Chirom Amit Singh

Cochlear implantation in Waardenburg syndrome: The Indian scenario.

Abstract Conclusion: Children with Waardenburg syndrome (WS) exhibiting normal inner ear anatomy, like those included in our cohort, derive significant benefit from cochlear implantation and results are comparable to those reported for the general population of implanted children. Objectives: The patient population of WS accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with WS who have undergone cochlear implantation. Methods: On retrospective chart review, there were four cases with WS who underwent cochlear implantation. These cases were assessed for age at implantation, clinical and radiological features, operative and perioperative course, and performance outcomes. Auditory perception and speech production ability were evaluated using categories of auditory performance (CAP), meaningful auditory integration scales (MAIS), and speech intelligibility rating (SIR) during the follow-up period. Results: In this group of children with WS, with a minimum follow-up of 12 months, the CAP score ranged from 3 to 5, MAIS from 25 to 30, and SIR was 3. These scores are comparable with those of other cochlear implantees.

Foreign Body Esophagus: When Endoscopic Removal Fails…

Aspiration or ingestion of a foreign body has frequently been reported in the pediatric and in the adult population. Among many foreign bodies to be ingested, artificial denture is one to be impacted in the esophagus, especially among the elderly. Radiolucency of dental prosthesis complicates early diagnosis of an impacted or ingested dental prosthesis. Rigid and flexible esophagoscopes have been used to retrieve the foreign body from the esophagus but the need for open surgery to remove the foreign body as a rescue procedure to endoscopy or the primary procedure has not been well defined. Here we report a case of impacted foreign body esophagus which was managed primarily by surgery and another case where surgery was performed after trials of endoscopic approach had failed.

A phase 2 randomized study to compare short course palliative radiotherapy with short course concurrent palliative chemotherapy plus radiotherapy in advanced and unresectable head and neck cancer.

BACKGROUND Treatment of unresectable HNSCC is not well defined and has a poor outcome. This study has been designed to address the unmet needs of such groups of patients with primary end points of (a) proportion of patients eligible for radical treatment in each arm (b) loco-regional disease control at 6months between two arms. MATERIALS AND METHODS Locally advanced and unresectable HNSCC patients (except Nasopharynx and Larynx) unfit for radical treatment were randomized to arm A [short course RT alone (4Gy/#/day for 5days)] or arm B [RT as arm A+concurrent cisplatin at 6mg/m(2)/day IV bolus for 5days]. Those with at least PR were taken for further RT to complete biological equivalent dose of 70Gy, in both the arms. In arm B, concurrent CDDP at a dose of 40mg/m(2)/week was administered. RESULTS 114 patients (57 in each arm) were randomized but 111 were analyzable. 15 (27.27%) patients in arm A and 28 (50%) patients in arm B had ⩾PR (p=0.01) however patients taken for FRT were 14 (25.45%) and 26 (46.42%) in arms A and B respectively (p=0.02). Locoregional control i.e. (CR+PR) at 6months was 16.36% in arm A versus 32.14% in arm B (p=0.15). Median PFS (arm A - 3.2months, arm B - 6.2months; p=0.02) and OS (arm A - 5.9months, arm B - 10.1months; p=0.03) was significantly more in arm B. There was relative improvement in quality of life for most parameters in arm B. CONCLUSION Concurrent low dose CTRT can be an effective treatment modality in advanced and incurable HNSCC. However, a larger phase III trial is required.

An Evaluation of the Surgical Trauma to Intracochlear Structures After Insertion of Cochlear Implant Electrode Arrays: A Comparison by Round Window and Antero-Inferior Cochleostomy Techniques

To evaluate the extent of intracochlear damage by histologic assessment of cadaveric temporal bones after insertion of cochlear implants by: round window approach and cochleostomy approach. Cochlear implantation was performed by transmastoid facial recess approach in 10 human cadaveric temporal bones. In 5 temporal bones, electrode insertion was acheieved by round window approach and in the remaining 5 bones, by cochleostomy approach. The bones were fixed, decalcified, sectioned and studied histologically. Grading of insertion trauma was assessed. In the round window insertion group, 2 bones had to be excluded from the study: one was damaged during handling with electrode extrusion and another bone did not show any demonstrable identifiable cochlear structure. Out of the 3 temporal bones, a total of 35 sections were examined: 24 demonstrated normal cochlea, 4 had basilar membrane bulging and 7 had fracture of bony spiral lamina. In the cochleostomy group, histology of 2 bones had to be discarded due to lack of any identifiable inner ear structures. Out of the 3 bones studied, 18 sections were examined: only 3 were normal, 4 sections had some bulge in spiral lamina and 11 had fracture of bony spiral lamina. The fracture of spiral lamina and bulge of basement membrane proportion is relatively higher if we perform cochleostomy as compared to round window approach. Therefore, round window insertion is relatively less traumatic as compared to cochleostomy. However, our sample size was very small and a study with a larger sample is required to further validate these findings.

Percutaneous Management of Systemic Fungal Infection Presenting As Bilateral Renal Fungal Ball.

Abstract Background: Zygomycoses are uncommon, frequently fatal diseases caused by fungi of the class Zygomycetes. The majority of human cases are caused by Mucorales (genus—rhizopus, mucor, and absidia) fungi. Renal involvement is uncommon and urine microscopy, pottasium hydroxide mount, and fungal cultures are frequently negative. Case Presentation: A twenty-one-year-old young unmarried lady presented to our emergency department with bilateral flank pain, fever, nausea, and decreased urine output of one-month duration. She was found to have azotemia with sepsis with bilateral hydronephrosis with a left renal pelvic obstructing stone. Even after nephrostomy drainage and broad spectrum antibiotics, her condition worsened. She developed disseminated fungal infection, and timely systemic antifungal followed by bilateral nephroscopic clearance saved the patient. Conclusion: Although renal fungal infections are uncommon, a high index of suspicion and early antifungal and surgical intervention can give favorable outcomes.

Malignant Peripheral Nerve Sheath Tumour of the Maxilla

A 38-year-old man was diagnosed with malignant peripheral nerve sheath tumour of the maxilla. He was treated with total maxillectomy. Histopathological examination of the resected specimen revealed a close resection margin. The tumour was of high grade with an MIB-1 labelling index of almost 60%. At six weeks following the surgery, he developed local tumour relapse. The patient succumbed to the disease at five months from the time of diagnosis. The present report underlines the locally aggressive nature of malignant peripheral nerve sheath tumour of the maxilla which necessitates an early therapeutic intervention. A complete resection with clear margins is the most important prognostic factor for malignant peripheral nerve sheath tumour in the head and neck region. Adjuvant radiotherapy may be considered to improve the local control. Future research may demarcate the role of targeted therapy for patients with malignant peripheral nerve sheath tumour.

Iatrogenic facial nerve palsy "Prevention is better than cure": Analysis of four cases

Iatrogenic facial nerve palsy in mastoid surgery is considered a crime or a taboo in the present scenario of medical science. But one has to accept the fact that every otologist encounters this entity at some point in his/her career. Hence it is of prime importance to be equipped to detect and to manage these cases. The obvious and disfiguring facial deformity it causes makes this a dreaded complication. Our article here discusses our experience in managing four cases of iatrogenic facial palsy. The etiology in all the cases was mastoidectomy for cholesteatoma. The detection of the site and repair was performed by the same surgeon in all cases. The facial nerve was transected completely in three cases, and in one case there was partial loss (>50%) of fibers. Cable nerve grafting was utilized in three patients. There was grade 4 improvement in three patients who underwent cable nerve grafting, and one patient had grade 2 recovery after end-to-end anastomosis. A good anatomical knowledge and experience with temporal bone dissection is of great importance in preventing facial nerve injury. If facial nerve injury is detected, it should be managed as early as possible. An end-to-end anastomosis provides better results in final recovery as opposed to cable nerve grafting for facial nerve repair.

SMARCB1 (INI1) -deficient sinonasal carcinoma: a series of thirteen cases with assessment of histological patterns

A significant proportion of sinonasal malignancies comprise poorly differentiated/undifferentiated carcinomas that defy accurate histologic classification and behave aggressively. Recent years have seen a refinement of this spectrum by inclusion of novel entities harboring specific genetic alterations, including SMARCB1 (INI1)-deficient sinonasal carcinoma (SDSC), characterized by inactivating alterations in SMARCB1 gene, as demonstrated by loss of INI1 immunoexpression. Cyclin D1 is a cell-cycle regulatory protein downstream of INI1. Loss of INI1 leads to derepression of cyclin D1 transcription, suggesting its role as a putative therapeutic target. However, cyclin D1 expression has not been assessed in SDSCs. We retrieved all sinonasal carcinomas, including sinonasal undifferentiated carcinoma, undifferentiated carcinoma, poorly differentiated squamous cell carcinoma, and adenocarcinoma. Histopathologic features were reviewed. INI1 immunohistochemistry was performed. Cyclin D1 was performed in cases showing INI1 loss. Loss of INI1 staining was seen in 13 cases (5.8%), including 11 males and 2 females (age range, 11-65 years). Original diagnoses included SDSC (3/13), sinonasal undifferentiated carcinoma (3/13), adenocarcinoma (3/13), poorly differentiated squamous cell carcinoma (2/13), and poorly differentiated carcinoma (2/13). Tumors were predominantly basaloid in 6 cases and plasmacytoid/rhabdoid in 5 cases. We identified 2 cases having oncocytoid cells arranged in a gland-like pattern. Significant cyclin D1 immunoexpression was absent. SDSC is a rare, emerging entity that resembles a poorly differentiated carcinoma. Histomorphologic spectrum of these tumors is evolving. In addition to basaloid and plasmacytoid/rhabdoid cells, oncocytoid/adenocarcinoma-like pattern can also be seen in SDSC and predicts INI1 loss. These histologic patterns can further be subjected to INI1 immunohistochemistry for correct diagnosis.

Radiation-Induced Sarcoma Originating in Recurrent Juvenile Nasopharyngeal Angiofibroma

Juvenile nasopharyngeal angiofibroma is a benign disease affecting young males with a propensity to invade intracranially and into the orbit along preformed pathways. Complete surgical excision is the mainstay of management. Patients with multiple recurrences along with tumour extension into skull base and orbit can be considered for external beam radiation as either adjuvant or definitive treatment. Possibility of radiation-induced malignancy has been speculated by many authors, proof of which exists in only two studies so far. This report focuses on radiation-induced leiomyosarcoma in a patient with recurrent juvenile nasopharyngeal angiofibroma.

Primary laryngeal tuberculosis-“The great masquerader”

We report a case of primary laryngeal tuberculosis in a 54 year old Indian male, who had hoarseness as his presenting complaint. This case highlights that laryngeal tuberculosis may occur even without pulmonary tuberculosis, and the characteristics of the lesions now appear to be more nonspecific than earlier. Otolaryngologists and airway physicians need to be more alert to the emergence of laryngeal tuberculosis with atypical clinical manifestations and consider it in their differential diagnosis of all laryngeal diseases. Correspondence to: Pirabu Sakthivel, Department of Otorhinolaryngology and Head & Neck surgery,All India Institute of Medical Sciences, New Delhi-110002, India, Tel: 9958744547; E-mail: pirabusakthivel@gmail.com