W. Andrew Clement

Unilateral Vocal Cord Paralysis Following Patent Ductus Arteriosus Ligation in Extremely Low-Birth-Weight Infants

OBJECTIVE To determine if unilateral vocal cord paralysis (UVCP) following patent ductus arteriosus (PDA) ligation is associated with respiratory and swallowing morbidities in extremely low-birth-weight (ELBW) infants. DESIGN Case-control study. SETTING Tertiary care neonatal intensive care units and pediatric hospital. PARTICIPANTS Twenty-three infants undergoing PDA ligation (subdivided into the main study group of 12 infants with UVCP and 11 without paralysis) and 12 weight- and gestational age-matched ELBW controls. MAIN OUTCOME MEASURES Incidence of UVCP, time requiring supplemental oxygen and ventilatory support, length of hospital stay, incidence and duration of tube feeding following discharge, and incidence of chronic lung disease. RESULTS The overall incidence of UVCP was 52% (12/23), increasing to 67% (12/18) in ELBW infants. Infants without UVCP following PDA ligation were heavier (P = .006), with a more advanced gestational age (P = .03). Patients with UVCP required longer tube feeding (relative risk, 8.25; 95% confidence interval, 1.93-46.98; P = .003), supplemental oxygen (P = .004), and ventilatory support (P = .001) and had a longer hospital stay (P < .001). In comparison to matched controls, infants with UVCP required longer tube feeding (relative risk, 9.00; 95% confidence interval, 2.08-51.30; P = .003), supplemental oxygen (P = .03), and ventilatory support (P = .002) and had a longer hospital stay (P < .001). CONCLUSIONS There was a high incidence of occurrence of UVCP (67%) associated with PDA ligation in ELBW infants. Unilateral vocal cord paralysis following PDA ligation does seem to be associated with increased requirements for tube feeding, respiratory support, and hospital stay in these ELBW infants.

A prospective randomized single-blind trial comparing ultrasonic scalpel tonsillectomy with tonsillectomy by blunt dissection in a pediatric age group.

OBJECTIVE: To determine if there was a difference in postoperative pain and dietary intake when comparing ultrasonic scalpel (US) with blunt dissection tonsillectomy (BD) in children. STUDY DESIGN AND SETTING: A prospective single-blind randomized controlled trial comparing US and BD tonsillectomy in children aged between 5 and 13 years who undergo tonsillectomy for recurrent acute tonsillitis in a specialized pediatric hospital. RESULTS: One hundred and twenty-two children were enrolled. Ninety-three (76%) patients completed dietary and pain scales. Pain was significantly greater in the US group on postoperative days 1 and 3 (P = 0.0466, P = 0.0084) and overall (P = 0.0003). Dietary intake scores were significantly better in the US group on days 1, 5, 7, and 9, however, these scores were inconsistent with dietary intake scores declining in both groups throughout the study. Blood loss was statistically significantly greater in the BD group (33.1 ml vs 3.0 ml, P < 0.0001). There were no significant differences for all other observed outcomes. CONCLUSIONS: US tonsillectomy causes significantly more pain overall and on postoperative days 1 and 3 when compared to BD tonsillectomy. US tonsillectomy causes less intraoperative blood loss, the clinical significance of which is questionable. SIGNIFICANCE: US tonsillectomy appears to cause more postoperative pain than BD tonsillectomy in children aged 5 to 13 years undergoing tonsillectomy for recurrent acute tonsillitis.

The management of laryngeal clefts

OBJECTIVE 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. PATIENTS AND METHODS 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. RESULTS 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. CONCLUSIONS 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.

Laser division of congenital complete tracheal rings

Congenital complete tracheal rings are a rare and life threatening problem in young children and they are often challenging to manage. Whilst historically associated with high mortality rates increasing experience with this tracheal pathology has led to much improved survival rates and slide tracheoplasty has become the treatment of choice. We present 3 cases in which an open procedure was not deemed possible and they underwent laser division (CO2, KTP) of their complete rings. Two patients subsequently required stent insertion. All patients are alive and well at a mean follow up of 3 years and 5 months. Whilst laser division of complete tracheal rings has only been described in a small number of cases it may provide an alternative approach in patients who are not able to undergo an open procedure or in an emergency situation.

Otolaryngological presentations of Cornelia de Lange syndrome.

AIM Children with Cornelia de Lange syndrome frequently present to otolaryngology services with hearing problems. Airway problems have not previously been reported. We wish to describe our experience of the overall management in a series of children with Cornelia de Lange syndrome. METHODS Retrospective case note review of children diagnosed with Cornelia de Lange syndrome presenting to our department between 2005 and 2014. RESULTS Six patients were seen. Airway problems consisted of laryngeal overspill with severe gastroesophageal dysmotility and reflux despite structurally normal airway (1 case), laryngomalacia requiring supraglottoplasty (2 cases), reflux laryngitis with secondary laryngomalacia and coincidental tracheal diverticulum (1 case) choanal atresia requiring stents (1 case) and obstructive sleep apnoea (1 case). Supraglottoplasty produced a dramatic improvement in feeding and breathing in both children who underwent the procedure. Two children had palatal anomalies and one underwent cochlear implantation for a profound sensorineural hearing loss. CONCLUSION Children with Cornelia de Lange syndrome have multifaceted ENT problems. Airway pathology has not previously been described in Cornelia de Lange syndrome but has been common in our experience. We wish to highlight that laryngomalacia in Cornelia de Lange syndrome responds well to supraglottoplasty.

Early identification of pituitary dysfunction in congenital nasal pyriform aperture stenosis: recommendations based on experience in a single centre.

Background: Congenital nasal pyriform aperture stenosis (CNPAS) is an increasingly recognised cause of upper airway obstruction associated with midline abnormalities. Studies have described pituitary dysfunction in 40% of patients. We aimed to develop guidelines for: (a) the early identification of pituitary insufficiency to minimise surgical risk and (b) to stratify patients for follow-up. Methods: Retrospective case note review of patients with CNPAS between 2000 and 2014 in a tertiary paediatric unit. Results: 20 patients (12 female:8 male) were analysed; 16 were diagnosed during the neonatal period while 4 were diagnosed later. There was no consistent approach in the evaluation of the pituitary axis at diagnosis. Pituitary dysfunction was identified in 3 (15%) patients, 2 of whom were found during evaluation of short stature in mid-late childhood. Hypoglycaemia and conjugated hyperbilirubinaemia, but not the degree of stenosis, were highly predictive of pituitary dysfunction (p < 0.05). Available height standard deviation score (SDS) data at 1 year of 70% of our patients identified both of the late-diagnosed growth hormone-deficient patients, with SDS of -2.6 and -3.6, respectively. Conclusion: All CNPAS patients should have MRI of the brain and baseline endocrine investigations at diagnosis. Growth monitoring for at least 1 year is recommended as low, or falling, height SDS at 1 year is a good predictor of pituitary dysfunction.

Management of congenital cartilaginous sleeve trachea in children.

AIMS Children with congenital tracheal cartilaginous sleeve may present to otolaryngology services with airway problems. We wish to describe our overall management in a series of four children with this very rare anomaly. METHODS Retrospective case note review of children diagnosed with congenital tracheal cartilaginous sleeve presenting to our department between 2006 and 2014. RESULTS Four patients were seen. One had Opitz G syndrome, two had Pfeiffers syndrome and one had no associated anomalies. Two children were successfully managed with laryngeal reconstruction using an anterior costal cartilage graft, while the third and fourth required a short period of tracheostomy only. All four are well and currently asymptomatic from an airway point of view. CONCLUSION Congenital tracheal cartilaginous sleeve is a very rare and potentially challenging problem. Otolaryngologists should be aware that it can occur in children with syndromes other than craniosynostosis (and indeed, those with no syndrome) and that it can be successfully treated using established airway management techniques. ETHICAL APPROVAL Registered with Clinical Governance Committee.