'Pai Syndrome' with anterior alveolar polyp: A variant of a rare clinical entity.

'Pai syndrome' (PS) is a rare congenital syndrome. Presented here, a new-born baby-girl who exhibited the characteristic features of having a midline nasal (septal) polyp, an anterior alveolar process polyp, and a pericallosal lipoma associated with corpus callosum dysgenesis of the brain. Both polyps were lined with stratified-squamous epithelium. The overall features were largely consistent with those described by Pai et al., in 1987. A midline cleft-lip (with or without cleft-alveolus) is one of the most common features of the syndrome which was however absent in this case. Instead, an anterior alveolar polyp is present, which is relatively rare.

Ancient schwannoma of cervical sympathetic chain masquerading as carotid body tumour.

Schwannoma of cervical sympathetic chain is a rare cause of neck swelling. We report a 73- year-old male presented with anterior neck triangle swelling mimicking a carotid body tumour. Surgical excision was done, and the histopathological examination reported as ancient schwannoma. We would like to discuss the important differential diagnoses and highlight the possibility of an ancient schwannoma of cervical sympathetic chain masquerading as carotid body tumour. Also, to emphasise the importance of imaging for pre-operative planning and counselling.

Management experience of subglottic stenosis by endoscopic bougie dilatation with mitomycin C and review of literature: case series.

The aim of the study was to examine and analyze the epidemiology and outcome of treatment for paediatric acquired subglottic stenosis treated with endoscopic bougie dilatation and topical mitomycin C. There were 15 patients identified from 2008 until 2013. All of them had acquired subglottic stenosis due to history of intubation. Majority of the patients had grade III stenosis, with the total of seven. Three patients had grade IV; three were grade II and two were grade I. All of the patients with severe stenosis (grade III and IV) needed tracheostomy while only one in mild stenosis group (grade I and II) required it for prolonged ventilation rather than obstruction due to subglottic stenosis. All of them underwent direct laryngoscopy under general anesthesia followed by endoscopic dilatation with bougie and topical mitomycin C 0.4 mg/ml for 5 min. Aim of success in our study was decannulation of tracheostomy or absence of symptoms at exertion. We achieved 6 (60 %) successful decannulation out of 10 patients with tracheostomy (excluded the patient with tracheostomy in grade I stenosis due to prolonged ventilation). As for those without tracheostomy, 3 (75 %) out of 4 patients were asymptomatic even at exertion. Average number of dilatation was 3.1 times, with mean duration of 28 min. No complications were reported in our series. One patient with grade I stenosis passed away due to severe pneumonia unrelated to the stenosis or dilatation, and she did not have any dilatation before she passed away. Multiple related risk factors were identified such as intubation, prematurity, movement of endotracheal tube, respiratory infection, traumatic intubation and gastroesophageal reflux disease. Experience of open surgical method was very limited in our centre in Sabah in East Malaysia. Endoscopic technique plays an important role in treatment of subglottic stenosis with adjunct like mitomycin C possibly booster the successful rate.

Endoscopic, endonasal decompression of spinal stenosis with myelopathy secondary to cranio-vertebral tuberculosis: two cases.

BACKGROUND: Tuberculosis can cause extensive osseo-ligamentous destruction at the cranio-vertebral junction, leading to atlanto-axial instability and compression of vital cervico-medullary centres. This may manifest as quadriparesis, bulbar dysfunction and respiratory insufficiency. AIM: We report two patients presenting with spinal stenosis and cord compression secondary to cranio-vertebral tuberculosis, who were successfully decompressed via an endoscopic, endonasal approach. STUDY DESIGN: Two case reports. METHODS AND RESULTS: Both patients were successfully decompressed via an endoscopic, endonasal approach which provided access to the cranio-vertebral junction and upper cervical spine. CONCLUSION: An endoscopic, endonasal approach is feasible for the surgical management of cranio-vertebral junction stenosis; such an approach minimises surgical trauma to critical structures, reducing post-operative morbidity and the duration of hospital stay.