Thoraco-abdominal asymmetry and asynchrony in congenital diaphragmatic hernia.

OBJECTIVE: Congenital diaphragmatic hernia (CDH) consists of an incomplete formation of the diaphragm and the subsequent herniation of abdominal bowels. Diaphragmatic defect can be repaired by primary closure or placing a patch. Respiratory follow up usually focuses on spirometric and clinical evaluation. The aim of the study was to assess thoraco-abdominal volumes in CDH patients and to verify whether the action of the diaphragm on the chest wall is altered leading to an asymmetric and asynchronous expansion of the different thoracoabdominal compartments. PATIENTS AND METHODS: Total and compartmental chest wall volumes and asynchronies were measured by Opto-Electronic Plethysmography in 14 CDH patients (7 M/7F, age 5 ± 2 years, 12 left side operated) and in 9 age matched healthy subjects during quiet spontaneous breathing in supine position. Patients were divided in two groups: five patients with suture (group S) and nine patients with diaphragmatic patch (group P). Pulmonary function was assessed by spirometry and spirometric parameters were expressed as Z-score. RESULTS: In group P abdominal contribution to tidal volume was lower than healthy controls and group S. Unlike controls, in both CDH groups the right side of pulmonary rib cage moved inward with a correspondent left side expansion during inspiration. In group S, thoraco-abdominal asynchronies were higher than in group P and controls, especially in the right side. Five patients belonging to group P had a spirometric obstructive pattern. CONCLUSIONS: In overall CDH patients a reduced action of the treated (left) hemi-diaphragm is evident. In patients treated by primary suture, a compensatory action of the right side allows to reach a normal total diaphragmatic displacement and a proper contribution of the whole diaphragm to tidal volume. In patients treated by diaphragmatic patch, instead, thoraco-abdominal asynchronies are prevented.

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 : In 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.

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.

Peritoneal tuberculosis due to multidrug-resistant Mycobacterium tuberculosis.

The emergence of drug-resistant Mycobacterium tuberculosis has been widely reported throughout the world, but there are very few data regarding children. We describe the case of a 14-year-old Peruvian adolescent who had been living in Italy since the age of 8 years and was diagnosed as having peritoneal tuberculosis (TB). While she was receiving first-line anti-TB therapy, she developed pyrazinamide-associated thrombocytopenia and cultures revealed a multidrug-resistant strain of Mycobacterium tuberculosis. Pyrazinamide, rifampicin and isoniazid were replaced by moxifloxacin, which was continued for 9 months together with ethambutol. The patient recovered without experiencing any drug-related adverse event or the recurrence of TB in the following year. In conclusion, this case illustrates some of the problems that can arise when multidrug-resistant TB has to be treated in children and adolescents, and also highlights the fact that further studies are needed to clarify which drugs should be used and for how long.

Anastomotic strictures and endoscopic dilatations following esophageal atresia repair.

PURPOSE: To identify risk factors that can predict prevalence of anastomotic strictures (AS) following esophageal atresia (EA) repair. METHODS: Of 46 consecutive patients with EA managed at our institution between 2004 and 2012, 35 underwent esophageal anastomosis and were included in this retrospective longitudinal study. Routine endoscopy was performed 1 month after surgical repair. According to stricture index (SI), endoscopically calculated as SI = (D - d)/D, where D is the diameter of the esophageal pouch and d the stricture diameter, population was divided into Group 1, SI ≤ 0.1 (no evidence of stricture); Group 2, 0.3 > SI > 0.1 (mild stricture); Group 3, SI ≥ 0.3 (high-grade stricture). Trends of subsequent endoscopic esophageal dilatations were compared between the groups using Wilcoxon-Mann-Whitney or Pearson's tests. Cox regression analysis was performed to estimate the hazard ratio. RESULTS: Gastro-esophageal reflux disease (P = 0.04), tension on the anastomosis (P = 0.02) and long-gap form (P = 0.008) have an increased risk of developing AS. SI at 1 month after surgery correlates with the average number of future dilatations: Group 2 and 3 compared to Group 1 required more dilatations (hazard ratio 2.291 and 12.765). CONCLUSION: AS remain frequent complications of esophageal surgery, especially in specific subgroups of patients. SI at 1 month after surgery could already predict the severity of the stricture and the need for subsequent endoscopic esophageal dilatations.

Single-stage surgical correction of anorectal malformation associated with rectourinary fistula in male neonates.

INTRODUCTION: The treatment of children affected by anorectal malformations (ARM) is characterized by some unsolved problems. The three-stage surgical correction has been known to be most effective in preventing complications, but recently new approaches have been proposed. We describe our experience with the newer approaches. METHODS: Twenty three male newborns, affected by ARM and recto-urinary fistula, were treated in 2 different centers in 8 years. Nineteen neonates (birth weight 2.4 - 3.5 kg) received a primary posterior sagittal anorectoplasty (PSARP) at the Department of Pediatric Surgery of the Chittagong Medical College Hospital (group 1). Four term neonates (birth weight 2.9 - 3.4 kg) received a primary pull-through with combined abdomino-perineal approach at the Pediatric Surgery Department of Fondazione Cà Granda of Milan (group 2). RESULTS: Among patients of Group 1, 11 patients had a recto-bulbar fistula and 8 a recto-prostatic fistula. Among the Group 2, 2 had a recto-bulbar fistula and 2 a recto-prostatic fistula. The site of fistula was decided at the time of surgery. In Group 1, 5 post-surgical complications were recorded (26%); 1 child died of sepsis, 3 had dehiscence and 1 stenosis, which resolved with dilatation. In Group 2, the only post-operative complication of small rectal prolapse resolved spontaneously after a few months on follow-up. Group 2 patients were followed-up in a dedicated multidisciplinary colorectal center. CONCLUSIONS: Primary repair of ARMs with recto-urinary fistula is a feasible, safe and effective technique in the neonatal period. A combined abdominal and perineal approach seems to guarantee better results. A dedicated team is mandatory, both for the surgical correction and for a long-term follow-up.

Feasibility of peritoneal dialysis in extremely low birth weight infants.

Acute renal injury is common in extremely low birth weight (ELBW) infants with a frequency ranging from 8% to 24%. Peritoneal dialysis (PD) has been used only occasionally in ELBW. We report our experience and share the solutions used to tackle the difficulties rising from the small size of this type of patients. PD was successfully performed in three ELBW infants with acute renal failure. A neonatal, single-cuff, straight Tenckhoff catheter was placed in 2 patients, while a Broviac single cuff vascular catheter was used in another. PD was feasible and effective in all children. Leakage was observed with Tenckhoff catheters, but this did not impair the PD efficacy. The technical difficulties were related to the size and shape of the peritoneal catheters, not easily fitting with the very thin abdominal wall of the preterm infants. We conclude that PD is feasible and effective, can be considered as the rescue therapy in preterm ELBW infants with acute renal failure.

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.

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). METHODS: A total of 62 CDLS patients were investigated for GERD (1 month-35 years). In all of them a pH-metry, an upper endoscopy with multiple biopsies and a complete radiologic digestive evaluation were carried out. BE was diagnosed in case of replacement of oesophageal mucosa by specialized intestinal-type columnar mucosa. Anti-reflux surgery was considered in case of persistence of BE after medical therapy. Follow-up (mean 3.5 years) consisted in endoscopy every 6 months . RESULTS: Gastro-oesophageal reflux disease was found in 50 CDLS patients (80%) and BE in six of them (12% of the GERD group, 9.6% of the entire population, mean age 17 years, range 6-32 years). A short segment BE was observed in three patients, a long one in two patients and an infiltrating adenocarcinoma of the lower oesophagus in one patient. CONCLUSIONS: A higher frequency of BE in CDLS patients than in a normal population is found. A delayed diagnosis because of atypical GERD symptoms and an altered intestinal motility as a result of neurological impairment can be recognized as the main cause.

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.

Conservative surgical management of catheter infections in children on peritoneal dialysis.

INTRODUCTION: Major indications for peritoneal dialysis catheter removal include chronic exit-site infection (ESI) or tunnel infection (TI). No consensus on the optimal treatment of these infections in children exists. PATIENTS: During the last 10 years, 13 patients (7 females, mean age 56 months) on peritoneal dialysis were treated for recurrent ESI (N: 4) or TI (N: 9). Staphylococcus aureus (12 patients) and Pseudomonas aeruginosa (1 patient) were isolated. All patients had a double-cuff straight Tenchkoff catheter and underwent the shaving of the external cuff, with a new tunnel tightly adherent to the catheter, and an exit-site in the opposite abdominal region. A mean follow-up of 31 months/catheter demonstrated no recurrence of ESI and TI. DISCUSSION: Little pediatric experience with cuff shaving exists: reported catheter salvage rates are 48-100%. In our experience, good results were obtained by shaving off the external cuff and re-creating a new tunnel, with a different course, strictly adherent to the catheter. This measure seems to guarantee an effective barrier against infections, while obviating the need of the external cuff. CONCLUSIONS: Sometimes, in case of recurrent ESI or TI, the external cuff may facilitate the persistence of bacteria. A conservative surgical treatment offers good results in children.

Right congenital diaphragmatic hernia associated with a complex heart disease.

A case of right diaphragmatic hernia is presented associated with a complex congenital heart disease (double-outlet right ventricle, transposition of the great arteries and left isomerism) diagnosed prenatally. Despite high-frequency oscillatory ventilation plus nitric oxide and uneventful repair of the hernia, the infant died after 6 days of uncontrolled pulmonary hypertension and severe aortic coarctation that developed postnatally.

Chronic peritoneal dialysis in children: catheter related complications. A single centre experience.

Despite advancements in catheter design and dialysis technique, catheter related complications still remain a common clinical problem in paediatric patients on chronic peritoneal dialysis (PD); in particular, infections are a common cause of patient's morbidity and technique failure. In the present paper, data on 89 catheters implanted between January 1986 and December 2002 are reviewed to analyse the major causes of complications and/or PD failure and to ascertain their optimal management. A total of 89 catheters were implanted in 78 patients at the start of chronic PD: 26 in children under 2 years of age, 14 in children aged 2-5 years and 49 in patients over 5 years. Mean age of patients was 76.1 +/- 73.0 months and median treatment time 14.5 +/- 13.1 months. All catheters were surgically implanted and partial omentectomy was performed in 70% of cases. Straight Tenckhoff catheters were used in 70 cases (78%), curled ones in 19 (22%). Sixty-three catheters (71%) had two cuffs, 26 (29%) a single cuff. The entry-site was the midline in 34 patients (38%) and the paramedian line in 55 patients (62%). Catheter survival rate was 80% at 12 months, 62% at 24 months and 58% at 36 and 48 months, respectively. The incidence of catheter-related complications was one episode every 6.4 PD-months, and they were mainly represented by peritonitis (61%), exit-site infections and tunnel infection (ESI + TI: 23%), catheter obstruction (5%), dislocation (3.5%), leakage (2.5%). After the introduction of curled single-cuff catheters, a considerable reduction in the peritonitis incidence was observed during the last 7 years. A more prolonged catheter survival was observed in older children (>5 vs. <2 years: P < 0.05). Leakage was less common in catheters with paramedian entry-site compared with catheters implanted on the midline. In 7 out of 11 (64%) patients with catheter obstruction, omentectomy had not been performed. Single-cuff catheters had a lower infection-rate than double-cuff catheter (P < 0.01). Single cuff-curled Tenckhoff catheter can be considered the first choice catheter. Single cuff-catheters are not associated with an increase of infections. The surgical technique requires a strict adherence to a standardized procedure and a dedicated team, in order to obtain a reduction of the complications, a prolonged catheter duration and a better quality of life.