response of infantile airway and facial hemangiomas to propranolol in a patient with phace syndrome

ric Otorhinolaryngology 77 (2013) 879–881

Laryngeal synovial cell sarcoma in an 11-year-old boy: Challenges
of management and rehabilitation
C. Simon a, D.P. Crampsey a, F.B. MacGregor b,*a Department of ENT, Gartnavel General Hospital, 1053 Great Western

Road, Glasgow, G12 0YN, United Kingdomb Department of ENT, Royal Hospital for Sick Children, Dalnair Street,

Glasgow, G3 8SJ, United Kingdom

Abstracts / International Journal of Pediat880

Synovial cell sarcoma is a rare subtype of soft tissue sarcoma. Itusually affects the extremities and laryngeal tumours areextremely rare. We present the case of an 11-year-old boy withsynovial cell sarcoma arising from the right aryepiglottic foldwhich was not deemed resectable at the time of diagnosis. Heunderwent pre-operative chemoradiotherapay followed by totallaryngectomy and remains disease free over 6 years aftertreatment. There were also multiple clinical, psychological andsocial challenges faced during the rehabilitation of this paediatriclaryngectomy patient.

http://dx.doi.org/10.1016/j.ijporl.2012.02.077

Recurrent pyogenic meningitis and Mondini dysplasia:Surgeons nightmare—Our experience

Roshan K. Verma *, Nishikant tripathi, Naresh K. PandaDepartment of Otolaryngology, Head and Neck Surgery, Post Graduate

Institute of Medical Education and Research, Chandigarh, 160012, India

History of recurrent meningitis in a deaf child warrantsradiological evaluation with MRI and high resolution computedtomography. Mondini’s dysplasia may be recognized duringevaluation of recurrent meningitis in children. Mondini’s dysplasiacommonly associated with deafness is frequently heralded byrecurrent pyogenic meningitis secondary to communication ofmiddle ear with subarachanoid space. Early diagnosis requireshigh clinical suspicion and radiologic investigation and successfulrepair can prevent sequelae.

We report a case of seven-year-old deaf child who presentedwith four episodes of recurrent meningitis and bilateral deafness.Mondini’s dysplasia of inner ear was diagnosed on high resolutioncomputed tomography and MRI. The defect in the middle ear wassuccessfully repaired. We report this case for its rarity and tohighlight the fact that Mondini’s dysplasia should be considered indifferential diagnosis in child presenting with recurrent meningitisand sensorineural deafness.


Retropharyngeal abscess: An unusual presentation of Kawasakidisease. Case report and review of the literature

Maria Elena Cavicchiolo a, Paola Berlese a, Silvia Bressan a,Elena Trincia b, Ingrid Inches b, Maria Stefania Strafella c,Chiara Stefani c, Liviana Da Dalt c,*a Department of Woman and Child Health, University of Padua, Italyb Neuroradiology Unit, Treviso Hospital, Treviso, Italyc Pediatric Unit, Treviso Hospital, Treviso, Italy

We report the case of a patient with Kawasaki disease whoseinitial presentation mimicked a retropharyngeal abscess and

review the literature of this topic (16 cases reported). Fever anddeep neck infection like symptoms were the only clinical findingsat admission in 87.5% children. All children had a neck CT scanperformed showing findings suggestive of retropharyngeal ab-scess. All children were started antibiotic therapy without clinicalimprovement and 31% of patients underwent unproductivesurgical drainage of the retropharyngeal space. Otolaryngologistshould be aware of atypical presentation of Kawasaki diseasepresentation mimicking retropharyngeal abscess. Early diagnosisis pivotal for preventing cardiac complications and avoiding therisk associated to unnecessary surgical intervention.


Response of infantile airway and facial hemangiomas topropranolol in a patient with PHACE syndrome

Brian C. Gross, Jeffrey R. Janus, Laura J. Orvidas *

Department of Otorhinolaryngology – Head and Neck Surgery, Mayo

Clinic, 200 First St SW, Rochester, 55905, MN, USA

PHACE syndrome is a neurocutaneous syndrome defined byposterior fossa malformations (P), hemangiomas (H), arterialanomalies (A), cardiac defects and coarctation of the aorta (C), andeye abnormalities (E). Propranolol is currently first line therapy fortreatment of head and neck hemangiomas that cause functionaldefects or extreme disfigurement. The safety and efficacy of thistreatment have been well documented in current literature, butfew authors have reported the use of propranolol in patients withPHACE syndrome. We report a case of a patient with PHACEsyndrome and associated infantile airway and facial hemangiomaswho was successfully treated with propranolol and a review of theliterature on this subject.


Velopharyngeal insufficiency as the initial manifestation of amyotonic dystrophy type 1: A case report

Andres Silva-Rojas a, Antonio Ysunza b,*, Daniel Diaz-Torres a,Mercedes Bardales-Lazcano a, Maria C. Pamplona c

a Department of Audiology and Phoniatrics, Hospital General de Mexico,

Mexico City, Mexicob William Beaumont Hospital, Royal Oak, MI, United Statesc Cleft Palate Clinic, Hospital Gea Gonzalez, Mexico City, Mexico

Velopharyngeal insufficiency (VPI) is a disorder affecting nasalresonance during speech. The most common cause of VPI is a cleftpalate. However, VPI can occur as a consequence of a neuromus-cular or neurologic disorder without a palatal malformation.Myotonic dystrophy type 1 is a hereditary muscular disorder. Inthese cases, the abnormality is located on the 19q13.3 region,including a myotonic dystrophy protein kinase CTG repeat. Thisdisease has several clinical manifestation features depending ofthe number of CTG repeats. The myotonic dystrophy can affectseveral muscle groups. VPI associated with the involvement of thevelopharyngeal muscles, has been reported as the main clinicalfeature in some cases. The purpose of this paper is to present a caseof VPI as the main clinical manifestation in a patient with amyotonic dystrophy type 1.







response of infantile airway and facial hemangiomas to propranolol in a patient with phace syndrome

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