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

Soave's extramucosal endorectal pull-through procedure.

The use of the abdominal extramucosal dissection of the rectal pouch was first proposed by Romualdi at the Roman Society of Surgery on 15 May 1955. The technique was first published in 1960 [1]. During the next few years, Rehbein [2] and Kiesewetter and Turner [3] also popularized this operation. In 1957, Soave started using Romualdi\u2019s procedure for the treatment of anorectal malformations with urethral fistula. Since his initial experience with Romualdi\u2019s technique, he thought that this principle could be applied for the treatment of Hirschsprung\u2019s disease (HSCR). So, in 1961, he performed his first operation on a 2-year-old boy with the classic form of HSC

Hirschsprung's Disease and Related neuromuscular Disorders of the Intestine

Hirschsprung's disease may present initially as perforated appendicitis, commonly in the newborn period.15,201,287 Serial biopsies of the colon are required in infants who present with such a clinical picture. In general, these perforations often denote longer lengths of aganglionosis. A great deal of controversy surrounds the use of the appendix to diagnose total colonic Hirschsprung's disease; however, histologic evaluation of the appendix is generally an excellent diagnostic tool.10 Innervation of the appendix differs slightly from that of the rest of the intestine; in cases of total colonic aganglionosis, the appendix has a paucity of nerve fibers and neuroendocrine cells.295 Evaluating the appendix at a relatively early stage in the initial surgical exploration of a child with Hirschsprung's disease often expedites the diagnosis. In one case, a child with presumed total colonic aganglionosis and aganglionosis of the appendix was subsequently found to have normal ganglion cells in the transverse colon.10 This extremely rare case represents segmental Hirschsprung's disease

The Nuss procedure after breast augmentation for female pectus excavatum

Pectus excavatum, the most common congenital chest wall malformation, has a higher incidence among men. Since 1987, when Donald Nuss performed his technique for the first time, the minimally invasive approach has become the most widely used technique for treating pectus excavatum. Few reported studies have focused on the repair of female pectus excavatum. Women with pectus excavatum often present with breast asymmetry that may require breast augmentation, either before or after pectus excavatum repair. To the authors' knowledge, no reports on the Nuss procedure after breast implant surgery have been published. This report describes the case of a 26-yearold woman who underwent minimally invasive repair after breast implant surgery. The authors believe that for women with severe pectus excavatum, the Nuss procedure should be the first choice for surgical correction. Moreover, for breast implant patients, this technique is absolutely feasible without major complications. \ua9 Springer Science+Business Media, LLC and International Society of Aesthetic Plastic Surgery 2009

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

SummaryObjectivesTo evaluate the association between the frequency of acid reflux (AR) and weakly acid reflux (WAR) and specific respiratory symptoms (RS) in childhood.Study designWe 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.ResultsA 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).ConclusionWAR 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