Ultrasound-guided Intralesional Bleomycin Injection (IBI) for Treatment of Cutaneous Hemangiomas and Vascular Malformations

Purpose: To report the therapeutic outcome of ultrasound-guided intralesional injection of bleomycin in the treatment of cutaneous hemangiomas and vascular malformations. Material & Methods: The medical records of patients with cutaneous hemangiomas and vascular malformations treated with the intralesional injection of bleomycin under ultrasound guidance between August 2009 and June 2013 at the Indus Hospital, Karachi were reviewed retrospectively using a computerized medical record information management system. Data were extracted using a pre-coded performa that included patient demographics, type and location of lesion, number of treatments, presenting/pre- and post-treatment clinical symptoms (pain, swelling, heaviness, size, discoloration), ultrasound appearance and vascularity, and post-treatment side effects. The dose range of bleomycin was 0.5-1.0 mg/kg, but not exceeding 15 mg in a single session. A maximum of four treatments were given in any given patient except for one, who presented with recurrence after a year of complete resolution. Therapeutic outcome was determined using review of ultrasound images and recorded clinical assessment. Treatment response was categorized as: (i) complete resolution [more than 90% reduction]; (ii) substantial reduction [more than 50% reduction]; (iii) mild reduction [25% reduction]; or, (iv) no improvement [ \u3c 10% reduction]. Results: A total of 30 patients (16 female, 14 male), ranging in age from 8 months to 48 years (mean age 10.2 years), were treated from 2009 to 2013. There were 23 hemangiomas. Seven were vascular malformations, of which five were lymphatic malformations and two were venous malformations. Twenty-eight lesions were located in the head and neck region, and two were peripheral.. In 24 of the 30 patients (76%), treatment had been completed. In six patients (21%) treatment was ongoing at the time of this report. Seventeen of the 23 hemangiomas (74%) were completely resolved clinically and on ultrasound, five (22%) showed substantial improvement and one (4%) showed mild improvement. In five of the seven vascular malformations (71%) lymphatic malformations resolved completely, and two (29%) venous malformations showed substantial improvement. Of the 13 patients presenting with discoloration, there was complete resolution in one (7.7%), marked reduction in 11 (84.6%) and mild reduction in one (7.7%). Of seven patients presenting with pain, there was complete resolution in two (28.6%), marked reduction in two (28.6%), mild reduction in two (28.6%), and no improvement in one (14.3%). There were no pulmonary complications. Conclusion: Ultrasound-guided intralesional injection of bleomycin is an option to consider for the treatment of certain types of cutaneous hemangiomas and vascular malformations. Prospective studies should be undertaken to understand the various factors contributing to therapeutic success

Distribution, Severity and Radiologic Features of Intracranial Stenosis in Asymptomatic Pakistanis.

Background: Intracranial atherosclerotic disease (ICAD) is the most common ischemic stroke subtype globally. It accounts for 30–50% of all ischemic strokes in Asians. Aims—The aim of the study is to report the frequency of asymptomatic ICAD and its associated Magnetic Resonance Imaging (MRI) findings. Methods: 200 adult participants were recruited from the Radiology Departments of two major diagnostic centers in Karachi. Eligible participants were confirmed for the absence of stroke symptoms via the Questionnaire for Verifying Stroke Free Status (QVSFS). QVSFS negative subjects underwent MRI on a 1.5 Tesla scanner. Images were centrally reviewed on Di com Viewer 3.0 with electronic calipers to calculate the degree of ICAD. Results: Mean age of subjects was 37.1 years (S.D 15.1) with 50.5% men (n=101) and 49.5% women (n=99). Asymptomatic ICAD was found in 34.5% (n=69) subjects. Of the 3800 intracranial arteries studied, 2.2% (n=88) had biological disease. 20.5% (n=18) of these vessels had atherosclerotic irregularities, 43.2% (n=38) had mild stenos is, 11.4% (n=10) had moderate stenos is, 5.7% (n=5) had severe stenos is while 19.3% (n=17) were completely occluded. The posterior cerebral artery (42% of stenosed arteries, n=37) was most affected. 23.5% (n=47) of subjects had peri-ventricular lucencies, 10.5% (n=21) had brain atrophy while 3.5% (n=7) had silent brain infarcts. There was a significant association between asymptomatic ICAD and peri ventricular lucencies (PR 1.59; 95% CI 1.35–1.99) Conclusion: Asymptomatic ICAD is common in young Pakistanis, with no gender predilection; it preferentially affects the posterior circulation. Silent infarcts are rare compared to peri ventricular lucencies and atrophy

Adenocarcinoma of the appendix presenting as bilateral ureteric obstruction

<p>Abstract</p> <p>Background</p> <p>Adenocarcinoma of the vermiform appendix is a rare neoplasm of the gastrointestinal tract. Presentation mimics acute appendicitis, but right iliac fossa mass and intestinal obstruction have also been reported. These presentations reflect various stages of a locally expanding tumour causing luminal obstruction of appendix. The investigation and subsequent management with a review of the literature is presented.</p> <p>Case presentation</p> <p>We report a case of appendicular adenocarcinoma found unexpectedly in a 43 year old male who presented with urinary symptoms. Cystoscopy and uretero-renoscopy showed normal bladder but external compression of the ureters and therefore bilateral stents were inserted. CT scan showed a caecal mass. After colonoscopy, that showed external compression, and diagnostic laparoscopy the patient underwent right hemicolectomy. Histopathology revealed well differentiated adenocarcinoma with signet ring morphology with multiple lymph node involvement. The patient was referred for chemotherapy where he received infusional 5 fluorouracil but died 7 months after surgery.</p> <p>Conclusion</p> <p>Patients with atypical manifestations related to right lower abdominal quadrant should be thoroughly investigated with an open mind. Every attempt should be made to make a precise diagnosis through all the available means to direct the treatment along correct lines.</p

Mutational landscape of head and neck squamous cell carcinomas in a South Asian population

Head and neck squamous cell carcinoma (HNSCC) is the sixth most common cancer type globally and contributes significantly to burden of disease in South Asia. In Pakistan, HNSCC is anmong the most commonly diagnosed cancer in males and females. The increasing regional burden of HNSCC along with a unique set of risk factors merited a deeper investigation of the disease at the genomic level. Whole exome sequencing of HNSCC samples and matched normal genomic DNA (n=7) was performed. Significant somatic single nucleotide variants (SNVs) were identified and pathway analysis performed to determine frequently affected signaling pathways. We identified significant, novel recurrent mutations in ASNS (asparagine synthetase) that may affect substrate binding, and variants in driver genes including TP53, PIK3CA, FGFR2, ARID2, MLL3, MYC and ALK. Using the IntOGen platform, we identified MAP kinase, cell cycle, actin cytoskeleton regulation, PI3K-Akt signaling and other pathways in cancer as affected in the samples. This data is the first of its kind from the Pakistani population. The results of this study can guide a better mechanistic understanding of HNSCC in the population, ultimately contributing new, rational therapeutic targets for the treatment of the disease

Environmental enteropathy in undernourished Pakistani children: Clinical and histomorphometric analyses

Despite nutrition interventions, stunting thought to be secondary to underlying environmental enteropathy (EE) remains pervasive among infants residing in resource-poor countries and remains poorly characterized. From a birth cohort of 380 children, 65 malnourished infants received 12 weeks of nutritional supplementation with ready-to-use therapeutic food (RUTF). Eleven children with insufficient response to RUTF underwent upper endoscopy with duodenal biopsies, which were compared with U.S., age-matched specimens for healthy, celiac disease, non-celiac villous atrophy, non-celiac intraepithelial lymphocytosis, and graft-versus-host disease patients. Of the 11 children biopsied, EE was found in 10 (91%) with one subject with celiac disease. Morphometry demonstrated decreased villus-to-crypt (V:C) ratios in EE relative to healthy and non-celiac lymphocytosis patients. Environmental enteropathy villus volumes were significantly decreased relative to healthy controls. In EE, average CD3+ cells per 100 epithelial cells and per 1,000 µm2 of lamina propria and the number of lamina propria CD20+ B-cell aggregates were increased relative to all other groups. Our results indicate that V:C ratios are reduced in EE but are less severe than in celiac disease. Environmental enteropathy intraepithelial and lamina propria T lymphocytosis is of greater magnitude than that in celiac disease. The increases in lamina propria B and T lymphocytes suggest that non-cytolytic lymphocytic activation may be a more prominent feature of EE relative to celiac disease. These results provide new insights into shared yet distinct histological and immunological features of EE and celiac disease in children

Chylous ascites following radical nephrectomy: a case report

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