A Case of Isolated Interhemisphere Fusion of Frontal Lobe with Intact Septum Pellucidum : A Variant of Holoprosencephaly / 대한소아신경학회지

Holoprosencephaly is a rare CNS developmental defect with midline cleavage of embryonal forebrain during the fourth week of fetal development characterized with s single ventricle. Holoprosencephaly is divided into three types by the degree of the brain cleavage : alobar, semilobar, and lobar. Depending on the degree of defected regions, there exist variable clinical symptoms and signs such as stillbirth, hypotonia, apnea, convulsion, delayed development and visual disturbance. It was frequently associated with the deformity of the brain as well as the midline facial structures. Advances in neuroimaging over the past decades have led us to a better understanding of the pathogenesis and the variability of holoprosencephaly. As a result, a lot of cases of holoprosencephaly with various clinical and pathologic findings have been reported. We experienced a case of isolated interhemisphere fusion of frontal lobes with intact septum pellucidum in a child with febrile status epilepticus. Is it a variant of holoprosencephaly?

A Case of Congenital Cystic Adenomatoid Malformation of the Lung in the Patient with Recurrent Pneumonia (Mixed type I and II) / 소아알레르기및호흡기학회지

Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon embryonic developmental anomaly, characterized by the presence of one or multiple interconnecting cysts. This disease may present various clinical aspects, stillborn or perinatal death, respiratory distress in newborns, and acute and chronic pulmonary infections in older infant and children. We experienced a case of CCAM in a 3-years, 8-month-old male who had frequent upper respiratory tract infection and recurrent pneumonia. Chest X-ray and chest CT showed a cavitary lesion in the right middle lobe. Histologic examination revealed numerous small and large sized cysts in the parenchyma. The large cysts were mainly lined by pseudostratified ciliated cells and the small cysts are lined by simple cuboidal cells and subclassified Stocker type mixed I and II. Wedge resection was done with satisfactory postoperative courses clinically and radiologically. We report a case of CCAM (I and II mixed type) with a brief review.