J Anesth. 2012 Feb 1;
Collard V, Segura LG, Daaboul DG, Yuki K

Retrograde intubation is part of the difficult airway algorithm in the American Society of Anesthesiologists, but its usage is rare in small pediatric patients with the advent of new intubation techniques. We present our experience of retrograde intubation for a 4-month-old patient who presented for laryngeal cleft repair on cardiopulmonary bypass. This case highlights the unique place for retrograde intubation in small patients in the current era.

Ital J Pediatr. 2011; 37: 51
Torre M, Carlucci M, Avanzini S, Jasonni V, Monnier P, Tarantino V, D'Agostino R, Vallarino R, Della Rocca M, Moscatelli A, Dehò A, Zannini L, Stagnaro N, Sacco O, Panigada S, Tuo P

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.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.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.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.

Eur Arch Otorhinolaryngol. 2012 Jan; 269(1): 345-8
Birkent H, Durmaz A, Karakoc O, Ilica T, Gerek M

Congenital clefts of the larynx are rare and usually found dorsally. An anterior or ventral cleft of the larynx is extremely rare. Only a few patients with this defect have been reported in the literature. The purpose of this paper is to present a patient having an anterior and posterior laryngeal cleft together. A 20-year-old man presented with a history of dysphonia since childhood. He did not report symptoms of swallowing or respiration, and had no history of neck trauma. Findings of videolaryngoscopy showed a grossly abnormal larynx. The anterior commissure was wider than normal, and the vocal folds did not show a fusion anteriorly. There was an interarytenoid cleft posteriorly. A neck CT with 3D reconstruction demonstrated a ventral cleft or nonfusion of the thyroid cartilage with a posterior cricoid cleft. Barium swallow study was in normal limits. Since the patient did not have any problem with swallowing or respiration, no surgical intervention was planned, and the patient was put on speech therapy, which revealed improvement in voice. To our knowledge, this is the first case of a combined laryngeal cleft. The diagnosis is established by the clinical symptoms, endoscopic evaluation, and radiographic examinations including 3D and barium studies.

Eur J Pediatr Surg. 2011 Sep 29;
Wood JA, Carachi R

A right-sided aortic arch (RAA) occurs in around 5% of patients with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF). This anatomical variation can complicate the operative management of these patients, as it is often not diagnosed preoperatively but only discovered at thoracotomy, and it remains unproven as to whether a right or left thoracotomy is the best operative approach. This retrospective study aimed to determine the prevalence of RAA in OA/TOF, review the accuracy of preoperative investigations, and investigate the best operative approach, by reviewing the literature and our own patient series.The case notes of all infants with OA/TOF over a 15 year period (1994-2008) were retrospectively analysed to identify those with a RAA. Birth weight, gestational age, associated anomalies, preoperative investigations, surgical management, postoperative complications and long-term prognosis were all extracted.A total of 107 case notes of OA/TOF infants were reviewed, identifying 4 with a RAA. Preoperative echocardiography was performed in all of the 4 RAA infants, but RAA was only identified in one. All 4 infants were managed surgically via a right thoracotomy, regardless of the echocardiography result, with primary anastomosis achieved successfully in all. A laryngeal cleft repair was performed in 1 infant due to an interarytenoid cleft. Laparoscopic fundoplication was performed in 1 patient, because of severe gastro-oesophageal reflux. There were no postoperative anastomotic leaks, bleeding, or deaths in this group.In our study, the incidence of RAA in OA/TOF was 3.7%. Preoperative echocardiography identified the RAA in only 1 of 4 cases. However, echocardiography was helpful for diagnosing other cardiac anomalies, which might have potentially affected the management of these patients. Previous studies have cited the operative difficulties associated with RAA and OA/TOF. However, in this series of 4 infants, primary anastomosis was achieved via conventional right thoracotomy without complication, and with no effect on outcome or prognosis. Therefore, we conclude that, where possible, a conventional right-sided thoracotomy should be performed in OA/TOF patients with a RAA.

Int J Pediatr Otorhinolaryngol. 2011 Dec; 75(12): 1525-8
Thiel G, Clement WA, Kubba H

To review the clinical presentation and management of all infants and children presenting with laryngeal clefts to a tertiary pediatric ENT centre and to identify changes in practice over time.A retrospective case note review of the management of all infants and children with a diagnosis of a laryngeal cleft identified in our Department between 01/11/2003 and 31/12/2010.Twelve children with laryngeal clefts were identified. Six clefts were grade 1, five grade 2 and one grade 3b. All grade 1 clefts were managed conservatively. Of the grade 2 clefts, four required surgery with one being managed conservatively. Two were repaired using an open technique and two using an endoscopic technique. The grade 3b cleft was repaired endoscopically. Two cleft repairs broke down post-operatively requiring further surgery.Conservative management remains the management of choice for lower grade clefts. Where a laryngeal cleft requires repair there has been a trend towards the endoscopic over open technique, even of more extensive clefts.

Pediatr Pulmonol. 2011 Nov; 46(11): 1128-33
Williams JL, Lee EY, Casey AM, Haver KE, Buonomo C, Zurakowski D, Rahbar R

The purpose of this study was to (1) evaluate chest radiography (CR) and computed tomography (CT) findings in pediatric patients with laryngoscopically confirmed laryngeal cleft and (2) determine whether CT provided additional information over CR in evaluating lung abnormalities in pediatric patients with laryngeal cleft.Two pediatric radiologists performed a retrospective review of CRs and CTs in pediatric patients with laryngoscopically confirmed laryngeal cleft from January, 2002 to January, 2010. Lungs were evaluated for pattern (airspace or interstitial), distribution (upper, middle, or lower lung zones), and extent (<25% or >25%) of abnormalities on CRs and CTs. In patients who had both CR and CT, CT findings were compared with concurrent CRs to determine whether CT provided additional information and resulted in alterations in patient management.The final study cohort consisted of 67 pediatric patients (37 males and 30 females; mean age 3.0 years ± 3.5 years; range 25 days to 17.5 years) who had CR. Sixteen (24%) of these 67 patients had both CR and CT. Chest radiographs were abnormal in 47 patients (70%) which included consolidation (n = 35; 74%) and reticular opacities in 12 patients (26%). Lung parenchymal abnormalities were observed in the upper (n = 10; 21%), middle (n = 2; 4%), or lower (n = 35; 75%) lung zones on CR. The extent of lung parenchymal abnormalities was >25% in 30 patients (64%) and <25% in 17 patients (36%). Chest CT examinations were abnormal in all 16 patients (100%) who had available chest CT examinations, which included consolidation (n = 9; 56%) and reticular opacities (n = 7; 44%). Lung parenchymal abnormalities were observed in the upper (n = 2; 12.5%) or lower (n = 14; 87.5%) lung zones on CT. The extent of lung abnormalities was >25% in 10 patients (60%) and <25% in six patients (40%). CT provided major additional findings in 12 patients (75%) and minor findings in four patients (25%). Additionally, CT improved the evaluation of the distribution and extent of lung abnormalities in 11 patients (69%). The additional information provided by CT resulted in alteration of patient management in seven patients (43.8%).Among pediatric patients with laryngeal cleft, CR and CT often show lung abnormalities such as consolidation and reticular opacities. CT frequently provides additional information, which may change patient management.

Otolaryngol Head Neck Surg. 2011 Feb; 144(2): 262-7
Silver AL, Yager P, Purohit P, Noviski N, Hartnick CJ

Assess the postoperative use of dexmedetomidine (Precedex) in pediatric patients following airway reconstruction.Historical cohort study.Tertiary medical center.A retrospective review of 24 children undergoing laryngotracheal reconstruction (LTR) or laryngeal cleft repair (LCR) was conducted. Twelve children were treated with standard sedation protocols where dexmedetomidine was administered in lieu of propofol (Diprivan); 12 age-, gender-, and procedure-matched controls were selected. Subjects were divided into groups based on duration of postoperative intubation for cross-comparison; group 1 was intubated <24 hours, group 2 was intubated 2 to 6 days, and group 3 was intubated 7 days or longer. Baseline heart rate and blood pressure measurements were compared to hourly measurements for the first 6 hours following initiation of dexmedetomidine or mechanical ventilation in the control group. Number of supportive respiratory interventions, adverse events, self-extubations, premature termination of dexmedetomidine, amount of muscle relaxants, agents to treat withdrawal, and length of stay were evaluated.Ten patients undergoing LTR and 2 patients undergoing LCR receiving dexmedetomidine were compared to 10 LTR and 2 LCR control patients. Overall, dexmedetomidine was well tolerated and without significant adverse effects, particularly in cases of short-term intubation or as a bridge to extubation.In cases requiring short-term intubation following airway reconstruction, dexmedetomidine may offer a safe alternative to propofol by providing readily reversible sedation during the periextubation period. Further studies are needed to determine the safety, efficacy, dosing, and potential complications of longer term dexmedetomidine administration in pediatric airway reconstruction.

Otolaryngol Head Neck Surg. 2011 May; 144(5): 789-93
Cohen MS, Zhuang L, Simons JP, Chi DH, Maguire RC, Mehta DK

To review the 2-year, single-institution experience with injection laryngoplasty for diagnosis and treatment of type 1 laryngeal clefts (LC-1).Case series with chart review.Tertiary care academic children's hospital.Patients at our institution who underwent injection laryngoplasty for LC-1 from January 2008 to December 2009. Outcome measures included patient demographics, surgical and anesthetic technique, effect on swallowing, and complications. Preoperative and postoperative swallowing evaluations were compared, and quality and duration of effects were calculated.Sixteen children, 9 male and 7 female, underwent injection laryngoplasty for LC-1. Mean gestational age was 36.4 weeks (SD, 4.0 weeks; range, 27-41 weeks). Six patients had a major congenital anomaly (37.5%). Mean age at injection was 11.8 months (SD, 8.9 months; range, 2.9-33.5 months). Nine patients (56%) demonstrated complete resolution of penetration and aspiration on postoperative modified barium swallow (MBS), 4 patients (25%) had some improvement, and 3 patients (19%) showed no change. There were no complications. Mean duration of symptom improvement was 3.3 months (SD, 3.0 months; range, 0-11 months). Five patients went on to have definitive surgical LC-1 repair.Injection laryngoplasty was found to have favorable results in this group of patients with LC-1. Most patients experienced complete resolution of aspiration on MBS, and no patients experienced complications. These findings support the further study of injection laryngoplasty for LC-1 as both a diagnostic and therapeutic technique. Longer followup intervals are necessary to elucidate the predictive value with regard to success of formal cleft repair.

J Laryngol Otol. 2011 May; 125(5): 513-6
Broomfield SJ, Bruce IA, Rothera MP

Traditionally, small laryngeal clefts may be closed endoscopically, while larger clefts necessitate an open anterior approach. We report the presentation, evaluation and outcome following endoscopic surgical repair of a series of laryngeal clefts.Retrospective study of children treated in a tertiary referral centre between 2003 and 2008. The presenting symptoms, patient demographics, cleft type, surgical outcome and complications were evaluated.Seven children underwent primary endoscopic repair of their laryngeal clefts (four Benjamin-Inglis type III clefts and three type II clefts). Presenting symptoms included stridor, cough and cyanosis with feeds, swallowing problems, weak cry, and recurrent lower respiratory tract infection. Treatment was ultimately successful in six of the seven children; treatment was ongoing for the remaining child, who underwent subsequent revision surgery via an open approach. Two children went on to require a second endoscopic repair, and two underwent an open repair of a residual defect. One child required a tracheostomy for failed extubation in the post-operative period.Endoscopic repair is a safe, useful technique in the management of laryngeal clefts. Laryngeal clefts must be excluded in a child presenting with persistent aerodigestive tract symptoms, as described here.

Genet Couns. 2010; 21(3): 289-92
Ipek MS, Zenciroglu A, Aydin M, Okumus N, Orun UA, Uner C

We report a case of VACTERL complex which had concomitant horseshoe lung, laryngeal cleft, and hypertrophic pyloric stenosis, which has not been previously reported.

Laryngoscope. 2010 Nov; 120(11): 2227-30
Maturo S, Hartnick CJ

Ir Med J. 2010 Mar; 103(3): 77-9
Zaid AA, Elnazir B, Greally P

This study aimed to determine the aetiology, clinical presentation, co-morbidity, severity and the lobar distribution of non cystic fibrosis bronchiectasis (NCFB). We performed a retrospective review of clinical, radiological, immunological and microbiological data from 92 non-CF patients with a High resolution thoracic CT (HRCT) diagnosis of bronchiectasis in the three Dublin Children's referral Hospitals for the period 1996-2006. Of 92 patients (50 female), the median age at diagnosis was 6.4 years. The aetiology of bronchiectasis was as follows; idiopathic 29 (32%), post-pneumonia 16 (17%), immune deficiency 15 (16%), recurrent aspiration 15 (16%), primary ciliary dyskinesia 8 (9%), chronic aspiration with immune deficiency 5 (5%), post foreign body inhalation 2 (2%), tracheomalacia 1 (1%) and Obliterative bronchiolitis 1 (1%). Bronchial asthma and gastroesophageal reflux disease (GORD) were concurrently present in 18 (20%) and 10 (11%) respectively. Left lower lobe was commonly involved followed next by the right middle lobe. The common isolates were Haemophilus influenza (50), Streptococcus pneumoniae (34) and Staphylococcus aureus (14), Moraxella catarrhalis (9) and Pseudomonas auerginosa (8). Surgical interventions were performed in 23 (25%) of patients, lobectomy 11 (12%), pneumectomy 2 (2%), laryngeal cleft repair 4 (5%), rigid bronchoscopy for foreign body removal 2 (2%), Nissan's fundoplication 2 (2%), tracheoesophageal fistula repair 2 (2%). We conclude NCFB is under-recognised in Irish children and diagnosis is often delayed and Bronchial Asthma and GORD are common co morbidity. A high index of suspicion and early HRCT can expedite the diagnosis.