A case of double cystic esophageal duplication in VACTERL syndrome: the first case report and a review of the literature.

Background: An esophageal duplication cyst (EDC) is a rare malformation resulting from the embryonic foregut. VACTERL syndrome is a genetic disorder affecting many systems of the human body. We report the first case of VACTERL syndrome associated to asymptomatic double EDC. Case report: A girl with anorectal malformation and rectovestibular fistula, kidney malformation, and various vertebral defects came to our attention at the time of birth. VACTERL disease was diagnosed. She underwent Peña anoplasty at 4 months of life without complications. MRI was conducted at the age of 2. It accidentally showed a double esophageal duplication (12 mm × 35 mm × 10 mm) at the D7-D9 level. We planned a thoracoscopy; previous intraoperative esophagogastroduodenoscopy showed an external compression of the native esophagus. Two duplicated esophageal lesions were removed. The patient made an uneventful recovery and was completely asymptomatic at long-term follow-up. Conclusions: VACTERL syndrome is still a not well-defined disease. Based on the current literature, this is the first case of a double esophageal duplication in a patient affected by VACTERL syndrome. According to us, the thoracoscopic approach of esophageal duplications can be followed by experts. Complete surgical excision is possible even if the cyst shares a common muscular wall with the esophagus. For this reason, we suggest to close the muscular wall by a simple interrupted suture.

Onabotulinum toxin A in children with refractory idiopathic overactive bladder: medium-term outcomes.

INTRODUCTION: Botulinum toxin-A (BtA) has been used for refractory idiopathic overactive bladder (IOAB) in children. Data on the optimum dose success rates, duration of effect, complications and medium-term outcomes are limited. This study aims to analyse the authors' experience to provide medium-term results of BtA in symptomatic refractory patients. MATERIALS AND METHODS: Patients with refractory IOAB who were treated with BtA (Botox®) were retrospectively analysed. All patients had urodynamic study before treating with BtA. Group A had low-dose BtA (LDBtA) at 5 units/kg (maximum 150 units), and Group B had high-dose BtA (HDBtA) at 10 units/kg (maximum 300 Units). Post-BtA clinical response, functional bladder capacity (FBC) and postvoid residual (PVR) were assessed in addition to the duration of response. RESULTS: Thirty-nine patients, 11 male and 28 female, were analysed. Forty-six percentage had symptom improvement (73% of males and 36% of females [P = 0.072, ns]). The difference in response rates between LDBtA and HDBtA was not statistically significant (P = 0.684). Increase in total bladder capacity (TBC) was greater in those given HDBtA (P ≤ 0.001), but the increase in FBC was not different between the groups, due to greater PVRs in the HDBtA group. Nine patients (23%) developed UTI; however, six of these patients suffered with UTI pre-BtA as well. Only three were asymptomatic after a single treatment with BtA. The remainder required further BtA or oral anticholinergic therapy. At a median follow-up of 35.4 months (interquartile range [IQR] 25.2-46.6), 12 (31%) were asymptomatic and off all therapy, 18 (46%) were still symptomatic despite therapy and 9 (23%) had their symptoms controlled on continuing treatment. DISCUSSION AND CONCLUSIONS: Botulinum toxin-A improves symptoms in 46% of children after the first injection in refractory IOAB. Although HDBtA resulted in greater increase in bladder capacity, it conferred no advantage in terms of success rate or duration of response. Five units/kg may be an optimum dose to use as a first treatment with the understanding that some patients will require a higher dose. And, there will be a cohort of patients who need a dose lower than 5 units/kg. A higher dose is more likely to lead to PVR leading to urine stasis and UTIs. The success of BtA only lasts until its effect wears off, and the majority of this cohort (36/39) required continuing treatment with repeat BtA or anticholinergic agents. However, it remains a useful option in patients who are intolerant or unresponsive to anticholinergic medication with symptomatic resolution in 30% at medium-term follow-up.

Isolated Vaginal Agenesis Associated with Multiple Gastrointestinal Anomalies: A Case Report.

More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature.

Association of Duodenal Atresia, Malrotation, and Atrial Septal Defect in a Down-Syndrome Patient.

Duodenal atresia is the frequent cause of neonatal intestinal obstruction. The association between duodenal atresia, intestinal malrotation, cardiac anomalies and Down syndrome is infrequently reported. We present a prenatally suspected case of duodenal atresia which was associated with malrotation and atrial septal defect in a patient of Down syndrome. Duodenotomy and resection of web was performed in addition to Ladd's procedure. Postoperative course remained uneventful.

Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy.

BACKGROUND: Bladder exstrophy and epispadias are severe congenital anomalies associated with an open bladder and urinary sphincter. Despite modern reconstruction, there is a significant incidence of residual or recurrent urinary incontinence that impacts on quality of life (QoL) and self-esteem, which in turn limits social interaction (Figure). The present study involved 14 patients, mainly from a Middle Eastern country, and reported the early findings with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique for both urinary and faecal control. STUDY DESIGN: Fourteen children, with a median age of 8.1 years, with poor quality of life and low self-esteem because of urinary incontinence and small polypoidal open bladders of 5-15 ml volume, mostly after bladder exstrophy surgery, were managed with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique keeping an intact anal sphincter. The retrorectal pulled-through colon was anastomosed to the posterior wall of the rectum just above the external sphincter complex, thereby avoiding any possible injury to the anal sphincter. All patients had a normal colon and a competent anal sphincter without lumbosacral spinal or nerve anomalies. RESULTS: Ten children had a 5- to 10-year follow-up, one child had a 15-year follow-up, and three others, that were also continent, were excluded because of a <5-year follow-up. There were no postoperative complications, and all were dry and odour-free by day within 2-4 weeks of surgery. Two children still had minor urinary loss at night. There were no UTIs and renal function remained unimpaired. Eleven years after surgery, one child underwent excision of a pedunculated benign inflammatory polyp from the tip of the left ureter because of recurrent torsion and bleeding, there was no recurrence at the 2-year follow-up. None of the rectal or ureteric biopsies from any of the children showed metaplasia or neoplasia; however, in view of the potential long-term risks, all children were placed on a lifelong 'proctoscopy and biopsy' protocol. DISCUSSION: The ability to be dry and odour-free, and to wear normal clothing had a striking impact on QoL and psychological well-being of the children and their families. This was reflected in their positive overall approach, voluntary school attendance, and enthusiastic participation in communal events. All agreed that their improved genital appearance markedly contributed to their better body image and increased self-esteem. CONCLUSION: These significant benefits, at a crucial time in the child's life, outweigh the potential risk of long-term neoplasia. Therefore, the Heitz-Boyer-Hovelacque rectal bladder technique is recommended with long-term proctoscopic follow-up.

Herlyn-Werner-Wunderlich syndrome: An "early" onset case report and review of Literature.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital mullerian anomaly consisting of uterus didelphys, hemivaginal septum, and unilateral renal agenesis [1,2]. Most authors reported cases of Herlyn-Werner-Wunderlich syndrome with prepuberal or postpuberal onset with cyclical abdominal pain and a vaginal mass (3-8). Only six cases are reported in Literature with early onset of this syndrome under 5 years (9-14). Our case is about 3 years old girl, with all the features of this syndrome who came to our attention for lower abdominal mass. The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment. The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis. It is common, in fact, an error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. For all these reasons, our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cystoscopy and vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis.

Esophagogastric dissociation reduces the re-operation rate for persistent gastroesophageal reflux in severely neurologically impaired children.

PURPOSE: In this study we want to demonstrate the effectiveness of the esophagogastric dissociation as a first level operation in treatment of the gastroesophageal reflux in severe neurologically impaired children, in term of a reduction of reoperation rate. METHODS: We divided patients operated from 1998 to 2005 in a group A, composed by children treated with fundoplication, and in a group AR, composed by the patients of group A who had a recurrence of reflux and that was treated with esophagogastric dissociation. Patients operated from 2005 to 2013 were selected on the basis of the severity of the neurological impairment and were divided in a group B, treated with fundoplication, and in a group C of more severe impaired children, treated with esophagogastric dissociation. Data regarding the complications of the A and C groups were analyzed with Fisher's test. RESULTS: We evaluated 63 patients: 34 (54%) in group A, 11 in group AR, 15 (23.6%) in group B, 14 (22.4%) in group C. The Fisher's test showed a non significant difference with a p value of 0.2. CONCLUSION: Despite of statistic result we believe that TOGD is a useful procedure as the first choice of surgical management in severe neurological impaired children affected by gastroesophageal reflux.

Uncommon surgical emergencies in neonatology.

Objective. Over the past decade, multiple factors have changed the pattern of neonatal surgical emergencies. An increase in prenatal screenings and the development of neonatal tertiary care centres have changed the clinical approach to these kids. Materials and methods. Between 1995 to 2011 were retrospectively reviewed 34 patients with diagnosis of uncommon rare neonatal surgical emergencies at our institute. We analyzed: sex, gestational age, weight at birth, primary pathology, prenatal diagnosis, associated anomalies, age and weight at surgery, clinical presentation, start of oral feeding and hospitalization. The follow-up was performed at 6,12, 24 and 36 months. Results. There were 21 male and 13 female. The gestational age ranged between 28 and 36 weeks. The weight at birth ranged between 700 and 1400 grams. Oral feeding was started between 4th and 10th postoperative day. The average hospitalization was about 70.47 days. To date, all patients have finished the followup. They are healthy. Conclusion. The outcome of the patients with uncommon surgical emergencies is different based on the etiology. Overall survival is generally good but is influenced by the associated anomalies.

Preoperative distraction in children: hand-held videogames vs clown therapy.

Anxiety in children undergoing surgery is characterized by feelings of tension, apprehension, nervousness and fear which may manifest differently. Postoperative behavioural changes such as nocturnal enuresis, feeding disorders, apathy, and sleep disturbances may stem from postoperative anxiety. Some Authors pointed out that over 60% of children undergoing surgery are prone to developing behavioural alterations 2 weeks after surgery. Variables such as age, temperament and anxiety both in children and parents are considered predictors of such changes.1 Studies were published describing how psycho-behavioural interventions based on play, learning and entertainment in preparing children for surgery, may reduce preoperative anxiety. Clown-therapy is applied in the most important paediatric facilities and has proved to diminish children's emotional distress and sufferance, as well as consumption of both analgesics and sedatives and to facilitate the achievement of therapeutic goals. The aim of our study was to evaluate the efficacy of clown-therapy during the child's hospital stay, with a view to optimizing treatment and care, preventing behavioural alterations and enhancing the child's overall life quality.

Ileostomy in extremely low birth weight and premature neonates.

AIM: The establishment of an ileostomy is a surgical option in the treatment of neonatal intestinal diseases, such as necrotizing enterocolitis (NEC) and meconial disease, in premature or extremely-low-birth-weight (ELBW) infants. METHODS: A prospective study was performed between July 2000-April 2011, with in exam all cases of acute abdomen in newborn premature babies. We perfomed a temporary ileostomy with a skin bridge and resection of the necrotic intestine. The temporary ileostomy was followed by anastomosis and the effect of possible confounding factors were assessed on the intestinal canalization. Data analysis and multiple monovariate were conducted. RESULTS: Thirty-three neonates, 14 males and 19 females, operated for intestinal perforation were identified. They were ELBW or premature neonates. There were 24 neonates with NEC, 4 with meconium peritonitis and 5 with complicated meconium ileus. In 4 cases of meconium ileus we found ileale atresia. Eight patients were excluded from the study because 3 died; 2 had cystic fibrosis and 3 with hydrocephalus. Thirteen patients developed complications: 7 related to ileostomy, 2 cholestasis and 4 recurrent NEC. Patients with meconium ileus gain rcovery of bowel function 4 days or more the others (OR=8.0; P=0.0455). CONCLUSION: In our experience, the establishment of ileostomy for the treatment of acute abdomen in child newborn premature or low birth weight allows optimal management of the child, excluding bowel sick and faster healing with a low rate of morbidity and mortality.

Cervical Lymphadenitis by Mycobacterium triplex in an Immunocompetent Child: Case Report and Review.

Mycobacterium triplex was first described in 1996. This nontuberculous Mycobacterium causes a severe pulmonary disease in immunocompromised patients but it can involve also healthy patients. A literature search was made on the PubMed database and it produced only few cases of children with cervical lymphadenitis due to this Mycobacterium Triplex. We are describing a case of M. triplex cervical lymphadenitis in an immunocompetent child.

Lactobacillus inhibitor production against Escherichia coli and coaggregation ability with uropathogens.

Previous investigations have shown that certain strains of lactobacilli can competitively exclude uropathogens from attaching to uroepithelial cells and from causing urinary tract infection in animals. The finding of an inhibitory effect produced by Lactobacillus casei ssp. rhamnosus GR-1 against the growth of uropathogens was investigated further using two Escherichia coli indicator strains Hu 734 and ATCC 25922. There were two phases to the inhibitor studies. The first one using an agar sandwich technique showed that the inhibitor activity was heat stable and inhibitory to the E. coli. The second phase showed that MRS broth provided optimum lactobacilli growth and inhibitor production. In addition, the inhibition was present under conditions buffering for acid and pH. The data indicated that the inhibitory effect was not due to bacteriophages or hydrogen peroxide. Strain GR-1 was found to coaggregate with E. coli ATCC 25922 in urine, a phenomenon that has not previously been reported for urogenital bacteria. An in vitro assay system was developed to study the coaggregation of various lactobacilli and uropathogens. The results demonstrated that highest coaggregation scores occurred after 4 h incubation at 37 degrees C with lactobacilli and two type-1 fimbriated E. coli strains. Of the nine lactobacilli strains tested, each was found to coaggregate with 2 or more of the 13 uropathogens. The dominance of inhibitor-producing lactobacilli on the urogenital epithelium and the ability of these organisms to interact closely with uropathogens would constitute an important host defense mechanism against infection.