Anna Morandi

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.

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.

Endoscopic Surveillance for Congenital Diaphragmatic Hernia: Unexpected Prevalence of Silent Esophagitis

Introduction Gastroesophageal reflux disease (GERD) is a frequent comorbidity after congenital diaphragmatic hernia (CDH) repair. Our aim was to evaluate the clinical and endoscopic prevalence of esophagitis in a long-term follow-up of CDH patients. Materials and Methods Patients operated on for posterolateral CDH and undergoing general anesthesia for concomitant pathologies between January and October 2013 were included in the study. GERD was investigated both clinically (Manterola questionnaire) and endoscopically. The severity of esophagitis was evaluated according to the Hetzel-Dent classification and multiple biopsies were performed. The correlation between clinical score and severity of esophagitis was evaluated. Results Twelve patients were included in the study (mean age: 14.5 years; range, 9-18 years). Only three children (25%) had a pathological questionnaire. At endoscopy, three children (25%) were affected by grade 1 esophagitis, six (50%) by grade 2, two (17%) by grade 3, and one (8%) by grade 4. One of the children presented Barrett esophagus. A moderate negative correlation was found between clinical data and endoscopic findings (r: -0.54 and p: 0.067). Conclusion Even in the absence of symptoms, esophagitis revealed to have a high prevalence in CDH patients. A long-term clinical and instrumental follow-up is mandatory to early diagnose and treat GERD.

Do we need imaging to diagnose appendicitis in children

Background: To evaluate the role of clinical assessment with selective use of imaging studies in the management of suspected acute appendicitis in children. Patients and Methods: Medical records of children referred to Emergency Room in 2010 for suspected appendicitis were retrospectively reviewed. Diagnostic investigations divided by age and sex were related to pathological findings. Negative appendectomy and complication rates were calculated. Results: 923 children needed surgical assessment : i0 n 75.7% of them surgical indication was excluded and 24.3% were admitted to surgical ward for observation. Appendectomy was eventually performed in 137 patients (61.9%), 82.4% of them without any preoperative imaging while 17.6% underwent selective studies, mainly abdominal ultrasonography (14.6%). Imaging was requested twice as frequently in not operated admitted children (39.3%) than in the operated ones (17.5%, P < 0.001). Overall complicated appendicitis rate (peritonitis and abscess) resulted 26.4% and negative appendectomy rate 8.8%. Females older than 10 years presented histologically not-confirmed appendicitis in 22.2% of cases, while the younger ones presented more frequently complicated appendicitis (29.3%). Conclusions: Clinical assessment is the key to diagnose appendicitis. Nevertheless, in girls older than 10 years, selected use of imaging should be implemented to avoid unnecessary appendectomies. Imaging of choice in equivocal cases should be ultrasonography.

Classification of Esophageal Strictures following Esophageal Atresia Repair

Introduction The aim of this study was to stratify anastomotic strictures (AS) following esophageal atresia (EA) repair and to establish predictors for the need of dilations. Materials and Methods A retrospective study on children operated on for EA between 2004 and 2014 was conducted. The stricture index (SI) was measured both radiologically (SIXR) and endoscopically (SIEND). A correlation analysis between the SI and the number of dilations was performed using Spearmans test and linear regression analysis. Results In this study, 40 patients were included: 35 (87.5%) presented with Grosss type C EA, 3 (7.5%) type A, 1 (2.5%) type B, and 1 (2.5%) type D. The mean follow‐up time was 101 ± 71.1 months (range: 7.8‐232.5, median: 97.6). The mean SIXR was 0.56 ± 0.16 (range: 0.15‐0.86). The mean SIEND was 0.45 ± 0.22 (range: 0.15‐0.85). Twenty‐four patients (60%) underwent a mean of 2 endoscopic dilations (range: 1‐9). The number of dilations was poorly correlated with SIXR, while significantly correlated with SIEND. Patients who did not need dilations had a SIEND < 0.33, patients who needed only one dilation had 0.33 ≤ SIEND < 0.44, and those with SIEND ≥ 0.44 needed two or more dilations. No significant association with other clinical variables was found. All patients were asymptomatic at the time of the first endoscopy. Conclusion SIEND is a useful tool to classify AS and can represent a predictor of the need for endoscopic dilation. The role of the SIEND becomes even more important as clinical characteristics have a low predictive value for the development of an AS and the need for subsequent endoscopic esophageal dilatations.

Correlation between magnetic resonance imaging findings after posterior sagittal anorectoplasty for anorectal malformations and the clinical outcome: Preliminary report

BACKGROUND/PURPOSE To assess the anatomical results after posterior sagittal anorectoplasty (PSARP) by magnetic resonance imaging (MRI) and to evaluate the correlation of these findings with clinical outcomes. METHODS Patients followed-up at our center after PSARP, being at least 6year old, with neither evidence of sacral abnormalities nor spinal dysraphisms were prospectively included. Complex ARMs were excluded. MRI was performed on a 1.5T unit. T1- and T2-weighted sequences were acquired, in orthogonal planes, according to the anal canal orientation. The degree of anorectal centering in the muscle complex (DARC), the pelvic floor symmetry, the rectal maximum diameter, the fat tissue interposition, and the presence of fibrosis were evaluated. A clinical questionnaire (Rintala score) was filled in by the parents. MRI findings were compared to the clinical outcomes assessed by the Rintala score. For statistical analysis the Spearman rho correlation coefficient was calculated and the Wilcoxon rank-sum test was performed. RESULTS We recruited 11 patients (mean age 12years, range 6-19) with MRI. DARC (range 252-360°) was strongly correlated with the degree of fecal incontinence (rho=0.70), mildly with the ability to hold back defecation (rho=0.58), constipation (rho=0.46) and total Rintala score (rho=0.41). Pelvic floor symmetry correlated with the frequency of defecation (rho=0.58). Rectal maximum diameter negatively correlated with the ability to feel the urge to defecate (rho=-0.60). Patients with fibrosis were slightly more constipated (p=0.056) and presented more social impairment (p=0.04). Fat tissue interposition had no correlation with the clinical outcome. CONCLUSIONS Thanks to soft tissue definition, multiplanar imaging, and lack of ionizing radiation, MRI is a valuable tool in the postoperative anatomical evaluation of patients with ARM. Our preliminary results show that abnormal anatomical findings can correlate to a nonoptimal functional outcome, thus helping in understanding the clinical course. The degree of anorectal centering in the muscle complex (DARC) seems to have the better correlation with the outcome, especially in terms of fecal continence. TYPE OF STUDY Prognosis study. LEVEL OF EVIDENCE level IV.

Supra-transumbilical laparotomy (STL) approach for small bowel atresia repair: Our experience and review of the literature

Background: Supra-Transumbilical Laparotomy (STL) has been used in paediatric surgery for a broad spectrum of abdominal procedures. We report our experience with STL approach for small bowel atresia repair in newborns and review previous published series on the topic. Patients and Methods: Fourteen patients with small bowel atresia were treated via STL approach at our Institution over a 5-year period and their charts were retrospectively reviewed. Results: STL procedure was performed at mean age of 3.1 day. No malrotation disorders were detected with pre-operative contrast enema. Eight patients (54.1%) presented jejunal atresia, five (35.7%) ileal atresia, and one (7.1%) multiple ileal and jejunal atresias. Standard repair with primary end-to-back anastomosis was performed in all but one patient. In the newborn with multiple atresia, STL incision was converted in supra-umbilical transverse incision due to difficulty of exposition. After surgery, one patient developed anastomotic stricture, and another developed occlusion due to adhesions: Both infants required second laparotomy. No infections of the umbilical site were recorded, and cosmetic results were excellent in all patients. Conclusions: Increasing evidence suggests that STL approach for small bowel atresia is feasible, safe and provides adequate exposure for small bowel atresia surgery. When malrotation and colonic/multiple atresia are pre-operatively ruled out, STL procedure can be choosen as first approach.

Appendicitis in a 14-month-old infant with respiratory symptoms

Although appendicitis is the condition that most commonly requires emergent abdominal surgery in the paediatric population, less than 2% of the disease occurs in infants and it is even more uncommon in neonates. In this report, we describe a rare case of a 14-month-old child presenting with abdominal pain first diagnosed with upper respiratory tract infection and then admitted to our Paediatric Surgery Department with a final diagnosis of acute appendicitis. A particular attention has to be kept on children presenting with an upper respiratory tract infection since symptoms can mask abdominal signs. Due to high morbidity and mortality rate related to a delayed diagnosis, appendicitis always has to be considered as a possible diagnosis, in order to ensure a prompt treatment.

Duodenal Hematoma and Pancreatitis Complicating Endoscopic Intestinal Biopsy in a Boy with Noonan Syndrome

Duodenal intramural hematoma is a rare condition, mostly described in children and young adults that can be a complication of duodenal biopsy, especially in patients with predisposing hemorrhagic diathesis. It can determine secondary pancreatitis because of ampullary hematoma. Noonan Syndrome (NS) is an autosomal dominant disorder characterized by short stature, typical facial dysmorphisms, congenital heart defects and other anomalies such as bleeding problems which have been reported in up to 55% of patients. We herein report a case of duodenal hematoma with pancreatitis developed after endoscopic biopsy in a boy who was initially suspected of having celiac disease on the base of his short stature and growth retardation. Afterwards a more careful past medical history collection and objective examination revealed characteristic features of NS which could have been picked-up in advance, thus avoiding an investigation, such as the duodenal endoscopic biopsy, which in NS patient is potentially more risky.