A case report of congenital arhinia and literature review

A case report of congenital arhinia and literature review

1934 Choanal stenosis in Crohn’s disease: A case report and review of literature Domenico Leonardo Grasso a, * , Margherita Bussani b, Stefano Martelo...

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1934 Choanal stenosis in Crohn’s disease: A case report and review of literature Domenico Leonardo Grasso a, * , Margherita Bussani b, Stefano Martelossi c a Ear, Nose and Throat Department, IRCCS ‘‘Burlo— Garofolo’’ Children Hospital, Trieste, Italy b Clinical Pathology Department, Ospedale Maggiore, Trieste, Italy c Pediatric Department, IRCCS ‘‘Burlo—Garofolo’’ Children Hospital, Trieste, Italy

Crohn’s disease is a granulomatous inflammatory bowel disease. Its pathologic findings include noncontiguous chronic inflammation and non-caseating granulomas sometimes with extra-intestinal localizations. Nasal manifestations of Crohn’s disease are quite rare. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforation. The authors describe the case of a 4-year-old girl with Crohn’s disease with recurrent epistaxis and narrowing of the right choana, secondary to fibrous tissue. DOI: 10.1016/j.ijporl.2007.06.029

A case report of congenital arhinia and literature review Tsvetan Marinov a,*, Petar Rouev b, Youri Anastassov c, Philippe Pellerin d, Katja Kovacheva e, Maxim Jonov f a Department of Otorhinolaryngology, Medical University Pleven, St. Kliment Ohridski Str. Nr. 1, 5800 Pleven, Bulgaria b Clinic of Otorhinolaryngology, Medical Faculty, Thracian University Stara Zagora, Bulgaria c Plastic and Craniofacial Clinic, Medical University Plovdiv, Bulgaria d Department of Plastic Surgery, University Hospital Lille, France e Department of Medical Genetics, Medical University Pleven, Bulgaria f Clinic of Neonatology, Medical University Pleven, Bulgaria

We report about a boy with congenital total arhinia and coloboma of the iris. The newborn had complete absence of external nose, nasal and paranasal cavities, with that area being flat with some elevation and firm on palpation. Congenital arhinia is a rare developmental abnormality characterised by

Abstracts lack of the formation of external and internal nasal structures. Since there were no life-threatening complications a tracheotomy was not performed on this newborn. Airway support with oropharyngeal tube was made and the feeding of the child was through an orogastral tube. The child learned to breathe and to eat through the mouth and at age of 3 years a reconstruction of the external nose was performed. Description of the treatment, embryological aspect and a literature review is made to suggest guidelines for the management of such cases. DOI: 10.1016/j.ijporl.2007.07.021

Para-meningeal rhabdomyosarcoma with critical airway compromise: Role of endoscopic debulking surgery Sharad Chawla a,*, Heather Tapp b, Mark Schembri c a Department of Otolaryngology, Modbury Public Hospital & Flinders Medical Centre, Adelaide, SA, Australia b Department of Haematology & Oncology, Women’s and Children’s Hospital, SA, Australia c Department of Otolaryngology, Women’s and Children’s Hospital & Modbury Public Hospital, Adelaide, SA, Australia

Paediatric para-meningeal rhabdomyosarcomas (RMSs) are treated with aggressive chemo-radiotherapy; so far, surgery has played a limited role in the management owing to the relative inaccessibility of lesion and the associated morbidity. We present the management of two cases of parameningeal embryonal RMS at our institution from 2005 to 2006, with critical airway compromise on presentation. Tumour stage and site were identical and both patients received chemo-radiotherapy based on the Malignant Mesenchymal Tumour Group MMT 953A protocol. In addition, one patient underwent debulking surgery to establish airway patency using trans-nasal endoscopic technique allowing a rapid return to normal activity. The second patient had higher morbidity and longer hospital stay due to prolonged intubation. We discuss the role of endoscopic surgery in the management of paediatric head and neck RMS. DOI: 10.1016/j.ijporl.2007.07.022