Perioperative management of circumcision in children: Is there a difference between African and European hospitals?

CONTEXT: The circumcision is the most frequent procedure in paediatric surgery worldwide, performed for medical and ritual purposes. In developing countries, because of the difficult accessibility to healthcare, even a common procedure could be unsafe. AIMS: The aim of the article is to compare the perioperative and anaesthesiological management of circumcision in children between two Italian and two sub-Saharan African hospitals. MATERIALS AND METHODS: Medical records of paediatric circumcision from January 2014 to December 2016 have been reviewed. The involved hospitals were: Padua (Italy), Ferrara (Italy), Sao José em Bor (Guinea Bissau) and Yaoundé (Cameroun). RESULTS: In Padua, 77 circumcisions were performed, 19 of these (24.6%) were ritual. In 75 children (97.4%), locoregional anaesthesia (LRA) together with sedation was used; only one complication (1.3%) occurred. In Ferrara, 200 interventions were done, 140 (70%) ritual; general anaesthesia was administered to 183 (93.5%) patients. There were five complications (2.5%). In Bissau, 53 procedures were performed, 21 (39.6%) ritual; in 34 children (64.1%), LRA with sedation was preferred. Two complications (3.8%) were reported. In Yaoundé, 60 children were circumcised, 15 (25%) for ritual purposes; in 51 (85%), only LRA was performed; there was only one (1.7%) complication. In the African hospital, no post-operative analgesia was administered. CONCLUSION: Despite the different anaesthesiological techniques, the study shows no difference in rate of complications for the in-hospital setting. Training of the local medical team in pain management and post-operative care should be emphasised.

Multicenter retrospective study on management and outcome of newborns affected by surgical necrotizing enterocolitis.

BACKGROUND: Necrotizing enterocolitis (NEC) is the most common surgical emergency in newborns and it is still a leading cause of death despite the improvements reached in the management of the critically ill neonate. The purpose of this study was to evaluate risk factors, surgical treatments and outcome of surgical NEC. METHODS: We retrospectively evaluated a multicentric group of 184 patients with surgical NEC over a period of 5 years (2008-2012). Indications to operation were modified NEC Bell stages IIIA or IIIB. The main outcome was measured in terms of survival and postsurgical complications. RESULTS: Data on 184 patients who had a surgical NEC were collected. The majority of patients (153) had a primary laparotomy (83%); 10 patients had peritoneal drainage insertion alone (5%) and 21 patients had peritoneal drainage followed by laparotomy (12%). Overall mortality was 28%. Patients with lower gestational age (P=0.001), lower birth weight (P=0.001), more extensive intestinal involvement (P=0.002) and cardiac diseases (P=0.012) had a significantly higher incidence of mortality. There was no statistically significant association between free abdominal air on the X-ray and mortality (P=0.407). Mortality in the drainage group was 60%, in the laparotomy group and drainage followed by laparotomy group was of 23-24% (P=0.043). There was a high incidence of stenosis (28%) in the drainage group (P=0.002). On multivariable regression, lower birth weight, feeding, bradycardia-desaturation and extent of bowel involvement were independent predictors of mortality. CONCLUSIONS: Laparotomy was the most frequent method of treatment (83%). Primary laparotomy and drainage with laparotomy groups had similar mortalities (23-24%), while the drainage alone treatment cohort was associated with the highest mortality (60%) with statistical value (P=0.043). Consequently laparotomy is highly protective in terms of survival rate. Stenosis seemed to be statistically associated with drainage. These findings could discourage the use of peritoneal drainage versus a primary laparotomy whenever the clinical conditions of patients allow this procedure.

Cyclosporine A in Ullrich congenital muscular dystrophy: long-term results.

Six individuals with Ullrich congenital muscular dystrophy (UCMD) and mutations in the genes-encoding collagen VI, aging 5-9, received 3-5 mg/kg of cyclosporine A (CsA) daily for 1 to 3.2 years. The primary outcome measure was the muscle strength evaluated with a myometer and expressed as megalimbs. The megalimbs score showed significant improvement (P = 0.01) in 5 of the 6 patients. Motor function did not change. Respiratory function deteriorated in all. CsA treatment corrected mitochondrial dysfunction, increased muscle regeneration, and decreased the number of apoptotic nuclei. Results from this study demonstrate that long-term treatment with CsA ameliorates performance in the limbs, but not in the respiratory muscles of UCMD patients, and that it is well tolerated. These results suggest considering a trial of CsA or nonimmunosuppressive cyclosporins, that retains the PTP-desensitizing properties of CsA, as early as possible in UCMD patients when diaphragm is less compromised.

Eosinophilic cystitis and nephrogenic adenoma of the bladder: a rare association of 2 unusual findings in childhood.

Neither eosinophilic cystitis nor nephrogenic adenoma is often diagnosed in children, with few pediatric cases being reported in the literature. Although these maladies share the same predisposing conditions, namely, chronic irritation or injury to the urothelium and lower urinary tract and symptoms such as dysuria, hematuria, and urinary frequency, their concomitance is exceptional. Herein, we report the case of an 8-year-old boy with a previous history of bladder surgery presenting histologically diagnosed eosinophilic cystitis and nephrogenic adenoma.

A case of portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy in a child.

We report the case of an 8-year-old boy with a red cell membrane disorder who developed, soon after undergoing laparoscopic cholecystectomy and splenectomy, complete thrombosis of the right branch and a partial occlusion of the left branch of the portal vein. The child was affected by a right hemiparesis because of a hypoxic-ischemic disorder that occurred in the first hours of life and was heterozygous for the methylenetetrahydrofolate reductase gene mutation 677C-T. Intravenous heparin and aspirin were initiated on postoperative day 7. Heparin treatment was switched to the subcutaneous route after the first 24 hours. The symptoms subsided 3 days after the beginning of treatment, whereas complete resolution of portal vein thrombosis was observed 2 months later. A review of the literature is reported, and the possible pathogenetic mechanisms underlying portal vein thrombosis are discussed.

Cystathionine beta-synthase c.844ins68 gene variant and non-syndromic cleft lip and palate.

Non-syndromic cleft lip with or without cleft palate (CL/P) is a common birth defect with substantial clinical and social impact and whose causes include both genetic and environmental factors. Folate and homocysteine (Hcy) metabolism have been indicated to play a role in the etiology of CL/P, and polymorphisms in folate and Hcy genes may act as susceptibility factors. We investigated a common polymorphism in the cystathionine beta-synthase (CBS) gene (c.844ins68) in 134 Italian CL/P cases and their parents using the transmission disequilibrium test (TDT). Although no overall linkage disequilibrium was observed, considering the parent-of-origin transmission of the CBS 68 bp insertion a significant (P = 0.002) transmission distortion was detected. When children receive the c.844ins68 allele from the mother compared to the father, they show a 18.7-fold increase in risk for CL/P. This evidence suggests CBS as a candidate gene for CL/P and supports a role of maternal-embryo interactions in the etiology of CL/P.