Retropharyngeal ganglioneuroma presenting with neck stiffness: report of a case and review of literature.

Ganglioneuromas rarely occur in the retropharynx with only three cases reported in the current literature. The most common symptom associated with retropharyngeal ganglioneuromas is dysphagia. We report a retropharyngeal ganglioneuroma with an unusual clinical presentation of neck stiffness and pain. A 42-year-old woman presented with incapacitating neck pain, neck stiffness, right upper extremity weakness, as well as dysphagia. Neurological workup was normal. Imaging revealed a hyperdense, ill-defined, diffuse right retropharyngeal mass suggestive of a possible nerve sheath tumor with no communication with the cervical spine. Surgical removal was uneventful and associated with a postoperative Horner's syndrome. In follow-up, dysphagia and neck symptoms improved. Retropharyngeal ganglioneuromas can occur in a wide age range of patients. Surgical excision via a cervical approach offers definitive therapy but may be associated with an iatrogenic Horner's syndrome for which the patients should be counseled prior to operative intervention. Neck pain is an atypical symptom that needs to be worked up to rule out a communication with the spinal column prior to surgical removal. Patients must be counseled that atypical symptoms may not completely resolve with surgical treatment.

Retropharyngeal ganglioneuroma presenting with neck stiffness: report of a case and review of the literature.

OBJECTIVE: Ganglioneuromas rarely occur in the retropharynx with only three cases reported in the current literature. The most common symptom associated with retropharyngeal ganglioneuromas is dysphagia. We report a retropharyngeal ganglioneuroma with an unusual clinical presentation of neck stiffness and pain. STUDY DESIGN: Case report and review of literature. METHODS: A 42 year old woman presented with incapacitating neck pain and neck stiffness as well as dysphagia. Neurological work up was normal. Imaging revealed a hyper-dense, ill-defined, diffuse right retropharyngeal mass suggestive of a possible nerve sheath tumor with no communication with the cervical spine. Surgical removal was uneventful and associated with a post-operative Horner's syndrome. In follow-up, dysphagia and neck symptoms improved. CONCLUSION: Retropharyngeal ganglioneuromas can occur in a wide age range of patients. Surgical excision via a cervical approach offers definitive therapy but maybe associated with an iatrogenic Horner's syndrome for which the patients should be counseled prior to operative intervention. Neck pain is an atypical symptom that needs to be worked up to rule out a communication with the spinal column prior to surgical removal. Patients must be counseled that atypical symptoms may not completely resolve with surgical treatment.

A rational approach to the use of tracheotomy in surgery of the anterior skull base.

OBJECTIVE: To offer an algorithm for airway management in anterior skull base surgery. METHODS: This is a retrospective review of 109 patients undergoing major anterior skull base surgery from a single senior surgeon's experience from September 1997 to May 2006. RESULTS: We report only one (1%) postoperative mortality in this series and only seven major complications in six patients, including two cases of stroke, one case of cerebrospinal fluid (CSF) leak, and four cases of delayed osteoradionecrosis. No patients in this series developed tension pneumocephalus. The total major complication rate is 6%. Fifty-one (47%) patients received prophylactic tracheotomy, and 58 (53%) patients did not receive prophylactic tracheotomy. Eighty-eight (81%) patients received anterior skull base reconstruction with local flaps. Six (5.5%) patients required primary reconstruction with a free flap. CONCLUSION: We attribute the very low rate of major complications in this series and, in particular, no cases of tension pneumocephalus and rarity of CSF leaks primarily to prophylactic tracheotomy in selected patients and to a reconstructive strategy that emphasizes use of local vascularized tissue to reconstruct the anterior skull base.

Aggressive maxillofacial disassembly in skull base surgery.

OBJECTIVE: To review our favorable experience with maxillofacial disassembly for exposure and resection of tumors of the skull base. PATIENTS AND METHODS: This is a retrospective review of 31 patients from 1997 to 2005 treated by maxillofacial disassembly and includes 22 patients with midface disassembly and 9 patients with mandible with or without midface disassembly. RESULTS: Follow-up ranges from 2 weeks to 7 years: 23 (74.2%) of 31 patients are alive and disease free; 3 patients are alive with disease; 3 patients have died of distant metastasis. There were 14 complications in 12 (38.7%) patients; 13 of 14 complications were minor. One major complication (cardiovascular accident) resulted in the single postoperative mortality in this series. CONCLUSION: Our technique of maxillofacial disassembly allows for tailored exposure of all regions of the anterolateral skull base with acceptable perioperative morbidity in appropriately selected patients. EBM RATING: C-4.