A Provisional Experience with Robot-Assisted Soave Procedure for Older Children with Hirschsprung Disease: Back to the Future?

Hirschsprung disease is a congenital disease characterized by intestinal aganglionosis of various extents. Most patients are younger than 1 year of age. Though, a minority of cases can be older or even adult. Older the patient the more difficult and prolonged is the endorectal dissection required for the pull-through procedure. Longer surgery leads to longer anal dilatation and trauma with subsequent higher likelihood of continence impairment. The article aims at describing the first case series of robot-assisted Soave procedure, which was adopted as an alternative minimally invasive approach to older patients with Hirschsprung disease. The technical principles are represented by intraoperative seromuscolar leveling biopsies, intracorporeal endorectal cranial dissection, and endorectal pull-through with colo-anal anastomosis. The authors report three procedures that were carried on without complication with a limited requirement for anal dilatation and trauma, given the reduced need for endorectal caudal dissection. The results demonstrate the feasibility of the proposed approach. The robotic approach should be considered as an alternative minimally invasive approach for older children or adults with Hirschsprung disease.

Use of failure modes, effects, and criticality analysis to compare the vulnerabilities of laparoscopic versus open appendectomy.

PURPOSE: To measure the feasibility of using FMECA applied to the surgery and then compare the vulnerabilities of laparoscopic versus open appendectomy by using FMECA. METHODS: The FMECA study was performed on each single selected phase of appendectomy and on complication-related data during the period January 1, 2009, to December 31, 2010. The risk analysis phase was completed by evaluation of the criticality index (CI) of each appendectomy-related failure mode (FM). The CI is calculated by multiplying the estimated frequency of occurrence (O) of the FM, by the expected severity of the injury to the patient (S), and the detectability (D) of the FM. RESULTS: In the first year of analysis (2009), 177 appendectomies were performed, 110 open and 67 laparoscopic. Eleven adverse events were related to the open appendectomy: 1 bleeding (CI: 8) and 10 postoperative infections (CI: 32). Three adverse events related to the laparoscopic approach were recorded: 1 postoperative infection (CI: 8) and 2 incorrect extractions of the appendix through the umbilical port (CI: 6). In the second year of analysis (2010), 158 appendectomies were performed, 69 open and 89 laparoscopic. Four adverse events were related to the open appendectomy: 1 incorrect management of the histological specimen (CI: 2), 1 dehiscence of the surgical wound (CI: 6), and 2 infections (CI: 6). No adverse events were recorded in laparoscopic approach. CONCLUSION: FMECA helped the staff compare the 2 approaches through an accurate step-by-step analysis, highlighting that laparoscopic appendectomy is feasible and safe, associated with a lower incidence of infection and other complications, reduced length of hospital stay, and an apparent lower procedure-related risk.

A prospective observational study of associated anomalies in Hirschsprung's disease.

BACKGROUND: Associated anomalies have been reported in around 20% of Hirschsprung patients but many Authors suggested a measure of underestimation. We therefore implemented a prospective observational study on 106 consecutive HSCR patients aimed at defining the percentage of associated anomalies and implementing a personalized and up-to-date diagnostic algorithm. METHODS: After Institutional Ethical Committee approval, 106 consecutive Hirschsprung patients admitted to our Institution between January 2010 and December 2012 were included. All families were asked to sign a specific Informed Consent form and in case of acceptance each patient underwent an advanced diagnostic algorithm, including renal ultrasound scan (US), cardiologic assessment with cardiac US, cerebral US, audiometry, ENT and ophthalmologic assessments plus further specialist evaluations based on specific clinical features. RESULTS: Male to female ratio of our series of patients was 3,4:1. Aganglionosis was confined to the rectosigmoid colon (classic forms) in 74,5% of cases. We detected 112 associated anomalies in 61 (57,5%) patients. The percentage did not significantly differ according to gender or length of aganglionosis. Overall, 43,4% of patients complained ophthalmologic issues (mostly refraction anomalies), 9,4% visual impairment, 20,7% congenital anomalies of the kidney and urinary tract, 4,7% congenital heart disease, 4,7% hearing impairment or deafness, 2,3% central nervous system anomalies, 8,5% chromosomal abnormalities or syndromes and 12,3% other associated anomalies. CONCLUSIONS: Our study confirmed the underestimation of certain associated anomalies in Hirschsprung patients, such as hearing impairment and congenital anomalies of the kidney and urinary tract. Subsequently, based on our results we strongly suggest performing renal US and audiometry in all patients. Conversely, ophthalmologic assessment and cerebral and heart US can be performed according to guidelines applied to the general population or in case of patients with suspected clinical features or chromosomal abnormalities. This updated diagnostic algorithm aims at improving overall outcome thanks to better prognostic expectations, prevention strategies and early rehabilitation modalities. The investigation of genetic background of patients with associated anomalies might be the next step to explore this intriguing multifactorial congenital disease.

Magnetic resonance urography and laparoscopy in paediatric urology: a case series.

Paediatric urology often presents challenging scenarios. Magnetic resonance urography (MRU) and laparoscopy are increasingly used. We retrospectively studied children affected by a disease of the upper urinary tract who after MRU were elected for laparoscopic treatment. This pictorial essay draws on our experience; it illustrates some specific MRU findings and highlights the usefulness of MRU for the diagnosis of upper urinary tract pathology in children. It also offers some examples of the potential additional diagnostic information provided by laparoscopy as well as its therapeutic role.

Induction of RET dependent and independent pro-inflammatory programs in human peripheral blood mononuclear cells from Hirschsprung patients.

Hirschsprung disease (HSCR) is a rare congenital anomaly characterized by the absence of enteric ganglia in the distal intestinal tract. While classified as a multigenic disorder, the altered function of the RET tyrosine kinase receptor is responsible for the majority of the pathogenesis of HSCR. Recent evidence demonstrate a strong association between RET and the homeostasis of immune system. Here, we utilize a unique cohort of fifty HSCR patients to fully characterize the expression of RET receptor on both innate (monocytes and Natural Killer lymphocytes) and adaptive (B and T lymphocytes) human peripheral blood mononuclear cells (PBMCs) and to explore the role of RET signaling in the immune system. We show that the increased expression of RET receptor on immune cell subsets from HSCR individuals correlates with the presence of loss-of-function RET mutations. Moreover, we demonstrate that the engagement of RET on PBMCs induces the modulation of several inflammatory genes. In particular, RET stimulation with glial-cell line derived neurotrophic factor family (GDNF) and glycosyl-phosphatidylinositol membrane anchored co-receptor α1 (GFRα1) trigger the up-modulation of genes encoding either for chemokines (CCL20, CCL2, CCL3, CCL4, CCL7, CXCL1) and cytokines (IL-1ß, IL-6 and IL-8) and the down-regulation of chemokine/cytokine receptors (CCR2 and IL8-Rα). Although at different levels, the modulation of these "RET-dependent genes" occurs in both healthy donors and HSCR patients. We also describe another set of genes that, independently from RET stimulation, are differently regulated in healthy donors versus HSCR patients. Among these "RET-independent genes", there are CSF-1R, IL1-R1, IL1-R2 and TGFß-1, whose levels of transcripts were lower in HSCR patients compared to healthy donors, thus suggesting aberrancies of inflammatory responses at mucosal level. Overall our results demonstrate that immune system actively participates in the physiopathology of HSCR disease by modulating inflammatory programs that are either dependent or independent from RET signaling.

Weakly acidic gastroesophageal refluxes are frequently triggers in young children with chronic cough.

OBJECTIVE: To evaluate whether the proportion of acid and weakly acidic refluxes preceding cough bursts could be different in infants, preschool- and school-aged children with chronic, unexplained cough. PATIENTS AND METHODS: One hundred six children with unexplained chronic cough, not receiving acid suppressive therapy, underwent impedance-pH monitoring. They were divided into Group A: <2 years (21 pts), Group B: ≥2 to ≤6 years (41 pts), and Group C: >6 years (44 pts). Reflux was defined as acid (pH <4), weakly acidic (WA; pH 4-7), and weakly alkaline (pH >7).Cough episodes were considered temporally associated with gastroesophageal reflux (GER) when occurring within 2 min after a GER episode. RESULTS: Impedance-pH detected 55.50 (39.00-76.00) reflux episodes/patient with an acid-to-WA reflux event ratio of 3.31 (1.55-8.33). This parameter was significantly lower in Group A (1.33 [0.41-3.40]) than in Group B (3.06 [2.00-6.50]; P < 0.05] and Group C (5.09 [2.34-12.43; P < 0.001]). No cough episode was preceded by weakly alkaline refluxes in any patient. During impedance-pH recording, 93 patients (87.7%) had at least one cough episode that had been preceded by impedance refluxes in 83 patients. In this latter group (83 pts), the median number of cough episodes was 12.0 (5.0-25.5), of which 48.15% (30.15-71.43%) were preceded by refluxes; GER-preceded cough episodes were similar in the three groups. The majority of children had cough episodes preceded by acid refluxes in the total population (78.3%), in Group B (80.5%) and in Group C (93.7%), but not in Group A (40.0%; P < 0.001). CONCLUSIONS: In addition to acid, also a significant proportion of WA GER may precede cough episodes in young children with persistent cough, possibly explaining the inconstant effects of anti-acid treatment on respiratory symptoms.

Early diverting colostomy for perianal sepsis in children with acute leukemia.

Perineal sepsis is a life-threatening complication of acute leukemia. Although conservative management (antibiotics, incision, and drainage, alone or in combination) is considered the criterion standard, it provides an outcome that is not fully satisfactory, with an overall mortality of roughly 30%. This report presents a case series of 4 children who underwent early defunctioning colostomy for the treatment of perineal sepsis during leukemia. This management proved to be successful and allowed prompt reestablishment of chemotherapy, thus improving overall results. Routine application of this "aggressive" management in these cases will presumably increase overall survival of children with leukemia.

The surgical approach for cervicothoracic masses in children.

BACKGROUND: The surgical approach to masses located in the cervicothoracic juncton represents a challenge for surgeons. Many techniques have been described with good results. METHODS: We analyzed and compared the results obtained in 2 Italian pediatric surgery centers using 2 different techniques in patients with tumors of the thoracic inlet: center 1, using anterior cervical transsternal approach on 7 patients, and center 2, applying "trap-door" technique on 5 patients. RESULTS: Excision was incomplete in 5 patients and complete in 7 patients. Histologic examination revealed 5 patients with neuroblastoma; 3, ganglioneuroblastoma; 1, mixoid liposarcoma; 1, desmoid fibromatosis; 1, Castleman disease; and 1, Schwann cell tumor. The median duration of the procedure was 345 minutes in center 1 and 245 minutes in center 2. The median blood loss was 200 mL in both centers. The median hospital stay was 11 days in center 1 and 9 days in center 2. Globally, 5 patients developed postoperative complications. No significant differences were encountered comparing the main surgical outcome parameters between the 2 approaches. CONCLUSIONS: Both techniques resulted in valid options to achieve a safe excision of thoracic inlet masses with a manageable complication rate and acceptable hospital stay. Surgical risk factors should be carefully investigated preoperatively. Postoperative pain control is important to guarantee early recovery.

Intraureteral injection of NASHA/Dx gel under direct ureteroscopic visualization for the treatment of primary high-grade vesicoureteral reflux.

OBJECTIVE: To present a preliminary experience with the modified technique of extravesical intraureteral injection of non-animal-stabilized hyaluronic acid/dextranomer (NASHA/Dx) gel under direct ureteroscopic visualization for the treatment of primary high-grade vesicoureteral reflux (VUR). PATIENTS AND METHODS: The medical records of all pediatric patients (age range, 0-14 years) who underwent intraureteral injection of NASHA/Dx gel under direct ureteroscopic visualization for the treatment of primary high-grade VUR during the period June 2006-June 2010 were reviewed. RESULTS: Eighty-nine children (61 boys, 28 girls; M:F ratio, 2.1) underwent intraureteral injection of NASHA/Dx gel under direct ureteroscopic visualization for the treatment of primary high-grade VUR during the study period. VUR completely disappeared after the injection of NASHA/Dx gel into 105 (73%) of 144 ureters, with no further treatment required. Thirty-five (24.3%) required a second injection, and 2 (1.4%) required a third injection for resolution of their VUR. No intraoperative complications were observed. No ureteral obstruction during follow-up was observed using ultrasound or micturition studies. CONCLUSIONS: Intraureteral injection of NASHA/Dx gel under direct ureteroscopic visualization is safe and effective in the treatment of primary high-grade VUR, including cases with ureteral duplication, if the ureteral meatus is easy to pass through without mechanical dilation. This approach represents an effective and safe alternative to antibiotic prophylaxis alone and open surgery.

Strictureplasty and intestinal resection: different options in complicated pediatric-onset Crohn disease.

BACKGROUND/PURPOSE: Surgical resection or strictureplasty (SP) are different options for intestinal Crohn disease (CD) strictures. The aim of this article is evaluation of long-term outcome of SP and resection. METHODS: From 1996 to 2011, 39 patients (23 male, 16 female) with symptomatic ileal and ileocolonic CD strictures resistant to medical/nutritional therapy and treated with surgery in 2 different surgical units were reviewed. The mean age at diagnosis was 11.82 years (range, 4-17 years). Mean age at surgery was 15.94 years (range, 4-24 years). Mean follow-up was 6.88 years (range, 0.5-15 years). Patients underwent resection (group A) or different SP techniques (group B). RESULTS: Twenty patients underwent intestinal resection (ileal or ileocolonic resection), and 19 patients underwent SP (jejunal, ileal, or ileocolic). Early postsurgical complications were observed in 2 patients of group A. Follow-up of group A patients revealed that 1 patient needed emergency treatment after 8 months surgery because of adhesions and 1 patient developed recurrence treated with medical therapy. In the follow-up group B, 3 patients experienced disease recurrence, 2 of them at the site of previous surgery. CONCLUSIONS: At long-term follow-up, no significant difference in relapsing rate was observed between the 2 groups. Strictureplasty and resection represent an effective treatment of pediatric CD strictures. Strictureplasty could represent the first option for intestinal preservation.

Provisional unicentric experience with an electronic incident reporting form in pediatric anesthesia.

OBJECTIVE: To present and compare with literature our experience with an electronic anesthesia-related incident reporting form as a quality control measure at Gaslini Children's Hospital over a 19-month period. METHODS: All events that occurred between March 2009 and September 2010 were recorded. We adopted an electronic reporting form included in the online recording process of every anesthetic procedure. Events were divided into near misses and adverse events. Adverse events were further divided into incidents, minor events, and major events. Patients were divided into three age-groups: <1, between 1 and 3, and >3 years. RESULTS: A total of 12,850 anesthetics were performed. Eight (0.06%) near misses and 108 (0.8%) adverse events were reported. Adverse events occurred more frequently in infants. Of 108 events, 35 (32.4%), 61 (56.5%), and 12 (11.1%) were classified as incidents, minor, and major events, respectively. Of all the adverse events, 66 (61%) were respiratory, 27 (25%) organizational, six (5%) drug-related, four (4%) cardiocirculatory, and five (5%) miscellaneous. CONCLUSIONS: Infants were at the highest risk to experience adverse events. Although experimental electronic incident reporting proved to be feasible, there is reason to suspect that there was underreporting of near misses. Overreporting of near miss events may be enhanced by easier and more straightforward reporting forms as well as by better education for anesthetic providers about the importance of recognizing and reporting near misses.

Near-miss events are really missed! Reflections on incident reporting in a department of pediatric surgery.

PURPOSE: The aim of this study was to evaluate the frequency of surgical and organizational events that occurred in the whole Department of Paediatric Surgery at Gaslini Children's Hospital through an incident-reporting system in order to identify the vulnerabilities of this system and improve it. MATERIALS AND METHODS: This is a 6-month prospective observational study (1st January-1st July 2010) of all events (including surgical and organizational events, and near misses) that occurred in our department of surgery (pediatric surgery, orthopedics and neurosurgery units). RESULTS: Over a 6-month study period, 3,635 children were admitted: 1,904 out of 3,635 (52.4%) children underwent a surgical procedure. A total number of 111 adverse events and 4 near misses were recorded in 100 patients. A total of 108 (97.3%) adverse events occurred following a surgical procedure. Of 111 adverse events, 34 (30.6%) required re-intervention. Eighteen of 100 patients (18%) required a re-admission, and 18 of 111 adverse events (16.2%) were classified as organizational. Infection represented the most common event. CONCLUSIONS: An electronic physician-reported event tracking system should be incorporated into all surgery departments to report more accurately adverse events and near misses. In this system, all definitions must be standardized and near misses should be considered as important as the other events, being a rich source of learning.

Intraoperative bronchoscopy for bronchial carcinoid parenchymal-sparing resection: a pediatric case report.

Bronchial carcinoid tumors are the most common primary pulmonary neoplasm in the pediatric population. The widely accepted treatment for carcinoid tumors is surgical, specifically aiming at being as much as conservative on lung parenchyma, while the entire tumor is resected. A brief case is described, highlighting the importance and advantages of a surgical and endoscopic combined approach.

Technical considerations in children undergoing laparoscopic ileal-J-pouch anorectal anastomosis for ulcerative colitis.

BACKGROUND: Sub-total colectomy and restorative proctocolectomy with j-pouch ileo-anorectal anastomosis is the treatment of choice in children with ulcerative colitis uncontrolled with medical therapy. OBJECTIVE: To present some technical considerations about children undergoing laparoscopic ileal-J-pouch anorectal anastomosis. SETTINGS AND PATIENTS: All patients with ulcerative colitis undergoing laparoscopic ileal-J-pouch anorectal anastomosis were evaluated from January 2006 to February 2011. INTERVENTION: The new technical innovations herein are (1) total laparoscopic approach, (2) a very short 3-cm J-pouch ileal reservoir created outside the stoma incision, (3) preservation of the entire anal canal and the Knight-Griffen double stapled anastomosis, less than 3 cm from the dentate line, (4) use of a Multiple Instrument Access Port system in the stoma skin incision to reduce the number of port site incisions and (5) proctectomy performed using only an electrosurgical vessels sealing device thus avoiding clips to close rectal pedicle. RESULTS: Seventeen laparoscopic ileo J-pouch low rectal anastomosis were performed by the same surgical staff. Three complications occurred postoperatively: one bowel obstruction, one ileostomy prolapse, and one anastomotic stricture. Satisfactory functional results were achieved in all, there was no significant perineal excoriation and quality of life was excellent. CONCLUSIONS: A Multiport Instrument Access Port placed in the stoma site allowed the use of more instruments through a single incision. The very short ileo J-pouch low rectal anastomosis has been shown to be a safe, feasible, and effective reconstructive procedure.

Phenotypic spectrum and management of sternal cleft: literature review and presentation of a new series.

Sternal cleft is a chest wall malformation that can expose mediastinal viscera and vessels to injuries. It can be classified into two forms, complete and partial. Its etiology and incidence are unknown and it is often associated with other defects. The aim of this article is to review the literature and report our experience with this rare anomaly, focusing on clinical presentation and management. We reviewed the English written literature about sternal cleft and collected the clinical data of all the published series. We present seven new cases that we have observed and treated since 1999. Literature reports 51 series including 86 patients, more frequently female (62%) and affected with partial superior form (67%). Sternal cleft is often asymptomatic (74%) and associated with other defects (72%). Surgical treatments include primary closure (73%), bone graft interposition (10%), prosthetic closure (7%), and muscle flap interposition (3%). In our series, primary closure was possible in four cases, while in three cases we placed a prosthesis. Five patients had associated defects and two were affected with PHACES (posterior fossa abnormalities, hemangiomas, arterial lesions, cardiac abnormalities/aortic coarctation, abnormalities of the eye, and sternum defects) syndrome. We report for the first time the association of sternal cleft with connectival nevi in three of our patients. At follow-up, we observed no major complication or recurrences. Although primary closure is the preferred option and should be performed in the neonatal period, the use of prostheses warrants good results as well. Prior to treatment, associated defects and syndromes should be excluded.

Gaslini's tracheal team: preliminary experience after one year of paediatric airway reconstructive surgery.

BACKGROUND: Congenital and acquired airway anomalies represent a relatively common albeit challenging problem in a national tertiary care hospital. In the past, most of these patients were sent to foreign Centres because of the lack of local experience in reconstructive surgery of the paediatric airway. In 2009, a dedicated team was established at our Institute. Gaslini's Tracheal Team includes different professionals, namely anaesthetists, intensive care specialists, neonatologists, pulmonologists, radiologists, and ENT, paediatric, and cardiovascular surgeons. The aim of this project was to provide these multidisciplinary patients, at any time, with intensive care, radiological investigations, diagnostic and operative endoscopy, reconstructive surgery, ECMO or cardiopulmonary bypass. Aim of this study is to present the results of the first year of airway reconstructive surgery activity of the Tracheal Team. METHODS: Between September 2009 and December 2010, 97 patients were evaluated or treated by our Gaslini Tracheal Team. Most of them were evaluated by both rigid and flexible endoscopy. In this study we included 8 patients who underwent reconstructive surgery of the airways. Four of them were referred to our centre or previously treated surgically or endoscopically without success in other Centres. RESULTS: Eight patients required 9 surgical procedures on the airway: 4 cricotracheal resections, 2 laryngotracheoplasties, 1 tracheal resection, 1 repair of laryngeal cleft and 1 foreign body removal with cardiopulmonary bypass through anterior tracheal opening. Moreover, in 1 case secondary aortopexy was performed. All patients achieved finally good results, but two of them required two surgeries and most required endoscopic manoeuvres after surgery. The most complex cases were the ones who had already been previously treated. CONCLUSIONS: The treatment of paediatric airway anomalies requires a dedicated multidisciplinary approach and a single tertiary care Centre providing rapid access to endoscopic and surgical manoeuvres on upper and lower airways and the possibility to start immediately cardiopulmonary bypass or ECMO.The preliminary experience of the Tracheal Team shows that good results can be obtained with this multidisciplinary approach in the treatment of complicated cases. The centralization of all the cases in one or few national Centres should be considered.

Removal of pectus bar fixed with absorbable vs metallic stabilizers.

BACKGROUND/PURPOSE: The removal of a pectus bar fixed with a metallic stabilizer can be time consuming and tedious, because in some cases, fibrous or new bone tissue covers the metallic devices. Our study aims to evaluate bar removal in 2 groups of patients with metallic and absorbable stabilizers, respectively. METHODS: A total of 162 patients underwent mini-invasive repair of pectus excavatum. In all the cases, the bar was stabilized with at least 1 stabilizer on the left side. We used both metallic and absorbable stabilizers. Absorbable stabilizers were preferred when they were available in the market. The bar was removed in 30 patients. We compared removal of the bar in 17 absorbable stabilizers with those bars fixed with 18 metallic stabilizers. Length of incision, operative time, postoperative pain, and complications were studied. RESULTS: No differences between metallic and absorbable stabilizers were found in terms of postoperative pain and complications. However, removal of the bar fixed with an absorbable stabilizer required a significantly smaller incision and shorter operative time. CONCLUSIONS: Removal of the pectus bar fixed with an absorbable stabilizer was simpler and faster.

Laparoscopic approach for children with inflammatory bowel diseases.

BACKGROUND: Minimally invasive surgery is being increasingly applied to inflammatory bowel diseases (IBDs). Few pediatric series from selected research have been described to date. This study describes a unicentric experience of laparoscopic treatment of children with IBDs. MATERIALS AND METHODS: All consecutive patients with IBDs between February 2006 and February 2010 who underwent laparoscopic treatment were included. We reviewed notes and recorded demographic data, indications, perioperative management, surgical details, length of surgery, complications, postoperative management, length of hospitalization and functional outcome. RESULTS: We performed 25 procedures on 16 patients (12 ulcerative colitis, 3 Crohn's disease, and 1 indeterminate colitis). Median age was 12 years. A total of 50% patients underwent elective surgery; 11 underwent staged laparoscopic subtotal colectomy (LSTC) followed by J-pouch ileorectal anastomosis (JPIRA). Three patients underwent straight LSTC + JPIRA. All procedures included protective ileostomy. Length of surgery ranged between 120 and 380 min depending on the procedure (LSTC ± JPIRA). No conversion was required. Length of hospitalization ranged between 3 and 18 days. We observed six complications (24%) mainly represented by adhesions that were effectively treated laparoscopically. Ten patients were restored (ileostomy closure) and were assessed for continence that turned out to be good in 80%. CONCLUSIONS: Laparoscopy proved to be feasible, safe and effective for the treatment of IBD in children. Although we observed a relatively low incidence of complications, stoma site adhesions still remain the major issue, which can be effectively dealt with laparoscopically. Functional outcome as well as cosmesis is satisfactory. As results are encouraging, at present we prefer laparoscopy for the surgical treatment of IBD in pediatric patients.

Acid and weakly acid gastroesophageal refluxes and type of respiratory symptoms in children.

OBJECTIVES: To evaluate the association between the frequency of acid reflux (AR) and weakly acid reflux (WAR) and specific respiratory symptoms (RS) in childhood. STUDY DESIGN: We retrospectively reviewed medical records of children with difficult-to-treat RS, not under acid suppressive therapy, and with a positive multiple intraluminal esophageal impedance (pH/MII) monitoring. To discriminate children with prevalent AR and WAR events, a ROC curve was designed and the distribution of the different RS in children with prevalent AR or WAR events was analyzed. RESULTS: A higher number of AR over WAR events was detected (p < 0.0001) but the WAR-to-AR events ratio progressively decreased with the age of the subjects (p < 0.01). Similar total number of reflux events was found in the three age group and in children with a more prevalent WAR or AR. The most prevalent RS, equally distributed among the three age groups, were persistent and/or nocturnal cough, wheezy bronchitis/asthma, and recurrent lower respiratory tract infections (RLRTI). Apnoea was most frequent in infants (p = 0.036). A higher frequency of RLRTI, but not of nocturnal cough or wheezy bronchitis/asthma, was shown in WAR as compared with AR patients (p = 0.040), and specifically those in the school-aged group (p = 0.013). Age and WAR were respectively identified as independent predictors of apnoea and RLRTI (p < 0.05). CONCLUSION: WAR events are common in children with gastroesophageal reflux and difficult-to-treat RS and often associated with RLRTI. These findings support the role of pH/MII monitoring in the evaluation of these patients and may explain the disappointing clinical results often observed with anti-acid treatments.

Hirschsprung's disease: what about mortality?

PURPOSE: Although significantly decreased during last decades, mortality rate for Hirschsprung's disease still ranges between 1 and 10%. The authors reviewed the main features of patients with Hirschsprung's disease treated in our Institution who died in the period between 1993 and 2010 in order to detect possible risk factors or prevention strategies. METHODS: The notes of all patients with Hirschsprung's disease admitted to our Institution between January 1993 and January 2010 were reviewed. All families were interviewed and possible unknown deaths were recorded. We reported demographic data, length of aganglionosis, type and number of associated anomalies, age and type of onset, age and cause of death, chronological relationship between death and pull-through, and all other significant details. RESULTS: Eight out of a series of 313 patients with Hirschsprung's disease died during the study period (mortality rate = 2.56%). Six patients were male for a male to female ratio of 3:1. Eleven associated anomalies were detected in five patients, including four congenital anomalies of the kidney and urinary tract, four heart, two central nervous system, and one skeletal malformations. One patient had Down Syndrome and one Cat Eye Syndrome. Two patients were born preterm. All patients had neonatal onset with delayed meconium passage. Five patients had aganglionosis confined to rectosigmoid colon (classic forms), two had total colonic aganglionosis and one had total intestinal aganglionosis. Enterostomy was performed in five patients. Median age at death was 75 days (range 30 days-8 years). The cause of death was enterocolitis in four patients, heart failure in three, and intestinal failure in one. Six patients died before the pull-through. Two patients died postoperatively. All patients but two died at home. CONCLUSIONS: Onset and clinical features do correlate with severity. Newborns and infants seem to be more likely to develop serious life-threatening complications, particularly in case of associated cardiovascular malformations. Although enterostomies do not have protective effects over cardiovascular issues, prophylactic stoma should be considered in high-risk patients. Radical treatment (pull-through) should be performed as soon as possible. Alternatively, protected and cautious discharge of newborns with associated heart anomalies is strongly recommended in order to prevent serious and uncontrolled complications, regardless of the presence of a protective enterostomy. Postoperatively, close follow-up appointments are recommended. Families should be acknowledged and educated for prompt recognition and treatment of severe life-threatening complications.