Congenital Cystic Adenomatoid Malformation (CCAM) Type II: A Rare Case of Sudden Infant Death

Abstract

Congenital cystic adenomatoid malformation (CCAM) is a developmental lesion of the lungs and terminal respiratory structures, which is characterized by pseudocysts, lesions, and cystically dilated airways. CCAM is also known as congenital pulmonary airway malformation (CPAM). Various classification systems for CCAM have been described, and based on a recent classification, CCAMs are classified morphologically into five different types (Type 0, I, II, III, and IV) based on lesion sizes. The most common manifestation of CCAM in neonates and children is respiratory distress (RD). Spontaneous pneumothorax is a rare manifestation of CCAM. In this case report, we discuss a CCAM type-II case of a 38-day-old female infant with a radiological post-mortem diagnosis of a large left-side spontaneous pneumothorax. The gross examination of the lungs revealed multiple emphysematous air bubbles up to 0.5 cm in diameter, and the histological examination revealed focal pleural fibrosis, accompanied by thickened septa and atelectasis. In this scenario, the routine use of prenatal ultrasonography would be important to obtain a timely prenatal diagnosis. At the same time, improvements in surgical techniques, as well as greatly improved imaging techniques, have improved the outcome of these patients. Finally, it is important to remark on the importance of autopsy in the case of sudden infant death with a suspected CCAM.

Two-stage surgical management of horseshoe lung associated with congenital pulmonary airway malformation in a neonate: A case report

“…Horseshoe lung has been described in association with Scimitar syndrome (Table 1), making the presence of CPAM associated with horseshoe lung even rarer and challenging. [3,5,6,7] Horseshoe lung and CPAM, both, can affect respiratory system physiology, ranging from asymptomatic patients to varying degrees of respiratory distress, spontaneous pneumothorax, and recurrent infections, depending on the degree of lung involvement and location of the malformation. [2,8] Diagnosis is typically made using imaging methods such as prenatal ultrasound, postnatal X-rays, computed tomography (CT), or magnetic resonance imaging (MRI) [2], as well as pulmonary function tests and histopathological reports following surgery.…”