Trocar-site hernia as a typical postoperative complication of minimally invasive surgery among preschool children.

BACKGROUND: Minimally invasive surgery (MIS) in preschool children (<5 years of age) is not common yet, and few reports evaluating typical complications are available. Trocar site hernias are well described in adult patients but also have been reported for preschool children. The goal of our study was to determine incidence and relevance of trocar site hernias as complications of minimally invasive surgery in preschool children. METHODS: Retrospective analysis of all pediatric patients who underwent minimally invasive surgery at a single institution. Review of the literature. RESULTS: Trocar site hernias are significantly more frequent in preschool children than in older ones (p = 0.006). Complication rates at all are not significantly different. Trocar site hernias in infants are mainly of type 3 (omental protrusion) and occur within the first postoperative week. CONCLUSION: Meticulous suturing of all layers (particularly the peritoneum) even in small incisions (2 mm) is recommended to prevent omental protrusion at trocar site in children up to 5 years of age.

Isolated ultra-long gap esophageal atresia - successful use of the foker technique.

OBJECTIVE: Ultra-long gap (> 3.5 cm) esophageal atresias (ULGEA) are very rare congenital disorders usually treated by esophageal substitution. Since the introduction of Foker's elongation technique for primary anastomosis, there is much controversy over whether the Foker method or esophageal substitution results in a better outcome. Until now, there was only one series, which had been reported by Foker himself. We therefore present the outcome 2.5 years after one of the first children was operated on with the Foker method in Europe (2003). PATIENT AND METHOD: A premature child (1820 g, 33 + 4 gestational week) born with isolated ULGEA (6-cm gap length/30.5-cm body length) was treated with the Foker technique. We present a critical review of the course together with any complications that can help in the clinical evaluation of the new technique. RESULTS AND CONCLUSION: Three thoracotomies and 5 dilating procedures over a period of 3 months were necessary to achieve primary anastomosis. Two major complications occurred (anastomotic leak and subsequent stricture). The boy was under mechanical ventilation for a total of 15 days. The Foker method seems to be quite feasible and quickly results in the expected primary anastomosis. The result in this single case is excellent.

Upregulation of vascular endothelial growth factor receptors is associated with advanced neuroblastoma.

BACKGROUND: Angiogenesis is essential for tumor growth and relies on the production of angiogenic factors. Vascular endothelial growth factor (VEGF) is a major regulator of angiogenesis that binds to tyrosine kinase receptors Flt-1 and KDR. The interaction of VEGF and its receptors at gene and protein levels in neuroblastoma remains widely unknown. METHODS: Tumor biopsy specimens and serum were obtained from 37 neuroblastoma patients; adrenal biopsy sections and sera of 7 normal children served as controls. Biopsy specimens were examined by real-time reverse transcription polymerase chain reaction (RT-PCR) and Western blotting; serum was analyzed by enzyme-linked immunosorbent assay (ELISA). VEGF-A(165), B, C, Flt-1, and KDR were analyzed. RESULTS: VEGF isoforms and its receptors' mRNA were expressed in neuroblastoma and control tissues. Whereas the ligands were increased in stages III and IV, the receptors were upregulated in stage III only. At protein level, VEGF-B and C, Flt-1, and KDR were not detectable in tissue lysates, whereas VEGF-A was increased in stages III and IV. Serum VEGF protein levels were upregulated in stage III. CONCLUSIONS: VEGF-A(165) is one of the major angiogenesis regulators among the ligands' family of VEGF, whereas its receptors KDR, and most probably Flt-1, may contribute to a poor prognosis (angiogenic) phenotype, as indicated by their upregulated MRNA levels in stage III neuroblastoma. VEGF-A(165) mainly contributes to increased serum VEGF levels and may serve as a diagnostic tool in advanced-stage neuroblastoma.

Expression of cytokeratin-18-related tissue polypeptide-specific (TPS) antigen in Wilms tumor.

BACKGROUND: So far, there is no approved tumour marker for diagnosis or follow-up in Wilms tumour (WT). Tissue polypeptide-specific antigen (TPS), a cytokeratin 18 proteolytic fragment, has been suggested to be of value in the clinical management of WT patients. Cytokeratin 18 fragments are an early indicator of apoptosis and cytokeratin 18 might influence tumour cell behaviour. We investigated TPS expression in specimens of WT and other paediatric renal malignancies PROCEDURE: Immunoreactivity of WT sections (n = 9), clear cell sarcomas (CCSK, n = 3), and a renal cell carcinoma (RCC), and two pediatric kidney tumour cell lines (WT: SK-NEP-1 and rhabdoid tumour of the kidney: G-401) were investigated using the monoclonal antibody M3. Additionally, immunoblotting and RT-PCR analysis were performed. Cell culture supernatants were evaluated for TPS release. Serum TPS was measured in five patients at diagnosis, during chemotherapy and after surgical resection. RESULTS: Moderate to strong immunoreactivity for TPS was found in tubular and blastemal components of nearly all (8/9) WT specimens. This was confirmed by Western-blotting. Cystic and epithelial-like portions of CCSKs and RCC showed distinct reactivity (3/3). The supernatant of G-401 but not of SK-NEP-1 showed a time- and cell number-dependent increase of TPS release. Interestingly, TPS synthesis was demonstrated in SK-NEP-1 cells. Median preoperative serum TPS was elevated (293 U/l) compared to healthy children and lowest after surgical resection (49.5 U/l). CONCLUSIONS: This is the first study demonstrating the synthesis and release of TPS by WTs and other paediatric renal malignancies. Considering the elevated levels of TPS in serum of these patients, a further investigation of this marker by larger clinical trials seems to be justified.

Bilateral Wilms' tumor--surgical aspects.

Four to eight percent of all Wilms' tumors are bilateral. Achieving curative resection of such tumors by partial nephrectomy or tumor enucleation while maintaining sufficient renal function represents a surgical challenge. Effective preoperative chemotherapy facilitates this aim considerably. Seven patients who were diagnosed to have bilateral synchronous Wilms' tumors between 1990 and 1994 are being reviewed. At the time of initial diagnosis, their mean age was 24.4 months, range 7 to 45 months. In 4 cases, pre-operative imaging did not reveal the full extent of the lesions, and in one of these, involvement of the second kidney was only detected by surgical exploration. Five of the children received at least 4 weeks of neo-adjuvant chemotherapy without primary biopsy, followed by bilateral tumor resection. One child had to be operated on as an emergency without any preliminary treatment, and in one, chemotherapy was interrupted after 3 weeks because of veno-occlusive disease. After a follow-up period of 66 months on the average (range 50 to 81 months), five of the patients are free of recurrence and clinically well--one with a kidney graft. The remaining two patients have died. Discussion is focussed on different management strategies of this rather rare pathology considering SIOP und NWTS protocols.

Solid-pseudopapillary tumor of the pancreas (Frantz tumor) in children: report of four cases and review of the literature.

BACKGROUND: Solid-pseudopapillary tumor of the pancreas (SPT) is an exceptionally rare neoplasm in children. Its origin remains enigmatic. It is of low malignant potential and occurs most frequently in young females. PATIENTS AND METHODS: A cumulative review of the tumor's clinicopathological characteristics from the world's literature is presented. The clinical course, pathohistologic data and outcome of surgery of four Austrian children treated at the general hospital of Vienna are analyzed. RESULTS: Between 1987 and 1999, four girls (age: 12--16 years) with SPT were diagnosed at our institution. All patients presented with an abdominal mass and uncharacteristic abdominal pain. Two tumors were located in the tail, one in the body and tail and one in the head of the pancreas (diameter: 7--15 cm). Surgical procedures included three distal pancreatectomies and one partial duodenopancreatectomy (Whipple procedure). One patient had two recurrences with metastases that could only be partially resected. Chemotherapy was initiated for this patient. In the follow-up period (range: 6 months to 12 years) all patients are alive with no evidence of recurrence. CONCLUSIONS: SPT is a rare differential diagnosis of a pancreatic mass in children. It is mandatory to establish this diagnosis since complete surgical removal of the tumor even in case of metastases or local invasion offers an excellent prognosis.

Open versus laparoscopic appendectomy in children: a comparison of complications.

Although between 4% and 20% of all appendectomies in adults are performed laparoscopically, this procedure is rarely done in the pediatric age group because of the substantially more difficult technique, the expected risks and suspected higher rate of complications. In a prospective study of 500 consecutive appendectomies, we tried to assess the actual rate of complications of each operative approach. We included 362 conventional and 138 laparoscopic appendectomies, the median age of the patients was 10.8 years. The mortality was 0 in both groups. We observed 89 minor and 11 major complications. All major complications (wound infections needing re-operation under general anesthesia, intra-abdominal abscesses, ileus due to adhesions and a case of renal insufficiency because of glomerulonephritis) occurred in the conventional group (n=11, ie, 3% of 362 open appendectomies). Seventy-two minor complications were seen in the same group (20%). In the laparoscopic group, there were 17 (13%) minor complications and no severe complications. This difference was statistically significant. We conclude that in children laparoscopic appendectomy does not carry a greater risk of intra- or postoperative complications and can therefore safely be established as a standard procedure.

[Progressive familial intrahepatic cholestasis (Byler disease): current genetics and therapy]. / Die Progressive Familiäre Intrahepatische Cholestase (Morbus Byler): Aktuelles zu Genetik und Therapie.

Progressive familial intrahepatic cholestasis (PFIC) is a congenital liver disease. First symptoms can frequently be seen shortly after birth. Quality and expectation of life are substantially reduced due to severe pruritus and the complications of progressive liver cirrhosis. PFIC is diagnosed on the basis of characteristic clinical and laboratory parameters and genetic analysis after exclusion of other liver diseases leading to intrahepatic cholestasis. Medical therapy is only effective in a proportion of children with PFIC. Partial biliary diversion (PBD) is nowadays considered the therapy of choice in patients with therapy-refractive pruritus. If performed in time, damage to the liver can be delayed or arrested, thus orthotopic liver transplantation (OLT) can be postponed or even avoided in at least some patients with PFIC. Besides providing a current overview of PFIC, we report on three patients who were successfully treated surgically. One patient was subjected to a new technique of PBD (cholecysto-appendicostomy), the other two had OLT.

CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients.

OBJECTIVE: The aim of this study was to describe the spectrum of abnormalities seen in generalized cystic lymphangiomatosis as shown by CT and MR imaging and to correlate these findings to gross pathology. CONCLUSION: MR imaging and CT may substantially broaden visualization of the spectrum of abnormalities seen in generalized cystic lymphangiomatosis by revealing the complete extent of disease and, thus, may contribute to clinical management of the disease by preventing initial misdiagnosis.

Perforating appendicitis. An indication for laparoscopy?

BACKGROUND: This pilot study was performed to reassess the widespread postulate that laparoscopic surgery is contraindicated in cases of perforating appendicitis. METHODS: A total of 75 children (appendiceal perforation: n = 67; perityphlic abscesses and peritonitis: n = 8) were analyzed in a prospective nonrandomized trial. Ten of them were treated by laparoscopic appendectomy. RESULTS: In the laparoscopy group, both pain and hospitalization were less time-consuming (i.e., by 50% and 19%, respectively). Antibiotics use was down from 2.6 over 6 days to 2.2. over 5.5 days, while the duration of surgery was up by 52%. Wound healing disturbances occurred in 10% (n = 1) and postoperative fever in 50% (n = 5) of patients, compared to 14% (n = 9) and 15% (n = 10) in the group treated by open surgery. All severe complications requiring reintervention (10%; massive subcutaneous abscess, n = 3; retrocolic abscess, n = 2; adhesion-related ileus, n = 3; appendicular stump, n = 1) were associated with open surgery. CONCLUSIONS: There was not a single major complication in the laparoscopy group. These unexpected results are in contrast to previous reports and have prompted us to initiate a prospective randomized trial.

Pulmonary echinococcosis (hydatidosis) in children: results of surgical treatment.

From 1986-1996, 33 children with 49 pulmonary hydatid cysts underwent surgical treatment in Vienna and Istanbul. Cysts were unilateral in 28 and bilateral in 5 cases; unruptured cysts (URC) were diagnosed in 19 patients, and 14 children presented with ruptured cysts (RC). Ten patients had cysts in other organs (liver, spleen, central nervous system) in addition to pulmonary cysts. Diagnosis was primarily based on chest X-ray and computed tomography scan. In Austrian children, a new combination of serological tests was used successfully (71% positive). The standard surgical procedure was cystotomy followed by capitonnage. The main postoperative complications were fever and wound infection. There were two recurrences after a mean follow-up of 4.8 years, and one patient died because of multiple organ involvement. We conclude that the therapy of choice in pediatric pulmonary hydatidosis is complete surgical elimination of the cyst by cystotomy and capitonnage, whereas more extended resections should be avoided. Ideally, benzimidazole treatment should be combined with surgery. New serological tests can improve diagnostic accuracy.

Diagnostic value of tissue polypeptide-specific antigen (TPS) in neuroblastoma and Wilms' tumour.

Although tissue polypeptide-specific antigen (TPS) has been described as a potentially useful serum marker of tumour activity in adult epithelial tumours, few data are available for childhood malignancies. Neuroblastomas and Wilms' tumours are the commonest types of solid malignancies found in the retroperitoneum of children. At this time, a widely used marker for Wilms' tumour is not available. Using an enzyme-linked immunosorbent assay (ELISA) kit, serum TPS levels in 23 children with neuroblastomas, nine with Wilms' tumours and 22 with benign tumours were evaluated to test the usefulness of the marker in identifying malignancies. Compared with healthy children (n = 110), the preoperative least-square means (LSM) of serum TPS were considerably elevated in both neuroblastoma (LSM = 209 U l(-1)) and Wilms' tumour (LSM = 235 U l(-1)), whereas values in benign tumours were only slightly elevated. Although the Wilms' tumours were associated with higher preoperative serum TPS levels, there was no statistically significant difference compared with neuroblastomas. Receiver operating characteristic analysis (ROC curves) showed a high sensitivity and specificity for both malignancies. Successful treatment resulted in decrease in TPS serum values. Serum TPS measurements in children presenting with abdominal masses can help in diagnosing the two commonest extracranial solid malignancies of childhood. Furthermore, TPS could acquire a pivotal role in monitoring therapy.

MR imaging in children with nonperforated acute appendicitis: value of unenhanced MR imaging in sonographically selected cases.

OBJECTIVE: The aim of this study was to describe the MR appearance of acute appendicitis and to determine the value of MR imaging for diagnosis of acute appendicitis. SUBJECTS AND METHODS: Forty-five children (28 girls, 17 boys), 7-16 years old (mean age, 13 years old), with clinically diagnosed acute appendicitis underwent independently graded compression sonography by two radiologists. MR imaging was performed when sonography revealed acute appendicitis (observer 1, 16 [36%] patients; observer 2, 18 [40%] patients), was inconclusive (observer 1, two [4%] patients; observer 2, one [2%] patient), and was interpreted as normal (observer 1, two [4%] patients; observer 2, one [2%] patient) (n = 20). Axial T1-weighted turbo spin-echo sequences, T2-weighted turbo spin-echo sequences in the axial and coronal planes, and fat-suppressed short inversion time inversion recovery turbo spin-echo sequences in the axial plane (4-mm slice thickness) were obtained and evaluated independently by two radiologists. The ability to see acute appendicitis with MR imaging was evaluated, the appearance and morphologic changes were described, and the most accurate sequence was determined. All children in whom MR imaging was performed underwent surgery. RESULTS: MR imaging revealed acute appendicitis in all cases (100%) by both observers. On T2-weighted ultra turbo spin-echo images, acute appendicitis appeared with a markedly hyperintense center, a slightly hyperintense thickened wall, and markedly hyperintense periappendiceal tissue. Unenhanced axial T2-weighted spin-echo imaging was the most sensitive sequence. CONCLUSION: In this study group, MR imaging was a valuable technique for depiction of acute appendicitis.

Retroperitoneal necrotizing fasciitis in a 4-year-old girl.

Necrotizing fasciitis is a rare but serious condition with a poor prognosis both in adults and in children. Retroperitoneal localization is mostly associated with fatal outcome. Early diagnosis, extensive and repeated surgical debridement, and use of antibiotics are necessary. Herein the authors report on a 4-year-old girl in whom retroperitoneal necrotizing fasciitis developed after she suffered from pyelonephritis. In this case, the outcome was favorable because of early surgical intervention, confirming the diagnosis.

Tissue polypeptide-specific antigen in pediatric patients: assessment of normal values.

Measurement of serum concentrations of tissue polypeptide-specific Antigen (TPS) has been demonstrated to the useful in diagnosis and monitoring of adult epithelial tumors. So far, no data have been available on normal or pathologic TPS values in children. Therefore, the present study was designed to evaluate the normal values of TPS in childhood. Using a commercial enzyme linked immunosorbent assay (ELISA) kit, serum TPS was determined in 361 healthy children. Median (M) TPS was found to be 107 U/l at birth (n = 124). By the end of the first week, the value rose to M = 150 U/l (n = 68) and then continuously decreased with age (1 week-1 year, n = 45, M = 88 U/l; 1-7 years, n = 75, M = 51 U/l) until reaching the adult level (8-18 years, n = 49, M = 34 U/l). Additionally, the serum TPS values of 45 mothers right after delivery (M = 161 U/l) were assessed, and there was no correlation to the marker levels determined in the cord blood of their children. The age-dependent distribution of serum TPS in healthy children must be taken into account in the clinical application of this tumor marker.

Tissue polypeptide-specific antigen (TPS) in pediatric malignancies.

Tissue Polypeptide Specific Antigen (TPS) may soon be routinely used as a proliferation marker in adult epithelial tumors. So far, no data have been available on normal or pathologic TPS values in children. Therefore the present study was designed to test the marker for the first time in pediatric malignancies. Using a commercial ELISA kit (Beki Diagnostics), serum TPS levels were determined in 270 healthy children and compared with various benign (n = 143) and malignant (n = 58) diseases. In healthy children, we found an age-dependent distribution of TPS values. Median (M) TPS was found to be 105.05 U/l at birth as determined from umbilical cord blood (n = 96). By the end of the first week, the value rose to M = 164 U/l and then continuously decreased with age until reaching the adult level at around 14 years. Patients with benign masses (n = 29) did not show elevated serum TPS in contrast to children with malignancies. Advanced tumor stage, metastases, and low dignity correlated with an increase in serum values. In many cases, chemotherapy and especially surgical resection of the tumor were followed by a decrease of the previously markedly elevated TPS levels. Severe infections and impaired renal function were however related with very high values and this must be taken into account when judging the validity of TPS measurements. The marker appears useful for the differential diagnosis between benign and malignant processes. While previous investigators described its use exclusively in epithelial tumors of adults, we cannot confirm this specifity in pediatric patients: Our data show that elevated TPS values in children can also be observed in nonepithelial tumors.

[Cardiovascular arrest after inhalation of leather glue]. / Herzkreislaufstillstand nach Inhalation von Schusterleim.

We are dealing with the rare case of a cardiac arrest of a 44 year old man, who has been using shoemakers glue. It could be proved in retrospect that the cause was the inhalation of trichlorethylene due to insufficient ventilating of the working place. The problem of this case was that at first the malign disorder of cardiac rhythm as well as the patient's state of coma did not provide any hint at the aetiology of the illness. By means of differential diagnosis we had to think of a myocardial infarction or a cerebral incident in the first place. Only by means of outside and self anamnesis could conclusions be drawn revealing a chronic poisoning at the working place. In particular an organic solvent, especially trichlorethylene as well as toluol was taken into consideration, the poisoning through trichlorethylene being proved conclusively. Other causes could be eliminated considering the clinical development and diagnostic parameter.