Kurzfassungen zum 9. Internationalen Donausymposium für Kinderchirurgie 21.-23.10.2005

Endorectal pull-through for rectosigmoidal aganglionosis

M-A. Ardelean, B. Detlefsen, T. Boemers, J. Bauer
Universitätsklinik für Kinderchirurgie, Paracelsus Medizinische Universität

Introduction
In 1988 De la Torre-Mondragon and Ortega-Salgado reported the one-stage endorectal pull-through (ERPT) procedure without the use of a defunctioning colostomy.
The mucosectomy, colectomy, and pull-through are performed transanally, and neither laparotomy nor laparoscopy are required. Over the next years, one-stage operations became increasingly popular and proof to be the best method for young infants with rectosigmoidal aganglionosis (RSA): about 85% of patients have this form of Hirschsprung disease.

Material and Methods
From 01/01 to 01/03, 8 children (3 female) with RSA underwent an ERPT procedure. Mean age at surgery was 6 months (range 2 - 10 months). The mean length of the resected bowel was 19 cm (range 12 - 26/30 cm). One patient (pt) had additional laparotomy. An other pt having an incomplete resection (false intraoperative histology), needed a redo (also transanlly) 3 weeks later. The enteral nutrition started on the 2nd or 3rd postoperative day. 7 children were discharged 5 - 7 days postoperative, one pt (primary incomplete resection) was discharged 4 weeks after the 1st- 6 days after the 2nd-operation. The bouginage of the anastomosis (started on 10th postoperative day) up to 1 month was done in 3 pts.

Results
Mean follow-up was 36 months (30 - 54 mo). All pts are continent. All pts have very acceptable stooling pattern: 1 - 3 defecations/day. No postoperative enterocolitis was observed.

Conclusions
ERPT is the best procedure for patients with RSA who are in good physical condition (no severe enterocolitis, perforation, malnutrition or massively dilated proximal bowel). ERPT avoids the morbidity of the stomas, gives better functional results, shortens the postoperative need for medication and the duration of hospitalization.

Treatment of faecal incontinence in patients operated on anorectal malformations

M-A. Ardelean, T.M. Boemers, C. Schimke, B. Ludwikowski
Kinderchirurgie, LKH Salzburg

The aim of the study is to assess the pre- and postoperative continence in patients (pts) with anorectal malformations (ARM) reoperated in our department.

Methods
From 1990 to 2002, 40 children (25 boys) and one adult with previously ARM repairs underwent surgery: 39 referred and 2 own pts. Initially anomaly was low in 9 pts (8 boys) and intermediate and high in 32 pts (18 boys). There were 3 pts with cloacal exstrophy (2 boys) and 10 girls with cloacal malformation. On examination 39 pts had incontinence and 2 complete stenosis of the neoanus and colostomy. Surgical therapy consisted on posterior sagittal anorectoplasty (PSARP) in 22 pts, posterior sagittal anorectovaginourethroplasty in 4, PSARP and total urogenital mobilisation in 3, PSARP and antegrade continent enema (ACE) in 2 pts, posterior sagittal anorectovaginoplasty in 1, ACE in 6, sigmoid resection in 1, anaoplasty in 1, definitive colostomy in 1 pt.

Results
All pts have follow-up examinations up to 14 years postoperative: 18 are continent without constipation. Social continent are 22: they need antegrade or retrograde enemas, diet or medications to be clean. Some of these pts had occasionally staining. Thus, 40 pts improved their condition. The remaining pt had definitive colostomy.

Conclusion
Surgery is a good alternative to improve the condition of most pts with incontinence previously operated on ARM.
PSARP should be used as secondary procedure, except for pts with correct placed rectum and without stenosis. In these last pts ACE, sigmoid resection or anoplasty may improve the continence.

Rotundum psoas hitch: a new method for colpohysteropexy in girls with bladder exstrophy

M-A. Ardelean, T.M. Boemers, C.M. Schimke, B. Ludwikowski
Universiätsklinik für Kinderchirurgie, Paracelsus Medizinische Universität, SALK, Salzburg, Austria

PURPOSE
Uterine prolapse is a frequent problem in females with bladder exstrophy. The cause is a defective pelvic floor anatomy with lack of support of the pelvic organs. As the incidence of procidentia is high in this group, prophylactic fixation is advocated, also because many children with exstrophy undergo continent reconstruction, making later repair of prolapse difficult. We describe a new method for hysteropexy.

MATERIAL AND METHODS
From 01/00 to 01/05, 9 girls with bladder exstrophy underwent a 'Rotundum Psoas Hitch' (RPH) procedure. In 6 hysteropexy was prophylactic and combined with continent reconstruction of the lower urinary tract. In 3 girls who had uterine prolapse, hysteropexy was therapeutic: in 2 of these girls, RPH was done without additional abdominal procedure, whereas the other girl also had continent urinary reconstruction.
The operative technique consists in detaching both round ligaments from the inner inguinal ring. Both ligaments are positioned cranially, pulling the uterus upward. They are attached to the right and left psoas muscle by pulling them underneath the tendinous aspect of the psoas muscle and fixing them with non absorbable sutures to the tendon and musculature. This leads to posterior and cranial fixation of the uterus.

RESULTS
Mean age at operation was 9.6 years (4 - 20 yrs) and mean follow-up was 27 months (6 - 52 mo). No complications due to hysteropexy occurred. One girl, had back pain for 2 weeks postoperatively. All except 2 girls had either a normal vaginal introitus or had undergone introitoplasty. In no case uterine prolapse developed during the follow-up period. 3 girls who had prophylactic rotundum-psoas-hitch reached puberty during the follow-up period. In all 3 cases of therapeutic hysteropexy, uterine prolapse was cured.

CONCLUSIONS
We describe a new method for prophylactic and therapeutic hysteropexy in girls with exstrophy: the rotundum-psoas-hitch. It is a simple procedure during urological operations, free of complications. We recommend prophylactic uterine fixation in exstrophy girls, especially in combination with reconstructive urological procedures.

Gastroschisis associated with urinary bladder evisceration

Jan Bauer
Univ.-Klinik für Kinderchirurgie PMU, SALK, Salzburg

Introduction
Gastroschisis associated with urinary bladder evisceration is very seldom and may be complicated by the development of the hydronephrosis.

Material and methods
We report here a case of a prenatally diagnosed simple gastroschisis. The child was delivered by a Cesarean section in the 35.gestational week. The immediate postnatal ultrasound examination showed a moderate hydronephrosis of the right kidney. Intraoperatively we found the evisceration of almost the whole intestine and, additionally an evisceration of the urinary bladder. After the transurethral drainage and the oversewing of a nearly perforated area of the bladder wall we succeeded in primary closure of the abdominal wall defect.

Results
The postoperative course was uneventful, 48 hours after the operation no more hydronephrosis was evident. A pre-discarge voiding cystography showed no reflux nor diverticulum or persistent urachus.

Discussion
Gastroschisis associated with urinary bladder evisceration is very rare. If prenatally diagnosed together with rapidly developing hydronephrosis, the timing of delivery shoulg be determined by the degree of the hydronephrosis and of the bowel dilatation.

Management of giant omphalocele: using the biological silo

1S. Berger, 2M. Göppl, 1Z. Zachariou
1Dept. Surgical Pediatrics, Inselspital, University of Berne, Switzerland
2Dept. Pediatric Surgery, University of Heidelberg, Germany

Introduction
Closure of the abdominal wall in large omphaloceles remains challenging due to the volume mismatch between the giant omphalocele and a small abdominal cavity or due to additional malformations that forbid a straightforward surgical procedure. The well known complications of silastic silo techniques in mind we used a stepwise approach with a conservative period of suspension and external compression of the omphalocele sac followed by delayed surgical closure.

Material and methods
From 1998-2004, 9 neonates with omphalocele were treated by secondary surgical closure after primary conservative external compression of the intact omphalocele sac. The omphalocele was covered by sterile moist swabs, suspended by the umbilical cord and gentle circular compression was applied by elastic bandages. Most children breathed spontaneously during this period, received no antibiotics but minimal enteral feeding and daily sterile dressing changes with increasing compression applied.

Results
2 infants with medium sized omphaloceles were operated after 2 and 3 days of suspension with complete closure. In 2 infants with large (>5 cm

Conclusions
External compression of the intact omphalocele and delayed surgical closure was successfully employed in a group of infants that either could not receive immediate closure or showed giant omphaloceles that would otherwise have required a silastic silo procedure. If after 5 days no significant daily improvement is observed, an attempt of closure or decision for creating a silo should be made. In most cases however, this method avoids the use of a silo and its possible complications.

Fetal magnetic resonance imaging of congenital diaphragmatic hernias

Brugger PC, Kasprian G, Tonnhofer U, Hafner E, Horcher E, Prayer D
Medical University of Vienna, Danube Hospital Vienna

abstract
Purpose Congenital diaphragmatic hernias (CDH) are a major concern to both prenatal diagnostics and pediatric surgeons, particularly with regard to the more recent possibilities of in-utero surgery. Aim of the present study was to evaluate the use of fetal magnetic resonance imaging (MRI) in assessing these conditions.

Methods and Materials
31 fetuses (19-39 gestational weeks (GW)) with ultrasound diagnosis of suspected CDH underwent 1-3 fetal MRI studies (total: 47) on a 1.5 Tesla magnet using a five-element phased-array surface coil and no sedation. Imaging protocols included T2-, T1-weighted and balanced-angiography sequences in different section planes.

Results
Fetal MRI identified three right-sided and 28 left-sided CDHs. In 24/28 left-sided CDHs the herniated content included stomach, small intestines and colon except for the descending and sigmoid, 4 fetuses had an intraabdominal stomach. The topography of the meconium filled intestines could be demonstrated with T1-weighted colonography. The extent of an intrathoracic left hepatic lobe (16/28) situated on the ventral thoracic wall was variable and associated with more dorsally positioned stomach. The ipsilateral lung was recognizable in 14/28 cases. Contralateral lung size corresponded to the mediastinal shift, which was more pronounced in cases with intrathoracic liver and associated with left heart compression. CDHs with a hernial sac could be identified by their morphology. In those fetuses with two studies increase in lung volume could be demonstrated in all survivors. All fetuses with suspected hernial sac by fetal MRI survived.

Conclusion
Detailed morphological description is possible with fetal MRI, which may have a bearing on prognosis as MRI can distinguish between CDHs with and without hernial sac. Fetuses with an unfavourable outcome can be identified as possible candidates for in-utero tracheal occlusion as early as 20 GW.

Can levels of interleukins and matrix metalloproteinases in the amniotic fluid predict postnatal bowel function in fetuses with gastroschisis ?

G. Fasching, M. Haeusler, J. Mayr , G. Schimpl, J. Haas, P. Puerstner
Dept of Pediatric Surgery, Klagenfurt, Dept of Obstetrics and Gynecology and Dept of Pediatric Surgery, Medical University of Graz

Purpose
To study whether interleukin or matrix metalloproteinase (MMP) levels in the amniotic fluid can predict the postnatal condition of the bowel in fetuses with gastroschisis.

Methods
Eleven pregnant women with a fetus with gastroschisis underwent amniocentesis. Levels of interleukin-1, interleukin-1 and interleukin-6 were measured in 10/11 specimens and MMP-1, MMP-3 and MMP-8 levels were measured in 7/11 specimens. Neonates who tolerated complete oral feeding within 21 days were considered to have good bowel status (GS/good: n = 4, 4 in IL study, or 3 in MMP study, respectively); those who did not were considered to have poor bowel status (Gs/poor: n =7, 6 in IL study or 4 in MMP study, respectively). Seventy-eight women undergoing amniocentesis for other reasons served as controls.

Results
Interleukin-1 levels were considerably lower in the pregnancies complicated by GS than in controls. MMP-3 and MMP-8 levels were significantly higher in the GS pregnancies than in controls. Neonates with poor bowel status had higher MMP-3 and MMP-8 levels than those with good bowel status.

Conclusion: Amniotic fluid matrix metalloproteinase-3 and 8 levels may prove useful to assess the condition of the bowel in fetuses with gastroschisis.

Spontaneous intestinal perforation (SIP) in very-low-birth-weight

Hager J.1, Häussler B.1, Reiter G.2, Trawöger R.2 , Sergi C.3
Universitätsklinik Kinderchirurgie Innsbruck

Purpose
During the last 15 years circumscribed intestinal perforations in VLBW infants were increasing, also increasing were the reports in literature about this "new (rare) disease", differentiated from the well known NEC perforations of the small bowel. Patho-genesis is still partially unclear. There are different guesses about the aetiology ranging from arterial hypotension and vascular compromises, focal absence of intestinal muscle layers in the terminal ileum, induction by indomethacin-, ibuprofen-, dexamethasone-therapy to disturbances of intestinal peristalsis.

Material and Methods
Within the last 10 years 17 VLBW babies (five times one twin) with one or more isolated intestinal perforations were observed. Symptoms were usual identical: Abdominal distension, greenish-blue discoloration of the abdominal wall but a stable general condition. Plain X-ray showed a gasless intestinal tract sometimes with free gaz in the abdomen and abdominal sonography free fluid without the well known bowel changes of NEC.

Results
All patients underwent operation. Perforations were mainly situated in the terminal ileum, five patients showed a leak in the jejunum and the upper ileum and additionally three patients had two or more (four) perforations in the upper ileum. Only the part of the bowel carrying the perforation was resected and one or more end-end-anastomoses combined with a double enterostomy in case of peritonitis were done. The mortality rate was 17%.

Conclusion
The surviving rate of VLWW babies is improving everywhere but local bowel perforations of unknown cause appear to increase in these babies. Usually laparotomy and anastomosis and/or double enterostomy are done. There are some reports preferring only drainage of the abdomen primarily, although laparotomy may still become necessary. We perform reduced resection of the sites of perforations and a double enterostomy, even if multiple anastomoses have to be done, to prevent a short bowel syndrome. Nowadays the challenge is to clear the aetiology and to find a possibility to prevent further bowel complications in these otherwise also highly endangered babies.

Key words
Spontaneous intestinal perforation - very-low-birth-weight infant - twins - reduced intestinal resection - enterostomy.

Interdisziplinäres Management angeborener Zwerchfelldefekte resp.-hernien

Hechenleitner P.1, Häussler B.1, Trawöger R.2, Skladal D.2, Hager J.1
Universitätsklinik Kinderchirurgie Innsbruck

Gundlagen
Angeborene Zwerchfelldefekte und -hernien gelten als Fehlbildungen mit hoher Letalität. Ursache dafür ist weniger die mit der Zwerchfellmalformation einhergehende beidseitige Lungenhypoplasie, sondern viel mehr die Reduktion der Lungenstrombahn durch Wandveränderungen des Lungengefäßsystems. Weiters prägen die in ca 50% der Fälle vorkommenden Begleitfehlbildungen wie z.B. Herzfehler oder ZNS-Anomalien die Prognose. Zur Minimierung dieser Probleme kommt der prä-und postoperativen intensivmedizinischen Stabilisierung der Kinder eine besondere Bedeutung zu.

Methodik
In den letzten 6 Jahren wurden an der Abteilung für Neonatologie und Neonatologischen Intensivmendizin der Universitätsklinik für Kinder-und Jugendheilkunde in Innsbruck 19 Kinder mit angeborenem Zwerchfelldefekt aufgenommen. Alle Neugeborenen wurden nach folgendem Schema behandelt: die initiale Therapie bestand in einer Beatmung mittels Hochfrequenzoszillation mit einem FiO2 von 1,0. Nach r erster Stabilisierung erfolgte eine Beurteilung der zu erwartenden pulmonalen Hypertonie mittles Echokardiographie. Bei entsprechendem Befund wurde die Therapie mit NO ( 20 ppm ) ergänzt. Die Zwerchfellkorrektur wurde angesetzt, wenn ein FiO2 von < 0,4 erreicht worden war und fand nach 1-7 tägiger präoperativen Stabilisierung statt.

Ergebnisse
Fünf Neugeborene verstarben präoperativ, bei ihnen war eine adäqate Stabilisierung war nicht zu erreichen.14 Kinder konnten operiert werden, bei 7 Fällen war ein linearer Defektverschluss möglich , 7x war eine Patchimplantation notwendig. 12 dieser 14 Kinder überlebten, ein Kind mit einem hämodynamisch aktiven ASD verstarb einen Tag nach der am 27. Tag nach der Zwerchfellkorrektur erfolgten Herzoperation aufgrund eines Herzstillstands, der andere Patient, ein Kind mit einem Fehlbildungssyndrom, verstarb postoperativ an respiratorischer Insuffizienz. Ein Kind musste 4 Jahre nach der Zwerchfellkorrektur eine Reoperation aufgrund eines Rezidivs erfahren, ein Patient eine Relaparotomie wegen eines Bridenileus 8 Monate nach der Zwerchfelloperation. Bei allen anderen Kindern war der postoperative Verlauf seitens der Zwerchfellkorrektur komplikationslos, wenngleich die postoperative intensivmedizinische Behandlung im Einzelfall durchaus nicht einfach war.

Schlussfolgerungen
Der Outcome von Kindern mit Zwerchfellfehlbildungen ist dank der verbesserten präoperativen resp. postoperativen intensivmedizinischen Therapie gegenüber früher besser geworden, aber immer noch nicht befriedigend. Die Technik des chirurgischen Eingriffs ist standardisiert und hat keinen Einfluß auf die Überlebenschancen der Betroffenen.

Congenital diaphragmatic hernia repair during and after ECMO

E.Horcher, G. Trittenwein
Div. of Pediatric Surgery, Dep.of Surgery1, Div. of Pediatric Intensive Care2,
Medical University of Vienna, Austria

Background
CDH is associated with variable pulmonary hypoplasia and pulmonary hypertension. Depending on the severity of the respiratory problem, the patients are treated with conventional ventilation or high frequency ventilation, NO inhalation and in severe cases by ECMO.

Material and methods
Since 1997, 32 newborns with CDH were reviewed,12 patients were treated by ECMO. The cut off point for ECMO treatment was an oxygenation index of 40.

Results
10 patients were operated after weaning from ECMO, 2 patients while still on ECMO. In the beginning (1997-99) only 1 of 3 patients survived, after 2000 only 3 of 9 patients died (1 bilateral CDH, 1 with associated esophageal atresia, 1 aortic isthmus stenosis). The over all survival rate was 66%, all of them have a normal neurological status.

Conclusion
Allmost one third of our newborns with CDH were high risk patients requiring ECMO. Extracorporal life support still has a mortality of 33%, but patients with an otherwise lethal disorder will benefit from ECMO.

Patch, drain and wait approach in neonate with congenital volvulus of the midgut.

Horn, F., Babala, J., Trnka, J., Pevalová, Ľ.
Dpt.Paed.Surgery, Bratislava

Introduction
Congenital volvulus with total bowel necrosis of the midgut is serious condition which endangering life of the newborn. Traditional surgical approach with bowel resection and anastomosis or enterostomies cannot be used. Patch, drain and wait approach is beneficial in life- and gut-saving good angiogenesis.

Patient and method
The patient was admitted to our paediatric intensive care unit 48 hours after delivery with the congenital volvulus of the midgut. At the first operation we found total necrosis of the midgut with thrombosis of the mesenteric artery except the last 7cm of the distal ileum. At the second look operation after 48 hours the bowel was unchanged and we decided to use “patch, drain and wait approach”. Two month later at the laparotomy we found 30cm of the small intestine revascularised and we used them. After that, we started oral feeding. Patient growth well. Unfortunately, two month later appeared stricture in duodeno-jejunal anastomosis. This leaded to reoperations. Nowadays the patient is at the Department of paediatric surgery in Graz. Nutrition is combined: oral feeding and parenteral.

Conclusion
It seems, that “patch, drain and wait approach” is giving hope for the patients with total bowel necrosis of the midgut.

Histiocytoid cardiomyopathy - rare cause for dramatic clinical course

Karpf Eva F., Ehringer-Schetitska Doris
Institut für Pathologie der Mediz. Universität Graz, G.v.Preyer´sches Kinderspital Wien

abstract
Case report about a girl of five months, born as the fourth child to a healthy family. After a short period of neonatal pneumonia, the infant did well until the age of 4 1/2 months when again bronchitis had to be treated. Pneumonia and fever induced hospitalization. There, vomiting and diarrhea preceded unexpected tachycardia of 200/min. Despite intensive care ventricular arrhythmias ended in untreatable right ventricular failure. Postmortal examination revealed a rare form of non-dilatative cardiomyopathy, typically occurring in females younger than 2 years. Its pathogenesis is still not fully understood and possible treatment is discussed.

Fetal magnetic resonance imaging (MRI) in oligohydramnios: detection of pulmonary hypoplasia and clinical outcome

Kasprian G.1,2, Messerschmidt A.3, Brugger P.C.1, Helmer H.4, Prayer D.2
1 Center of Anatomy and Cellbiology, 2 Department of Neuroradiology, 3 Department of Neonatology, 4 Department of Obstetrics and Gynecology; Medical University, Vienna, Austria

Background and Purpose:
Prolonged oligo/anhydramnios may lead to pulmonary hypoplasia. Using MR volumetry and the assessment of signal intensities of fetal lungs, MRI allows non invasive detection of restricted pulmonary development. In this follow up study we have compared MR data with postnatal clinical outcome.

Methods:
23 singleton pregnancies complicated by oligohydramnios due to premature rupture of membranes underwent MRI between the 18th gestational week (GW) and birth (mean 27,8 GW). Using MR volumetry, fetal lung volumes, fetal body volumes and the amount of amniotic fluid were measured and compared to the results of 111 measurements in normal fetuses.

For follow up 4 groups were defined: newborns with (1)lethal postnatal course (2 )intensive respiratory care >6 days and/or pulmonary hypertension (3)intensive respiratory care <6 days and (4)no need for additional respiratory support.

Results:
In fetuses with oligohydramnios fetal lung volumes were significantly reduced (p<0,01). The mean deficit of lung volumes was 31% compared to normals. Fetuses with a modal deficit of 58,7% of lung volume deceased postnatally. Reduced signal intensity of lung tissue was associated with lethal outcome in 6 of 8 newborns.
Differences of lung volumes and signal intensities between the groups 2-4 were less pronounced. Fetuses with a high risk of pulmonary hypoplasia (group 2) showed significantly reduced amniotic fluid volumes, early onset and a long persistence of oligohydramnios.

Conclusion:
Fetal MR is able to reveal the degree of pulmonary hypoplasia in fetuses with oligohydramnios. Reduction of fetal lung volumes over 50% of the expected value together with pronounced darkness of lung tissue on T2- weighted MR sequences is highly conspicuous for lethal outcome.

MANAGEMENT OF CHILDREN WITH CLEFT LIP/PALATE SPEECH THERAPY AND ENT - THE DSP CONCEPT

E.LINDNER
SMZ OST - DONAUSPITAL, WIEN

Our care for the new-born starts shortly after birth providing feeding tips and evoking otoacustic emissions (OAE). At the time of the lip repair grommets are inserted surgically if required. Beginning with six months audiological testings including ear inspection, tympanometry, OAE, children´s audiology and brainstem evoked response audiometry (BERA) are performed. Orofacial therapy may also be applied at this time.
Logopedic treatment starts at the age of three with a speech status, phonation exercises and voice therapy are employed. Logopedic testings are scheduled at six month intervals till the age of six. At the age of six articulation therapy will be continued. Once a year ENT testings are made beginning with eight years. At the age of twelve a final speech status is performed.

Epithelioid hemangioendothelioma of external iliac vein with multiple metachronous localizations in liver and lung

B.Ludwikowski, N. Jones
Univ.-Klinik für Kinderchirurgie PMU-Salk 5020 Salzburg Müllner Hauptstr.48

Introduction:
Epithelioid hemangioendothelioma (EH) with metachronous liver and lung localizations are rare. The therapeutic concepts are controversial: from lung-liver transplantation to a wait and see concept. This case illustrates the difficulty and pitfalls of making the correct diagnosis and treatment.

Case-report:
A 11-year old girl was presented with left leg swelling since 3 weeks. She had signs of ileo-femoral venous obstruction, because of multiple lymph nodes in the left groin (confirmed by US and CT). Bartonella IgG was positive and she was treated with Doxycyclin. After 6 weeks treatment there was no change of the size of lymph nodes in CT. Laparoscopy and exploration of the left groin detected tumor infiltration into the external inguinal vein and biopsy was performed. Histology was juvenile myofibrosis and extramural expert consultation give the final diagnosis of EH. At this time bilateral lung metastases were found in CT (X-ray of the thorax was normal) and map like structures in the liver was seen, but in US no signs for malignancy. Thoracoscopic biopsy of lung metastases and total resection of the tumor in the left groin with interposition of the vein was performed. Swelling of the left leg disappeared and the child has no symptoms. CT of the thorax 4 weeks later show!
s no change of lung metastases. In the follow-up diffuse liver infiltration was again seen in MRI, but never in US. Laparoscopic biopsy was performed and histology shows EH metastases.

Discussion and Conclusion:
In the literature approximately 50 cases are reported. The overall mortality is less 20% at 3 to 5 years. Spontaneous regression of pulmonary EH are described in asymptomatic patients after 5, 13 and 15 years. Patients with pleural effusion and clinical symptoms died within 1 year. Adjuvant chemotherapy, radiation therapy or both have no proven benefit. Interferon is described, but with severe complications in children. In this case we did not perform any systemic therapy, because the girl is now over 18 months in control without changing of metastases size in lung and liver and without symptoms.

Erfahrungen mit der Behandlung des VUR mit Deflux

B. Ludwikowski, C. Schimke, M. Ardelean
Univ. Klinik für Kinderchirurgie Müllner Hauptstr.48 5020 Salzburg

Einleitung: 
Im letzten Jahr hatten wir über unsere ersten Erfahrungen mit Defluxâ) zur Behandlung des vesicoureteralen Refluxes berichtet. Unsere Ergebnisse, im besonderen die Kinder mit fortbestehenden Reflux nach Unterspritzung, wollen wir analysieren.

Methoden:
In dem Zeitraum von 01/ 2002 - 02/ 2005 wurden 54 Patienten (I°= 7, II°= 50, III° = 34, IV° = 3) mit Deflux behandelt. Diese Patienten haben eine Nachkontrolle über mindestens 3 Monate (3-37 Monate) inklusive klinische Kontrolle, MCU und Sonographie. Eine Solitärniere fand sich 2 mal, Doppelniere einseitig 7 mal, beidseitig 1 mal, neurogene Blase bei MMC 2 mal, st. p. Kloake 2 mal und st. p. Blasenekstrophie 2 mal. Eine Blasentrabekulierung ohne neurologische Grunderkrankung fand sich 4 mal. Das Alter zum Zeitpunkt des Eingriffs lag zwischen 2 und 12 Jahren. Die Unterspritzung erfolgte in Allgemeinnarkose: 9,5 Ch Cystoskop, injizierte Bolus im Mittel 0,7 ml (0,2 -3,2 ml) bei 6 Uhr, gelegentlich zusätzlich bei 3 und 9 Uhr. 2 Patienten mit neurogener Blase bei MMC erhielten zusätzlich eine Botoxbehandlung. Die Patienten wurden am 1. post operativen Tag nach durchgeführter Ultraschallkontrolle entlassen.

Ergebnisse:
42 Patienten (78%) benötigten nach einer Defluxunterspritzung keine weitere Behandlung und waren kuriert. 12/54 Patienten (22%) benötigten einen weiteren Eingriff. Bei 3 von diesen Patienten (5,5%) kam es zu einer Verschlechterung des Refluxgrades (II°®III°=3x, II°®IV°=1x), die zur Operation (Cohen) führte. Die übrigen 9 Patienten (16,5%) erhielten eine 2. Unterspritzung. Bei 2 Patienten (MMC) führte auch dies nicht zum Ziel. Eine passagere Nierenstauung wurde einer Ureterschiene versorgt, bei mehrmals voroperiertem Ureter.

Zusammenfassung:
Wir haben bei uns im 1. Jahr eine Lernkurve beobachtet und hatten mit zunehmender Erfahrung die Technik (Menge und Lokalisation) verändert. Die Kinder, die operiert wurden waren alle im 1. Jahr unterspritzt worden. Hier würden wir rückblickend ie Menge erhöhen und eine 2. Unterspritzung anbieten. Bei Kindern mit ausgeprägter Blasentrabekulierung, insbesondere bei neurologischer Ursache, ist es extrem schwierig ein passendes Depot an die richtige Stelle des Ostiums zu platzieren. Hier ist die offene Operation zu empfehlen. Auch wenn die Heilungsrate längst nicht, die der offenen Operation erreicht, hat sie doch den Vorteil, das sie minimal invasiv ist mit einem sehr kurzen Kliniksaufendhalt und die Operationsbedingungen durch eine submuköse Injektion nicht verschlechtert wird und eine sehr gute Akzeptanz bei Patienten und Eltern hat.

Totale Urogenitale Sinus Mobilisation (TUM) bei persistierendem Urogenitalsinus und kloakalen Fehlbildungen: ein follow-up bezüglich Harnkontinenz und genitaler Sensibilität

B. Ludwikowski, T.M. Boemers
Univ. Klinik für Kinderchirurgie 5020 Salzburg Müllner Hauptstr.48

Einleitung: 
TUM ist eine etablierte Methode zur Korrektur des persistierenden Urogenitalsinus (UGS), kloakaler Fehlbildungen, des Adrenogenitalem Syndroms (AGS) und der Blasenekstrophie. Wir präsentieren unsere postoperativen Ergebnisse bei Kindern mit kloakalen Fehlbildungen und beim persistierenden UGS bezüglich funktionellem (Harnkontinenz und Sensibilität im Genitalbereich) und kosmetischem Resultat.

Methoden: 
In den vergangenen 9 Jahren (5/1996 -5/2005) wurden in unserer Abteilung insgesamt 17 Patienten mit einer kloakalen Fehlbildung (13 P.) oder einem persistierendem UGS (4 P.) durch eine totale urogenitale Mobilisation korrigiert. Der durchschnittliche Nachbeobachtungszeitraum betrug 4,2 Jahre (16 Monate - 9 Jahre). Die Krankengeschichten wurden analysiert, die Patienten telefonisch kontaktiert und klinisch nachuntersucht. Patienten, die Probleme mit der Harnkontinenz angaben, erhielten zusätzlich eine Cystomanometrie. Eine Uroflowuntersuchung wurde bei allen Patienten mit persistierendem UGS durchgeführt.

Ergebnisse: 
Die Patienten wurden in zwei Gruppen unterteilt: Kinder mit kloakaler Fehlbildung (13) und mit persistierendem UGS (4). Die Genital- und Klitorissensibilität war bei allen Patienten erhalten. Dreizehn von insgesamt 17 Patienten sind kontinent, 11 haben ein komplett unauffälliges Harnverhalten, 2 Patienten mit kloakaler Fehlbildung sind kontinent mit CIC. Zwei Mädchen mit kloakaler Fehlbildung und Sakrumdefekten leiden an neurogener Blase und wurden durch entsprechende Behandlung (Blasenaugmentation, medikamentös) kontinent. Zwei Mädchen sind inkontinent, wobei sich bei einem Mädchen die Symptome nach der Operation verschlechterten. Drei Mädchen haben eine Vaginaleingangsstenose entwickelt und benötigten eine Vaginalplastik.

Zusammenfassung: 
Die Technik der totalen urogenitalen Mobilisation beeinflusst nicht die Harninkontinenz und eignet sich zur Korrektur des UGS, wenn eine auseichend lange Urethra vorhanden ist. Mädchen, die bereits präoperativ Zeichen einer Inkontinenz hatten, konnte durch diese Methode nicht geholfen werden, jedoch der anatomische situs korrigiert werden. Schwierig ist der Eingriff bei voroperierten Kindern durch das vernarbte Gewebe, so dass eine Nerven schonende Operation schwierig ist. In unserer Sicht ist es eine ideale Methode zur Korrektur des persistierenden Urogenitalsinus und bei Ersteingriffen der kloakalen Malformation.

Clinical evidence of the role of the notochord in the common etiology for esophageal atresia, forgut duplication and vertebral anomaly

Ágnes Magyar MD., Tamás Józsa MD., Zoltán Sz&#337;l&#337;si
MD., István Csízy MD.,Tamás Cserni MD.
Departmant of Paediatrics Universcity of Debrecen; Hungary; H-4012
Debrecen, Nagyerdei krt. 98

INTRODUCTION: 
The Adriamycin treated rat model (ARM) is reliable model of esophageal atresia (EA), tracheoesophageal fistula (TEF) and vertebral anomaly (VA). Foregut duplications cyst (FD) has been observed 24% in ARM. The abnormal nothocord detachment from the foregut has been confirmed in the etiology. Only few human clinical cases of coexistent occurrence of FD, EA and TEF have been reported yet without mentioning associated vertebral anomaly (VA), and the role of the notochord was not considered in the etiology. The coexistence of these malformations described in our case supports the observations in the ARM and can be considered as real complete clinical evidence that the failed notochord detachment is responsible for their common etiology.

MATERIAL/METHODS: 
A neonate underwent a primary EA repair. VA and a 2x3 cm large cyst, communicating with the upper pouch of the esophagus was observed on the praeoperative X-ray image. The cyst was resected, primary anastomosis was performed. Common histological and immunhistochemical investigations were done.

RESULTS: 
A hamartomatous structure with columnar epithelium, disorganized smooth muscle layers, islands of cartilage and gland were seen on the hematoxylin-eosin-stained sections. Positive staining for carcinoembryogenic antigen (CEA) confirmed the intestinal origin. Reactions with cytokeratin 7 (CK7) and chromogranin demonstrated the presence of pancreatic origin in the glands. An esophageal diverticulum lined by mature gastrointestinal or squamous epithelium was excluded by the negative staining for CK14 and CK20.

CONCLUSION: 
It appears that the notochord by failed detachment drawing a cyst-like structure (FD) away from the foregut leading to abnormal bending of this structure causing EA and TEF and it arrest normal vertebral development.

If the Teflon Depot is not visible... Long-term results of the STING therapy

Zsolt Oberritter, Zsolt Juhasz, Reka Somogyi, Andrew B. Pinter
Surgical Unit of Department of Pediatrics, Pécs University, Pécs, Hungary

BACKGROUND / PURPOSE: 
This is a long-term retrospective study to investigate whether detection of Teflon depot in the bladder has a role in the recurrence of vesicoureteric reflux (VUR) following a successful subureteric Teflon injection (STING).

METHODS: 
Between 1989 and 2001 STING was performed in 136 patients (pts) at the authors' institute. 98 pts were followed for 2 to 12 years. These pts were included in this study. The mean follow-up time was 7 years. The disappearance of VUR was proved by voiding cystourethrography (VCUG) 6 months post-STING. All pts in the study were underwent VCUG following various VUR free periods and simultaneous bladder ultrasonography was performed.

RESULTS: 
Recurrence of VUR was detected in 14/98 (14.3 percent) patients. It was of grade I in 5 pts, grade II in 5 pts, grade III in 3 pts, and grade IV-V in 1 pt. The VUR was bilateral only in one patient (grade III). In one patient VUR was up-graded (from II to III). We found Teflon depot in 42/98 (42.8 percent) pts. We did not detect Teflon depot in those pts who had recurrent VUR, however, VUR was not detected in whom the Teflon depot was visible in the bladder.

CONCLUSIONS: 
Our study suggests that detectable Teflon depot after a successful STING procedure might exclude the long-term recurrence of VUR. Nevertheless lack of Teflon depot does not mean the recurrence of VUR, but it increases its possibility.

Oesophageal atresia and Schärli´s gastroplasty - Long - term surgical complications - 2 Cases

G. Pelizzo, M. Monai, P. Guastalla*, A. Sarti**, J. Schleef
Paeditric Surgery, Paediatric Radiology*, Anaestehsia** - IRCCS Burlo
Garofolo Trieste, Children´s Hospital, Via dellÌstria 65/1, I - Trieste, Italy

Schärli`s gastroplasty represents a challenging surgical option for the repair of long gap oesophageal atresia. Up to 6-8 centimetres of neo oesophagus can be tailored from the lesser gastric curve in order to achieve a primary anastomosis. Long term results and complications of this technique are still under discussion in the current literature. We report our experience with 2 affected patients who underwent gastrostomy at birth. Primary anastomosis by Schärli´s gastroplasty was performed at 4 month of life after a progressive bouginage of the upper pouch. Both patients showed feeding problems due to the persistence of an important gastroesophageal reflux, oesophageal stenosis, and esophagitis. Several oesophageal dilatations were performed in the firs year of life. A subsequent ant reflux Toupet procedure was performed one year after Schärli operation. Because of the intrathoracic position of the wrap, one patient underwent redo-anti reflux surgery, 12 months later. Three years later, both of the patients are thriving well under omeprazole medication. Although the gastroplasty provides the sufficient length to reconstruct the oesophagus, it implies the ant reflux wrap on the gastric fundus so that insufficient fundoplication becomes the most common issue in this technique. Furthermore, gastroplasty implies the intrathoracic displacement of the gastro - oesophageal junction. This condition favours the development of an ulcerative esophagitis and stenosis. An additional might be a concomitant Schärli technique implies a prolonged surveillance of the GERD due to the intrathoracic fundoplication. Redo-surgery for incontinent wrap should always be kept in mind to avoid the well documented serious complications associated with this condition. Nevertheless all these problems, Schärli´s technique might be useful to avoid oesophageal replacement by colon or stomach and all associated problems due to these techniques.

The cystic type of meconium peritonitis - prenatal diagnosis and perinatal management

G. Pelizzo, A. Giannotta, G.d´Ottavio*, S. Demarini**, J. Schleef
Paeditric Surgery, Prenatal Diagnostic*, Neonatology** - IRCCS Burlo
Garofolo Trieste, Children´s Hospital, Via dellÌstria 65/1, I - Trieste, Italy

Meconium peritonitis, a life threatening condition, is present in several forms: meconium ascitis, meconium pseudocyst and intra abdominal calcium deposit. Cystic fibrosis must be a strong consideration in the differential diagnosis, accounting for 25% to 40% of the cases. We describe 3 cases (female) with prenatal serial ultrasonographic scanning of type II pseudocyst meconium peritonitis. The severe ultrasound presentations suggested a potentially letal course for the neonates. The perforated bowel sealed in utero required a preterm labor respectively at 32, 34 and 36 WG. Postnatal abdominal X-rays showed peritoneal calcification, no free peritoneal gas. In all infants the clinical conditions worsened rapidly and perinatal progression in ascitis increased. The older baby (36WG) underwent perinatal abdominal drainage with a transient amelioration. Babies were submitted to urgent laparotomy in the first 2 days of life and meconium peritonitis was confirmed.
In the smallest baby (32WG) a perforated necrotizing tubular duplication of the transverse colon was detected. Removal of duplication followed by limited segmental colonic resection and double colostomy was carried out. Histology of resected cysts confirmed a colonic duplication. A perforated mass of the right abdomen was discovered in the two other neonates. Dissection allowed a complete excision of the mass in the older patient (36 WG). The other baby showed several clusters of calcification along the anterior peritoneal wall. Multiple intestinal perforations were present at the level of the righ colon requiring a right double colostomy. Suggestion was made to test for cystic fibrosis. All patients survived. Any source of bowel obstruction may result in meconium peritonitis (bowel atresia, volvulus, intussusception, vascular insufficiency). A detailed prenatal diagnosis of meconium peritonitis is necessary for a better understanding of the natural history of meconium peritonitis. Type II cysts, or large pseudocysts, resulted to be associated, in our experince, with a preterm labour, a very critical perinatal condition, and an emergent surgical treatment. Prenatal diagnosis of this form of meconium peritonitis could change prognosis and ameliorate management at birth. Proper prenatal counseling and risk evaluation should also be discussed with the parents of affected fetuses.

Fetal magnetic resonance imaging (MRI): Indications

Daniela Prayer, Peter C. Brugger, Gregor Kasprian, Linde Witzani, Marcus Hoermann, Elisabeth Krampl, Gerhard Bernaschek, Hanns Helmer, Erich Hafner
Medical University of Vienna, Danube Hospital Vienna

Purpose: 
Based on a 7-year experience, indications for the application of prenatal MRI shall be reviewed

Material and methods: 
600 fetal MRI-examinations were done between the 18th and 39th gestational week for clinical purposes. Patients were refered to MRI because of impaired ultrasound (US) assessment and/or unclear US results, and/or complementary information was expected from MRI. MRI was performed on a 1.5T superconducting unit.

Results: 
MR-findings consisted of: malformations of the central nervous system (CNS) 25%, acquired lesions of the CNS 6.5%, premature rupture of membranes (PROM) 10,3% extra-CNS malformations 34,4%, complex malformative syndromes 3.1%, placenta- related pathologies 14.9%, inconclusive and/or normal findings (1,4%) miscellaneaous (maternal pathologies, normal variants) (5,5%). Based on a preliminary US-examination, complementary MR-information confirmation included extension/change of diagnosis with impact on prognosis and/or counseling/therapy/maintanance or termination of pregnancy/ time of delivery in 152 cases (25%), while in the remaining cases MR-information did not change the further management.

Discussion and Conclusion: 
Our results suggest a useful application of prenatal MRI with following (most important) indications: impairment of US-assessment (oligohydramnios, maternal obesity, adverse position of the fetus), intra-and extra CNS-malformation (prognosis/therapy options depend on extent of organ involvement, a genetically determined condition with impact on counseling may be defined). Suspect acquired CNS pathologies (may be inconceivable with US), IUGR (detection of an underlying morphological pathology), maternal disease with possible impact on fetal development (infection, autoimmune diseases, eclampsy), twin-to twin transfusion syndrome (degree of organic damage, placenta pathology, posttreatment changes), and previous history of unclear abortion, stillbirth, genetic abnormalities.

Management of treatment of velopharyngeal incompetence in cleft palate patients

P.Schachner, B. Specht-Moser, C.Brandtner, C. Krenkel, T. Hofstetter, E. Pointner
Dep. of Oral and Maxillofacial Surgery, Private Medical University Salzburg - St. Johanns-Spital, Müllner Haupt Str. 48, A-5020 Salzburg

Due to international literature in up to 10 percent of all patients with complete clefts of palate or lip, alveolus and palate additional surgery is necessary because of velopharyngeal incompetence. The decision which surgical procedere is most convenient in the particular case requires very careful preoperative diagnostic evaluation. An interdisciplinary approach has to be performed including exact analysis by speech therapists with aerophonoscopy, ENT-examination with nasometry and endoscopy and videocinematography. The success of surgical treatment depends on exact preoperative diagnostics, optimal timing of the operation and surgical technique. The diagnostic steps and different surgical techniques are demonstrated.

The Bern Concept for the Treatment of Lip and Palate Clefts

I. Schnyder, G. Kaiser
Dep. Surgical Paediatrics, University of Bern, Inselspital, CH-3010 Bern, Switzerland

Since 1958 paediatric surgeons in Bern perform the primary surgical treatment of lip and palate clefts. During the years a specific surgeon performed all operations together with a second younger surgeon helping and learning for the future in order to gain large experience and increase treatment quality. In addition a multidisciplinary, interprofessional team was established for the continuous therapy and follow-up in an outpatient setting with fixed appointments. Our cleft team consists of a gynaecologist, breast feeding advisor, orthodontist, maxillar surgeon, logopedist, otorinolaryngologist, social worker, psychologist and geneticist. The paediatric surgeon is of course also medically involved, performing the primary operations, however she/he is responsible for the management and coordination of the team and its work. We treat between about 10 new cases per year and during the same period we perform 120 - 150 consultations of our patients. We would like to present our concept in detail by presenting our procedure in a new patient with the follow-up till the completion of the therapy. We think that the Bern model for lip and palate clefts is a way to achieve high expertise and increase the quality of the treatment of this rare congenital disease.

Present Results in the Treatment of Omphalocele and Gastroschisis

Šimsová M., Rygl M., Kalousová J., Pýcha K., Stýblová J., Šnajdauf J.
Department of Pediatric Surgery, 2nd Medical Faculty Charles University and Faculty Hospital Motol, Prague

Objective: 
The aim of study was to evaluate current possibilities of treatment of newborns with abdominal wall defects: omphalocele and gastroschisis.

Setting: 
Depatrment of Pediatric Surgery 2nd Medical Faculty Charles University and Faculty Hospital Motol, Prague

Methods: 
A retrospective cohort study of the treatment results in newborn with defect of abdominal wall.

Results: 
Fifty newborns with omphalocele and gastroschisis were treated at the authors‘ department in the period of 1995 – 2004. Primary closure of defect was possible in 31 newborns (62%), Goretex patch or ¨silo¨technique was used in 17 patients (34%), two newborns (4%) were not operated . The overall survival in children with omphalocele was 77%, 92% in children without chromosomal anomalies. Seven infants died: three with trisomy 13, one with trisomy 18, two infants died of sepsis and one had primary pulmonary hypertension and congenital heart defect. The survival in children with gastroschisis was 95%, one child died of multiorgan failure during sepsis.

Conclusion:
For prenatal diagnosis of gastroschisis or omphalocele is recommended complex examination and consultation in specialized center. The prognosis for most isolated defects of abdominal wall is good as far as survival and quality of live are concerned.

The work was supported by Reasech Project FNM MZO 00064203/6309

Swenson´s colorectoplasty: Prevention of postoperative enterocolitis in transanal surgery for Hirschsprung´s disease?

Skaba R, Kalousova J, Rouskova B, Frantlova M, Mixa V.
Motol Children Hospital , Prague, Czech Republic

Introduction: 
The transanal endorectal pull through ( TERP) has gradually become a standard operation in the treatment of classical form of Hirschsprung´s disease ( HD). However in TERP a relatively high percentage of postoperative enterocolitis has been reported. In oposite Swenson´s colorectoplasty with partial sphincteromyectomy of internal anal sphincter has low risk of postoperative enterocolitis.

Aim of study: 
To introduce Swenson´s type of transanal resection (STAR) as an alternative to TERP.

Patients and surgical technique: 
Between January 2003 and July 2005 27 patients with HD ( 19 M, 8 F ) aged 6 days to 16 years underwent STAR. 9 patients were neonates at operation. Association with Down sydrom was found in two female patients. The extent of resection was determined by peroperative biopsy. Antiobiotic prophylaxis was secured by three doses of cefoxitin and one dose of isepamycin.

Results: 
Intestinal passage was reestablished 8 – 12 hours after surgery, and complete oral intake was achieved on day 3 or 4 after the operation. Three patients developed anal stricture. In one of them it was treated by anal dilatation, in one colostomy and subsequent open endorectal pull - through were done. One patient has temporary colostomy. In two neonates and two older children with both aganglionic segment longer than 25 cm and pulled-down colon under tension STAR was switched to open surgery. The period of hospitalisation was 7 days on average. There were no cases of postoperative enterocolitis on follow-up.

Conclusions: 
In our series of patients STAR seems to be good surgical technique in prevention of postoperative enterocolitis in HD.

CONGENITAL DIAPHRAGMATIC HERNIA IN NEWBORNS – TREATMENT AND RESULTS IN ONE HUNDRED CASES

J.Šnajdauf, K.Pýcha, Z.Straňák, M.Rygl, J.Melichar, R.Škába
Dpt. Of pediatric Surgery, Charles University Prague, 2nd Medical School, Institute for Mother and Child Podoli, Charles University Prague, 1st Medical School, Czech

Background/purpose: 
Evaluation of survival and incidence of recurrence according to management of the newborns with congenital diaphragmatic hernia (CDH) presenting within the first 24 hours of life.

Methods: 
A retrospective study of 104 newborns treated for CDH by one team of neonatologists and surgeons. The infants were operated either after stabilization or on ECMO, if required. If the diaphragmatic defect was too large, Gore-tex 1 mm patch was inserted. Nonabsorbable interrupted suture was used, distance between stitches was 3-4 mm and they were anchored around rib.

Results: 
104 newborns were treated for CDH from January 1994 to December 2004, total survival was 73,1% (76/104), 83 infants were operated after preoperative stabilization, their survival was 91,6% (76/83), in 62 infants primary closure was performed, the survival was 96,8% (60/62), 21 infants received Gore-tex patch, the total survival was 76,2% (16/21), 15 non ECMO infants had 86,7% (13/15) survival, 6 ECMO infants had 50% (3/6) survival. No recurrence occurred in children with primary closure of diaphragm. Only in one infant with bilateral CDH and giant omphalocele early recurrence occurred after secondary closure of an abdominal defect. This child is alive and in good condition.

Conclusions: 
1) ECMO therapy was always associated with large diaphragmatic defect. 2) ECMO therapy was associated with poor outcome. 3) The Gore-tex membrane is suitable material for diaphragm reconstruction, we suppose that recurrence is caused by incorrect attachment of the patch to the thoracis wall.

MANAGEMENT OF CHILDREN WITH CLEFT LIP/PALATE SURGICAL REPAIR OF CLEFT PALATE

MARKUS STRASSER, INGEBORG M. WATZKE
SMZ OST- DONAUSPITAL, WIEN

INTRODUCTION
In children born with cleft lip and palate the rate of palatal affection is 80 to 85%. The palatal cleft may either occur isolated or in combination with cleft lip and/or alveolus. Except for feeding tips in most cases medical treatment is not necessary immediately after birth.We only use feeding plates subsequently to feeding problems or in very broad clefts if alveolar moulding is desired. In our department the entire palate is closed with 12 months. Our aim is a full anatomical rehabilitation, which should manifest itself in perfect feeding, good middle ear function and normal speech development.

PATIENTS
Within the last 12 months 20 palatoplasties have been performed at the department of oral and maxillofacial surgery of the DSP. We treated 8 female and 12 male patients with a mean age of 2,7 years. In 3 cases the primary cleft affected the soft palate, in 7cases hard and soft palate, in 7 cases lip, alveolus and palate unilaterally and 3 cases bilaterally. Amongst these cases 5 were rerepairs.

METHOD
The technique of palatal closure being used is similar to the one described by V. Veau, modified by B.Sommerlad. The underlying philosophy is a radical reconstruction of the palatal musculature.This method has replaced the two stage palatal closure by Widmaier/Perko formerly performed at our department.

Results:
17 cases showed no fistula at all. One rerepair case had a fistula at the junction of hard and soft palate . Another rerepair showed a relatively short soft palate due to loss of tissue at the rear end of the soft palate p.o. In one primary closure case a small hole developed at the soft palate/hard palate junction. Due to the the short follow up our assessment did not include functional aspects. Nevertheless the motility of the soft palate appeared to be satisfactory.

SUMMERY
We believe that the method we use is a reliable technique in palatal closure.

Management of intestinal atresia and perforation with intraluminal stenting via appendix

Peter Vajda*, Karoly Adamovich**, Andras Farkas*, Attila Vastyan* and Andras Pinter*
Surgical* and Neonatal Intensive Care** Units of Department of Paediatrics, Pecs University, Jozsef A. str. 7., Pecs, H-7623 Hungary

Purpose
The aim of this study was to present a complementary method in the surgical treatment of small intestinal atresia and perforation and milder form of meconium ileus in neonates. Methods Over a 15-year period (1991-2005), 17 neonates were treated for ileal atresia or perforation using intraluminal stenting through appendicostomy at the authors' institute. The medical records of these 17 patients were analyzed and evaluated retrospectively. Results The gestational age of the 10 female and 7 male newborns ranged from 30 to 41 weeks (mean 36). Mean birth weight was 2.5 (0.9-4.02) kilograms. The operations were performed at the 1-15 days of life (mean 6.6). In 9 cases appendicostomy was performed for intraluminal stenting to protect the primary anastomosis due to distal ileal atresia(s). Two of the atresias were multiple. In 8 newborns ileal perforation was found because of bowel ischemia (NEC) (5 pts) or meconium ileus (3 pts) without Bishop-Koop or Santolli stomas. Eleven patients were successfully treated and the stent (8F feeding catheter) was removed at the 7-12 postoperative days. In cases of meconium ileus the stent was used for enema, too. However, 6 newborns died of postoperative sepsis and/or leak of anastomosis, re-perforation were detected only in 2 patients at autopsy. In 5 cases the appendicostomy closed spontaneously. Stoma closure was needed in 6 patients 3 to 9 months postoperatively. Conclusions Intraluminar stenting via appendicostomy is a promising supplementary method in the management of ileal atresia or perforation, especially in multiple lesions. The catheter decreases intraluminal pressure, improves circulation of bowel wall, therefore helps to avoid anastomotic leaks or strictures, permits radiological evaluations and early enteral feeding, as well as enema in cases of meconium ileus.

MANAGEMENT OF CHILDREN WITH CLEFT LIP/PALATE SURGICAL REPAIR OF CLEFT LIP

INGEBORG M.WATZKE, MARKUS STRASSER
SMZ OST- DONAUSPITAL, WIEN

INTRODUCTION
Clefts of lip and/or palate affect between 1 per 500 to 1 per 700 live births. The management of children born with this malformation is best conducted in organized centers - as highly specialised corrective surgery in the early months of life is necessary. There are several surgical steps to improve function and appearance. The optimal time for primary lip repair, closure of soft and/or hard palate is controversial. We describe our time management and surgical procedure for primary lip repair.

PATIENTS
Between January 2004 and July 1005 fourteen children with cleft lip and/or alveolus and/or palate had primary lip repairs. There were eight boys and 6 girls. Thirteen children had a unilateral cleft and one child a bilateral cleft. Six patients had a unilateral cleft on the right side. There were four girls and two boys. There were left sided clefts in seven children, five boys and two girls. The child with bilateral clefts was a boy.

METHOD
The surgical repair is performed at six months of age. We prefer the surgical technique introduced by G. Pfeifer in the 1960's. This wave line procedure results in a straight line. It is a flexible system of wavy and curvilinear incisions and dislocated soft tissue is replaced into an anatomically correct location. Basically there are several therapeutical goals depending on the severity of the cleft:

  1. lifting of the nose,
  2. forward movement of the membraneus septum
  3. medialization of the laterally placed ala nasi
  4. anatomical end-to-end union of labial musculature
  5. shaping of Cupid's bow
  6. intraoral maintainance of frenulum
  7. placement of scar above central incisor

SUMMERY
In our patients we found this method reliable and save to repair lip clefts. In short time follow up there was a good aesthetic and functional result. However a long term follow up is necessary for decisive answer of rehabilitation of aesthetic and function.

Tissue Reactivity of Prosthetic Materials for Reconstruction of Defects in Surgery; a comparison of Gore-Tex, vicrylnet and dura in a rat modell.

Z. Zachariou
Dep. of Surgical Paediatrics, University of Bern, Inselspital, CH3010 Bern, Schweiz

Prosthetic materials are implanted for a wide range of purposes, such as for the closure of big tissue defects in congenital malformations, the repair of traumatised organs or hernias. Nowadays, we can choose between biologic and synthetic materials the latter being subdivided into absorbable or nonabsorbable. In the sixties, Marlex was one of the first synthetic materials on the market, but more demanding requirements soon led to the development of more sophisticated materials, such as Gore-Tex and vicryl-net. The problem is that biologic materials are not always available, immunotolerance is uncertain and infection, especially in the AIDS-era unclear, while rigidness, a defined size and the elicitation of a foreign body reaction (FBR) are problems with synthetic materials. Conflicting results have been arrived at in studies using prosthetic materials for surgical treatment of congenital defects. Only a few studies examined FBR in contrast to a significant number of studies dealing primarily with stability and subsequent adhesions. This was mainly due to lack of an adequate animal model in which FBRs could be reproducibly studied. In this study, we compared under controlled laboratory conditions two biological implants (fetal membrane, lyophilised dura) and two meshes (Gore-Tex and vicryl-net) with respect to lightmicroscopic and immunohistological appearance 15, 30 and 90 days after implantation in the rat defining the characteristics of each material. Our studies revealed that dura is an inert material. However, it should be considerd as a prosthetic material of second choise, as it is not clear whether diseases might be transmitted. Gore-Tex induces a longlasting and extensive foreign body reaction, however, adhesions formed in the beginning resolve with time. In the vicryl-net group lasting and intensive adhesions occur but foreign body reaction was temporary. The originality of this study is that we then combined fetal membane and vicryl-net in order to reduce unwanted effects while securing the advantages of each material.

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