Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly.

Ectrodactyly‑ectodermal dysplasia and clefting syndrome or “Lobster claw” deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case.

Effects of type 3 thyroplasty on voice quality outcomes in puberphonia

@#p style=text-align: justify;strongOBJECTIVES:/strong To assess effects of type 3 thyroplasty on outcomes of voice quality in puberphonia.METHODS:br /Design:/strong Prospective cohortbr /strongSetting:/strong Tertiary Referral Hospitalbr /strongParticipants:/strong Six patients with puberphonia who failed voice therapy, aged 16-25 years, who consulted at the ENT Outpatient department between September 2010 and September 2012, underwent type 3 thyroplasty.Pre-operative and 6-month post-operative voice analysis by voice recordings, Voice Handicap Index (VHI), GRBAS score and real time acoustic analysis (perturbation) using Dr Speech software (University version 4.0, Voice Tech Corporation, USA [Tiger Electronics] with habitual fundamental frequency (FO), jitter % and shimmer % as parameters were performed.Results: Mean pre-operative VHI and GRBAS scores were 53 and 75.67, respectively, whereas post-operative scores were 29 and 25.00, respectively. (P-value for VHI was 0.004 and that of GRBAS was 0.00). On acoustic analysis, mean pre-operative habitual fundamental frequency (FO), jitter % and shimmer % was 245.82 Hz, 0.21 and 2.34, respectively, whereas post-operative mean was 140.78 Hz (P=0.00), 0.19 (P=0.04) and 1.52 (P=0.00), respectively.Conclusion: The mainstay of treatment of puberphonia is voice therapy. Thyroplasty provides a suitable management option in those cases who fail to respond by voice therapy./p

Carcinoma ex pleomorphic adenoma in the parapharyngeal space

@#<p><strong>OBJECTIVE:</strong> To present a case of carcinoma ex pleomorphic adenoma in the parapharyngeal space and discuss its management.<br /><br /><strong>METHODS:</strong> <br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary Government Teaching Hospital<br /><strong>Patient:</strong> One<br /><br /><strong>RESULTS:</strong> A 40-year-old male patient with dysphagia for three months and a left-sided mucosa-covered oropharyngeal mass was found to have a prestyloid parapharyngeal lesion on CT scans. Fine Needle Aspiration Cytology (FNAC) revealed a pleomorphic adenoma. With a past history of parapharyngeal pleomorphic adenoma excised transorally three years before, the present mass was excised by mandibular swing approach. Post-operative recovery was uneventful but the final histopathological report was carcinoma ex pleomorphic adenoma. <br /><br /><strong>CONCLUSION:</strong> Malignant transformation should be suspected in recurrent salivary tumors in the parapharyngeal space. Provided there was truly no pre-existing malignant focus in the originally-excised tumor, and that early recurrence was not due to inadequate initial excision, this patient had a rare condition where the same tumor underwent malignant transformation within three years only. To the best of our knowledge, such an early transformation to malignancy of a minor salivary gland tumor of the parapharyngeal space has not been reported in the English literature.</p>

Ectopic thyroid tissue- A case series

@#<p><strong>OBJECTIVE:</strong> To describe the clinical presentation and course of seven ectopic thyroid patients. <br /><br /><strong>METHODS:<br />Design:</strong> Retrospective Chart Review<br /><strong>Setting:</strong> Tertiary Government Teaching Hospital<br /><strong>Patient:</strong> Seven patients<br /><br /><strong>RESULTS:</strong> Five patients were female and two were male (ratio of 5:2). Three belonged to the 20 to 30 year-old age group, whereas two were below 10 years of age. All seven were biochemically hypothyroid and ectopic thyroid was found to be the only functioning thyroid tissue. Three patients were managed medically with levothyroxine, while ectopic thyroid was excised in four. Ectopic thyroid tissue was autotransplanted in two cases following excision.<br /><br /><strong>CONCLUSION:</strong> The ages of presentation in the present series correspond with the increased physiological demand of thyroid hormone. Thyroid substitution therapy is a must in the presence of clinical and/or biochemical hypothyroidism. Surgical excision should be avoided as far as possible especially if the ectopic tissue is the only functioning thyroid in the body. Surgery is required in selected cases presenting with obstructive symptoms or hemorrhage which are unresponsive to substitution therapy. Auto transplantation of the ectopic thyroid may not provide significant benefit to the patient and more research is warranted in this aspect.</p>

Giant cell tumor of the maxilla

@#OBJECTIVE/strong: To present a rare case of maxillary swelling; its investigation and management.br /br /strongMETHODS/strong:br /strongDesign/strong: Case Report br /strongSetting/strong: Tertiary Government Teaching Hospital br /strongPatient/strong: One br /br /strongRESULTS/strong: A 45-year-old female presented with a right maxillary swelling of six months duration. Radiological investigation revealed a radiolucent lesion arising from the inferior aspect of the right maxilla with no areas of calcification. Incisional biopsy report was consistent with giant cell tumor. The mass was excised via a Weber Ferguson incision under general anesthesia.CONCLUSION/strong: Though rare, giant cell tumor should be considered as one of the differential diagnosis in cases of maxillary swelling. Adequate surgical excision with long-term follow-up should be the treatment of choice for managing a giant cell lesion of the maxilla./p

Orbital and para-orbital tumours--a 10 years retrospective study.

A total number of 631 cases of orbital and para-orbital tumours causing proptosis was analysed in a retrospective way in relation to age, anatomic location, histological and clinical profile. CT-scan was considered as the primary modality of investigation as it correctly delineates the extent of the tumour, integrity of bony structures and intracranial spread. Different modalities of surgery like lateral orbitotomy, medial orbitotomy, lateral rhinotomy and endoscopic excision, etc, had been performed depending upon the anatomical locations. Surgical approaches, histological types of various tumours are analysed in detail.