Kartagener's syndrome presenting as bilateral recurrent nasal polyposis in a young boy

Kartagener's syndrome is a very rare congenital disease consists of a classic triad, sinusitis, situs inversus and bronchiectasis. Approximately one half of patients with primary ciliary dyskinesia have situs inversus and Kartagener syndrome. We are presenting a case of Kartagener's syndrome in a 10-year-old boy presented with chronic sinusitis leading to bilateral multiple nasal polyposis. He also had situs inversus and chronic bronchiectasis. He had undergone surgery two years back for nasal polyposis but now again presenting as recurrent nasal polyposis. In order to prevent the dreadful complications correct diagnosis in early life is very important in such patients

Complications of open surgical Tracheostomies and their management - our experience

Objective: To justify the role of open surgical tracheotomy in patients admitted in surgical or medical intensive care units for ventilator support or secretion management

Study Design: A descriptive study

Place and Duration of Study: Department of otorhinolaryngology- Head and Neck Surgery, Dr Ruth K M Pfau Civil Hospital Karachi, Dow Medical College- Dow University of Health Sciences and Otolaryngology-Head and Neck Surgery Ziauddin University Karachi Jan 2014 to Jan 2018

Material and Methods: This study includes all elective and emergency tracheotomies performed in intensive care units (ICU). Patients with cervical spine injuries, bleeding diathesis and patients below 12 years of age were excluded from our study

Results: We encountered complications in 20 patients out of 534, on whom tracheotomies were carried out in intensive care setting. Hemorrhage during and after tracheotomy procedure occurred in eight patients, which was controlled by pressure, ligation or diathermy. Procedure related surgical emphysema occurred in two patients; it was limited to cervical region and subsided with conservative management. The inadvertent decannulation of tracheotomy tube occurred in two cases. Subglottic stenosis developed in four patients. Tracheo esophageal fistula was encountered in four cases

Conclusions: A low morbidity and mortality rate in our series justifies the role of open surgical tracheostomy in patients admitted in surgical or medical ICU for ventilator support or secretion management. It is also effective in a situation of unsecured airway

Frequency, presentation and management of juvenile nasopharyngeal angiofibroma

To determine frequency, clinical presentation and management of juvenile nasopharyngeal angiofibroma [JNA] at Civil Hospital Karachi. A descriptive study. Department of Otorhinolaryngology - Head and Neck Surgery, Dow Medical College, Civil Hospital Karachi and Dow University of Health Sciences from, January 2002 to December 2008. This study included 54 cases of nasopharyngeal angiofibroma over a period of 7 years. Follow up period ranges from 1 month to 7years. All patients were male with an age range of 14 - 17yrs. Epistaxis and nasal obstruction were the two most common presenting symptoms. Majority of our patients 39 [72%] had stage III a disease, stage II disease as seen in 13 [24%] cases while 2 [4%] cases presented in stage I disease according to Fisch classification. Surgery was done in all patients. Tumor recurrence was seen in 4 [7%] of cases. Angiofibroma usually presents in adolescent males. The triads of nasal obstruction, nasopharyngeal mass and recurrent epistaxis indicate the presence of the neoplasm. Surgery is the treatment of choice. Lateral rhinotomy is the most common approach. The sublabial endoscopic assisted approach is cosmetically better than other approaches and has definite advantages. Long term outcome for our patients treated by endoscopic assisted sublabial technique is required to further strengthen our view

Juvenile nasopharyngeal angiofibroma: stage and surgical approach

Objective: to study various surgical approaches and the outcome of surgery in juvenile nasopharyngeal angiofibromas [JNA]

Design: a descriptive study

Setting: department of Otorhinolaryngology - Head and Neck Surgery, Civil Hospital Karachi

Method: this study included 40 consecutive cases of nasopharyngeal angiofibroma over a period of 4 years. Patients were regularly followed up for any recurrence up to 3 years

Results: majority of our patients had stage III [72.5%] disease [Chandlers staging] i.e. extension in pterygopalatine fossa, maxillary antrum, nose and sphenoid sinuses; and infratemporal fossa in 72.5% patients, tumor was removed through lateral rhinotomy approach while Weber Fergusson in 17.5% , mid facial degloving in 7.5% and only in 2.5% patient transpalatal approach was used. Tumour recurrence were seen only in 5% of cases

Conclusions: angiofibromas usually present at the late stage. Surgery is the treatment of choice. Most of the tumours are excisable by a lateral rhinotomy approach, with minimal chance of recurrence

clinic pathological study of laryngeal malignancies - an institutional experience

Objective: to determine the risk factors and clinic-pathological pattern of the laryngeal malignancies

Design: descriptive case-series

Setting: department of Otorhinolaryngology - Head and Neck Surgery, Dow Medical College, Civil Hospital Karachi and Dow University of Health Sciences from, March 1998 to March 2009

Methods: all patients of either gender having malignant lesion of larynx were included. Recurrent cases after surgery or chemo radiotherapy and benign Tumours of the larynx were excluded from the study. Patients were studied with particular importance to the risk factors, mode of presentation, topography and histopathology of the tumour

Results: out of 100 patients, 89 were males and 11 were females with male to female ratio of 8.1:1. Majority of the subjects was in 5th decade of their life. Tobacco intake in the form of smoking or chewing was present in 91% . Presenting symptoms were hoarseness, odynophagia, sore throat, dysphagia and dyspnea/stridor. Tran's glottic area was the commonest site [39%] for laryngeal cancer. Squamous cell carcinoma [SCC] found in 98% of patients. Well differentiated category seen in 57% of patients

Conclusions: SCC was the most frequent malignant lesion of larynx. Most of the patients presented in the late stages [stage III and IV]. Commonest presenting symptom was hoarseness. Smoked tobacco [cigarette smoking] was found to be the major risk factor

Frequency of un-safe chronic suppurative otitis media in patients with discharging ear

Objective: To determine the frequency of un-safe chronic suppurative otitis media and its complications in patients with chronic discharging ear

Methods: This study was carried out in the Department of Otorhinolaryngology, Civil Hospital Karachi, Sindh - Pakistan between January 2004 and June 2006. This study included 390 cases of chronic discharging ears attending out patient department. All were examined according to protocol, and relevant investigations were carried out. 107 cases underwent mastoid exploration and were observed for cholesteatoma, granulations, aural polyps and their complications. A structured questionnaire was designed to record all information which was later analyzed

Results: Majority of patients was belonging to poor families. Female-male ratio was 1.2:1. Age range was between 6 months and 70 years. Most of the patients presented with discharging ears. Central perforation was seen in 89% cases. One hundred seven cases [27.5%] underwent mastoid exploration, in which cholesteatoma was found in 11.5% cases. Ossicular damage was the commonest complication in all cases having cholesteatoma, while 1% cases presented with mastoid abscess, 1.5% with exposed facial nerve, 1.2% with exposed sigmoid sinus and 0.25% with Bezold abscess. Intracranial complications were seen in nine patients

Conclusion: Cholesteatoma was found to be associated with most of the complications in cases of un-safe type of C.S.O.M. The complication rate in this study is lesser than that observed in our neighboring countries like Bangladesh, but still higher as compared to the developed countries. Cholesteatoma was also found in two cases having central perforation which is unusual presentation

Xeroderma pigmentosa - squamous cell carcinoma lip

A 10 Years old female patient of xeroderma pigmentosa [XP] presented with an oval shaped swelling on left upper lip for 2 months. Excisional biopsy was done and histopathology revealed moderately differentiated Squamous cell carcinoma. We report this case as Xeroderma pigmentosa with squamous cell carcinoma is rarely seen in ENT practice

Laryndeal Cancer Surgery Techniques, Management and Complications

Management of laryngeal cancer experienced a vital change during the last few decades. Indeed laryngeal cancer is a significant malignancy as it also has high cure rate. Surgery is an effective economical mode of treatment in our country, where facilities of time duration of study other modalities such as radiotherapy and chemotherapy are not uniformly available. This study evaluates the different surgical techniques, management and post operative complications of 50 cases of laryngeal carcinoma. Total laryngectomy was the commonest operation i.e. in 31 cases [62 percent]. The commonest complication was found to be pharyngocutaneous fistula [18 percent]. 72 percent patients were living disease free, 20 percent patients were living with disease and 8 percent patients expired in follow up period, of 3 years. It was concluded that radical surgery is still the most suitable modality of treatment in our environment where we deal advanced pathology