International Journal of Pediatric Otorhinolaryngology Extra RSS feed: Current Issue. International Journal of Pediatric Otorhinolaryngology Extra is an online-only companion to the International Journal of Pediatric Otorhinolaryngology. International Journal of Pediatric Otorhinolaryngology Extra publishes case reports which have an important educational value but cannot be published in the printed journal due to lack of space. Case reports published in International Journal of Pediatric Otorhinolaryngology Extra are: •Peer reviewed to the usual high standards of IJPORL •Fully citable through digital object identifiers (DOI) •Rapidly available online via ScienceDirect, the world's leading online journal platform. Please note that as a new journal International Journal of Pediatric Otorhinolaryngology Extra is NOT currently covered by PubMed/MEDLINE® and does not have an impact factor.http://www.ijporlextra.com/?rss=yesElsevier Inc.en © 2009 Elsevier Ireland Ltd. All rights reserved. International Journal of Pediatric Otorhinolaryngology Extra1871-404854December 2010 © 2009 Elsevier Ireland Ltd. All rights reserved. healthpermissions@elsevier.comhttp://www.ijporlextra.com/article/PIIS187140480900046X/abstract?rss=yesAbstract: Although actinomycosis may occur anywhere in the body, the most common site of this infection is the head and neck region. Actinomyces, the usual cause of actinomycosis, is a commensal bacterium with low pathogenicity that is a normal resident in the oral cavity, thus odontogenic injury is a common antecedent event. Therefore, cervicofacial actinomycosis commonly occurs in the mandibular area, submandibular gland, and tongue in order of decreasing frequency.The authors encountered a case of a child with a history of slowly progressing swelling in the left side of the lower neck and no history of oral or odontogenic trauma. A pyriform sinus fistula found in this patient could be the unusual root of the cervicofacial actinomycosis occurring in the perithyroidal region, via a direct connection between the site of infection and the upper aerodigestive tract.A case of perithyroidal actinomycosis in a child with pyriform sinus fistulaBon Seok Koo, Gun Ho Lee, Sung Tae Seo, Yeo-Hoon Yoon10.1016/j.pedex.2009.08.002International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-09-02International Journal of Pediatric Otorhinolaryngology Extra2009-09-0254S1871-4048(10)X0004-1Case reports149151http://www.ijporlextra.com/article/PIIS1871404809000513/abstract?rss=yesAbstract: Lemierre's syndrome is characterised by an oropharyngeal infection leading to secondary septic thrombophlebitis of the internal jugular vein. Most of the cases are caused by Fusobacterium necrophorum. Here, we report an unusual variant of Lemierre's syndrome developed after modified radical mastoidectomy. There was right sigmoid sinus and right internal jugular vein thrombosis along with multiple abscess foci seen in bilateral lung with right pyopneumothorax. The organism isolated from chest foci was Citrobacter freneli. We also briefly review the literature of Lemierre's syndrome.Lemierre's syndrome after modified radical mastoidectomy: An unusual variant caused by Citrobacter freneliPrakash Adhikari, Rajendra P. Guragain, Chop Lal Bhusal, Rabindra B. Pradhananga10.1016/j.pedex.2009.08.003International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-09-25International Journal of Pediatric Otorhinolaryngology Extra2009-09-2554S1871-4048(10)X0004-1Case reports152154http://www.ijporlextra.com/article/PIIS1871404809000525/abstract?rss=yesAbstract: Introduction: Neuroglial heterotopia is a rare condition in which mature neuroglial tissue is found in the body other than in the central nervous system. Nasal glioma is neuroglial heterotopia in the nasal cavity. We report an extremely rare case of heterotopic neuroglial tissue in the temporal bone with tumoral transformation. Case report: Our patient was a 1-year-old Chinese girl. She was born with a left facial swelling. CT and MRI revealed a heterogeneous mass with cystic component occupying the parapharyngeal space. The upper airway was significantly compromised by the mass which extended into the middle cranial fossa. A combined approach to its resection was undertaken by ENT and neurosurgeons which was uneventful with all cranial nerves in its vicinity preserved. Intraoperative findings confirmed the absence of direct communication between the mass and the brain. Pathological examination confirmed mature neural tissue with tumoral differentiation. Conclusion: Heterotopic neuroglial tissue is a rare condition in the temporal bone. Ganglioglioma formation within the heterotopic neuroglial tissue is an even rarer condition. Careful pre-operative investigations and planning is the key to successful and complete excision of the abnormal tissue whilst preserving normal function.Parapharyngeal space neuroglial heterotopia with tumoral differentiationJoseph Y. Chan, Chris K.C. Lai, Victor J. Abdullah, Micheal C.F. Tong, C.A. vanHasselt10.1016/j.pedex.2009.08.005International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-10-16International Journal of Pediatric Otorhinolaryngology Extra2009-10-1654S1871-4048(10)X0004-1Case reports155158http://www.ijporlextra.com/article/PIIS1871404809000537/abstract?rss=yesAbstract: Extraskeletal Ewing's Sarcoma (EES) of head and neck region, is a rare soft tissue tumour morphologically similar to the commoner Ewing's Sarcoma arising from bone. We report a case of EES in parapharyngeal space in a 6-year-old male; who presented with stridor. CT scan of neck showed soft tissue mass in right parapharyngeal space with intraorbital and intracranial extention. Histopathological examination showed round cell tumour and immunohistochemistry was positive for CD99 and Vimentin. The patient responded very well to Concurrent Chemoradiation. An extensive review of English literature, to the best of our knowledge, did not reveal any previous case of EES in parapharyngeal space in children; although one similar case in a 53-year male is reported.Extraskeletal Ewing's Sarcoma of parapharyngeal spaceNeena Chaudhary, Deepak Gupta, V. Natesh, Sarvejeet Singh10.1016/j.pedex.2009.08.004International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2010-02-08International Journal of Pediatric Otorhinolaryngology Extra2010-02-0854S1871-4048(10)X0004-1Case reports159161http://www.ijporlextra.com/article/PIIS1871404809000549/abstract?rss=yesAbstract: Duplication cyst of the cervical esophagus represents a rare congenital anomaly. The children with esophageal duplication cyst usually present with respiratory distress or as asymptomatic thoracic mass found on incidental chest X-ray. We here present a case of a 3-month-old male infant who presented with noisy breathing and progressive respiratory distress and with no other abnormality. After laryngoscopy, barium esophagography and MRI study, congenital esophageal duplication cyst was diagnosed. Transcervical excision of the cyst was done using a harmonic scalpel. Histopathological examination of the specimen confirmed the diagnosis. The patient remains asymptomatic after one year of follow up.Congenital esophageal duplication cyst: A rare cause of infantile stridor and a diagnostic challengeV. Kumar, A.P. Singh, R. Meher, A. Raj, S.K. Aggarwal10.1016/j.pedex.2009.09.001International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-09-30International Journal of Pediatric Otorhinolaryngology Extra2009-09-3054S1871-4048(10)X0004-1Case reports162164http://www.ijporlextra.com/article/PIIS1871404809000550/abstract?rss=yesAbstract: Ascaris lumbricoides is found in various parts of the world, mainly in poor sanitation conditions and in developing countries with very crowded areas. Although the prevalence of ascariasis has decreased in Iran due to improved hygiene situation and the use of pesticides in farms, we recently encountered a rare case complication in which the worm was found exiting the middle ear.Ascariasis into the middle ear: Report of two cases from IranD.V.M. Mehdi Vejdani, Farangis Namvar10.1016/j.pedex.2009.09.002International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-10-05International Journal of Pediatric Otorhinolaryngology Extra2009-10-0554S1871-4048(10)X0004-1Case reports165166http://www.ijporlextra.com/article/PIIS1871404809000562/abstract?rss=yesAbstract: Cystic fibrosis (CF) is one of the most common inherited life-shortening diseases with an incidence of 1:2.500–3500 and a carrier frequency of 4–5% . It is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, which encodes a protein expressed in the apical membrane of exocrine epithelial cells, resulting in changes to the fluid and electrolytes on cell surface. More than 1500 mutations have been described so far in the CFTR gene, grouped in 5 classes on the basis of the impact on protein synthesis or activity .In the last years a mounting number of reports described patients that did not meet all diagnostic criteria for CF ; in particular, atypical or nonclassic CF is characterized by normal or borderline sweat test, pancreatic sufficiency and a monosymptomatic phenotype; the most studied forms are: congenital bilateral absence of vasa deferentes, acute or chronic recurrent pancreatitis, idiopathic bronciectasis . These patients typically bear a severe and a mild (classes 4–5) CFTR mutation, the latter being dominant .The involvement of upper airways is observed in up to 100% of classic CF patients, including recurrent sinusitis and rhinitis. Nasal polyps are present in 6–48% of overall CF patients and in 5–15.2% of less than 10 years old CF patients.Surgery is required for syntomatic polyps. It is the second most common class of operations performed on CF patients. In spite of the best of operations CF patients will have recurrence, because of systemic disease.We describe a patient diagnosed as “atypical CF” on the basis of persisting chronic rhinosinusitis, that showed a very rare CFTR genotype.Nasal polyposis in atypical cystic fibrosis: A case reportMassimo Mesolella, Mariano Cimmino, Elena Cantone, Rossella Grassia, Rossella Tomaiuolo, Giuseppe Castaldo, Valeria Raia10.1016/j.pedex.2009.09.003International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-11-02International Journal of Pediatric Otorhinolaryngology Extra2009-11-0254S1871-4048(10)X0004-1Case reports167169http://www.ijporlextra.com/article/PIIS1871404809000574/abstract?rss=yesAbstract: The third arch branchial fistula is a rare congenital abnormality that presents as an inflammatory process in the anterior neck, often in the region of the left lobe of thyroid. Incomplete fistula is not an uncommon finding extending from base of pyriform sinus to the thyroid or perithyroidal space, which predisposes to recurrent suppurative thyroiditis during episodes of upper respiratory infection. Complete fistula of third arch is extremely uncommon, especially of congenital origin. A 10-year-old male child presented with a small opening in the anterior neck since birth, with a swelling around it. Computerized tomography revealed a patent tract from neck skin to base of pyriform sinus. Total excision of the tract up to the left pyriform sinus with hemithyroidectomy was performed. There was no recurrence at 22 months of follow-up.Thyroid abscess with branchial anomalyJ. Madana, Deeke Yolmo, Sunil Kumar Saxena, S. Gopalakrishnan10.1016/j.pedex.2009.09.004International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-10-26International Journal of Pediatric Otorhinolaryngology Extra2009-10-2654S1871-4048(10)X0004-1Case reports170173http://www.ijporlextra.com/article/PIIS1871404809000586/abstract?rss=yesAbstract: Hypoglossal nerve palsy frequently occurs with other cranial nerve palsies; an isolated hypoglossal nerve palsy is rare. Most hypoglossal nerve palsies are caused by tumors. However, several reports have shown an association with oropharyngeal manipulation such as intubation, laryngeal mask airway or as a complication of surgery such as carotid endarterectomy, and tooth extraction. When the nerve was injured, a deviation to the affected side, atrophy, and tongue fasciculation could be observed. We treated a patient with isolated hypoglossal nerve palsy after open repair of a fracture of the humerus in the beach-chair position.Isolated hypoglossal nerve palsy following open surgery in the beach-chair position under general anesthesia: A case reportJun-Yeon Hwang, Ho-Ryun Won, Young-Ho Hong, Seog-Kyun Mun10.1016/j.pedex.2009.09.005International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-11-02International Journal of Pediatric Otorhinolaryngology Extra2009-11-0254S1871-4048(10)X0004-1Case reports174176http://www.ijporlextra.com/article/PIIS1871404809000598/abstract?rss=yesAbstract: Occipital teratomas are anecdotal and may mimic meningoencephaloceles in children. We describe a neonate presenting with an occipital mass that was diagnosed to be a mature teratoma and discuss its differentiation from neural tube defects in the region.Mature occipital teratoma in a neonateS.R. Raghu, A.M. Shubha, Kanishka Das10.1016/j.pedex.2009.10.001International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-11-05International Journal of Pediatric Otorhinolaryngology Extra2009-11-0554S1871-4048(10)X0004-1Case reports177179http://www.ijporlextra.com/article/PIIS1871404809000604/abstract?rss=yesAbstract: Johanson–Blizzard syndrome is a hereditary disorder extremely rare. The characteristic features include aplastic alae nasi, midline ectodermal scalp defects, deafness, dental abnormalities and malabsorption related to pancreatic exocrine deficiency. This paper presents a case of an 18-year-old patient with Johanson–Blizzard syndrome and emphasizes the importance of knowledge of the potential anaesthetic concerns of this syndrome for providing appropriate treatment for these patients.Johanson–Blizzard syndrome—A case study of oral and systemic manifestationsKeila Martha Amorim Barroso, Debora Farias Batista Leite, Pollianna Muniz Alves, Paula Frassinetti Vasconcelos de Medeiros, Gustavo Pina Godoy10.1016/j.pedex.2009.10.002International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-12-07International Journal of Pediatric Otorhinolaryngology Extra2009-12-0754S1871-4048(10)X0004-1Case reports180182http://www.ijporlextra.com/article/PIIS1871404809000616/abstract?rss=yesAbstract: Nontuberculous mycobacterial infections are seen frequently in the head and neck region in immunocompetent young children in their toddler years. These infections usually present as an inflammatory neck mass that is refractory to standard antibiotic therapy. We describe a previously healthy 16-month-old boy presenting with persistent wheezing despite treatment for reactive airway disease. At bronchoscopy a large carinal mass compressing both main stem bronchi was found. Subsequent thoracoscopic biopsy and culture revealed Mycobacterium avium complex. The rationale for serial bronchoscopies as well as diagnostic, medical and surgical management options are discussed.Mediastinal nontuberculous mycobacteria as a cause of pediatric airway obstructionCristian M. Slough, Sagila George, Holger Link, Carol J. MacArthur10.1016/j.pedex.2009.10.003International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-11-13International Journal of Pediatric Otorhinolaryngology Extra2009-11-1354S1871-4048(10)X0004-1Case reports183185http://www.ijporlextra.com/article/PIIS1871404809000628/abstract?rss=yesAbstract: Intracochlear schwannoma (ICS) is a rare tumor that can cause significant hearing loss. Previous clinical reports on ICS have described this disease in adults. We present a pediatric patient with left-sided ICS who initially presented with a mild conductive hearing loss. We demonstrate the progression of the hearing loss over a span of several years through serial audiograms, as well as the process of diagnosis and treatment. This rare and unique case demonstrates that ICS can be found in young children.Atypical presentation of intracochlear schwannoma in a childMarilyn Neault, Guangwei Zhou, Margaret Kenna, Dennis Poe10.1016/j.pedex.2009.10.004International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-11-09International Journal of Pediatric Otorhinolaryngology Extra2009-11-0954S1871-4048(10)X0004-1Case reports186189http://www.ijporlextra.com/article/PIIS187140480900063X/abstract?rss=yesAbstract: Peripheral facial nerve paralysis is relatively common in the pediatric age group. Infectious agents convincingly associated with acute facial palsy include varicella-zoster virus, herpes simplex virus, cytomegalovirus, Epstein–Barr virus, rubella virus, and more recently, human immunodeficiency virus. However, facial palsy has rarely been documented in patients with mumps virus infection. Facial palsy caused by mumps infection is believed to exist in two distinct forms, one representing an initial infection with mumps parotitis and another representing a reinfection without parotitis. We recently saw the two different forms of mumps-associated facial palsy with and without parotitis.Two different forms of mumps-associated facial palsyTatsuo Fuchigami, Ikuko Kimura, Junichi Suzuki, Michio Miyashita, Kenichi Watanabe, Koji Hashimoto, Yukihiko Fujita, Yasuji Inamo, Hideo Mugishima10.1016/j.pedex.2009.10.005International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-11-09International Journal of Pediatric Otorhinolaryngology Extra2009-11-0954S1871-4048(10)X0004-1Case reports190191http://www.ijporlextra.com/article/PIIS1871404809000641/abstract?rss=yesAbstract: Objective: Symptoms of pediatric eosinophilic esophagitis (EoE) include dysphagia, emesis, regurgitation and feeding difficulties. This symptom complex has been mistaken for refractory gastroesophageal reflux disease (GERD). Whether EoE and GERD are related is controversial. Recently, EoE has been associated with upper airway manifestations including recurrent sinusitis, cough, wheezing, pneumonia, laryngeal edema, and subglottic stenosis. Laryngospasm secondary to EoE has not been reported. The purpose of this study is to increase the awareness of laryngospasm as a presenting symptom in this population. Methods: A 1-year retrospective chart review of 2 children with laryngospasm as the presenting symptom with endoscopic and histopathologic evidence of EoE. Results: Two patients presented to an otolaryngologist with a history consistent with laryngospasm in addition to GI complaints. Laryngospasm was identified during endoscopy. Both patients presented with GERD and symptoms refractory to medical management. Esophagoscopy and biopsy revealed >20 eosinophils per high power field. Symptoms were completely resolved following oral fluticasone therapy at follow-up. Conclusions: The eosinophilic esophagitis symptom complex includes airway manifestations. Pediatric eosinophilic esophagitis should be considered in the differential diagnosis of patients with laryngospasm, dysphagia and gastroesophageal reflux refractory to treatment.Laryngospasm and pediatric eosinophilic esophagitisCarrie L. Francis, Troy Gibbons, Gresham T. Richter10.1016/j.pedex.2009.11.001International Journal of Pediatric Otorhinolaryngology Extra 5, 4 (2010)2009-12-21International Journal of Pediatric Otorhinolaryngology Extra2009-12-2154S1871-4048(10)X0004-1Case reports192194