Monozygotic twins and cholesteatomas: nature or nuture?

PURPOSE: Cholesteatoma is a rare middle ear pathology. It can be classified into acquired and congenital forms. Although benign, cholesteatomas can cause significant morbidity including hearing loss, infection, facial palsy and thrombosis. Congenital cholesteatomas are incredibly rare and bilateral disease has not commonly been published in the literature. METHOD: We describe the case of female identical (monozygotic, monochorionic, diamniotic) twins who both developed congenital cholesteatomas. In this report, we review the aetiology, treatment, embryology and pathology of cholesteatoma. RESULTS: The patients have been followed up 15 years after their initial surgery with promising results - pure-tone audiometry and repeat scans have not illustrated any disease recurrence. CONCLUSION: This paper presents one of the only cases of female monozygotic twins presenting with unilateral and bilateral cholesteatomas.

Vocal Cord Palsy in Children With Cancer: A 10-Year Analysis of UK Pediatric Intensive Care Admissions.

Vocal cord palsy (VCP) is a rare but potentially life-threatening complication in children with cancer. This study reviews UK Intensive Care admissions for children with cancer and VCP using data obtained from the Pediatric Intensive Care Audit Network (PICANet) database. 26 children with cancer and VCP were admitted to intensive care from 2002 to 2012. The majority of admissions (23/26) required respiratory intervention (17 invasive ventilation, 8 noninvasive ventilation, and 5 tracheostomy). VCP should be considered early in children with cancer who present with signs of upper airway obstruction, especially in those receiving vinca-alkaloids as VCP is likely to be reversible.

Management of third branchial pouch anomalies - an evolution of a minimally invasive technique.

OBJECTIVES: The management of third branchial pouch anomalies has evolved in recent times with the popularisation of the endoscopic diathermy technique to sclerose the pyriform fossa sinus opening. We present our experience in managing 3 children with third branchial pouch anomalies and propose a minimally invasive management algorithm avoiding open neck surgery. METHODS: Retrospective case review of 3 patients including demographics, mode of presentation, investigations, management and complications. RESULTS: Three children, two male and one female of mean age 9.6 years presented with painful left anterior neck swelling. Axial neck imaging showed a superficial abscess with air locules and a sinus tract leading towards the left pharynx. Diagnosis was confirmed by endoscopic examination of the pyriform fossa revealing a sinus opening. Two patients underwent open excision; one combined with diathermy to the sinus opening. The last patient was diagnosed at his initial presentation and managed with endoscopic diathermy of the sinus opening combined with percutaneous needle aspiration of the neck abscess at the same sitting. One patient had two recurrences, the first after initial open surgery and the second after the first cautery. Two patients developed temporary hoarseness after the procedure, which resolved within two weeks. All patients were free from recurrences at follow-up. CONCLUSIONS: Introduction of the technique of endoscopic diathermy to the pyriform fossa sinus opening in children with third branchial pouch anomalies has revolutionised their management avoiding open and potentially morbid surgery. Our algorithm takes this further by advocating percutaneous needle aspiration of the infective component and performing diathermy to the sinus opening at the first presentation.

Cervicofacial nontuberculous mycobacterium lymphadenitis in children: is surgery always necessary?

INTRODUCTION: The optimal treatment of cervicofacial nontuberculous mycobacterium lymphadenitis (CFNTB) in children is yet to be established. There is a general consensus that surgical excision results in a definitive resolution of the disease. The main aim of surgery is to remove affected nodes so that they do not discharge through the skin. Recently there are some investigators who are reporting successful antibiotic treatment and advocating medical therapy as the first line treatment. METHODS: 16 children consecutively presenting to otolaryngology in a tertiary referral centre over an 8-year period with CFNTB. Inclusion criteria were chronic cervicofacial lymphadenitis with either: (1) a culture positive for atypical mycobacteria (from either a lymph node or fine needle aspirate (FNA) specimen); or (2) acid-fast bacilli identified (from either a lymph node or FNA specimen); or (3) post excision histological findings consistent with mycobacterial infection (i.e. non-caseating granulomas) in the absence of other clinical features suggestive of other granulomatous conditions. Lesions with superficial skin change were treated preferentially with surgery. Children presenting with lymph nodes contained deep to sternocleidmastoid were assessed with FNA cytological and microbiological analysis and MRI or CT, and treated preferentially with antibiotics or watchful waiting. RESULTS: 4 children (2 culture positive, 2 with acid-fast bacilli on needle aspirate) presented with lymphadenopathy deep to sternocleidmastoid and were managed non-surgically. All 4 resolved without cutaneous involvement. 11 children with a clinical presentation of CFNTB underwent complete excision of all involved nodes for superficial lesions (6 were culture positive, and all had granulomatous histology). None recurred. 1 patient presented late with a mature, discharging parotid sinus, which was managed with watchful waiting as the lesion was clinically close to natural resolution. CONCLUSIONS: Depth at presentation may help decide which patients with CFNTB can be treated non-surgically without cutaneous sequelae. We propose that a watch and wait management is an option for deep nodes.

Antenatal carbimazole and choanal atresia: a new embryopathy.

Carbimazole embryopathy is a recently recognized and defined phenotype. Choanal atresia; gastrointestinal anomalies, particularly esophageal atresia; athelia or hypothelia; developmental delay; hearing loss; aplasia cutis; and dysmorphic facial features all can occur after exposure to the antithyroid drugs carbimazole or methimazole during gestation. Although full expression of this phenotype appears to be an uncommon sequelae of antenatal carbimazole exposure, infants with less overt manifestations may remain with the condition undiagnosed. It is clearly important when an infant with choanal atresia is assessed to take an adequate drug history and consider this embryopathy as a potential causative factor.

Ectopic thymus presenting as a subglottic mass: diagnostic and management dilemmas.

The commonest subglottic mass in infants is a congenital haemangioma, which is usually managed conservatively without a histological diagnosis. Ectopic cervical thymus is rare and usually presents as a cervical mass, with only one case of subglottic ectopic thymus reported to date. Due to its rarity, the diagnosis in most cases relies on surgical excision and histological examination. However, histological diagnosis may not always be easily reached, as is demonstrated in this case report. In this article, an infant with congenital stridor secondary to a subglottic mass is described and the clinical and diagnostic difficulties in its management are discussed.

Retropharyngeal and parapharyngeal infections in children: the Toronto experience.

OBJECTIVE: To review the Hospital of Sick Children, Toronto's experience of the diagnosis and management of retropharyngeal and parapharyngeal infections with particular emphasis on the role of computed tomography (CT) imaging in diagnosing the presence of an abscess. METHODS: A retrospective analysis of all patients diagnosed with retropharyngeal and parapharyngeal infections from 1987 to 1999 was performed. Demographic data, presenting symptoms, season of presentation, management and complications were reviewed. The CT scans of 27 patients who underwent surgical treatment were retrospectively examined by two neuroradiologists who were blinded to the patient's history and outcome. The sensitivity, specificity and predictive values for the specific features and overall assessment were calculated. RESULTS: Fifty-four children were identified. There were 46 retropharyngeal infections, 6 parapharyngeal infections and 2 patients had both retropharyngeal and parapharyngeal infections. All patients were treated with parenteral antibiotics. Thirty-seven patients underwent surgical drainage and in 27 there was a positive finding of pus. The retrospectively assessed CT scans of the 21 patients who underwent surgery were found to have a sensitivity of 81% in detecting an abscess by CT scan but the specificity was 57%. There were four complications including mediastinitis, aspiration pneumonia, internal jugular vein thrombosis and common carotid artery aneurysm. All patients recovered but abscess recurred in five patients. CONCLUSION: Not all patients with retropharyngeal and parapharyngeal abscesses require surgery. Whilst CT scans are helpful in diagnosing and assessing the extent of these infections they are not always accurate in detecting an abscess. A decision to drain an abscess should therefore not be made based solely on the CT findings.

Visibility of puncture sites after external osteotomy in rhinoplastic surgery.

BACKGROUND: External lateral osteotomy performed through a small puncture incision in the skin has numerous advantages, but it has been criticized by its opponents who claim it may leave visible scars. OBJECTIVE: To examine whether the external lateral osteotomy actually leaves visible scars. METHODS: Eighty-one postoperative photographs of patients who had undergone the external lateral osteotomy technique were reviewed. The photographic slides were a mixture of some taken 3 months after surgery and others taken 6 months after surgery. Scar visibility was assessed. RESULTS: Percutaneous puncture site visibility as assessed by 3 surgeons independently was 0% at 6 months after surgery and about 3% at 3 months after surgery. CONCLUSION: The external osteotomy procedure produces an excellent cosmetic result at the puncture sites in the skin.

Nontuberculous mycobacterial cervical adenitis: a ten-year retrospective review.

OBJECTIVE: To review the authors' institutional experience with nontuberculous mycobacterial cervical adenitis in children over a 10-year period. STUDY DESIGN: Retrospective chart review. METHODS: The case histories of 79 children with nontuberculous mycobacterial lymphadenitis who presented to a large tertiary care referral center from 1989 to 1999 were retrospectively analyzed, and the demographics, clinical presentation, methods of diagnosis, and types of surgical intervention they underwent were reported. RESULTS: The mean age at presentation was 3.6 years. The male-to-female ratio was 1:2, and the frequency of presentation was highest in the winter and spring months. The diagnosis was not immediately apparent to most primary health care physicians because there was a delay between the appearance of the lump and referral for treatment, which was in excess of 8 weeks. Involved nodes were mainly treated surgically. The recurrence rate of complete surgical resection was less than 1%. CONCLUSION: The study highlights the fact that a high index of clinical suspicion is needed to make an early diagnosis of nontuberculous mycobacterial lymphadenitis and lends further weight to the already existing evidence that surgical excision remains the mainstay of successful treatment for lymphadenitis caused by nontuberculous mycobacteria.

Microbiologic findings and risk factors for antimicrobial resistance at myringotomy for tympanostomy tube placement--a prospective study of 601 children in Toronto.

CONTEXT: There is limited information on the identity and antibiotic susceptibility of bacterial pathogens in children with chronic otitis media whose repeated antibiotic use may place them at increased risk of antibiotic-resistant bacteria. OBJECTIVE: To determine, at myringotomy for tympanostomy tube placement, (1) the prevalence of bacteria, (2) the extent and patterns of antibiotic resistance, and (3) the risk factors associated with the presence and resistant status of pathogens. DESIGN: Prospective, multi-site, cohort study. SETTING AND PATIENTS: Children undergoing myringotomy for tympanostomy tube placement between November 1, 1999 and March 31, 2000 in seven hospitals in Toronto, Ontario, were identified. If fluid was present, aspirates were submitted for bacteriologic testing. A follow-up telephone questionnaire was administered to patient caregivers in order to identify risk factors for the presence of (1) culturable pathogens and (2) resistant pathogens. MAIN OUTCOME MEASURES: The identification and prevalence of bacteria cultured from the middle ears of subjects, and the degree of nonsusceptibility to commonly prescribed antibiotics. RESULTS: Among 601 patients (mean age 3.9 years, 60.7% male), both a telephone interview (n=544) and an ear specimen (n=527) were obtained for 478. Pathogens were found in middle ear effusions of 37% of the children in the study; including at least one 'definite' pathogen in 189 children (31.4%), and a further 32 children (5.3%) with at least one 'possible' pathogen. Definite pathogens included Haemophilus influenzae in 17% of the children, followed by Moraxella catarrhalis (9%) and Streptococcus pneumoniae (6%); ampicillin nonsusceptibility was found in 40, 100 and 24%, respectively. Overall, 123 children (20.5%) were found to have definite pathogens with resistance to ampicillin/penicillin, trimethoprim-sulfamethoxazole, or clarithromycin/erythromycin. Patient characteristics included premature birth and/or long length of stay in the nursery (23%), first infection before the age of 6 months (26%), put to bed with a bottle (28%), household smoker (34%), in out-of-home child care (38%), history of eczema, bronchiolitis and/or asthma (39%), and use of pacifiers (40%). Household characteristics were smoking (34%), married/common law parents (85%), and 60% had completed college or university; in 26% both parents were born outside of Canada; 73% of children were Caucasian. Of the 75% who responded to the question regarding income, 42% had household income over $60,000 (CAN). Risk factors for the presence of a pathogen and for a resistant pathogen in multivariate analysis included younger age, lower maternal education, day care centre attendance, no previous adenoidectomy and bilateral, primarily winter infections as well as amoxicillin use in the previous 6 months. CONCLUSION: Modifiable risk factors for otitis media including household smoking and pacifier use are present in many children undergoing tympanostomy tube placement; child care centre attendees are over-represented. Multiple antibiotic courses were commonly prescribed prior to surgery. H. influenzae and M. catarrhalis are important pathogens and therapy in clinical failures should be directed against them. The 7-valent protein conjugate polysaccharide vaccine (Prevnar) would have covered 73% of the serotypes of S. pneumoniae isolated in this study.

Nasal nitric oxide in children: a novel measurement technique and normal values.

OBJECTIVES: To develop and standardize a technique for measuring nasal nitric oxide (NO) output in children and to determine normal values in this population. STUDY DESIGN: Prospective study evaluating a new technique for measuring nasal nitric oxide in a cohort of normal patients and a cohort of patients with nasal disease. METHODS: Nasal NO was measured using an aspiration technique, aspirating room air through the nasal cavities by means of a Teflon nozzle placed in one nasal vestibule while maintaining velopharyngeal closure using a party "blow-out" toyRESULTS Nasal NO measurements were performed in 45 children (mean age, 11.0 y; age range, 3.2-17.6 y) There were 20 girls and 25 boys. All children were able to perform the maneuvers necessary for measurement of nasal NO output. Among the subgroup of normal healthy children (30), there was considerable variation in NO output between subjects, with a mean NO output of 481 nL/min and an SD of 283 nL/min. CONCLUSIONS: Nasal NO can be readily measured in children using the presented technique. There is considerable variability in the values for nasal NO output in normal children.