Ernesto Leva

Esophageal atresia with proximal tracheoesophageal fistula: A missed diagnosis ☆ ☆☆ ☆☆☆ ★ ★★ ★★★

AIM OF THE STUDY This retrospective study was performed to compare the relative incidence of esophageal atresia (EA) with proximal tracheoesophageal fistula (PTEF) at our institution with those reported in literature and to test the hypothesis that our higher relative incidence is caused by the routine use of tracheoscopy. METHODS A total of 204 children with EA were managed at our institution from 1981 to 2012. The type of EA and the diagnostic assessment were noted, and the relative incidence of PTEF was calculated. For patients managed from 1981 to 2003 (Group 1), the PTEF was diagnosed by contrast esophagogram or during surgical repair. For those born after 2004 (Group 2), the final diagnosis was made by routine rigid tracheoscopy performed preoperatively. The relative incidence of PTEF was compared between these two groups and with those reported in 15 selected published large series, encompassing 4197 patients with EA. MAIN RESULTS Of 204 patients with EA, 10 had PTEF, with a relative incidence of 4.9%, statistically higher than those reported in reference group (1.14%, P<0.001). The routine employ of tracheoscopy involved a higher relative incidence of PTEF (Group 2=11.11%, Group 1=3.14%, P=0.038). The age of diagnosis of PTEF was 2.8 days for children of Group 2 and 4.2 days for Group 1 (P=0.038). CONCLUSION The presence of the proximal TEF should be always ruled out before surgery. Routine employ of rigid tracheoscopy avoids delay of the diagnosis, improves diagnostic accuracy, and involves a higher relative incidence of proximal fistul. This procedure should be recommended in children undergoing EA repair.

Management of traumatic complete pancreatic fracture in a child: case report and review of literature.

UNLABELLED Blunt abdominal trauma is the most common cause of pancreatic injury in children. Laparoscopic distal pancreatectomy in a child with complete duct disruption has not been reported in the literature in children, although it has been well described in adults. METHODS In this paper report a case of a 7-year-old male, with grade 4 pancreatic trauma, who was treated nonoperatively in the acute phase and subsequently by laparoscopic distal pancreatectomy 3 months after the trauma. DISCUSSION Although in adults the surgical management of grade 3-4 pancreatic traumatic injury is well described, including the laparoscopic approach, no report of laparoscopic distal pancreatectomy was found in the literature. We would like to emphasize the importance of using a conservative management in the acute phase of pancreatic injury, including grade 4 injuries. After this phase, the use of the high-definition computed tomography scan and endoscopic retrograde pancreatography were fundamental. CONCLUSION Magnification of laparoscopic technique allowed us to identify the structures much better than open surgery.

Peristeen(®) transanal irrigation in paediatric patients with anorectal malformations and spinal cord lesions: a multicentre Italian study.

In paediatric and adult patients with neurogenic bowel, transanal irrigation (TAI) of the colon has gained popularity due to the introduction of a specifically designed device. The aim of this pilot study was to present the results of TAI using the Peristeen® TAI system in a group of paediatric patients with anorectal malformation (ARM) and congenital or acquired spinal cord lesions (SCLs).

Effect of maternal human immunodeficiency virus status on the outcome of neonates with necrotizing enterocolitis

AIM The aim of the study was to assess the impact of human immunodeficiency virus (HIV) exposure on survival and extent of disease in necrotizing enterocolitis (NEC). PATIENTS AND METHODS All patients with NEC requiring surgery between June 1998 and June 2008 were analyzed. Three groups were identified: those born to HIV-positive (HIV+) mothers, those born to HIV-negative (HIV-) mothers, and those with an unknown HIV status. Primary outcome measure was survival to discharge. Secondary outcome measure was extent of disease. RESULTS One hundred nine infants (mean gestational age, 31 weeks; birth weight, 1413 g) underwent surgery for NEC. Gestational age, birth weight, and day of presentation were similar in all 3 groups, showing no statistical difference. The HIV+ group consisted of 22 infants, of which 13 (59%) died and 2 (9%) had panintestinal necrosis. The HIV- group consisted of 48 infants, of which 11 (23%) died, with 3(6%) having panintestinal necrosis. The remaining group of HIV (unknown) consisted of 38 infants, of which 14 (37%) died, with 2 (5%) having panintestinal necrosis. The latter group was not included in the analysis; but comparing the HIV+ and HIV- groups, there was a statistically higher chance of death (odds ratio = 4.8, P = .05). There was no difference statistically in the extent of disease. CONCLUSION Neonates with NEC born to HIV+ mothers have a higher mortality.

Barrett’s esophagus and Cornelia de Lange Syndrome

Aim:  To review the records of Cornelia de Lange Syndrome (CDLS) children, affected by Gastro‐oesophageal reflux disease (GERD), to detect the presence of Barrett’s Esophagus (BE).

Anorectal malformations with good prognosis: Variables affecting the functional outcome

BACKGROUND/PURPOSE The purpose of this study was to investigate the outcome of patients operated for anorectal malformations (ARMs) with good prognosis. METHODS Thirty patients underwent clinical evaluation by Rintala score and anorectal manometry recording anal resting pressure (ARP), rectoanal inhibitory reflex (RAIR), and rectal volume (RV). The results were analysed with regard to sex, type of ARM, surgical timing of posterior sagittal anorectoplasty (PSARP), neurospinal cord dysraphism (ND), neonatal colostomy, and institution where they underwent surgery. RESULTS 6/30 (20%) presented ND despite normal sacrum. 17/30 (57%) patients had a normal Rintala score. ND and neonatal colostomy were significantly associated with a pathologic score (p=0.0029 and p=0.0016). Patients with ND had significantly lower ARP compared to patients with normal spine (23.5±7.2mmHg vs 32±7.9mmHg, p=0.023). ARP was significantly lower in patients with neonatal colostomy compared to patients with primary repair (25.22±10.24mmHg vs 32.57±6.68mmHg, p=0.026). RAIR was present in only 2/6 (33%) patients with ND, while in 21/24 (87.5%) without ND (p=0.015) and in 4/9 (44%) patients with neonatal colostomy, while in 19/21 (90.5%) patients submitted to primary repair (p=0.014). CONCLUSIONS Neurospinal cord dysraphism may be present despite normal sacral ratio. From a clinical point of view, patients with good prognosis ARMs are not completely comparable to healthy children. Neurospinal cord dysraphism and neonatal colostomy seem to worsen the clinical and manometric (ARP and RAIR) outcomes of these patients.

Cervical/thoracotomic/thoracoscopic approaches for H-type congenital tracheo-esophageal fistula: A systematic review

PURPOSE Aim of this systematic review is to investigate the thoracic and cervical surgical approaches of H-type tracheo-esophageal fistula (TEF) according to the position of the fistula. METHODS The PubMed database was searched for original studies on H-type TEF treatment published between 1977 and 2012. Manuscripts finally included were divided into open and thoracoscopic surgery groups. RESULTS Seventeen studies were selected for open surgery group, and most of them agree on the importance of pre-operative diagnosis of the fistula by preliminary tracheoscopy. Right cervicotomy was used in 70 cases (76.9%), left cervicotomy in 12 (13.2%), and thoracotomy only in 9 (9.9%). Five studies were included in thoracoscopic group (6 patients). Indications for the surgical approach (cervical vs thoracic) according to the position of the TEF were clearly described in 10 manuscripts, and all stated differences in surgical technique details. Complications and mortality rates were not statistically correlated to the different surgical approaches. CONCLUSIONS The evidence base in regard to the treatment of H-type fistula in children is poor and the skills and preferences of the surgeons guide the choice of the procedure. Surgical division of the fistula is curative, and the key to a successful repair is the pre-operatively identification of the level of the fistula with tracheoscopy. Right cervicotomy seems to be the approach of choice in the majority of case, with the thoracic approach appropriate only for fistulae opening below T2. Further well-designed prospective studies which take into account of selection and performance bias are strongly required.

The Role of Laparoscopy in Newborns Affected by NEC

INTRODUCTION For many years, laparoscopic procedures have been reported in the literature in pediatrics also. In this article, we report their experiences of the use of gasless laparoscopy in 8 newborns affected by necrotizing enterocolitis (NEC). MATERIALS AND METHODS From January 2007 to May 2008, 8 patients affected by stage 1-2 NEC were treated at the Department of Pediatric Surgery, Fondazione Policlinico Milan (Milan, Italy). Of those, 3 patients presented with a birth weight below 1.5 kg. RESULTS All patients were submitted at gasless laparoscopy. In 6 of 8 patients, a covered perforation was detected; in 5 cases, the perforation was on the posterior wall of the ascending colon, and in 1, a perforation of the transverse colon was detected. In these 6 of 8 patients, the procedure was converted to formal laparotomy, with colonic resection and primary anastomosis. In 2 of 8 patients, a diffuse necrotizing enteritis of the small bowel was reported, without evidence of perforation; two drains were placed and continued abdominal washout with antibiotics solution was maintained for 48 hours, associated with systemic therapy. All patients were maintained on systemic antibiotic therapy for 7 days with regression of sepsis; all patients survived and were discharged in good general condition. At follow-up of 3 months, none of the patients presented with complications. DISCUSSION We believe that the decision to perform a laparoscopy, despite the very low weight of the patient, was crucial in the management of nondetected perforation at X-ray. Retrospectively, laparoscopy would be the best option to define the presence of NEC without a perforation, which may only require washout of the cavity that can be also managed with this technique. CONCLUSIONS We believe that laparoscopy can be easily managed also in newborns and small for gestational age neonates, reducing the morbidity of laparotomy for suspicion of perforation in patients affected by NEC who do not respond to medical treatment.

Treating acid reflux disease in patients with Down syndrome: pharmacological and physiological approaches

Down syndrome (DS) is often accompanied by gastrointestinal disease, occurring mainly in early infancy and frequently requiring therapy. Among motility disorders, the most frequent is gastroesophageal reflux disease (GERD), which may often be misdiagnosed because of its atypical manifestations. Early diagnosis of esophageal functional disorders is essential to prevent respiratory problems, growth retardation in children, weight loss in adults, and to establish the correct type of surgery if needed. Furthermore, the involvement of the enteric nervous system in the pathophysiology of GERD in DS is not yet completely understood but seems supported by much evidence. In fact DS is often associated with motor disorders and this evidence must be considered in the choice of therapy: in particular all options available to improve motility seem to be effective in these patients. The effectiveness of therapy is strictly related to the rate of mental impairment, so that modulating therapy is essential, especially in view of the severity of the neurological status.

Long term follow-up and transition of care in anorectal malformations: An international survey

BACKGROUND The aim of the study was to assess current international practice in the long term follow-up, in managing active problems and transition of care for teenagers born with anorectal malformations (ARM). METHODS An original survey was administered to delegates attending two large colorectal surgical meetings in 2015. The 21 questions covered long term follow-up, specific issues for teenagers and transition of care. RESULTS 96/236 delegates completed the survey. Follow-up was routinely suspended before 10 year of age by 33% of respondents. 90% of them did not use a scoring system to assess or risks stratify patients, despite 81% stating that an objective score would be beneficial. 40% of respondents felt that >30% of their teenagers had ongoing active medical or psychosocial issues. 42% thought their patients were not ready to be transitioned. The process of transition should start around 13-16 years according to 54% of respondents. 72% had no protocol for transition and 82% did not hold multidisciplinary meetings with adult practitioners before transition. CONCLUSIONS International consensus on the following aspects of the care in ARM is needed: structured long term follow-up, objective assessment and risk stratification scores, pathways of transition and methods to prepare patients, parents and adult practitioners.