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 Published by Elsevier Inc. International Journal of Pediatric Otorhinolaryngology Extra1871-404872March 2012 Published by Elsevier Inc. healthpermissions@elsevier.comhttp://www.ijporlextra.com/article/PIIS1871404811000141/abstract?rss=yesAbstract: Juvenile rheumatoid arthritis (JRA), now termed juvenile idiopathic arthritis (JIA), is a well-described group of arthritides in the pediatric population with known associations with airway compromise. Subclasses are based on associated symptoms and number of joints involved, namely pauciarticular, polyarticular, and systemic onset JRA. Cricoarytenoid arthritis (CA), as a cause of airway compromise, is rarely associated with JRA as compared to adult onset rheumatoid arthritis, however, there are reports of CA with JRA. Additionally, JRA is associated with other rheumatologic diseases, which include a reported case of Wegener's granulomatosis. Wegener's granulomatosis has a well-established association with subglottic stenosis. Described below is the case study of an 18 year-old African American female with a 5-year history of pauciarticular juvenile rheumatoid arthritis developed worsening shortness of breath, orthopnea, and stridor over a 1-month period. The patient had a firm circumferential subglottic mass obstructing approximately 80% of the lumen and requiring tracheostomy tube placement. Biopsies of the mass revealed only acute and chronic inflammation. There was no evidence of rheumatoid nodules, granulomas, acid fast, or fungal organisms. To our knowledge, this is the first case history reported of a patient with JRA and idiopathic subglottic stenosis; this potentially establishes an association between the two disease processes.Subglottic stenosis in juvenile rheumatoid arthritisChafeek Tomeh, Nicholas Jones, Parker Velargo, Jerome Thompson10.1016/j.pedex.2011.01.009International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-01-19International Journal of Pediatric Otorhinolaryngology Extra2012-01-1972S1871-4048(12)X0003-0Case reports4547http://www.ijporlextra.com/article/PIIS1871404811000475/abstract?rss=yesAbstract: Pott's puffy tumor is a rare entity, described as a subperiosteal abscess associated with underlying cranial osteomyelitis. It is more commonly seen in the adolescent age group and may result from frontal sinusitis or trauma. We present a case of a 5-year-old male with forehead swelling, meningitis, and brain abscess communicating between epidural and subdural spaces. Treatment included 8 weeks antibiotic therapy with broad spectrum coverage of anerobic and aerobic organisms. Initial surgical approach included soft tissue incision and drainage of abscess, right frontal sinus trephine, and right endoscopic sinus surgery. One week after his initial procedure, he returned to OR for right pterional craniotomy for drainage of multiple intracranial abscesses. Total inpatient care was 4 weeks duration, and a full recovery was achieved with no long term neurological sequelae. We include an updated review of the literature of pediatric Pott's puffy tumor in children less than 10 years of age.Pott's puffy tumor in a 5-year-old male and a review of the literatureNeil Gildener-Leapman, Aaron Lin10.1016/j.pedex.2011.05.003International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2011-07-04International Journal of Pediatric Otorhinolaryngology Extra2011-07-0472S1871-4048(12)X0003-0Case reports4851http://www.ijporlextra.com/article/PIIS1871404811000839/abstract?rss=yesAbstract: Arachnoid cysts are benign, intracranial mass filled with cerebrospinal fluid that are usually occurred in middle cranial fossa. If located in posterior cranial fossa, especially in cerebellopontine angle (CPA), they may present with symptoms such as dizziness, tinnitus and hearing loss. Sudden hearing loss associated with arachnoid cyst in middle cranial fossa is extremely rare. Herein, we present the case of a 17-year-old boy who complained of sudden sensorineural hearing loss associated with a huge arachnoid cyst of the middle cranial fossa in the left hemisphere.A huge arachnoid cyst presenting with sudden hearing lossJinsu Choi, Bosung Kim, Kisik Kim10.1016/j.pedex.2011.10.001International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2011-11-09International Journal of Pediatric Otorhinolaryngology Extra2011-11-0972S1871-4048(12)X0003-0Case reports5254http://www.ijporlextra.com/article/PIIS1871404811000840/abstract?rss=yesAbstract: We report a 1-year-old child with overlooked foreign body entrenched in arytenoid and false vocal cord in vertical plane of airway for 3 months leading to ulcer on posterior pharyngeal wall. The foreign body was missed on bronchoscopy and intubation. However, ulcer on the posterior pharyngeal wall aroused the suspicion and child was reintubated and foreign body was removed from arytenoids which caused ulcer because of repeated movements.Overlooked foreign body at arytenoid causing ulcer on posterior pharyngeal wall: A case report and review of literatureRavin K. Verma, Aayush Mittal, Sunil Kumar, J.K. Sahni10.1016/j.pedex.2011.10.002International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2011-12-02International Journal of Pediatric Otorhinolaryngology Extra2011-12-0272S1871-4048(12)X0003-0Case reports5556http://www.ijporlextra.com/article/PIIS1871404811000864/abstract?rss=yesAbstract: Wegener's granulomatosis (WG) is a fatal disease. Facial nerve paralysis in association with WG is rare, being present in about 5% of patients, alone or in combination with hearing loss and rarely may be the only presenting feature. We are reporting a case of WG presenting with bilateral facial paresis and its peculiarity consists in its acute onset, lack of a significant previous history of ear disease and its association with bilateral profound sensorineural hearing loss. A Medline search revealed that there has been no reported case of WG presenting with bilateral facial involvement with bilateral profound sensorineural hearing loss.Wegener's granulomatosis with bilateral facial nerve paresis and bilateral profound sensorineural hearing lossRaman Wadhera, Sat Paul Gulati, Vijay Kalra, Anju Ghai10.1016/j.pedex.2011.11.002International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2011-12-16International Journal of Pediatric Otorhinolaryngology Extra2011-12-1672S1871-4048(12)X0003-0Case reports5758http://www.ijporlextra.com/article/PIIS1871404811000876/abstract?rss=yesAbstract: Primary ciliary dyskinesia (PCD) is a hereditary disorder with structural and functional impairment of the cilia of the entire body. We report on two siblings (brother and sister) with PCD in whom electron microscopy identified ultrastructural abnormalities and genetic mutations were detected. The 11-year-old sister had repeated pneumonia, acute otitis media and sinusitis since infancy, with a persistent wet cough and purulent sputum developing over the past few years. Chest CT showed atelectasis and bronchodilatation in the middle lobes. However, the patient had no situs inversus. The 8-year-old brother had repeated respiratory infection since infancy and had been treated by an ENT doctor without success. Chest CT showed atelectasis and bronchodilatation in the middle lobes and he had no situs inversus. Electron microscopy studies were performed on biopsy specimens of the nasal mucosa from both cases and revealed a loss of dynein arms of the cilia. DNA analysis in both cases showed a homozygous mutation in the DNAI1 (dynein arm intermediate chain 1) exon 13. Making a diagnosis of PCD without situs inversus can sometimes be very difficult. Thus, when evaluating cases with repeated respiratory infection since infancy, both consideration of PCD along with a careful examination of family histories should be a part of the diagnostic process.Primary ciliary dyskinesia in two siblingsTakaaki Tanaka, Satoru Ogawa, Kaname Nakatani, Takao Fujisawa, Satoko Usui, Sawako Masuda, Kazuhiko Takeuchi10.1016/j.pedex.2011.11.003International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2011-12-16International Journal of Pediatric Otorhinolaryngology Extra2011-12-1672S1871-4048(12)X0003-0Case reports5963http://www.ijporlextra.com/article/PIIS1871404811000918/abstract?rss=yesAbstract: Trifid epiglottis is an extremely rare congenital laryngeal anomaly. Trifid anomaly of epiglottis has not been described in literature. In the embryo, epiglottis, hypothalamus, and digital buds develop synchronously. Some disturbances during this stage may account for the concurrence of trifid epiglottis and polysyndactyly. We report a trifid epiglottis in a 6 year old child with congenital bilateral polydactyly of hand and foot who was asymptomatic and developed postexubation mild stridor after general anesthesia for excision of the extra digit which was managed conservatively.Postextubation stridor in a child with asymptomatic trifid epiglottis with polydactyly – A rare case reportSameer Sethi, K.S. Vadaje, Ashok K. Gupta10.1016/j.pedex.2011.12.001International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-01-06International Journal of Pediatric Otorhinolaryngology Extra2012-01-0672S1871-4048(12)X0003-0Case reports6466http://www.ijporlextra.com/article/PIIS187140481100092X/abstract?rss=yesAbstract: Squamous cell carcinoma (SCC) of the esophagus usually occurs between the 4th and 6th decades of life. Esophageal SCC in children is an extremely rare entity – only a few pediatric and adolescent patients have been reported. Herein we present a 17-year-old male progressive dysphagia patient that developed SCC of the esophagus, as well as a literature review of esophageal SCC in pediatric and adolescent patients.Esophageal squamous cell carcinoma in children: A case presentation and review of an unusual entityNilda Süslü, Rezarta Taga Senirli, A. Şefik Hoşal, Burce Özgen Mocan, Mustafa Cengiz10.1016/j.pedex.2011.12.002International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-01-09International Journal of Pediatric Otorhinolaryngology Extra2012-01-0972S1871-4048(12)X0003-0Case reports6769http://www.ijporlextra.com/article/PIIS1871404812000020/abstract?rss=yesAbstract: A six months old male presented to our Otolaryngology Department with a long -standing history of chronic nasal congestion and difficulty of breathing. The child was initially treated with nasal steroids without improvements. On follow up investigations, bilateral sinus mucoceles were seen on CT scan. The diagnosis of cystic fibrosis was confirmed by the chloride sweat test and the child was taken to the operating room for functional endoscopic sinus surgery and marsupialization of mucoceles. The child's brother was then investigated and diagnosed with CF.Cystic fibrosis presenting as bilateral maxillary mucoceles in an infantLana Wazqar, Alipasha Rassouli, Nader Emami Esfahani, Lucia Carpineta, Sam J. Daniel10.1016/j.pedex.2012.01.001International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-02-01International Journal of Pediatric Otorhinolaryngology Extra2012-02-0172S1871-4048(12)X0003-0Case reports7072http://www.ijporlextra.com/article/PIIS1871404812000032/abstract?rss=yesAbstract: Nasal dermal sinus cysts are congenital malformations that result from anomalous embryological development and are not prescribed to any specific genetic defect. The occurrence of a supernumerary ring 20 that causes a partial trisomy 20 mosaicism is a rare chromosome abnormality and no common phenotype has been described yet. We present a unique case of a 3.5-year-old child with a supernumerary ring chromosome 20 mosaicism associated with nasal dermoid with intracranial extension. It is possible that this genetic defect contribute to embryonic developmental errors of the frontonasal region. The clinical presentation, surgical treatment, and literature review of this case are discussed.Nasal dermal sinus cysts with intracranial extension in a child mosaic for a supernumerary ring chromosome 20Bernarda Lozić, Draško Cikojević, Vlatko Ledenko, Marisa Klančnik, Ružica Lasan, Tatijana Zemunik10.1016/j.pedex.2011.12.003International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-01-27International Journal of Pediatric Otorhinolaryngology Extra2012-01-2772S1871-4048(12)X0003-0Case reports7378http://www.ijporlextra.com/article/PIIS1871404812000081/abstract?rss=yesAbstract: Extracranial meningiomas are reported to occur from 0.9% to 2% of cases. The presentation of this rare parapharyngeal space meningioma reflects the difficulty in diagnosing parapharyngeal space tumors especially in the pediatric population. Furthermore, the histopathological features of this meningioma offer a learning experience. We describe our multi disciplinary team approach to management and surgical techniques employed to treat this tumor.Surgical management of extracranial meningioma presenting in the parapharyngeal space in a pediatric patientMajestic Tam, Kestutis Paul Boyev, Kondi Wong, Tapan Padhya10.1016/j.pedex.2012.01.002International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-02-13International Journal of Pediatric Otorhinolaryngology Extra2012-02-1372S1871-4048(12)X0003-0Case reports7981http://www.ijporlextra.com/article/PIIS1871404812000093/abstract?rss=yesAbstract: This is a case report of a child who presented with an unusual presentation of acute mastoiditis, where the only feature was a gradually enlarging parieto-occipital mass. There was no significant other otological features at the time of presentation, where otoscopy revealed a normal middle ear and audiogram/tympanogram were normal. CT of the temporal bone revealed left mastoiditis with erosion of the inner cortex with extension of the abscess along the epidural space. A skull defect in the vault of the skull allowing communication of this extradural collection with a scalp abscess. MRI noted transverse and sigmoid sinus thrombosis. The child underwent left cortical mastoidectomy via extended post auricular approach. This was followed by a six weeks’ course of intravenous antibiotics and subcutaneous low molecular weight heparin. This case highlights the need to identify unusual modes of presentation for acute mastoiditis and to keep a high index of clinical suspicion to avoid complications of delayed treatment.Mastoiditis presenting as parietooccipital scalp abscess—A case report and literature reviewSomasundaram Subramaniam, Annette Ang, Henry K.K. Tan10.1016/j.pedex.2012.01.003International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-02-10International Journal of Pediatric Otorhinolaryngology Extra2012-02-1072S1871-4048(12)X0003-0Case reports8284http://www.ijporlextra.com/article/PIIS187140481200010X/abstract?rss=yesAbstract: In the post-Haemophilus influenza type b vaccination era, the etiology of pediatric supraglottitis has been evolving. In addition to traditional bacteriology, viral and fungal infectious agents must also be considered, particularly in immunocompromised patients. We report the case of a 15 year old female with cyclic neutropenia, a rare hematologic disorder, who presented with airway compromise secondary to Candida and Herpes Simplex Virus 1 (HSV1) supraglottitis. A review of viral causes of supraglottitis is also presented.Case report: Candida and Herpes Simplex Virus supraglottitis in a patient with cyclical neutropeniaYvonne L. Richardson, Amy E. Lawrason, Tulio A. Valdez10.1016/j.pedex.2012.01.004International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-03-05International Journal of Pediatric Otorhinolaryngology Extra2012-03-0572S1871-4048(12)X0003-0Case reports8588http://www.ijporlextra.com/article/PIIS1871404812000111/abstract?rss=yesAbstract: Supernumerary nostril is a rare congenital anomaly of the nose which presents as a previous deformity. In this work, we report a case of supernumerary nostril which is extremely rare. In this rare anomaly, the nasal cavity on one side is completely normal and on the affected side, a supernumerary nostril located above the left nostril, with an internal diameter measuring 0.4cm. The normal ipsilateral nostril was a little smaller than the contralateral one. This anomaly occurred as an isolated defect without any other anomalies.Supernumerary nostril: An extremely rare caseYihao Xu, Fei Fan, Huan Wang10.1016/j.pedex.2012.02.001International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-03-02International Journal of Pediatric Otorhinolaryngology Extra2012-03-0272S1871-4048(12)X0003-0Case reports8991http://www.ijporlextra.com/article/PIIS1871404812000196/abstract?rss=yesList of Societies10.1016/S1871-4048(12)00019-6International Journal of Pediatric Otorhinolaryngology Extra 7, 2 (2012)2012-03-01International Journal of Pediatric Otorhinolaryngology Extra2012-03-0172S1871-4048(12)X0003-09296