Paolo Gandullia

Esophageal Impedance/pH Monitoring in Pediatric Patients: Preliminary Experience with 50 Cases

This paper describes multiple intraluminal impedance (MII) in 50 children with typical and atypical gastroesophageal reflux (GER) symptoms and discusses the possible clinical significance of objective numeric data provided by MII computed analysis. Patients underwent 24-hr pH/MII monitoring. Reflux parameters were analyzed with relation to age and reported symptoms. Nonacidic MII events occurred as frequently as acidic ones. A Pathologic Bolus Exposure Index associated with a normal pH Reflux Index was detected in 26% of our series. Significant correlations were found regarding acid and bolus clearing times and their ratio.We conclude that the low rate of symptom occurrence in the pediatric population represents a limit on MII evaluation. Our study confirmed that nonacid GER is at least as frequent as acid GER. As MII provides interesting objective data that could be used in clinical practice, we suggest further research to define normal ranges in the pediatric population.

Laparoscopic vs open approach for the treatment of gastroesophageal reflux in children

BackgroundThe laparoscopic approach has become increasingly popular for fundoplication over the last few years; however many surgeons are skeptical about its real advantages.MethodsWe conducted a prospective comparative study of children operated on for gastroesophageal reflux (GER). Exclusion criteria included age <1 year and >14 years, previous surgery on the esophagus or stomach, and neurologic impairment. We compared two groups of patients who met the same inclusion/exclusion criteria. One group was treated via a laparotomic approach between January 1993 and December 1997; the other was treated via a laparoscopic approach between September 1998 and December 2000. A 360° wrap was performed in each group.ResultsGroup 1 (laparotomic approach) included 17 patients; mean operative time was 100 min and postoperative time was 7 days. Group 2 comprised 49 children operated on via a laparoscopic approach; mean operative time was 78 min and postoperative time was 48 hours. No major complications were encountered in either group. In postoperative period, two patients in group 1 had complications. One had a prolonged bout of gastroplegia, which required nasogastric drainage, and then recovered spontaneously after 20 days; the other had stenosis of the wrap, which required dilation. No relapses occurred during a follow-up of 6 months. Longterm follow-up data are not presented. Comparative analysis of the short-term functional results indicated that there were no differences between the two groups.ConclusionThis study confirms that the minimally invasive approach is safe and effective for the treatment of primary gastroesophageal reflux disease in children.

Long-term home parenteral nutrition in children with chronic intestinal failure: A 15-year experience at a single Italian centre

BACKGROUND AND AIMSnChronic intestinal failure is a condition causing severe impairment of intestinal functions; long-term total parenteral nutrition is required to provide adequate nutritional support.nnnMETHODSnThis is a 15-year follow-up study of paediatric patients with intestinal failure receiving long-term home parenteral nutrition.nnnRESULTSnThirty-six patients were included in the study, all aged <16 years. Total parenteral nutrition and home parenteral nutrition were administered respectively to 100.97 and 85.20 patients-year. Today, 12 out of 36 patients are still on parenteral nutrition. A total of 99 central venous catheters were inserted, for mean 2.75 catheters/patient. The overall incidence rates of catheter-related complications was 1.79 per 1000 days-catheter for sepsis and 3.37 per 1000 days-catheter for mechanical complications. Two multivariate Cox-models have been used to examine the role of some predictors for septic or mechanical complications. The only risk factor for septic complications was the indication for parenteral nutrition, and the only predictor of mechanical complications was the insertion period.nnnCONCLUSIONSnOur experience in the treatment of paediatric patients with gastrointestinal diseases confirms that long-term parenteral nutrition has become a safe and appropriate method in the treatment of severe chronic intestinal failure.

Laparoscopic approach for children with inflammatory bowel diseases.

BackgroundMinimally invasive surgery is being increasingly applied to inflammatory bowel diseases (IBDs). Few pediatric series from selected research have been described to date. This study describes a unicentric experience of laparoscopic treatment of children with IBDs.Materials and methodsAll consecutive patients with IBDs between February 2006 and February 2010 who underwent laparoscopic treatment were included. We reviewed notes and recorded demographic data, indications, perioperative management, surgical details, length of surgery, complications, postoperative management, length of hospitalization and functional outcome.ResultsWe performed 25 procedures on 16 patients (12 ulcerative colitis, 3 Crohn’s disease, and 1 indeterminate colitis). Median age was 12xa0years. A total of 50% patients underwent elective surgery; 11 underwent staged laparoscopic subtotal colectomy (LSTC) followed by J-pouch ileorectal anastomosis (JPIRA). Three patients underwent straight LSTCxa0+xa0JPIRA. All procedures included protective ileostomy. Length of surgery ranged between 120 and 380xa0min depending on the procedure (LSTCxa0±xa0JPIRA). No conversion was required. Length of hospitalization ranged between 3 and 18xa0days. We observed six complications (24%) mainly represented by adhesions that were effectively treated laparoscopically. Ten patients were restored (ileostomy closure) and were assessed for continence that turned out to be good in 80%.ConclusionsLaparoscopy proved to be feasible, safe and effective for the treatment of IBD in children. Although we observed a relatively low incidence of complications, stoma site adhesions still remain the major issue, which can be effectively dealt with laparoscopically. Functional outcome as well as cosmesis is satisfactory. As results are encouraging, at present we prefer laparoscopy for the surgical treatment of IBD in pediatric patients.

Multiple congenital anomalies, brain hypomyelination, and ocular albinism in a female with dup(X)(pter→q24::q21.32→qter) and random X inactivation

We report on an 18-month-old girl with multiple congenital anomalies (prominence of the metopic suture, fine hair, club foot, absence of the 12th rib, brachydactyly) and severe mental retardation. The funduscopic examination showed diffuse retinal hypopigmentation. Brain magnetic resonance image (MRI) showed signs of diffuse hypomyelination. On cytogenetic and molecular evidence, the karyotype was 46,X,dirdup(X) (pter-->q24::q21.32-->qter). The duplication of the PLP gene, involved in Pelizaeus-Merzbacher disease, was confirmed by fluorescent in situ hybridization (FISH). Both cytogenetic and molecular studies on the X chromosome inactivation status indicated a random pattern in lymphocytes and fibroblasts. This patient appears to be the first case of a female bearing a large duplication of Xq with a random X inactivation. The phenotype of this patient is compared to that of previously reported cases with Xq duplication.

Complications of percutaneous endoscopic gastrostomy in children: Results of an Italian multicenter observational study

BACKGROUNDnPercutaneous endoscopic gastrostomy is the preferred way to achieve an artificial feeding route for patients requiring long-term enteral nutrition. Although the procedure is well-standardized, it carries early and late complications.nnnAIMnTo establish the mortality and morbidity of this technique in a large cohort of children.nnnMETHODSnA multi-centre prospective clinical data collection from children undergoing percutaneous endoscopic gastrostomy tube implantation has been conducted from January 2004 to December 2007. Previous abdominal surgery was the only exclusion criterion. Follow-up visits were carried out at 1, 3, 6, 12, and 24 months after the procedure.nnnRESULTSn239 children (males, 55.2%; mean age 6.05±6.1years) were enrolled from nine tertiary Italian centres. Major complications occurred in 8 patients (3.3%). The cumulative incidence of complications was 47.7% at 24 months. The presence of thoraco-abdominal deformity was an independent predictor of complications at 12 months. No risk factors were identified in association to complications during the 1st tube replacement.nnnCONCLUSIONnIn children undergoing percutaneous endoscopic gastrostomy placement minor complications are common, while severe morbidities are rare. Accurate follow up is essential to recognize every complication, in particular when risk factors such as thoraco-abdominal deformity exist.

Childhood Thalidomide Neuropathy: A Clinical and Neurophysiologic Study

Thalidomide was recently reintroduced to treat several immune-mediated pathologies. Peripheral neuropathy is a significant side effect limiting its clinical use. Our aims include: (1) describing and identifying the incidence of clinical or electrophysiologic peripheral neuropathy in children, (2) determining whether peripheral neuropathy correlates with cumulative dose of thalidomide and with age, and (3) defining its reversibility rate. We studied 13 children manifesting immune-mediated pathologies treated with thalidomide at doses ranging from 25-100 mg/day. Clinical and neurophysiologic evaluation was performed before and after starting treatment. Seven children (53.8%) showed neurophysiologic signs of sensory peripheral axonal polyneuropathy. Five presented associated clinical symptoms, while the other two only presented subclinical, neurophysiologic signs of peripheral neuropathy. We found a significant correlation between the incidence of peripheral neuropathy and thalidomide cumulative dose (P = 0.02). We observed a lower incidence of peripheral neuropathy at a cumulative dose <20 gm, and a correlation with age (P < 0.01). The clinical and electrophysiologic recovery rate was 40%, and clinical improvement alone was observed in another 40%. Thalidomide induces dose-dependent and age-dependent peripheral neuropathy at a significant frequency in childhood (53.8%). In our experience a cumulative dosage at >20 gm and long-term administration for >10 months seem to increase the risk of peripheral neuropathy. We propose clinical and neurophysiologic follow-up every 3 months to identify and monitor possible side effects.

Chronic eosinophilic ascites in a very young child

A case of chronic eosinophilic ascites with onset in early infancy is described. An intensive diagnostic work-up ruled out other known causes of ascites in childhood. The final diagnosis was made at 2 years of age when a large number of eosinophils was detected in the ascitic fluid. The outcome was complicated by an ex vacuo intraperitoneal haemorrhage. Steroids were able to control the disease only after complete aspiration (1600xa0ml) of the ascitic fluid. On discontinuation of treatment, peritoneal inflammation recurred indicating steroid-dependency. Conclusion: eosinophilic ascites, a very rare disorder in children, should be considered in the differential diagnosis of even very young children presenting with ascites.

Medium-term results after cholecystectomy in patients younger than 10 years

Background: Gallbladder stones are a well-known and widely studied problem in children. Hematological disorders are the most common diseases that can cause cholelithiasis. However, in the last few years, the proportion of children with idiopathic cholelithiasis has increased 50%. Herein, we present a prospective study on laparoscopic cholecystectomy in a selected group of patients aged < 10 years. Methods: Fifty-eight patients aged < 10 years underwent laparoscopic cholecystectomy for stones in the period 1992‐99. The female/male ratio was 1.5, the mean age was 8 years (range, 2‐10), and the mean weight was 30 kg. In all patients, parenteral nutrition, fasting state, and prolonged use of antibiotics had been suspended for

Usefulness and safety of double endoscopy in children with gastroesophageal reflux and respiratory symptoms

6 months, and conservative treatment had been tried for