Plexiform neurofibromatosis presenting as dysphagia

Plexiform neurofibromatosis is a autosomal dominant disease characterized by multiple cafe aulait spots, cutaneous neurofibromatosis, CMS tumours and skeletal abnormalities. We report a child with plexiform neurofibromatosis presenting with dysphagia, an unsual presentation.     

Vallecular cyst-revisited

We report a 3 months-old-male infant presented with recurrent cough, noisy breathing and regurgitation of feeds since 15 days of life. Examination revealed inspiratory stridor. CECT showed cystic lesion in base of tongue. After excision it was proved a case of vallecular cyst on histopathology. Although rare, vallecular cyst should be included in the differential diagnosis of congenital laryngeal stridor in neonates. The literature of vallecular cyst is being reviewed in the current article.     

Audio Vestibular Status in CML Patients on Imatinib Mesylate with Review of Literature

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm occasionally presenting with features of leukostasis as primary symptoms. Hearing loss is occasionally reported in CML patients. Further whether Imatinib mesylate has any effect on vestibular functions is not known. We conducted a preliminary study to assess hearing pattern in patients with CML on long term TKI therapy. This is a single center, cross sectional study from northern-India. Patients of CML who were on regular Tyrosine kinase inhibitors (TKI) therapy for at least 6 months underwent audiovestibular evaluation. A total of 44 CML patients on TKI therapy were assessed over a period of 6 months. The median age of the patients was 41 years, the mean duration of TKI therapy was 36 months. Four patients were found to have otological disorder clinically. On pure tone audiometry of 88 ears normal hearing pattern were found with at low and mid frequencies. There was a down sloping type of curve at higher frequencies in PTA in most of the patients. Cold caloric tests in 42 patients were found as equal response in both ears. We conclude from this preliminary study that there are no audio vestibular dysfunctions amongst patients of CML on TKI. It’s a negative study wherein we have ruled out any auditory deficits secondary to Imatinib therapy. Further studies are required to evaluate the audiometric profile in CML patients before Imatinib therapy and to be compared with the patients already on Imatinib in a large cohort.     

Safety Profile of Bone Marrow Mononuclear Stem Cells in the Rehabilitation of Patients with Posttraumatic Facial Nerve Paralysis—A Novel Modality (Phase One Trial)

Objectives The objectives of this study were to study the safety profile and role of mononuclear stem cells in the rehabilitation of posttraumatic facial nerve paralysis not improving with conventional treatment.Study Design This is a prospective nonrandomized controlled trial.Study Setting This study is conducted at Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh between July, 2007 and December, 2008.Patients We included eight patients of either sex aged between 18 and 60 years of posttraumatic facial nerve paralysis not improving with conventional treatment presented to PGIMER, Chandigarh between July 2007 and December 2008.Methods All patients underwent preoperative electroneuronography (ENoG), clinical photography, and high-resolution computed tomography (HRCT) temporal bone. All patients then underwent facial nerve decompression and stem cell implantation. Stem cells processing was done in well-equipped bone marrow laboratory. Postoperatively, all patients underwent repeat ENoG and clinical photography at 3 and 6 months to assess for objective and clinical improvement. Clinical improvement was graded according to modified House–Brackmann grading system.Intervention Done All patients of posttraumatic facial nerve paralysis who were not improving with conventional surgical treatment were subjected to facial nerve decompression and stem cell implantation.Main Outcome Measures All patients who were subjected to stem cell implantation were followed up for 6 months to assess for any adverse effects of stem cell therapy on human beings; no adverse effects were seen in any of our patients after more than 6 months of follow-up.Results Majority of the patients were male, with motor vehicle accidents as the most common cause of injury in our series. Majority had longitudinal fractures on HRCT temporal bone. The significant improvement in ENoG amplitude was seen between preoperative and postoperative amplitudes on involved side which was statistically significant (0.041). Clinical improvement seen was statistically significant both for eye closure (p < 0.010) and for deviation of angle of mouth (p < 0.008) at 6-month follow-up in 85% of our patients, far better than the results of previous conventional surgeries.Conclusion Stem cell therapy can be used safely in human beings without any adverse effects on humans, and it appears to be a promising modality for rehabilitation of patients with posttraumatic facial nerve paralysis not improving with conventional surgical treatment but few more clinical series are required for validation.     

Ameloblastic fibrosarcoma: a cytologist's perspective

Fine-needle aspiration cytology of a case of ameloblastic fibrosarcoma (AFS), an unusual odontogenic tumor related to ameloblastoma (AB), was performed in a 25-year-old female with a 1 cm swelling in the left lower orbital region along with involvement of zygomatic region. Aspiration of the tumor yielded a cellular sample composed predominantly of mesenchymal element and few clusters representing epithelial component showing tall columnar cells with peripheral palisading. Detailed cytomorphological features of AFS are discussed along with differential diagnosis from other tumors such as AB, desmoplastic AB, odontogenic fibroma, ameloblastic fibrodentinoma and ameloblastic fibro-odontoma, ameloblastic fibroma.     

Plexiform neurofibromatosis presenting as dysphagia

Plexiform neurofibromatosis is a autosomal dominant disease characterized by multiple cafe aulait spots, cutaneous neurofibromatosis, CMS tumours and skeletal abnormalities. We report a child with plexiform neurofibromatosis presenting with dysphagia, an unsual presentation.     

Parapharyngeal and retropharyngeal space abscess: an unusual complication of chronic suppurative otitis media

Parapharyngeal and retropharyngeal abscess as a complication of cholesteatoma is an uncommon entity. We present the unusual case of a 32-year-old man with chronic suppurative otitis media, presenting with parapharyngeal and retropharyngeal abscess. This was treated with incision and drainage of the abscess followed by modified radical mastoidectomy for chronic suppurative otitis media.     

Second branchial cleft cyst causing internal jugular vein thrombosis — A case report

The branchial cleft cyst also known as lateral cervical cyst is usually present in the lateral part of neck deep to sternocleidomastoid muscle at the junction of its upper third and lower two thirds. Branchial cysts are known for repeated infection with sudden increase in size and pain and for its recurrence. Thrombosis of major vessels of neck secondary to recurrent infection and inflammation of branchial cyst is extremely rare. Here we present the first case of internal jugular vein thrombosis; a rare complication seen with branchial cyst in a 54-year-old male. The patient was treated initially with antibiotics followed by excision of cyst and ligation of internal jugular vein without any complication.     

Parapharyngeal and retropharyngeal space abscess: an unusual complication of chronic suppurative otitis media

Parapharyngeal and retropharyngeal abscess as a complication of cholesteatoma is an uncommon entity. We present the unusual case of a 32-year-old man with chronic suppurative otitis media, presenting with parapharyngeal and retropharyngeal abscess. This was treated with incision and drainage of the abscess followed by modified radical mastoidectomy for chronic suppurative otitis media.     

Differential independent impact of the intraoperative use of navigation and angled endoscopes on the surgical outcome of endonasal endoscopy for pituitary tumors: a prospective study

Neurosurgical Review - Despite widespread popularity of navigation and angled endoscopes in endonasal endoscopy, there are hardly few studies on their efficacy with the extent of resection or...