Comparison between true and partial hemifacial hypertrophy.

Hemifacial hypertrophy (HFH) is rare and characterized by unilateral enlargement of the head and teeth. Hemifacial hypertrophy is classified as true HFH (THFH) with unilateral enlargement of the viscerocranium, and partial HFH (PHFH) in which not all structures are enlarged. We present a case of THFH and compare and contrast it with a case of PHFH. Hemifacial hypertrophy may cover a wide spectrum of defects or may involve only muscle or bone. Myohyperplasia, reported previously as a separate syndrome, may actually represent a forme fruste of PHFH or THFH. The PHFH patient lead a healthy, normal life without significant psychosocial problems and was reluctant to accept his deformity. Contrastingly, the THFH patient had significant social stigma and compromised health together with major aesthetic morbidity. Treatment planning in THFH is arduous and involves multiple modalities. Therefore, it is imperative to differentiate THFH and PHFH for better understanding and management of the condition.     

Contemporary staging and prognosis for primary tracheal malignancies: a population-based analysis.

OBJECTIVE: Determine staging characteristics and survival outcomes for primary malignancies of the trachea. Design Cross-sectional analysis of national cancer database. METHODS: Cases of primary tracheal malignancy were extracted from the Surveillance, Epidemiology, and End Results database for the time period 1988-2000. T-stage, N-stage, and overall stage of presentation were determined. Mean, median, and 5-year survival statistics were computed using Kaplan-Meier survival analysis for each tumor histology and for the overall cohort according to stage. RESULTS: Ninety-two cases with adequate histologic information were identified. Mean age at presentation was 59.3 years with an equal sex distribution. Squamous cell carcinoma was the most common tumor type (41 cases) followed by adenoid cystic carcinoma (19 cases). Forty-nine cases (53%) presented with stage 3 or stage 4 disease. Squamous cell carcinoma exhibited poorer survival (mean survival, 44.0 month, 5-year survival, 34%) than adenoid cystic carcinoma (mean survival, 115 month, 5-year survival, 78%). Five-year unadjusted survival rates according to overall stage were 52.8%, 70.0%, 75.0%, 15.1%, respectively. CONCLUSIONS: Primary tracheal malignancies often present with advanced stage. Patients with squamous cell carcinoma of the trachea have poorer prognoses when compared with adenoid cystic carcinoma and other tumor types. Staging tracheal cancer with a TNM-based system helps predict survival. EBM rating: C.     

Extended trans-septal approach for mitral valve surgery: Our early experience

Background Extended trans septal (ETS) approach for mitral valve surgery often divides the artery to the Sino-Atrial node. The clinical implication of this is contentious. We analyzed our early results with ETS approach. Methods Between June 1998 and September 2003 eleven patients underwent mitral valve surgery by ETS approach. Six were females. Age ranged from 19 years to 67 years (median 40 years). Six underwent mitral valve replacement (MVR). Four underwent aortic and mitral (double) valve replacement (DVR). One had mitral valve repair. Three had additional procedures (tricuspid valve repair=1, Coronary artery bypass=1, Aorto bifemoral graft=1). Cardiopulmonary bypass ranged from 64 minutes to 77 minutes (median 72 minutes) for MVR and 112 minutes to 178 minutes (median 140 minutes) for DVR. Aortic cross clamp times ranged from 39 minutes to 52 minutes (median 47 minutes) for MVR and 74 minutes to 120 minutes (median 95 minutes) for DVR. Results There was no mortality or morbidity attributed to the ETS approach. One early death in emergency DVR was due to heart failure. Three patients needed seqeuntial pacing in the immediate post-operative period. Nine out of ten survivors were back to their preoperative rhythms on hospital discharge (6 sinus rhythm; 3 atrial fibrillation). One patient with preoperative trifascicular block who underwent reoperation to fix a paravalvular mitral leak needed a permanent pacemaker (VVI). The follow-up ranged from 1 month to 64 months (median 6 months) and is 100% complete. There was no late death or new arrhythmia. Conclusions Extended trans septal approach is safe. It gives excellent exposure of the mitral valve. division of the sinus node artery is not deleterious in the short to intermediate term. Presented at the 50th Annual Meeting of IACTS. New Delhi, Feb. 2004.     

Factors affecting survival in maxillary sinus cancer.

PURPOSE: The study goal was to determine survival parameters and clinical factors influencing survival for maxillary sinus malignancies. METHODS: Cases of maxillary sinus malignancy for the time period 1988 to 1998 were extracted from the Surveillance, Epidemiology and End Results database. Cases with distant metastatic disease at presentation were excluded. Clinical information, including tumor histology, grade and stage, and extent of surgery and radiation therapy, were determined. Kaplan-Meier survival and Cox proportional hazards analyses were conducted to determine the influence of these factors on overall survival. RESULTS: The study sample was composed of 650 patients with maxillary sinus cancer (mean age, 64 years; male-to-female ratio, 3:2). The most common histology was squamous cell carcinoma (61.7%), followed by adenoid cystic carcinoma (9.8%). The overall mean (median) survival was 52 months (25 months), and 77.5% and 7.4% of patients presented with advanced (T3/T4) disease or cervical metastasis, respectively. Radiation therapy was administered in 441 patients (67.9%) and significantly improved survival mainly for those with T4 lesions. On multivariate analysis, increasing age, T stage, N stage, and tumor grade independently predicted poorer survival, whereas gender did not. Adenoid cystic carcinoma exhibited a significantly improved overall survival (P <.001). CONCLUSIONS: Survival for patients with maxillary sinus cancer is determined not only by TNM staging but also by tumor histology and grade. TNM staging effectively stratifies patients according to survival. Radiation therapy significantly improves survival for those with T4 lesions.