Mode of delivery and risk of intracranial haemorrhage in newborns with severe haemophilia A: a multicentre study in Gulf region.

INTRODUCTION: The optimum mode of delivery in a known carrier of a haemophilia A is still an issue of debate. AIM: This study was conducted to report a multicentre experience in Gulf Cooperation Council (GCC) on the incidence of intracranial haemorrhage (ICH) in newborns with severe haemophilia A delivered by different modalities. METHODS: We have conducted a retrospective/prospective multicentre cohort study including a total of seven hospitals distributed in four GCC countries between 1998 and Jan 2015. A total of 163 patient with severe haemophilia A (factor VIII <1%) were enrolled in this study, age ranged between 2 weeks to 18 years. RESULTS: Most of the patients were born by spontaneous vaginal delivery (SVD) (131, 80.4%), whereas 26 patients (16%) were born by CS and only six patients were born by instrumental delivery (3.7%), five of them by vacuum and one was delivered using forceps. Five out of 163 patients developed ICH during the first 2 weeks of life (3.1%). Two of them were born by SVD (2/131; 1.5%) and two were born by instrumental delivery (2/6; 33.3%). Only one patient among those who were born by caesarean section developed ICH (1/26; 3.8%). Assisted vaginal delivery was associated with a significant risk of ICH, in comparison to SVD and CS (P = 0.0093). CONCLUSION: Normal vaginal delivery is still considered a safe journey through the birth canal for haemophilic newborns particularly in this area of the world. Larger prospective studies might be needed to define an evidence-based optimal mode of delivery for the haemophilia carrier expecting an affected child.

Pattern of liver diseases in Oman.

UNLABELLED: Arab Gulf countries including Oman have a high rate of consanguineous marriage with an associated increased frequency of a variety of genetic disorders including liver diseases. AIM: To describe the pattern of chronic liver diseases at The Royal Hospital, Muscat during the period January 2005 to January 2007. METHODS: A retrospective study of all children with chronic liver diseases seen at the Royal Hospital, which is a tertiary referral centre for gastro-enterology and hepatology for the whole of Oman. RESULTS: Seventy-nine patients with chronic liver disease were identified. The consanguinity rate amongst parents was 78%. The mean (SD) age of patients at the time of final diagnosis was 5 years (4). The two most common liver disorders were progressive familial intrahepatic cholestasis (30%) and fibrocystic disease of liver and kidney (21%). One-third of the patients came from the Sharqiya region. Complications included growth retardation (75%), cirrhosis (26%) and portal hypertension (39%). Three children died and three others received living related liver transplant. CONCLUSION: About half of the chronic liver disorders in Oman are hereditary in origin. At least three children per year will require liver transplant.

Surgical management of retroperitoneal sarcomas associated with external and intraoperative electron beam radiotherapy.

AIMS: To report outcomes of adults with retroperitoneal sarcoma (RS) treated by surgery, external beam radiotherapy (EBRT) and intraoperative electron beam radiotherapy (IORT). METHODS: From July 1988 to February 2001; 24 patients with primary and recurrent RS were diagnosed and treated. The median dose and energy of IORT delivered was 15 Gy/9meV. EBRT dose varies between 45-50 Gy. RESULTS: There were five primary and 19 recurrent tumours. One primary and five recurrent tumours underwent R0 resection. There were 12 liposarcomas and 19 grade I tumours; 13 patients developed local recurrence and three developed distant metastases.Twenty-two patients received IORT associated with EBRT: 11 developed recurrences. Six patients developed Neurotoxicity (4 grade II and 2 grade III). Disease free survival and overall survival at 5 years was 28 and 56% respectively. CONCLUSIONS: EBRT with IORT treatment is a promising technique for local control. Lower recurrence rates are associated with radical (R0) surgical procedures.

Sentinel lymph node biopsy in the Sultanate of Oman.

OBJECTIVE: To establish the feasibility of newly started sentinel lymph node (SLN) biopsy in our center at the Royal Hospital in the Sultanate of Oman. METHODS: Twenty five patients with Breast cancer and melanoma were staged with sentinel lymph node (SLN) biopsy, followed by Axillary lymph Node Dissection (ALND). Axillary SLN were detected by injection of blue dye alone or with radioisotope. Intraoperatively, a gamma probe detector identified the isotope-labeled SLN. Two patients underwent preoperative lymphoscintigraphy. The SLNs were examined histologically by hematoxylin-eosin staining and, if negative by this method an immunohistochemical staining (IHC) was done. RESULTS: The only failure to detect inguinal SLN was one case after neoadjuvant chemotherapy. Total detection rate of SLN was of 96%. Metastatic spread occurred in 11 patients of these 9 had other lymph node (LN) involved. Nine patients had no SLN involvement and no other LNs involved. CONCLUSIONS: Ethically, according to the international recommendations and looking to the statistics of the Sultanate all clinical NO should have the choise of SLN biopsy prior to ALND.

Hook wire localization procedure in biopsy and diagnosis of early breast cancers: Oman experience.

INTRODUCTION: Between January 2000 and December 2005, a total of 30 cases of impalpable suspicious breast lesions (microcalcifications or impalpable lumps classified as BRADS IV and above) were biopsied after hook wire localization. This is a retrospective review of these cases. RESULTS: One third of the suspicious lesions were malignant or pre-malignant. All these had further oncological treatment and follow up. CONCLUSIONS: Hook wire localization biopsy remains an important tool for the diagnosis of impalpable lesions of the breast. The incidence of malignancy in our series was similar to the published international levels. Our series is small, so there is a need to review the data with bigger number.