RESUMO
Congenital cystic adenomatoid malformation (CCAM) occurs secondary to the cystic adenomatous over-growth of terminal bronchioles, which results in the secondary inhibition of alveolar growth. In most of the cases, respiratory distress is the presenting feature during the neonatal period. In about 90% of patients, recurrent respiratory infections necessitating chest imaging reveal CCAM before the age of two years. We describe here the occurrence of congenital cystic adenomatoid malformation of right lung in a 12-year-old girl presenting with haemoptysis and hypovolaemic shock. She underwent right middle lobectomy; and histopathological examination confirmed the diagnosis. She has been doing well on follow-up.
Assuntos
Malformação Adenomatoide Cística Congênita do Pulmão/complicações , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Hemoptise/etiologia , Choque/etiologia , Criança , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Feminino , Humanos , Tomografia Computadorizada por Raios XRESUMO
Ethmocephaly is the rarest form of holoprosencephaly, which occurs due to an incomplete cleavage of the forebrain. Clinically, the disease presents with a proboscis, hypotelorism, microphthalmos and malformed ears. Amniotic band syndrome is another rare congenital malformation with ring-like constriction bands in the limbs, head, face or trunk. We present a case of ethmocephaly with amniotic band syndrome, which is likely the first of its kind, published in the literature.
Assuntos
Anormalidades Múltiplas , Síndrome de Bandas Amnióticas/diagnóstico , Holoprosencefalia/diagnóstico , Tronco/anormalidades , Evolução Fatal , Humanos , Recém-Nascido , Masculino , Tomografia Computadorizada por Raios XRESUMO
Amniotic band syndrome manifests at birth with a variety of malformations ranging from constriction ring to defects incompatible to life, in various parts of the body. Although some theories have been proposed for the development of this syndrome, the exact cause remains unknown. The median facial cleft is an extremely rare manifestation of amniotic band syndrome with a relative paucity of reports available in the literature. Here, we report one such case.
RESUMO
Primary cardiac tumours are mostly benign and rare in paediatric practice. Here, a male baby was presented with history of birth asphyxia. On examination he was found to have a systolic murmur and was initially thought to be either a case of ventricular septal defect or tricuspid regurgitation due to asphyxial cardiomyopathy. Echocardiography revealed normal LV systolic function, intact interatrial septum and interventicular septum without intracardiac shunt and normal cardiac valve morphology. There was a multilobulated solitary intracardiac mass with a narrow stalk arising from RV free wall. Thus the diagnosis of cardiac tumour was clinched.
