Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

INTRODUCTION: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. METHODS: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. RESULTS: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

Risk factors for adverse outcome in infancy in meconium ileus cystic fibrosis infants: A multicentre Italian study.

BACKGROUND: Meconium ileus (MI) is a risk factor for poor outcomes in cystic fibrosis (CF) patients. The aim of this study was to identify the risk factors for poor 12-month clinical outcomes in MI-CF newborns. METHODS: This retrospective, multicentre, observational study of MI-CF infants born 2009-2015 recorded their pre- and neonatal histories, intestinal occlusion treatments, post-surgical history, nutrition, CF diagnosis, and compared the patients with 12-month faltering growth or chronic Pseudomonas aeruginosa respiratory infection (cases) with the others (controls). RESULTS: About 25% of the 85 patients enrolled by 13 Italian CF centres (24% premature, 18% of low birth weight) had prenatally diagnosed bowel obstruction, and 39% had complex MI. Seventy-one required surgery (the 33 with complex MI and 38 with simple MI), of whom 58 (82%) required post-surgical intensive care, including 25 (35%) needing ventilatory support. Forty-six (54%) were breastfed; exclusively parenteral nutrition was started in 52 (61%). Cholestasis was diagnosed in 21%. Thirty-one (37%) experienced negative outcomes: the only risk factors were prenatally diagnosed intestinal obstruction and a need for intensive care and oxygen therapy. The cases had significantly higher first blood immunoreactive trypsinogen (b-IRT) levels (P = .008). Logistic regression showed that the probability of having negative outcome is decreased in the absence of cholestasis (Odds Ratio = 0.125) and a need for intensive therapy (OR = 0.141), and increased by not having been breastfed (OR = 2.921). CONCLUSIONS: High b-IRT levels, prenatally diagnosed intestinal obstruction, a severe post-surgical clinical picture and early liver disease are risk factors for negative outcomes. Breastfeeding may be protective.

Clinical manifestations and gastrointestinal pathology in 40 patients with autoimmune enteropathy.

Autoimmune enteropathy (AIE) is a rare condition that may affect pediatric and adult patients, frequently associated with primary immunodeficiencies. We performed a retrospective study on clinical and histological findings from 40 AIE patients. Histological presentation showed a prevalent celiac disease pattern (50%), followed by the mixed pattern (35%), independently of age, chronic active duodenitis (10%), and GVHD-like pattern (5%). Patients with primary immunodeficiencies (24/40) presented mainly with the celiac disease pattern (72.2% versus 22.2%; p < .0001), while patients without primary immunodeficiencies presented with a mixed histological pattern (61.1% versus 13.6%; p < .0001). Our study shows that the prevalent histological presentation is the celiac disease-like pattern, independently of age, and, for the first time, that the histological presentation of AIE differs significantly between patients with and without primary immunodeficiencies. These findings may be helpful for more precise and timely diagnosis and management of this rare disorder.

Heterotopic gastric mucosa in the cystic duct.

Heterotopic gastric mucosa in the gallbladder is rare (with about 100 reported cases), and its occurrence is even rarer in the biliary ductal system.The authors report a pediatric case of heterotopic gastric mucosa in the cystic duct associated with cholelitiasis.

Update on management of caustic and foreign body ingestion in children.

The following recommendations for management of caustic and foreign body ingestion in children have been developed following a multicentre study performed by the Italian Society for Paediatric Gastroenterology, Hepatology and Nutrition (SIGENP). They are principally addressed to medical professionals involved in casualty. Because there is paucity of good quality clinical trials in children on this topic, many of the recommendations are currently extrapolated from adult experiences or based on experts opinions. The document represents a level 2 to 5 degree of evidence (according to the Oxford Centre for Evidence-based Medicine Levels of Evidence), gathered from clinical experience, recent studies, and expert reports discussed during a consensus conference of the Endoscopic Section of the Italian Society of Paediatric Gastroenterology Hepatology and Nutrition. This working group comprises paediatricians, endoscopists, paediatric surgeons, toxicologists, and ENT surgeons, who are all actively involved in the management of these children. Recommendations are intended to serve as an aid to clinical judgement, not to replace it and therefore do not provide answers to every clinical question; nor does adherence to them ensure a successful outcome in every case. The ultimate decision on the clinical management of an individual patient will always depend on the specific clinical circumstances of the patient, and on the clinical judgement of the health care team.

Reversal of severe parenteral nutrition-associated liver disease in an infant with short bowel syndrome using parenteral fish oil (Omega-3 fatty acids).

Total parenteral nutrition is an important adjunct in the care of neonates with surgical disorders. Cholestasis is at present the most worrisome complication of this technique; it is difficult to treat and may progress to eventual cirrhosis and liver failure. This article reviews the pertinent clinical and nutritional data in a surgical patient with short bowel syndrome who developed parenteral nutrition-associated liver disease successfully treated with fish-oil based lipids.

Caustic ingestion in children: is endoscopy always indicated? The results of an Italian multicenter observational study.

BACKGROUND: The ingestion of caustic substances can represent a serious medical problem in children. OBJECTIVE: Whether or not an urgent endoscopy should be performed is still a matter of debate, particularly in asymptomatic patients. DESIGN: We conducted a multicenter observational study to investigate the predictive value of signs and symptoms in detecting severe esophageal lesions. SETTING AND PATIENTS: The records of 162 children who presented with accidental caustic substance ingestion were analyzed. INTERVENTIONS: Signs and symptoms were divided into minor (oral and/or oropharyngeal lesions and vomiting) and major (dyspnea, dysphagia, drooling, and hematemesis). An endoscopy was performed in all patients within 12 to 24 hours of the substance being ingested. MAIN OUTCOME MEASUREMENTS: The types of substance ingested, signs and symptoms, age, sex, and severity of esophageal injury were correlated. RESULTS: Mild esophageal lesions were identified in 143 of 162 patients (88.3%), and severe (third degree) esophageal lesions in 19 patients (11.7%). The risk of severe esophageal lesions without signs and/or symptoms was very low (odds ratio [OR] 0.13 [95% CI, 0.02-0.62], P = .002). Indeed, the presence of 3 or more symptoms is an important predictor of severe esophageal lesions (OR 11.97 [95% CI, 3.49-42.04], P = .0001). Multivariate analysis showed that the presence of symptoms is the most significant predictor of severe esophageal lesions (OR 2.3 [95% CI, 1.57-3.38], P = .001). CONCLUSIONS: The results demonstrated that the incidence of patients with third-degree lesions without any early symptoms and/or signs is very low, and an endoscopy could be avoided. The risk of severe damage increases proportionally with the number of signs and symptoms, and an endoscopy is always mandatory in symptomatic patients.

Management of cholelithiasis in Italian children: a national multicenter study.

AIM: To evaluate the management of Italian children with cholelithiasis observed at Pediatric and Surgical Departments linked to Italian Society of Pediatric Gastroenterology Hepatology and Nutrition. METHODS: One-hundred-eighty children (90 males, median age at diagnosis 7.3 years; range, 0-18 years) with echographic evidence of cholelithiasis were enrolled in the study; the data were collected by an anonymous questionnaire sent to participating centers. RESULTS: One hundred seventeen patients were treated with ursodeoxycholic acid; in 8 children dissolution of gallstones was observed, but the cholelithiasis recurred in 3 of them. Sixty-five percent of symptomatic children treated became asymptomatic. Sixty-four patients were treated with cholecystectomy and in only 2 cases a postoperative complication was reported. Thirty-four children received no treatment and were followed with clinical and echographic controls; in no case the development of complications was reported. CONCLUSION: The therapeutic strategies were extremely heterogeneous. Ursodeoxycholic acid was ineffective in dissolution of gallstones but it had a positive effect on the symptoms. Laparoscopic cholecystectomy was confirmed to be an efficacy and safe treatment for pediatric gallstones.

Diagnostic value of serological assays in pediatric inflammatory bowel disorders.

BACKGROUND: Pediatric studies reported that the combined use of the anti-neutrophil cytoplasm autoantibodies (ANCA) and the anti-Saccharomyces cerevisiae mannan antibodies (ASCA) may be a specific useful noninvasive test in the diagnosis of inflammatory bowel diseases (IBD). AIMS: To evaluate the diagnostic accuracy of ANCA and ASCA in children with suspected IBD, and to see whether different commercially available assays (indirect immunofluorescence vs. ELISA) agree well enough in terms of analytical performance. PATIENTS AND METHODS: Sixty-nine children (30 males, 39 females, age range 2-18 years) with suspicion of IBD entered the study. Before colonoscopy, a blood sample was also drawn to assess ASCA and ANCA. RESULTS: A diagnosis of IBD was established in 47 patients; the remainder had infective or other causes of colitis. For ulcerative colitis, the association ASCA-/ANCA+ had 70% sensitivity and 86% specificity, with a positive predictive value of 82%. The association ASCA+/ANCA- had 86% sensitivity and 93% specificity for Crohn's disease, with a positive predictive value of 75%. CONCLUSION: Although more experience is needed to state the diagnostic power of serologic assay, determination of ANCA and ASCA in IBD children may help both in distinguish these conditions from other entities and ulcerative colitis from Crohn's disease, particularly in doubtful cases.

Splenic cyst in a wandering spleen: laparoscopic treatment with preservation of splenic function.

BACKGROUND: Wandering spleen and splenic cyst are rare benign congenital conditions that can both cause severe complications related to torsion or trauma. CASE REPORT: A 14-year-old girl presented a mobile 10-cm-long abdominal mass in the left lower quadrant associated with mild abdominal pain. The diagnosis of an 8-cm-long nonparasitic cyst in a wandering spleen was confirmed by computerized tomography and negative serum indirect hemagglutination titer for hydatid disease. Laparoscopic unroofing of the cyst and splenopexy in a vycril mesh was performed. RESULTS: No problems were encountered during laparoscopic surgery. Postoperative course was uneventful, and at a 1-year follow-up, the spleen is viable and maintains a normal position in the phrenorenal angle. CONCLUSIONS: In the child, treatment of wandering spleen associated with a cyst should aim at the prevention of vascular accidents and at conservation of the spleen. We achieved these goals with unroofing and splenopexy through laparoscopic surgery.

Diversion of the fecal stream resolves ulcerative colitis complicating chronic granulomatous disease in an adult patient.

Chronic granulomatous disease (CGD) is a primary phagocytic disorder characterized by greatly increased susceptibility to severe bacterial and fungal infections. Patients with CGD may have gastrointestinal manifestations, commonly colitis, usually mimicking Crohn disease. We report an adult case, the second in literature, of CGD with severe colitis displaying histologic features of ulcerative colitis. The patient had a prompt improvement (continuing up to more than 5 y of follow-up) of the clinical picture after ileostomy and fecal diversion.

Further evidence on totally transanal one-stage pull-through procedure for Hirschsprung's disease.

BACKGROUND/PURPOSE: Fifteen consecutive children aged 20 days to 12 years with biopsy-proven Hirschsprung's Disease (HD) underwent a transanal pull-through procedure over a 17-month period. These patients have been divided into 2 groups. The first was a series of 9 patients, which helped us gain familiarity and confidence with technical and postoperative gestational problems, and the second series was of 6 patients, which fully corroborates and adds further evidence on the minimally invasive nature of the technique. Mucosectomy of aganglionic bowel, access to the peritoneal cavity, division of rectosigmoid mesenteric vessels, pull-through of normoganglionic colon, colectomy, and coloanal anastomosis all were performed transanally. Patients underwent a program of progressive anal dilatations and were assessed for postoperative clinical course, continence, constipation, diarrhea, postoperative enterocolitis, perianal excoriations, and anal stricture. RESULTS: Mucosectomy was done under direct vision. Operating time ranged from 150 to 350 minutes. The average length of bowel resected was 13.5 cm with a range of 8 cm to 25 cm. There were neither intraoperative nor significant postoperative complications. All but 2 patients accepted full oral feedings on postoperative day 2. Mean hospital stay in the first series of 9 patients was 7 days, range, 5 to 12 days; that of the second series of 6 patients was 5 days, range, 4 to 8 days. All children currently experience 1 to 6 bowel movements per day at a follow-up period of 1 to 17 months. CONCLUSIONS: A one-stage pull-through procedure for HD can be performed successfully with a completely transanal approach. This technique is associated with excellent early clinical results. Many more cases and a longer follow-up period will be required to compare long-term results with other one-stage procedures for definitive treatment of HD.

Correction of congenital chordee penis by 'ventral separation and outward rotation of corpora'.

BACKGROUND/PURPOSE: The formation of congenital chordee penis in patients with or without hypospadias is multifactorial and not completely clear. In most cases, after release of all known causes of chordee, "residual" penile curvature can persist. The authors discuss the etiopathogenetic mechanisms of congenital chordee penis and describe their experience in surgical correction of "residual" penile curvature by "ventral separation and outward rotation of corpora." METHODS: In 22 patients with congenital chordee penis with or without hypospadias, "residual" penile curvature was corrected by "ventral separation and outward rotation of corpora." RESULTS: Ventral separation and outward rotation of corpora resulted in satisfactory correction of penile curvature. In only 2 cases of chordee penis without hypospadias was further intervention necessary: a Nesbit's plication was carried out in one and a neourethra placed in the other. At follow-up no residual penile curvature was observed. CONCLUSIONS: An arrest of development of the penis caused by failure of outward ventral rotation of the corpora, may contribute to the formation of chordee penis. When "residual" penile curvature persists, "ventral separation and outward rotation of corpora" can be used to correct this complex malformation.