Ultrasound assessment of cranial spread during caudal blockade in children: effect of the speed of injection of local anaesthetics.

BACKGROUND: Despite caudal blockade being the most widely used regional anaesthetic procedure for infants and children undergoing subumbilical surgery, the question whether the injection velocity of the local anaesthetic itself affects its spread in the epidural space has not yet been investigated. Thus, the aim of the present study was to measure the cranial spread of caudally administered local anaesthetics in infants and children by means of real-time ultrasonography, with a special focus on comparing the effect of using two different speeds of injection. METHODS: Fifty ASA I-II infants and children, aged up to 6 yr, weighing up to 25 kg, undergoing subumbilical surgery, were enrolled in this prospective, randomized, observer-blinded study. Caudal blockade was performed under ultrasound observation using ropivacaine 1 ml kg(-1) 0.2% or 0.35% and an injection given at either 0.25 ml s(-1) or 0.5 ml s(-1), respectively. RESULTS: Ultrasound observation of the local anaesthetic flow and the extent of cranial spread was possible in all patients. All caudal blocks were considered successful, and all surgical procedures could be completed without any indications of insufficient analgesia. No statistically significant difference could be observed between the two injection speeds regarding the cranial spread of the local anaesthetic in the epidural space. CONCLUSIONS: The main finding of the present study is that the speed of injection of the local anaesthetic does not affect its cranial spread during caudal blockade in infants and children. Therefore, the prediction of the cranial spread of the local anaesthetic, depending on the injection speed, is not possible.

Decreased interferon-gamma response in respiratory syncytial virus compared to other respiratory viral infections in infants.

An inappropriate interferon-gamma response has been implicated in the pathogenesis of severe respiratory syncytial virus (RSV) lower respiratory tract illness (LRTI). To assess whether this is unique for RSV primary LRTI compared to a first non-RSV LRTI, intracellular interferon-gamma was determined by flow cytometry in peripheral blood mononuclear cells from 32 infants with a primary RSV infection, 28 with a first non-RSV LRTI due to adenoviral, parainfluenzaviral and rhinoviral infection and 13 healthy infants. Interferon-gamma responses were increased significantly during adenoviral, parainfluenzaviral and the majority of the rhinoviral infections, but remained low during RSV and severe rhinoviral infection. Low interferon-gamma responses were associated with a more severe clinical course of LRTI. This indicates that depending on the nature of the viral pathogen, respiratory virus infections in infants differ significantly with regard to the quantity of the interferon-gamma production and that this may contribute to the clinical course of the disease.

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.

[Chest X-ray of the neonate]. / Thoraxröntgen beim neonatologischen Patienten.

In diagnostic imaging of thoracic pathologies in mature and especially immature neonates, chest X-ray has a leading position. Profound knowledge of the normal chest X-ray and the potential physiological perinatal changes is the basic requirement for interpretation of the X-ray of a neonate. Childhood pathology: Many congenital and acquired diseases that the radiologist is faced with in neonatology are unknown in the imaging of adults. Many of these changes are life-threatening or may have an impact on the patient's future quality of life. Therefore early diagnosis in close cooperation with the paediatrician is essential. We give here an overview of the most important pathologic changes that the radiologist may be confronted with in daily routine.

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.

Reduced interferon-gamma expression in peripheral blood mononuclear cells of infants with severe respiratory syncytial virus disease.

We examined the in vivo cell-mediated immune response in infants with respiratory syncytial virus (RSV) infection in order to gain information about the pathogenesis of severe RSV disease in infancy. Semiquantitative reverse transcription-polymerase chain reaction and three-color flow cytometry were used to determine the levels of messenger RNA (mRNA) for interferon (IFN)-gamma in peripheral blood mononuclear cells, and the distribution of lymphocyte subsets in infants with acute RSV infection. The findings were correlated with the severity of the patients' illness and the production of RSV-specific IgE antibodies (RSV-IgE). Significantly lower IFN-gamma levels and T-lymphocyte counts in the acute phase of illness were observed in infants with severe RSV disease than in those with a milder clinical course of illness. The induction of RSV-IgE was not related to IFN-gamma levels in the acute phase of illness, but rather correlated with IFN-gamma expression during convalescence. The data indicate that reduced IFN-gamma expression may be an important factor in the pathogenesis of severe RSV disease in infancy.

Successful conservative treatment of severe renal candidosis with fungus balls.

Partial fungal obstruction of the renal collecting system is an unusual finding among infants that poses specific management problems. We report a patient with sepsis and fungal infection of the kidneys post surgery who presented with bilateral fungus balls and was successfully managed by conservative measures. Sonography is the imaging technique of choice in the diagnosis and follow-up of such patients. The need for prompt diagnosis in high-risk patients and the role of sonography are discussed.

Prognostic significance of tumor angiogenesis in primary fallopian tube cancer.

Tumor angiogenesis has been found to be prognostically significant in many types of malignant tumors. We assessed tumor vascularity in 43 cases of histologically proven primary fallopian tube cancer, FIGO stage I-IV, using the highly specific endothelial cell marker CD34. Microvessel count was determined by counting CD34-positive cells at 200 x magnification. The 5-year disease-free survival probability was 43.8% (+/- 11.5%) in 24 patients whose tumors had a microvessel count < or = 19 microvessels/field and 19.7% (+/- 9.5%) in the > 19 microvessels/field group (P = 0.046). Stage and microvessel count were statistically significant for disease-free survival in univariate analysis. Therefore, a larger sample size would be required to detect an independent and statistically significant prognostic effect of microvessel density in primary fallopian tube cancer in multivariate analysis.

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.

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.

Neurenteric cyst mimicking pleurodynia: an unusual case of thoracic pain in a child.

This study reports an 8-year-old boy with chronic recurring thoracic pain. Magnetic resonance imaging of the spine revealed an intradural cyst at T1-T2. Despite severe compression of the spinal cord, there was no neurologic deficit. After minimally invasive cystectomy, the patient recovered completely. Histological examination established the diagnosis of a neurenteric cyst. The clinical, pathohistologic, and radiologic aspects are discussed, and the decisive role of magnetic resonance imaging in the diagnosis of intraspinal cyst is emphasized.