Ana Laín, Alberto Parente, Agustín Cañizo, María Fanjul, María antonia García-Casillas, José Antonio Matute, Juan Vázquez

Department of Pediatric Surgery. Thoracic and Airway Surgery Unit, Hospital General Universitario Gregorio Marañón, Madrid, España.

Email: jamatute.hgugm@salud.madrid.org


AIM OF THE STUDY: To determine the risk factors influencing failure of treatment of patients with paediatric subglottic stenosis.


METHODS: The clinical records of 114 patients with subglottic stenosis were retrospectively reviewed. Study variables were: age, gender, type and grade of stenosis according to Cotton’s classification, previous treatment, actual treatment employed, presence or absence of gastro-oesophageal reflux, Down’s syndrome, prematurity, bronchopulmonary dysplasia (BPD), tracheostomy, complications, associated cardiac anomaly, post-op intubation time and outcome. Comparative analysis was done by Chi-Square with continuity correction when necessary and T Test. Data are presented as mean +- standard error of the mean.


RESULTS: Age at treatment was 1.79 +- 0.27 years (0-13.7 years). El 24.56% of the cases were considered failures. No influence was seen in outcome related to age and post-op intubation time (1.92+-0.32 years and 1.73+-0.42 years, p=0.899; 8.80+-0.63 days and 10.16+-1.12 days, p=0.499 respectively). Only two of the classical risk factors (prematurity and BPD) published elsewhere had a clear influence in outcome (14.1%vs.48.57%, p=0.002 and 18.52%vs.40.62%, p=0.027 respectively). Patients with more than one risk factors presented a higher failure rate (15.28% al 40.48%; p=0.0053), reaching a 66.67% failure rate with four risk factors (p=0.008). Although previous laryngeal surgery (21.43%vs.43.75%, p=0.054) and the complication rate (21.78%vs.46.15%, p=0.054) did not reach statistic significance, probably due to small study groups.


CONCLUSIONS: Prematurity and BPD are treatment failure risk factors in patients with subglottic stenosis. Concomitancy with other risk factors statistically non-significant by themselves also increases the risk of failure of the treatment of subglottic stenosis.



Mohamed Jallouli1, Ahmed Hakim2, Abir Znazen3, Zouhir Sahnoun2, Hatem Kallel4, Khaled Zghal2, Adnene Hammami3, Riadh Mhiri1

1-Pediatric surgery, Hedi Chaker. Hospital , Sfax, Tunisia.

2-Departement of Pharmacology,School of Medecine, Sfax, Tunisia

3-Departement of microbiology, Habib Bourguiba Hospital , Sfax, Tunisia

4-Intesif care unit, Habib Bourguiba Hospital, Sfax, Tunisia


Hypothesis : Peritoneal lavage with rifamycin reduce the number of intra peritoneal bacteria and adhesion and improve the outcome of intra abdominal infection (IAI).


Materiel and methods: Experimental IAI was induced in wistar rat. After 24 hours, the animals underwent relaparotomy, peritoneal fluid sample was obtained and lavage of the abdominal cavity was performed. Animals were randomly assigned to 3 groups; lavage with 0.9 % sodium chloride solution (S group), lavage with rifamycin at the dose of 25 mg/kg (R25 group) and lavage with rifamycin at the dose of 12.5 mg/kg (R12.5 group). Mortality was recorded every 8 hours for 7 days. All animals that died had a necropsy. Surviving rats were later sacrificed and also underwent a necropsy. At necropsy, intraperitoneal adhesions were noted and peritoneal fluid sample was obtained for bacterial analysis.


Results: Peritoneal lavage with rifamycin reduces mortality from 50% in the S group to 87.5% and 100% in the R25 group and R12.5 group respectively. Adhesion formation was significantly reduced in the R25 group and R12.5 group compared with the S group(P = 0.01and P< 0.01 respectively). There was a greater reduction in bacterial counts in peritoneal fluid in the R25 group compared with the S group (P = 0.037) but there was no significant difference in the reduction of bacterial count between R25 group and R12.5 group.


Conclusion: These results suggest that peritoneal lavage with rifamycin improve the outcome of IAI.



Katerina Kambouri1, Stefanos Gardikis1, Alexandra Giatromanolaki2, Sotiris Botaitis3, Michalis Pitiakoudis3, Alexandros Polychronidis3, Efthimios Sivridis2, Constantinos Simopoulos2

1-Pediatric Surgery, Democritus University of Thrace, Alexandroupolis, Greece.

2-Pathology,Democritus University of Thrace, Alexandroupolis, Greece.

3-Experimental Surgery,2nd department of Surgery,Democritus University of Thrace, Alexandroupolis, Greece.

Email: sgardik@med.duth.gr


Introduction: The purpose of the present study was to determine whether the exogenous administration of vascular endothelial growth factor (VEGF) influences the angiogenic activity of a free penile graft in urethral reconstruction in an animal model.


Materials and methods: Twenty-two male White New Zealand rabbits were divided into four groups. In the control group (group O, n=4) a simple urethrotomy and closure was performed, whereas a ventral urethral defect was created in groups A, B, and C and then bridged using free penile skin onlay patches: in group A(n=6) without administration of VEGF and in groups B(n=6) and C(n=6) with subfascial injection of 0.5ml VEGF (0.5mg/ml) and 0.5ml VEGF (1mg/ml) respectively at the margins of the flaps. On the 21st postoperative day, the animals were sacrificed and the retrieved implants were subjected to macroscopic and microscopic analysis. The angiogenic activity was assessed with immunohistochemistry, using the anti-CD31 MoAb and the phosphatase antialkaline phosphatase procedure. Statistical analysis was performed using the one-way ANOVA.


Results: The angiogenesis in a magnification of x200 in groups O, A, B, and C was 34.1±4.1 (mean±SD), 61.7±6.4, 67,3±1,9 and 84,5±6,3 vessels per optical field, respectively. There were, statistically significant differences between groups A and C, B and C (p<0.001) but not between groups A and B (p>0.05).


Conclusion: The viability of free penile grafts with injection of VEGF is better than that of a free penile graft without VEGF because of higher angiogenic activity. The angiogenic activity seems to be VEGF dose dependent.



Raphaël Moog, Isabelle Talon, Isabelle Kauffmann, Laure Mefat, Stéphane Grandadam, François Becmeur

CHU Hautepierre, Service of pediatric surgery, Strasbourg, France.

Email: raphael.moog@chru-strasbourg.fr


Introduction: Actually there is no ideal bulking agent to treat endoscopically vesico-renal reflux in children. Many teams try to find a safe and efficient, ideally autologous material. We describe a protocol of use of autologous adipocytes in the treatment of vesico-renal reflux in children.


Patients and methods: We decided to treat children aged from 3 to 15 years with a primitive symptomatic vesico-renal reflux stage I to III. Fat harvesting was obtained from thigh subcutaneous tissue by manual aspiration. Samples were centrifuged to pull the graft from blood and lipid. The last step was fat injection beneath pathologic uretere by a conventional endoscopic STING technique. A voiding cystourethrography (VCUG) closed the procedure. Follow up included a renal ultrasonography the day after surgery, one and three months later. A VCUG was realised at 3 months.


Results: At the end of the procedure, we obtained systematically a very well heightening of the pathologic meat and VCUG were normal in all cases. None presented an obstruction or an infection during the follow up. VCUG was normal in 50% of cases at 3 months and it shown an improvement in 38% of cases. One child had a surgical reimplantation at 4 months.


Conclusion: This is the first step of a study. Those preliminary results bring us to modify the technique in order to improve our results. Our first observation is the feasibility and the safeness of this technique regardless of others synthetic bulking agents which innocuousness is uncertain



Ferdinand Kosch, Katrin Langhammer, Béatrice Ferdilus, Paul Philippe

Pediatric Surgery Department, Centre Hospitalier de Luxembourg, Luxembourg, Luxembourg.

Email: kosch.ferdinand@chl.lu


Congenital urethral fistula is an extremly rare anomaly. It does occure with anorectal malformations or can occure without any other anomalies. In congenital urethral fistula you will not find a cordee and the aspect of the glans is normal and the distal part of the urethra is patent with a normal prepuce.

We present a case of a child with a high anorectal malformation without fistula and an isolated urethrocutaneous fistula. There was a normal straight penis with a normal glans and foreskin. The urethra was open at mid-shaft. An orthotopic meatus was patent, though urination was per the fistula only. The repair of the anorectal malformation was done at the second day of life according to Pena. There were no anomalies of the kidneys. Closure of the congenital urethral fistula was performed at the age of two. We performed a simple closure of the fistula with additional subcutaneus layers, over a 10 Fr stent. The child was treated on a day care basis. No complications occured. The child has normal micturitions 10 months after repair.

The management of a congenital urethral fistula is easy. We discuss the embryology and review of the literature is done.



Mourad Hamzaoui, Manef Gasmi, Sondes Sahli, Afif Essid, Taoufik Houissa

Pediatric surgery "A", Children hospital, Tunis, Tunisia.


Hydatid cyst disease is still a significant problem in endemic countries.The lung and the liver are the most commonly involved organs in pediatric population.Mediastinal localisation represents 0.3 to 3% of all hydatidosis.The authors report on two cases and review the literature.

In a retrspective review of the last ten years, we had two cases of mediastinal localisation out of total 200(1%) intrathoracic hydatid cysts treated in our department.Imaging investigation consisted of chest x-ray and computed tomography.

Two girls aged 12 and 13 years were admitted for history of chest pain.Chest x-ray revealed a left posterior mediastinal opacity of 150 mm in size in the first case and 50 mm in the second.Computed tomography performed in the second case showed a cyst in the middle mediastinum.

Abdominal ultrasound was normal in both cases.By a posterolateral thoracotomy,the cysts were found intact and diagnosis was confirmed macroscopically.The operative field was isolated and protected with scoliocidal agent.Hydatid fluid was aspirated followed by extraction of the hydatid membrane and subtotal excision of the residual cavity.Because of adherence with pericarduim and aorta, a small part of the wall was left in place.There were no complication.Hospital stay was eight days in both.Following postoperative course was unventful without relapse or other organ involvement, respectively during 6 and 12 months.


The authors focus on that hydatid disease should be considrerd in the differential diagnosis of mediastinal cyst mass, especially in endemic areas.Because of its benignity, they emphasize that the ideal treatment must be complete excision of the cyst without any spillage.



Mourad Hamzaoui, Manef Gasmi, Sondes Sahli, Afif Essid, Taoufik Houissa

Pediatric Surgery, Children hospital, Tunis, Tunisia.


Renal abscess is an uncommon lesion in children.It often raises diagnosis problems and can compromise renal prognosis.The authors report on their experience and discuss diagnosis difficulties and therapeutic modalities.

Seven renal abscess were managed in our department during the last 11 years.Their medical records were retrospectively reviewed.Imaging investigation consisted of ultrasonography,computed tomography and magnetic resonance imaging.Patients were treated with antibiotic and in one case surgical exploration was indicated.

There were 5 boys and 2 girls,aged from 4 to 12 years (mean 9 years).The time at diagnosis ranged from 7 days to 1 month.The clinical features comprised fever(7), flank tenderness(6) and flank mass(3).Leukocytosis was present in six cases.Elevated C-reactive protein were observed in all cases.Urine and blood cultures were negative in six cases.Ultrasound and computed tomography confirmed diagnosis.Magnetic resonance imaging was performed in one case.Four patients had a right abscess of 50 mm or less and three had a left abscess of 70 mm in diameter.Percutaneous scan guided aspiration was performed in four cases with antibiotic therapy in all cases.The abscess completely regress after a mean therapeutic of six weeks.In one case,clinical improvement with no resolution of the mass raise the suspicion of renal tumor and leads to surgical exploration.There is no recurrence during 9 months to 10 years follow-up period.

The authors point out the importance of early diagnosis and highlight the efficacity of antibiotic therapy.They suggest that therapeutic duration depends on volume of the abscess.They thought that the extension beyond the renal capsule and surgical drainage seem to decrease therapeutic period.



Mohamed Ben Brahim, Abdellatif Nouri, Mongi Mekki, Mohsen Belghith

Pediatric surgery, Fattouma BOURGUIBA Hospital, Monastir, Tunisia.

Email: mohamed.benbrahim@voila.fr


Multiple echinococcosis (ME) is a severe disease in childhood inaccessible to an initial radical surgical treatment. The aim of this study was is to evaluate the efficacy of Albendazole in ME and to discuss the role of surgery in this pathology.

Eleven patients were included in a prospective study between 1996 and 2004. ME was defined by the presence of 10 or more cysts in the same organ. Albendazole was given as 10 mg/kg daily continuously. Treatment outcome was defined as cure, improvement or failure. Surgery was discussed after 1 year of treatment.

Our patients totalised 296 cysts located essentially on the liver (178 cysts) and the lungs (78 cysts). The other localisations interested the peritoneum, spleen, heart, kidney and soft tissues. With exclusive Albendazole therapy, 57.7% pulmonary cysts and 96% peritoneal cysts were considered as cured. This rate was only 31.5% in hepatic localisation. After surgical therapy, 67.4% of hepatic cysts were cured. No productive biliary fistula was observed. Two patients were operated laparoscpically. The total treatment duration ranged between 1 and 5 years.

Albendazole has proven a strong efficacy in pulmonary and peritoneal localisations. A complementary surgical treatment is often necessary in hepatic localisations. Surgery is facilitated with previous Albenazole therapy.



Alberto Parente1, Aida Huerga2, Agustín Cañizo2, Iciar Marsinyach2, Ana Laín1, Paz Chimenti2, María Fanjul1, Cecilio López-Menchero2, Manuel Sánchez-Luna2, Juan Vázquez1

1-Servicio Cirugía Pediátrica, Hospital Infantil Gregorio Marañón, Madrid, España.

2-Sección Neonatología. Servicio Pediatría. Hospital Infantil Gregorio Marañón. Madrid. España


Introduction: There are some infants with Congenital Diaphragmatic Hernia (CDH) that needs some other therapy when maximal medical therapy fails.


Objectives: The aim of the present study is to analyze and compare those babies with CDH managed with or without ECMO.


Methods: Retrospectively study of all infants with CDH treated in a tertiary center. Standard therapy includes gentle ventilation an high frequency oscillatory ventilation, inhaled nitric oxide therapy, delayed surgery until stabilization. ECMO was indicated if cardio-pulmonary support was needed after failure to respond to medical therapy. Severe lung hipoplasia defined by severe preductal hypoxemia from delivery (<80%) with maximal respiratory support, was an exclusion criteria for ECMO.


Results: From October 1997 until December 2005, 56 CDH infants were treated, 44 /56 without ECMO and 12 with ECMO. 36/44 of the standard therapy group survived (82%) and 8/12 (66%) in the ECMO group were recovered with ECMO and 4/12 (33%) survived to hospital discharge. Mean duration of ECMO was 12,4 days (6-19). Surgical repair was done before ECMO in 3/12 with 1/3 survival , 4/12 during ECMO with 1/4 survival because of severe hemorrhagic complications, and 4/12 after ECMO with 2/4 survival. In 1 patient surgical repair was not done because of bad respiratory prognosis.


Conclusions: ECMO in CDH can recovers 65% of infants with severe cardio-pulmonary failure. This group of patients represents the most severe forms of CDH with a final hospital survival of 33%. Surgical repair during ECMO associates severe hemorrhagic complications. ECMO criteria and management of CDH patients differs to any other respiratory ECMO patients and constitutes a different approach.



Zarko Micovic

Pediatric Surgery Clinic, Podgorica, Yugoslavia.

Email: zarmic@cg.yu


Does a typically located, right iliac abdominal pain, approved by a positive palpatory finding, represents an indication enough to make a decision for the explorative surgery with expectance that an acute appendicitis is going on?

It was done a prospective study of 543 cases of children that were submited to the surgical exploration as suspect for acute appendicitis.The surgery was indicated bacesally by a typically located right iliac abdominal pain that was doubtless palpatory approved, regardless of the fact that the other clinical,lab. and ultrasound parameters were not always in a full accordance with the previous.

In 34 (6,26%) cases of our series a pathological substratum different than acute apendicitis was found, although indicated for the surgery too.In only 18(3.31%)of 543 explorated cases neither macroscopic nor microscopic changes of acute appendicitis were found. In all other cases of our series an acute appendicitis was certainly confirmed.

Only a clinical diagnosis based on a typically located and palpatory approved right iliac abdominal pain concretes an indication for the axplorative surgery expecting an acute appendicitis.

The diagnosis of an acute appendicitis without typically located and palpatory approved abdomional pain, practicaly does not exist.

The surgical epilogue of a such based exploration should not always be an acute appendicitis but appendectomy certainly yes.



Alexandra Weltzien, Martin Schwind, Thomas Huckstadt, Steffen Richter, Felix Schier

Department of Pediatric Surgery, University Medical Centre Mainz, Mainz, Germany.

Email: weltzien@kinderchir.klinik.uni-mainz.de


The association of preduodenal portal vein, annular pancreas, malrotation and biliary atresia is very rare. We report the case of a full term infant with prenatally diagnosed duodenal obstruction. At the time of laparoscopic evaluation, a preduodenal vein, an annular pancreas and a malrotation type I were found. The liver and gallbladder were highly suspicious of biliary atresia, therefore a liver biopsy was taken. After the assessment of the anatomical situation, a laparoscopic duodenoduodenostomy was performed. The postoperative course was uneventful. Currently, the diagnosis of biliary atresia is being established and a laparoscopic Kasai procedure will be carried out shortly, facilitating further liver transplantation. This case confirms the advantages of the laparoscopic approach in complex anomalies in the newborn.



Haitham Dagash, Nick Lansdale, Jenny Walker

Paediatric Surgery, Sheffield Children’s Hospital, Sheffield, United Kingdom.



Intestinal type mucinous borderline tumour (IMBT) of the ovary is by definition a

malignant mucinous tumour with atypical proliferation of the goblet cell-containing epithelium, which is thought to arise from the appendix. We present our experience with one such case.


Case Report:

A 14-year-old girl presented with a 3-month history of ‘fullness’ in her lower abdomen, accompanied by intermittent pain. There was no significant medical history. On examination there was a large, firm, smooth mass arising from the pelvis. An ultrasound revealed a multicystic mass 20 cm x 12 cm, with numerous daughter cysts but no focal solid components or calcification. Blood parameters were all within the normal range including alpha-feto protein (AFP) and beta human chorionic gonadotrophin (hCG) levels. At laparotomy the findings were a large cyst arising form the left ovary and a left oopherectomy was performed. Histology of the tumour revealed this to be a IMBT. In view of the histological diagnosis the decision was then made to remove her appendix, and she underwent a semi-urgent laparoscopic appendicectomy. At laproscopy no focal mucinous deposits were identified in the peritoneal cavity. The appendix was found to be histologically normal.

Four years on she remains well and is under regular review.



The concordant staining pattern for cytokeratin-7 (CK-7) of simultaneous mucinous tumours of the appendix and ovary supports an appendiceal origin for these tumours.

Paediatric surgeons need to be aware of this rare tumour which can behave in a malignant fashion and its association with the gastrointestinal tract.



Pilar Abad1, Carmina Duran2, Begoña Sanvicente1, Ana Perez3, Salvador Rigol1, Pere Obiols1

1-Paediatric surgery, hospital de Sabadell Parc Tauli, Sabadell, España.

2-Radiology UDIAT


Email: 29084mac@comb.es


Introduction: The Waugh’s Syndrome is the association of malrotation and intussusception .


Case Report: A boy 17 months of age arribed at hospital because vomits during three days, dehydration, and acute renal failure.


X-ray : show dilated stomach and loops of bowel with fluid levels.


Ultrasound : dilated stomach and proximal duodenum with anomalous disposition of mesenteric vessel compatible with midgut volvulus and malrotation.

In the operation , a volvulus around the base of the narrowed midgut mesentery ,it is untwiste. The bowel appears congested and dusky, rapidly improves. A Ladd’s procedure was carried out.It didn’t fixed the caecum. On the 3 postoperative day the abdomen was very distended and hypoactive.


Untrasound : didn’t show volvulus , but small bowel is dilated .


Contrast studies show obstruction in distal small bowel.

In the 5th postoperative day , reopened the laparotomy and found an 15 cms intussusception ileo-ileal, at 60 cms ileocecal valve. It is made manual reduction, and pexia caecum-sigma.


Discussion: A case of Waugh’s Syndrome, association of malrotation and intussusception , is being reported. It is suggested that this possibility must be kept in mind and looked for, when a child develops intestinal problems like obstruction, secondary to malrotation, in the post operative period.

We suggest that malrotation by its very nature is associated with a mobile right colon, which may be a prerequisite for intussusception.


Conclusion: Aggressive investigation, including reexploration, should not be delayed if a child has symptoms of prolonged ileus after undergoing a Ladd procedure.



Pilar Abad1, Salvador Rigol1, Cesar Martin2, Montserrat Marti1, Marta Fletas3, Pere Obiols1

1-Paediatric surgery, Hospital de Sabadell Parc Tauli, Sabadell, España.

2-Radiology UDIAT


Email: 29084mac@comb.es


Introduction: Duodenal atresia has being associated with prematurity(46%), maternal polyhydramnios (33%), Down syndrome(24%), annular pancreas(33%), and malrotation in 28%.


Case Report: A girl, prenatal diagnosis of duodenal obstruction with malrotation by magnetic resonance imaging. Before, the ultrasound realised at 26 weeks show a “double-bubble”sign, which persists after birth on a plain abdominal radiograph.

At 24hours of life , at operation, duodenal atresia due to annular pancreas and intestinal , partially volvulated malrotation became apparent. The operation, reduction of the volvulated bowel loops, division of obstructing bands, and creation of a duodenoduodenal, proximal tranverse to distal longitudinal anastomosis.


Discussion: During the fifth and sixth weeks of intra-uterine life the mucosal cells of the duodenum could proliferate an alteration in recanalization or a vascular catastrophe could be responsible of atresia or stenosis duodenal.

After the tenth week of intra-uterine life the intestine returns into the abdominal cavity . This is accompanied by rotation in an anti-clokwise direction around the axis of the superior mesenteric artery. By the end of the 11th to 12th week the total rotation has been completed.

But in the case reported , when took place the alteration ? At fifth weeks, At 12th ? Since 5th until 12th? It was a different injury?



Magnetic resonance imaging provide superb anatomic detail and added diagnostic specificity like in this case.

The malrotation make easy to performe the duodeno-duodenal anastomosis because after the Ladd procedure all the bowel is more free.



Abdellatif Nouri, Mohamed Benbrahim, Oumama Elezzi, Kais Maazoun, Lassaad Sahnoun

Department of peadiatric surgery, CHU of Monastir, Monastir, Tunisia.

Email: nouri.abdellatif@rns.tn


There are only few articles about trans anal Soave for old children and most of them mentioned some difficulties.

From January 2001 to December 2005, 68 primary trans anal Soave were performed for Hirschprung disease confirmed by biopsy. These patients were classified in 2 groups:

·        .Group 1: 50 patients operated before the age of 1 year, with 75% new born

·        .Group 2: 18 patients operated after the age of 1 year , the oldest was 16 years.

The procedure was possible in all cases , but the median operating time was 106 minutes for the group 1 and 207 minutes for the group 2. For the 2 groups, there were no operative incident or sepsis . During the 3 post operative months, the frequency of peri anal skin rash was 60%(group1) and 10%(group2). The anal stenosis needing calibration was noticed only in the group 1 ( 16% ).At the 6th post operative month, there was no anal stenosis, the peri anal skin rash disappeared nearly completely .The follow up ranged from 9 months to 4 years, there was no constipation or anal prolapse , and for patients older than 3 years ,there was no soiling or incontinence .Two patients of the group 1 have presented enterocolitis.

The trans anal Soave is feasible even with adolescent and huge dilation of the colon. It is a safe procedure but recurs in these cases a good colon preparation and a relatively long operating time.



Evangelia Livaditi, Michael Soutis, Areti Panagou, George Christopoulos-Geroulanos

Surgical Department, Aghia Sophia Childrens Hospital, Athens, Greece.

Email: qcz-ms@hol.gr


Primary torsion of the vermiform appendix is an extremely rare entity. Its clinical presentation is similar to acute appendicitis. Diagnosis is usally made at laparotomy. We report a case of appendiceal torsion in an 11year-old boy presenting with acute appendicitis.


Case report: An 11year-old boy was admitted with a two day history of abdominal pain which was initialy colicy and periumblical and later became constant and was localized in the right lower quadrant of the abdomen. There were also vomiting and low grade fever. Physical examination and hematologic tests where similar to acute appendicitis. Abdominal ultrasonography revealed a blind tubular structure 16mm in diameter without peristalsis and decreased blood flow within its wall. With the diagnosis of acute appendicitis, a laparotomy was performed which revealed an engorged, gangrenous vermiform appendix, with normal length, twisted 360° anticlokwise of its base. The mesoappendix was extremely narrow and the appendiceal vessels were parallel and in close proximity to the mesenderic margin of the appendix. A routine appendicectomy was performed after detorsion. The postoperative course was uneventfull. Histological examination of the appendix demonstrated ischemic necrosis of the appendiceal wall. There were no other predisposing factors such as lipoma, mucocele or fecalith.


Comment: Torsion of the vermiform appendix is extremely rare. Allthough clinical presentation is identical to acute appendicitis, ultrasonographic findings may lead to suspicion of this entity.



Michael Soutis

Surgical Department, Aghia Sophia Childrens Hospital, Athens, Greece.

Email: qcz-ms@hol.gr


Primary closure of a giant omphalocele remains a challenge for the pediatric surgeon. As a rule it is not feasible and usually staged procedures are performed with unsatisfactory cosmetic results. We present an 8 hour old female with a giant omphalocele in which primary full thickness closure of the defect was possible after oral and rectal administration of gastrografin solution.


Case report: A female neonate with a giant omphalocele was addmited in our department.The abdominal wall defect was 10cm in diameter, containing the entire left lobe of the liver and part of the right one, the spleen and intestinal loops. Surgical correction was preceded, while already under general anesthesia, by oral and rectal administration of gastrografin solution 1:1 with normal saline. Decompression of the gastrointestinal tract helped reduce the contents. In addition, the abdominal wall was manually stretched and full thickness defect closure became feasible. Postoperatively, paralysis and ventilator support for 3 days, eliminated the risk of caval compression. The postoperative course was uneventful and the patient was discharged from the hospital on the 20th day.


Comment: Intraoperative enteric gastrografin solution in combination with abdominal wall stretching and postoperative paralysis with ventilator support, enhances the possibility of primary closure of a giant omphalocele, contributing significantly to a successful outcome.



Shant Shekherdimian

General Surgery, Kaiser Permanente Medical Center, Los Angeles, CA.


The majority of babies with gastroschisis have a defect in the abdominal wall occuring to the right of the umbilicus. Furthermore, 10-20% have an associated intestinal atresia. Other anomalies, however, are extremely rare. We report a case of a female born with a left-sided gastroschisis (LSG). Upon inspection at birth, the exposed intestine was matted together, however on close examination a jejunal and transverse colonic atresia were noted. The bowel was placed back into the abdomen and a jejunostomy was created. The patient was taken back to the OR one month later and intestinal continuity was established via ostomy take-down and transverse-colonic resection. Post-operatively, she was able to tolerate enteral feeds.

Upon review of all reported cases of LSG, differences between left and right-sided gastroschisis(RSG) were noted. Whereas no known predisposition to gender exists in RSG, 83% of LSG’s occurred in females. Furthermore, extra-intestinal anomalies were recognized in approximately one-third of babies with LSG.

Conclusion: Significant differences exist between right and left-sided gastroschisis. These differences may suggest that LSG’s are etiologically a separate entity from the more common RSG, and as such arise from embryologic derangements that are unique from those responsible for RSG’s. Given the propensity for LSG’s to develop in females, differences in abdominal embryology between the sexes may have an etiologic role in its pathogenesis. Additionally, although the general management of babies with an LSG remains the same, the presence of an LSG should prompt a thorough evaluation for coexisting anomalies given their higher incidence in this population.



Javier Bueno, Jose Luis Peiró, Begoña Sánchez, Jose Lloret, Vicente Martinez-Ibañez

Pediatric Surgery, Hospital Valle de Hebron, Barcelona, España.

Email: jbueno@vhebron.net


The STEP is an alternative bowel lengthening operation to Bianchi’s procedure in short gut (SGS) with dilated intestine The result is a zig-zag shaped channel of bowel longer and narrower than the original dilated loop. The first clinical report data of 2003 and few cases have been described.


Case report: 11-year-old female (height/weight 22kg/123cm) with Hirschsprung’s disease and SGS on home partial parenteral nutrition. At 1 year of age she underwent the Lester-Martin operation. Since then, she had multiple hospital admissions due to electrolytes disturbances and dehydrations. Her intestinal transit was 1 ½ hour. The length from duodenum until the jejuno-colic loop of Lester-Martin was 120 cm. A total of 70 cm of jejunum were dilated (diameter >25 cm in the distal area). The STEP was performed in those 70 cm with endoscopic GIA stapler of 35 and 45 mm (19 shots). Thel firings were performed transversally to the intestine leaving a 4 cm bowel channel. The dilated segment was lengthened from 70 cm to 170 cm. With the distal jejunum an stoma was created and the jejunocolic Lester-Martin loop was closed. The postoperative course was uneventful. The oral diet was resumed at day 10. One year later her height/weight of 27kg/135cm later and the stomal output is solid. No electrolytes disorders occurred.


Conclusion: The STEP was an easy and effective operation. This is the first case of this technique in complicated Hisrschsprung’s disease with SGS. The distal lengthened intestine can be converted as stoma.



Javier Bueno, Alfredo Escartín, Begoña Sánchez, Joaquín Balsells, Gloria Royo, Vicente Martinez-Ibañez, Carlos Margarit

Pediatric Surgery and Liver Transplantation, Hospital Valle de Hebron, Barcelona, España.

Email: jbueno@vhebron.net


Our center, pionnered pediatric liver transplantation (LTX) in Spain (June 1985). Since then more than 200 LTX have been performed.


AIM: To evaluate the current status of our LTX recipients with >10 years of f/u.


MATERIAL AND METHODS: 52 patients who underwent LTX have >10 years of f/u. Mean age at LTX was 5.6 years and 25% had weight <10 kg. LTX indication was biliary atresia in 60%. All but one (tacrolimus) received cyclosporine as principal immunosuppressant. Technical complications were:3 arterial thrombosis (days 2,17 and 3 years), 2 portal vein thrombosis (days 2,30), and 14 biliary complications.


RESULTS: No patient loss ocurred in patients with f/u longer than 10 years. 22 patients have f/u >15 years and 3 >20 years. The current mean age is 20,5 years (range:10,3–33,4). The incidence of acute rejection was 51%, chronic rejection 19% and lymphoproliferative disorders 8%. Seven patients required retransplant (mean of 4,2 years (range: 0,05-11 years) of the first LTX due to: chronic rejection (n=4), arterial thrombosis (n=2) and after interventional radiology procedure (n=1). After 10 years of f/u 1 patient developed portal vein thrombosis (15 years), and 4 biliary strictures. All patients remain on immunosupression. In 18 patients cyclosporine was switched to tacrolimus (n=16) or rapamycin (n=2). One patient developed acute rejection at 19,2 years of f/u. Three recipients had offspring.


CONCLUSIONS: LTX is an effective treatment also in long term f/u with an excellent quality of life. After 10 years of f/u, biliary stricture is the most frequent complication.



Maria Fernanda Roll1, Markus Bettendorf2

1-Pediatric Surgery , University Hospital Heidelberg, Heidelberg, Germany

2-Pediatric Departament, University Hospital Heidelberg, Heidelberg

Email: maria_roll@med.uni-heidelberg.de


45,X0/46,XY mosaicism is associated with a broad spectrum of phenotypes such as Turner Syndrome, mixed gonadal dysgenesis, male pseudohermaphroditism and apparently normal male. Gender assignment and early management still remain difficult aspects in the treatment of these patients. We report on a 3 month old baby with 45,X0 /46,XY mosaicism, who was assigned female gender before referral to us. Phenotypically the child showed ambiguous genitalia with poor signs of virilization including left sided scrotal testis and severe hypospadia. Sonographic examination suggested the presence of a uterus supporting the female gender assignment. Unfortunately the patient underwent bilateral inguinal hernial surgery before completing diagnosis. Consequently the gonad on the left side was placed intra-abdominally.

After a new and thorough evaluation including clinical, radiological, cytogenetic, endocrinologic and molecular biological findings, we decided to reassign gender to male. The parents received extensive psychological support concerning the condition of their child. Nevertheless this was a very traumatic step for them. Explorative laparoscopy confirmed the presence of mixed gonadal dysgenesis. The streak gonad, fallopian tube and hypoplastic uterus on the right side were removed. The left sided cryptorchic testis was exteriorised to the scrotum. Gonadal biopsies were taken. Histologic findings confirmed the presence of mullerian structures and a streak gonad and normal testicular tissue. Hypospadia repair was planed in a separate surgery.

Although female gender assignment is usually preferred, male assignment is a justifiable alternative in instances of virilization and descended testis. Early diagnosis, proper management of the gonadal tissue and appropriate reconstructive genitoplasty are therefore vital management issues.