Chronic Myeloid Leukemia Presenting with Visual and Auditory Impairment in an Adolescent: An Insight to Management Strategies

A 15-year-old girl presented with progressive deterioration in vision and hearing over 1 week. A huge spleen was palpated below the left costal margin laying down to inguinal region. Blood count showed hyperleukocytosis with a white blood cell count of 455 × 109/l. Peripheral smear yielded myeloid precursor cells with basophilia. Bone marrow aspiration revealed a blast count of 5% morphologically and 4% by flow cytometry. Fundoscopic examination revealed bilateral retinal exudates, edema and hemorhages. Partial sensorioneural hearing loss was also detected on the right ear. The diagnosis of chronic myeloid leukemia was confirmed by positive t(9;22) by RT-PCR. After commencing on hydroxyurea and intrathecal methotrexate-prednisolone, progressive improvement in hearing and vision was obtained. In our brief report, we aimed to emphasize rare presentation with visual and hearing impairment of chronic myeloid leukemia during childhood, especially in “chronic phase”

Surgical Repair Of A Sternal Cleft Malformation

Sternal cleft is a unique congenital chest wall malformation that results from failure of sternal fusion early in the embryological development. Surgical correction is advised both to protect mediastinal structures and to restore respiratory dynamics. Early surgical correction, preferably in the neonatal period, is recommended in order to benefit from the elasticity of the thoracic cage. In this article, we present a two-month-old female patient with a superior V-shaped sternal cleft, which was successfully corrected with posterior periosteal flap, sliding chondroplasty and cartilage graft interposition techniques.PubMedWo

Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children

Background  Pulmonary Langerhans's cell histiocytosis (PLCH) is a rare cause of interstitial lung disease in children and more than half of the cases are bilateral. Persistent respiratory distress due to spontaneous pneumothorax (SP) in bilateral PLCH may refractory to conservative treatment and posed a great challenge to surgical modalities. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children. , Case Report  The patient was admitted to the emergency department with severe respiratory distress and SP. After chest tube insertion, biopsy from neck mass revealed Langerhans's cell histiocytosis. Chemotherapy including vinblastine and prednisone was initiated. Due to persistent respiratory difficulty and air leaks, talc pleurodesis and thoracoscopic bullae excision with pleural decortication were performed. Two months after the admission, due to nosocomial infection and severe respiratory distress, extracorporeal membranous oxygenation (ECMO) support was initiated. The patient was died of ECMO complications on 24th day of ECMO. , Conclusion  Despite the use of chemotherapy and surgical excision of cystic lesions, bilateral PLCH in children may have lethal outcome. Other treatment options including respiratory support with ECMO and lung transplantation should be considered as last resort of treatment alternative in persistent cases.PubMedWo