Abstract:The baby with Beckwith Wiedemann Syndrome (BWS) usualy presents with exomphalos, macroglossia and gigantism. In addition, some children with BWS have other findings including: nevus flammeus, prominent occiput, midface hypoplasia, hemihypertrophy, genitourinary anomalies (enlarged kidneys), cardiac anomalies, musculoskeletal abnormalities, and hearing loss. Incidence of one in 13,700 live birth. As children with BWS are at increased risk of childhood cancer, they should follow up strictly for cancer screening. We are reporting this type of a case who has got Exomphalos, Macroglossia, Gigantism and Hemihypertrophy. We have corrected the exomphalos and advised him for follow up for cancer screening.

Abstract:Hemangioma is one of the most common benign tumors of infancy and occurs in approximately 5-10% of infants. The treatment options for problematic haemangioma are intralesional and systemic steroids, chemotherapeutic agents including vincristine and interferon-alpha, laser therapy or surgical intervention. In this case series effectiveness of propranolon was observed on haemangioma. This study was conducted in the Department of Pediatric Surgery, Faridpur Medical College Hospital, Faridpur and Shahid Ziaur Rahman Medical College, Bogra. This study period was from January 2013 to December 2015. A total of 38 patients of haemangioma were admitted in Pediatric Surgery ward in FMCH and 26 patients in SZRMCH. These 54 patients were counseled about the study in detail. First dose of propranolol was given at a dose of 3 mg/kg/day in three divided doses. Pulse and blood pressure were recorded during initial 3 hours. Patients were then advised to continue with this treatment at home, with monthly follow-up. Total number of patients were 54. Of them 11 were male and 43 were female, giving a male: female ratio of 1:4. 60 % of the patients (32) were in <6 months. After 1 month, sign of regression was marked in all cases. Complete regression was seen in 6 patients (11.1 %) during second follow-up after 3 months of treatment. Only 4 patient required 9 months to reach the complete recovery. This case series contributes to the growing evidence that oral propranolol is efficacious and safe, with a careful dosing and monitoring. We hope a national guidelines will be developed in time for use of propranolol to treat the haemangioma.