Comparison of environmental risk factors for esophageal atresia, anorectal malformations, and the combined phenotype in 263 German families.

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and anorectal malformations (ARM) represent the severe ends of the fore- and hindgut malformation spectra. Previous research suggests that environmental factors are implicated in their etiology. These risk factors might indicate the influence of specific etiological mechanisms on distinct developmental processes (e.g. fore- vs. hindgut malformation). The present study compared environmental factors in patients with isolated EA/TEF, isolated ARM, and the combined phenotype during the periconceptional period and the first trimester of pregnancy in order to investigate the hypothesis that fore- and hindgut malformations involve differing environmental factors. Patients with isolated EA/TEF (n = 98), isolated ARM (n = 123), and the combined phenotype (n = 42) were included. Families were recruited within the context of two German multicenter studies of the genetic and environmental causes of EA/TEF (great consortium) and ARM (CURE-Net). Exposures of interest were ascertained using an epidemiological questionnaire. Chi-square, Fisher's exact, and Mann-Whitney U-tests were used to assess differences between the three phenotypes. Newborns with isolated EA/TEF and the combined phenotype had significantly lower birth weights than newborns with isolated ARM (P = 0.001 and P < 0.0001, respectively). Mothers of isolated EA/TEF consumed more alcohol periconceptional (80%) than mothers of isolated ARM or the combined phenotype (each 67%). Parental smoking (P = 0.003) and artificial reproductive techniques (P = 0.03) were associated with isolated ARM. Unexpectedly, maternal periconceptional multivitamin supplementation was most frequent among patients with the most severe form of disorder, i.e. the combined phenotype (19%). Significant differences in birth weight were apparent between the three phenotype groups. This might be attributable to the limited ability of EA/TEF fetuses to swallow amniotic fluid, thus depriving them of its nutritive properties. Furthermore, the present data suggest that fore- and hindgut malformations involve differing environmental factors. Maternal periconceptional multivitamin supplementation was highest among patients with the combined phenotype. This latter finding is contrary to expectation, and warrants further analysis in large prospective epidemiological studies.

Randomized controlled trial of taurolidine citrate versus heparin as catheter lock solution in paediatric patients with haematological malignancies.

BACKGROUND: A catheter lock solution containing 1.35% taurolidine and 4% citrate could potentially disrupt bacterial surface adherence and consecutive biofilm production due to the anti-adherence properties of taurolidine and the anticlotting and chelator activities of both compounds. AIM: To compare the impact on microbial catheter colonization and infectious complications of heparin and taurolidine citrate as central venous catheter (CVC) lock solutions in paediatric patients with haematological malignancies. METHODS: Seventy-one patients aged 1.4-18 years were randomized to two treatment groups using either heparin (N = 36) or taurolidine citrate (N = 35). Infectious complications and clinical side-effects were prospectively monitored and microbial colonization of catheters was assessed at the time of removal. FINDINGS: There were two bloodstream infections in the taurolidine citrate group versus nine in the heparin group (0.3 vs 1.3 infections per 1000 catheter-days; P = 0.03). Fever of unknown origin and catheter occlusions were observed with a similar frequency in both groups. Microbial colonization was found in 25.4% catheters. The time of no-lock use, but not the type of lock solution or time of observation, was a significant predictor of catheter colonization (P = 0.004). Colonization was not observed in CVCs used immediately with taurolidine citrate lock. Seven patients in the taurolidine citrate group (20%) experienced side-effects (nausea, vomiting, abnormal taste sensations). CONCLUSION: The use of taurolidine citrate lock solution was associated with a significant reduction in bloodstream infection in immunocompromised paediatric patients. Taurolidine citrate may prevent colonization of CVCs if used from the time of insertion, but not after a period of no-lock catheter use.

High reliability of scrotal ultrasonography in the management of childhood primary testicular neoplasms.

BACKGROUND: The role for testis-sparing surgery in the treatment of primary intratesticular lesions in childhood is growing. The reliability of scrotal ultrasonograpy (US) in the management of these lesions is still controversial. PATIENTS: Between 1991 and 2007, 383 children and adolescents presented with testicular abnormalities. Ultrasound results and records of patients with primary testicular neoplasms were analyzed. RESULTS: 12 of 383 patients (3.1%) had a histologically proven primary intratesticular neoplasm. Scrotal US was highly sensitive for the detection of these lesions. Patients' mean age at initial US was 6 years (9 prepubertal, 3 juvenile patients). The most frequent symptom was a painless unilateral scrotal mass (75%). Tumor markers or testosterone were elevated in 6/12 boys. Histology was intratesticular germ cell in 7, sexcord stromal tumor in 4 and capillary hemangioma in 1 patient. US correctly distinguished between benign and malignant lesions in all cases. When combined with clinical symptoms, US predicted 75% of histologies. After including hormone and tumor marker levels, a correct preoperative diagnosis was made for all boys with germ cell, and for 75% of boys with sexcord stromal tumor. CONCLUSION: Scrotal US is highly sensitive for the detection of childhood primary intratesticular tumors and, when combined with clinical data, highly reliable for differential diagnosis. It may help clinicians to decide when to opt for testis-sparing surgery.

The effect of surgery on lung volume and conventional monitoring parameters in ventilated newborn infants.

In newborn infants, thoraco-abdominal surgery is a serious intervention with respect to gas exchange and lung mechanics. This prospective clinical study compared surgery-induced changes in functional residual capacity (FRC) and ventilation inhomogeneity (VI) indices with changes in conventional monitoring parameters. Of 29 ventilated newborns (mean weight 2,770+/-864 g at surgery), 13, nine and seven underwent thoracic, abdominal or congenital diaphragmatic hernia (CDH) surgery, respectively. The multiple breath washout (MBWO) technique using heptafluoropropane as tracer gas (Babylog 8000; Dräger, Lübeck, Germany) was performed <6 h before surgery, 22-24 h after surgery and <6 h before extubation. Gas exchange, respiratory mechanics, FRC and VI index data were recorded. Thoraco-abdominal surgery resulted in changes to FRC and VI indices in a procedure-specific manner; however, these changes were not reflected in conventional mechanical or ventilatory monitoring parameters. FRC decreased in non-CDH infants, while FRC increased and VI indices decreased in CDH infants. Despite improvements, the differences in FRC and VI between CDH and non-CDH infants indicated persistent impaired lung function in CHD infants. MBWO can be advantageously used to measure the effect of surgery on the lung. While FRC and VI indices changed following surgery, conventional monitoring parameters did not.

Reduction of postoperative adhesions by perfluorocarbons: an experimental study in a rat model.

Peritoneal adhesions are a well-known and frequently occurring postoperative complication. Many published studies have looked into the prophylaxis of adhesions following abdominal surgery, but only few clinically relevant agents have been reported. Most publications refer to adult patients and not to paediatric patient collectives. This experimental study in a rat model compares the effect of perfluorocarbons as adhesion prophylaxis with those of a well-known anti-adhesive agent Adept and with an untreated control group. We hypothesized that PFC might have a double effect: initially it could suppress the accumulation of monocytes and neutrophilic granulocytes, and subsequently it would work as a barrier to prevent contact between the visceral and parietal layers of the peritoneum. After a standardised operation, PFC was injected into the abdominal cavity of rats in the study group. Macroscopically, the PFC group did not fare significantly better, but nevertheless a clear tendency towards fewer adhesions after the application of PFC could be ascertained.

Paediatric paranasal sinus mucoceles.

Mucoceles of the paranasal sinuses are extremely rare in children and adolescents and most cases described in the literature are associated with cystic fibrosis. The condition is potentially dangerous but frequently diagnosed late or inaccurately due to its non-specific symptoms or to an absence of inflammation parameters or other clinical signs. We present 3 children with mucoceles (sphenoid sinus n = 2, ethmoid sinus n = 1) in whom cystic fibrosis was discounted and who were managed in our medical centre during the last 3 years. No aetiological factor was identified in the 2 cases of sphenoid mucocele. The main symptom in these two patients was therapy-resistant cephalgia which shifted location and varied in intensity. One child had had recurrent sinus infection which was a possible aetiological factor of the ethmoidal mucocele. All patients successfully underwent endoscopic sinus surgery. During a 2-year follow-up, all patients have remained free of symptoms and no mucocele recurrence has been observed so far. The rareness of paranasal sinus mucoceles in children, in particular its sphenoid occurrence, coupled with its relatively non-specific symptomatology prompted the authors to outline and discuss the aetiological factors, clinical findings, and therapy and to review the literature.

Huge intra- and extrathoracic lymphangioma in a baby successfully treated by sclerotherapy with OK-432.

Lymphangiomas are rare congenital malformations of the lymphatic system. They typically appear within the first two years of life. They present as soft, indolent and benign tumors. As they have a tendency to grow in an infiltrating manner and rarely regress spontaneously, treatment may be necessary if the lymphangioma's location is functionally and cosmetically disturbing. Surgical therapy, which has been the treatment of choice so far, includes a risk of complications, depending on the location and extent of the tumor. Therefore alternative treatments such as sclerotherapy, especially with OK-432, have been developed. We present the case of a female baby with a huge macrocystic lymphangioma, extending over one half of the trunk, located intra- and extrathoracically, which was treated successfully by surgical resection and several intralesional injections of OK-432.

Echogenic foci in the dilated fetal colon may be associated with the presence of a rectourinary fistula.

Intraintestinal meconium calcification in the fetus and newborn is a rare finding, occurring in patients with intestinal obstruction. The intraluminal location of the enterolithiasis enables differentiation from calcifications that are secondary to bowel perforation and meconium peritonitis. We report on a term male neonate with VACTERL (vertebral defects, imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association and rectourethral fistula, in whom enterolithiasis was documented by prenatal ultrasound imaging at 21 weeks' gestation. A review of the literature and a summary of all previously described cases of neonates with intraluminal meconium calcifications are provided. Prenatal ultrasonographic detection of enterolithiasis in fetuses with anal atresia may indicate the presence of an enterourinary fistula.

[The VIP-secreting tumor as a differential diagnosis of protracted diarrhea in pediatrics]. / Der VIP-produzierende Tumor in der Pädiatrie als Differenzialdiagnose der protrahierten Diarrhö

BACKGROUND: Vasoactive intestinal peptide (VIP) can be produced by mature neurogenic tumors. Pathologically elevated VIP plasma levels cause secretory diarrhea with excessive loss of water and electrolytes. Despite the clinical severity diagnosis of a VIP-secreting tumor is often delayed and subsequently its extirpation as the mainstay of therapy. PATIENTS: We report on two patients with ganglioneuroblastoma and secretory diarrhea. We contrast the case of a 13-month-old boy with advanced symptoms of secretory diarrhea, high VIP plasma levels, and late diagnosis to the case of a 14-month-old boy with mild secretory diarrhea and normal VIP plasma levels but positive proof of VIP in tumor tissue. Reviewing the literature we found 57 cases of pediatric VIP-secreting tumors. RESULTS: The clinical situation is characterized by the typical symptoms of secretory diarrhea with hypokalemia and metabolic acidosis. Histopathology predominantly reveals ganglioneuroblastoma or ganglioneuroma. The symptoms mostly stop after complete resection of the tumor whereas lack of resection is associated with elevated mortality rates. CONCLUSIONS: In case of prolonged therapy-resistant secretory diarrhea the existence of a VIP-secreting tumor should be considered. Diagnostic work-up should include the assessment of VIP plasma levels, catecholamines in urine, and appropriate imaging techniques in order to rule out or confirm the possibility of a VIP producing tumor.

[Sonographic detection of a "VIPoma" in a small child with intractable gastroenteritis]. / Sonographische Detektion eines "VIPoms" bei einem Kleinkind mit therapieresistenter Gastroenteritis.

As case report we describe a rare cause of intractable "gastroenteritis" detected by ultrasonography. The 14 months-old boy was admitted to hospital because of intensive dehydration due to massive vomiting and diarrhoea. A salmonella enteritis with intractable hyponatraemia and hypokalaemia was thought to be the cause. After a dramatic relapse during oral treatment measures, further extensive laboratory tests finally disclosed an elevated serum level of vasoactive intestinal polypeptide ("VIP"). The VIP secreting tumor ("VIPoma") was detected ultrasonographically in a retroperitoneal localization mediocaudally of the right kidney. Diffuse distinct calcifications and an increased perfusion could be demonstrated. Intraspinal tumour spread was excluded by magnetic resonance imaging. After complete surgical removal of the tumour the clinical symptomatology normalized promptly and permanently. A VIP-excreting ganglioneuroblastoma with low grade growth fraction ("VIPoma") was diagnosed histologically. Common gastroenteritis in childhood represents no indication for ultrasound. In cases of unclear and therapy-resistant symptomatology, however, diagnostic work-up should include ultrasonography to search for retroperitoneal or pancreatic VIP-excreting tumours.

Latex allergy in pediatric surgery is dependent on repeated operations in the first year of life.

BACKGROUND/PURPOSE: Patients with spina bifida represent the highest risk group for hypersensitivity to latex. Recognized risk factors for these patients are repeated surgery and an atopic disposition. Little is known about children operated on in the first year of life for reasons other than spina bifida. METHODS: Eighty-six patients (mean age, 10.2 years) with gastrointestinal or urologic surgery were investigated for the number, type, and date of surgical interventions. Additionally, skin prick tests and provocation tests were performed to classify sensitized and symptomatic latex-allergic individuals. RESULTS: Twenty-seven patients were regarded as sensitized to latex (31.4%). Twenty patients were classified as being atopic (25.6%). Atopic patients were significantly more often sensitized and provocation positive compared with nonsensitized and provocation-negative ones (P <.01). Children already operated on in the first year of life (n = 44) with a positive provocation showed significantly higher latex-specific IgE-values than individuals with a negative outcome (P <.0001). The total number of operations and degree of sensitization showed a significant correlation; more than 8 surgical interventions during the first year of life significantly increased the risk of clinically relevant allergy to latex. CONCLUSION: This study emphasizes that individuals undergoing surgical interventions during infancy should be handled latex free from the very beginning of life.

[Is the Bishop-Koop anastomosis in treatment of neonatal ileus still current?]. / Ist die Bishop-Koop'sche Anastomose bei der Behandlung des Neugeborenenileus noch zeitgemäss?

Fourty-three cases of ileus in newborns are presented. Twenty-seven newborns received a Bishop-Koop anastomosis. In 19 cases, the Bishop-Koop anastomosis was performed primarily and in 8 cases as a second intervention. We consider the Bishop-Koop anastomosis to be a safer procedure than primary end-to-end or end-to-side anastomosis. Only one anastomotic leak occurred in our patients. We prefer the Bishop-Koop anastomosis not only in cases of meconium ileus, but also in other types of intestinal atresia and stenosis, especially for the management of greatly different intestinal diameters. In our experience, this method is also suitable for re-anastomosing a double-barrel anastomosis. The Bishop-Koop procedure minimizes the risks of primary anastomosis without enterostoma, and later extraperitoneal closure of the stoma is easy and safe.