectovestibular fistula. Upper gastrointestinal contrast study revealed partial duodenal obstruction, whereupon a side-to-side duodeno- duodenostomy was performed. A small number of gastrointestinal malformations associated with TAR syndrome has been described. An association with annular pancreas and anal atresia, however, has not been reported previously.—Thomas A. Angerpointner The Effect of antenatal diagnosis on the management of small bowel atresia Basu R, Burge DM. Pediatr Surg Int 2004 (March);20:177-179. Antenatal ultrasound discovery of small bowel atresia (SBA) is possible in up to 50% of cases. Prenatal diagnosis allows counselling of parents and permits planned delivery near or at an adequate equipped neonatal and pediatric surgical center. The study asked for prognostic information usable for counselling and postnatal management. Duodenal and complicated jejunal or ileal atresias (meconium ileus, gastroschisis) were excluded. Thirty-nine neonates within 15 years were born with uncomplicated small bowel atresia. Twelve children (31%) were antenatally diagnosed (AND) on the basis of polyhydramnios and dilated bowel loops between the 20 th and 36 th gestational week, and 27 (69%) were not (postnatal diagnosis, PND). The AND group included 7/19 jejunal and 5/20 ileal atresias. Gestational age at birth was the same, but birth weight was lower in AND. AND patients were operated more quickly, required parenteral nutrition in 83%, had a significantly longer time of parenteral nutrition and a longer stay in hospital. PND children were most frequently diagnosed one or two days after birth following bilious vomiting, operated later, needed parenteral nutrition in 44% for a shorter time and were discharged earlier. Jejunal atresias required more often (74% vs. 40%) and longer parenteral nutrition than ileal atresias. Antenatal diagnosis of small bowel atresia pointed out lower birth weight, frequent and longer parenteral nutrition and longer hospital stay with a slight accentuation on jejunum atresias. These aspects are useful in counselling the parents. The number of PND children was low, but the positive predictive value of ultrasound for gut anomalies is even low and the expertise in the examination is limited in a wide geographic region. Additionally, late ultrasound detects more gut anomalies, but many women underwent only a single and early scan. If small bowel atresia is detected, the distension of the prestenotic segment is more pronounced and this may explain the longer period of postoperative gut dysfunction.—Peter Schmittenbecher Efficacy of real-time monitoring to determine motility in porcine small intestinal transplantation Nishimoto Y, Taguchi T, Masumoto K, et al. Pediatr Surg Int 2004 (January);20:9-13. To improve graft survival in small bowel transplantation, early detection of rejection is a key point. Because mucosal biopsy is unable for real-time

Familial syndromic duodenal atresia: Feingold syndrome

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Page 1: Familial syndromic duodenal atresia: Feingold syndrome

International Abstracts 449

umbilical incision. The authors compared aspects of bowel recovery

between an extracorporal (ECP) and an intracorporal pyloromyotomy

(ICP) via supraumbilical incision.

Between 1994 and 1997, 29 patients were treated with extracorporal

pyloromyotomy, whereas 75 patients had an intracorporal myotomy

between 1995 and 2002. The technique was chosen by the surgeon.

Medical charts were retrospectively reviewed for alkalosis, muscle

thickness, operating time, time to return to full feeding without vomiting,

any complications and outcome.

Diagnosis was made by ultrasonography. After supraumbilical skin

incision, the abdomen was entered via a longitudinal incision of the linea

alba. Pylorus was delivered manually for extracorporal myotomy or

exposed with three pairs of traction sutures for the intracorporal approach.

Postoperative feeding began with 20 ml of regular formula the next

morning and was increased by 20 ml at every other feeding as tolerated.

Patients received an iv cephalosporin for 3 days postoperatively.

Preoperative conditions were similar. Average operation time in the ICP

group was significantly longer (56.4F 13.6min vs. 48.7F 16.3min), but the

average time to return to full feeding without vomiting (2.1 F 1.6 days vs.

2.9F 1.9 days) was significantly shorter. Extension of skin and/or fascia was

necessary in 3% in ICP compared with 38% of ECP. One ICP patient had a

mucosal perforation. Wound infections occurred in 5.3% of ICP and 6.9% of

ECP (not significant).

The intracorporal pyloromyotomy was initially described for a large un-

deliverable pylorus. The authors recommend this technique to further reduce

skin incision, to minimize the force to deliver the pylorus, to improve the

cosmetic outcome and to shorten recovery in all pyloromytomies regardless

of the size of the pylorus. Economic standpoints make this procedure prefe-

rable to laparoscopy. The increase in operation time seems to be negligible.

No explanation was given for the prophylactic use of antibiotics.

—Peter Schmittenbecher

Diaphragmatic duodenal atresia: Laparoscopic repairSteyaert H, Valla JS, Van Hoorde E. Eur J Pediatr Surg 2003

(December);13:414-416.

Stenosis due to a diaphragm is a type of intrinsic duodenal obstruction in

newborns and even childhood when obstruction is partial. The author

present a case of a 13-month-old girl with diaphragmatic stenosis associated

with dilatation of the prestenotic duodenum. Surgical management

consisted of partial excision of the diaphragm after vertical incision of

the anterior part of the duodenum, followed by transverse suture. This

diamond-shaped anastomosis was successfully carried out laparoscopically.

No tapering of the duodenum was performed as is suggested by some

authors in cases of megaduodenum. The rapid resumption of peristalsis and

fewer adhesions after such a minimally invasive procedure could render

more invasive approaches unnecessary.—Thomas A. Angerpointner

Familial syndromic duodenal atresia: Feingold syndromeHolder-Espinasse M, Ahmad Z, Hamill J, et al. Eur J Pediatr Surg 2004

(April);14:112-116.

Familial duodenal atresia appears as part of Feingold syndrome. Other

features of this variable autosomal dominant condition include tracheo-

esophageal fistula and esophageal atresia, microcephaly, hand and foot

anomalies, facial dysmorphism, and developmental delay. The authors

report a father and two sons with Feingold syndrome. One has bi-

lateral dysplastic kidneys, which have not been reported previously.—

Thomas A. Angerpointner

Duodenal atresia and gastric antral web.A significant lesion to learnFerguson C, Morabito A, Bianchi A. Eur J Pediatr Surg 2004

(April);14:120-122.

The authors report a case of a child who had a duodenal atresia and also a

gastric-antral web. The two conditions were not known to be associated.

The child had a duodenoduodenostomy for the duodenal atresia that had

been diagnosed as bdouble-bubbleQ appearance on antenatal ultrasound. Sheeventually had a second laparotomy due to persistent gastric outlet

obstruction where a pyloroplasty was performed and an antral web excised.

Post-operative course was uneventful. This case confirms, in the context of

congenital anomalies, the absolute necessity to consider other possible

lesions, and particularly those lying proximal to a bowel anastomosis.—

Thomas A. Angerpointner

TAR syndrome with annular pancreas and anal atresia—A case reportKaraman I, Karajan A, Ozalevli SS, et al. Eur J Pediatr Surg 2004

(April);14:123-125.

TAR syndrome (thrombocytopenia with absent radii) is manifested by the

presence of hypomegakaryocytic thrombocytopenia and bilateral absence

of the radii. An 8-day-old female newborn was admitted with diagnosis of

TAR syndrome and symptoms of intestinal obstruction. She also had anal

atresia with a rectovestibular fistula. Upper gastrointestinal contrast study

revealed partial duodenal obstruction, whereupon a side-to-side duodeno-

duodenostomy was performed. A small number of gastrointestinal

malformations associated with TAR syndrome has been described. An

association with annular pancreas and anal atresia, however, has not been

reported previously.—Thomas A. Angerpointner

The Effect of antenatal diagnosis on the management ofsmall bowel atresiaBasu R, Burge DM. Pediatr Surg Int 2004 (March);20:177-179.

Antenatal ultrasound discovery of small bowel atresia (SBA) is possible in

up to 50% of cases. Prenatal diagnosis allows counselling of parents and

permits planned delivery near or at an adequate equipped neonatal and

pediatric surgical center. The study asked for prognostic information usable

for counselling and postnatal management.

Duodenal and complicated jejunal or ileal atresias (meconium ileus,

gastroschisis) were excluded. Thirty-nine neonates within 15 years were

born with uncomplicated small bowel atresia. Twelve children (31%) were

antenatally diagnosed (AND) on the basis of polyhydramnios and dilated

bowel loops between the 20th and 36th gestational week, and 27 (69%) were

not (postnatal diagnosis, PND). The AND group included 7/19 jejunal and

5/20 ileal atresias. Gestational age at birth was the same, but birth weight

was lower in AND. AND patients were operated more quickly, required

parenteral nutrition in 83%, had a significantly longer time of parenteral

nutrition and a longer stay in hospital. PND children were most frequently

diagnosed one or two days after birth following bilious vomiting, operated

later, needed parenteral nutrition in 44% for a shorter time and were

discharged earlier. Jejunal atresias required more often (74% vs. 40%) and

longer parenteral nutrition than ileal atresias.

Antenatal diagnosis of small bowel atresia pointed out lower birth weight,

frequent and longer parenteral nutrition and longer hospital stay with a slight

accentuation on jejunum atresias. These aspects are useful in counselling the

parents. The number of PND children was low, but the positive predictive

value of ultrasound for gut anomalies is even low and the expertise in the

examination is limited in a wide geographic region. Additionally, late

ultrasound detects more gut anomalies, but many women underwent only a

single and early scan. If small bowel atresia is detected, the distension of the

prestenotic segment is more pronounced and this may explain the longer

period of postoperative gut dysfunction.—Peter Schmittenbecher

Efficacy of real-time monitoring to determine motilityin porcine small intestinal transplantationNishimoto Y, Taguchi T, Masumoto K, et al. Pediatr Surg Int 2004

(January);20:9-13.

To improve graft survival in small bowel transplantation, early detection

of rejection is a key point. Because mucosal biopsy is unable for real-time