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Recurrent pyogenic meningitis and Mondini dysplasia: Surgeons nightmare—Our experience
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Abstracts / International Journal of Pediatric 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 Kingdom b Department of ENT, Royal Hospital for Sick Children, Dalnair Street, Glasgow, G3 8SJ, United Kingdom
Synovial cell sarcoma is a rare subtype of soft tissue sarcoma. It usually affects the extremities and laryngeal tumours are extremely rare. We present the case of an 11-year-old boy with synovial cell sarcoma arising from the right aryepiglottic fold which was not deemed resectable at the time of diagnosis. He underwent pre-operative chemoradiotherapay followed by total laryngectomy and remains disease free over 6 years after treatment. There were also multiple clinical, psychological and social challenges faced during the rehabilitation of this paediatric laryngectomy 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. Panda Department 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 warrants radiological evaluation with MRI and high resolution computed tomography. Mondini’s dysplasia may be recognized during evaluation of recurrent meningitis in children. Mondini’s dysplasia commonly associated with deafness is frequently heralded by recurrent pyogenic meningitis secondary to communication of middle ear with subarachanoid space. Early diagnosis requires high clinical suspicion and radiologic investigation and successful repair can prevent sequelae. We report a case of seven-year-old deaf child who presented with four episodes of recurrent meningitis and bilateral deafness. Mondini’s dysplasia of inner ear was diagnosed on high resolution computed tomography and MRI. The defect in the middle ear was successfully repaired. We report this case for its rarity and to highlight the fact that Mondini’s dysplasia should be considered in differential diagnosis in child presenting with recurrent meningitis and sensorineural deafness.
review the literature of this topic (16 cases reported). Fever and deep neck infection like symptoms were the only clinical findings at admission in 87.5% children. All children had a neck CT scan performed showing findings suggestive of retropharyngeal abscess. All children were started antibiotic therapy without clinical improvement and 31% of patients underwent unproductive surgical drainage of the retropharyngeal space. Otolaryngologist should be aware of atypical presentation of Kawasaki disease presentation mimicking retropharyngeal abscess. Early diagnosis is pivotal for preventing cardiac complications and avoiding the risk associated to unnecessary surgical intervention. http://dx.doi.org/10.1016/j.ijporl.2012.09.042
Response of infantile airway and facial hemangiomas to propranolol 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 by posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), cardiac defects and coarctation of the aorta (C), and eye abnormalities (E). Propranolol is currently first line therapy for treatment of head and neck hemangiomas that cause functional defects or extreme disfigurement. The safety and efficacy of this treatment have been well documented in current literature, but few authors have reported the use of propranolol in patients with PHACE syndrome. We report a case of a patient with PHACE syndrome and associated infantile airway and facial hemangiomas who was successfully treated with propranolol and a review of the literature on this subject. http://dx.doi.org/10.1016/j.ijporl.2012.09.043
Velopharyngeal insufficiency as the initial manifestation of a myotonic 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, Mexico b William Beaumont Hospital, Royal Oak, MI, United States c Cleft Palate Clinic, Hospital Gea Gonzalez, Mexico City, Mexico
http://dx.doi.org/10.1016/j.ijporl.2012.09.041
Retropharyngeal abscess: An unusual presentation of Kawasaki disease. 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, Italy b Neuroradiology Unit, Treviso Hospital, Treviso, Italy c Pediatric Unit, Treviso Hospital, Treviso, Italy
We report the case of a patient with Kawasaki disease whose initial presentation mimicked a retropharyngeal abscess and
Velopharyngeal insufficiency (VPI) is a disorder affecting nasal resonance during speech. The most common cause of VPI is a cleft palate. However, VPI can occur as a consequence of a neuromuscular or neurologic disorder without a palatal malformation. Myotonic dystrophy type 1 is a hereditary muscular disorder. In these cases, the abnormality is located on the 19q13.3 region, including a myotonic dystrophy protein kinase CTG repeat. This disease has several clinical manifestation features depending of the number of CTG repeats. The myotonic dystrophy can affect several muscle groups. VPI associated with the involvement of the velopharyngeal muscles, has been reported as the main clinical feature in some cases. The purpose of this paper is to present a case of VPI as the main clinical manifestation in a patient with a myotonic dystrophy type 1. http://dx.doi.org/10.1016/j.ijporl.2012.09.044
Abstracts / International Journal of Pediatric 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 Kingdom b Department of ENT, Royal Hospital for Sick Children, Dalnair Street, Glasgow, G3 8SJ, United Kingdom
Synovial cell sarcoma is a rare subtype of soft tissue sarcoma. It usually affects the extremities and laryngeal tumours are extremely rare. We present the case of an 11-year-old boy with synovial cell sarcoma arising from the right aryepiglottic fold which was not deemed resectable at the time of diagnosis. He underwent pre-operative chemoradiotherapay followed by total laryngectomy and remains disease free over 6 years after treatment. There were also multiple clinical, psychological and social challenges faced during the rehabilitation of this paediatric laryngectomy 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. Panda Department 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 warrants radiological evaluation with MRI and high resolution computed tomography. Mondini’s dysplasia may be recognized during evaluation of recurrent meningitis in children. Mondini’s dysplasia commonly associated with deafness is frequently heralded by recurrent pyogenic meningitis secondary to communication of middle ear with subarachanoid space. Early diagnosis requires high clinical suspicion and radiologic investigation and successful repair can prevent sequelae. We report a case of seven-year-old deaf child who presented with four episodes of recurrent meningitis and bilateral deafness. Mondini’s dysplasia of inner ear was diagnosed on high resolution computed tomography and MRI. The defect in the middle ear was successfully repaired. We report this case for its rarity and to highlight the fact that Mondini’s dysplasia should be considered in differential diagnosis in child presenting with recurrent meningitis and sensorineural deafness.
review the literature of this topic (16 cases reported). Fever and deep neck infection like symptoms were the only clinical findings at admission in 87.5% children. All children had a neck CT scan performed showing findings suggestive of retropharyngeal abscess. All children were started antibiotic therapy without clinical improvement and 31% of patients underwent unproductive surgical drainage of the retropharyngeal space. Otolaryngologist should be aware of atypical presentation of Kawasaki disease presentation mimicking retropharyngeal abscess. Early diagnosis is pivotal for preventing cardiac complications and avoiding the risk associated to unnecessary surgical intervention. http://dx.doi.org/10.1016/j.ijporl.2012.09.042
Response of infantile airway and facial hemangiomas to propranolol 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 by posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), cardiac defects and coarctation of the aorta (C), and eye abnormalities (E). Propranolol is currently first line therapy for treatment of head and neck hemangiomas that cause functional defects or extreme disfigurement. The safety and efficacy of this treatment have been well documented in current literature, but few authors have reported the use of propranolol in patients with PHACE syndrome. We report a case of a patient with PHACE syndrome and associated infantile airway and facial hemangiomas who was successfully treated with propranolol and a review of the literature on this subject. http://dx.doi.org/10.1016/j.ijporl.2012.09.043
Velopharyngeal insufficiency as the initial manifestation of a myotonic 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, Mexico b William Beaumont Hospital, Royal Oak, MI, United States c Cleft Palate Clinic, Hospital Gea Gonzalez, Mexico City, Mexico
http://dx.doi.org/10.1016/j.ijporl.2012.09.041
Retropharyngeal abscess: An unusual presentation of Kawasaki disease. 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, Italy b Neuroradiology Unit, Treviso Hospital, Treviso, Italy c Pediatric Unit, Treviso Hospital, Treviso, Italy
We report the case of a patient with Kawasaki disease whose initial presentation mimicked a retropharyngeal abscess and
Velopharyngeal insufficiency (VPI) is a disorder affecting nasal resonance during speech. The most common cause of VPI is a cleft palate. However, VPI can occur as a consequence of a neuromuscular or neurologic disorder without a palatal malformation. Myotonic dystrophy type 1 is a hereditary muscular disorder. In these cases, the abnormality is located on the 19q13.3 region, including a myotonic dystrophy protein kinase CTG repeat. This disease has several clinical manifestation features depending of the number of CTG repeats. The myotonic dystrophy can affect several muscle groups. VPI associated with the involvement of the velopharyngeal muscles, has been reported as the main clinical feature in some cases. The purpose of this paper is to present a case of VPI as the main clinical manifestation in a patient with a myotonic dystrophy type 1. http://dx.doi.org/10.1016/j.ijporl.2012.09.044