Open Access Maced J Med Sci. 2022 Feb 23; 10(C):123-125. 123 Scientifc Foundation SPIROSKI, Skopje, Republic of Macedonia Open Access Macedonian Journal of Medical Sciences. 2022 Feb 25; 10(C):123-125. https://doi.org/10.3889/oamjms.2022.8539 eISSN: 1857-9655 Category: C - Case Reports Section: Case Report in Pediatrics Congenital Cystic Adenomatoid Malformations Type 1: Case Report Andi Dwi Bahagia Febriani 1,2 * , Nurul Sylvana Shoraya 1 , Ema Alasiry 1,2 1 Departement of Pediatrics, Faculty of Medicine, Hasanuddin University, Makassar, Indonesia; 2 Dr. Wahidin Sudirohusodo General Hospital, Makassar, Indonesia Abstract BACKGROUND: Congenital cystic adenomatoid malformation (CCAM) is a group of multicystic and non-cystic masses due to abnormal lung development. The incidence ranges from 1: 10,000 to 1: 35,000 pregnancies. CASE REPORT: We report a case of a 3-day male baby presented with worsening respiratory distress, X-ray and chest CT were suggestive of CCAM, and sepsis. CONCLUSION: The current case adds to the collective clinical and radiological knowledge of this rare congenital lung disorder. Edited by: Ksenija Bogoeva-Kostovska Citation: Febriani ADB, Shoraya NS, Alasiry E. Congenital Cystic Adenomatoid Malformations Type 1: Case Report. Open Access Maced J Med Sci. 2022 Feb 25; 10(C):123-125. https://doi.org/10.3889/oamjms.2022.8539 Keywords: Congenital cystic adenomatoid malformations; Respiratory distress; Lung malformation *Correspondence: Andi Dwi Bahagia Febriani, Departement of Pediatrics, Faculty of Medicine, Hasanuddin University, Dr. Wahidin Sudirohusodo Hospital, Perintis Kemerdekaan Km. 10, Tamalanrea, Makassar, Indonesia. E-mail: bahagiadwi@med.unhas.ac.id Received: 05-Jan-2022 Revised: 10-Feb-2022 Accepted: 15-Feb-2022 Copyright: © 2022 Andi Dwi Bahagia Febriani, Nurul Sylvana Shoraya, Ema Alasiry Funding: This research did not receive any fnancial support Competing Interests: The authors have declared that no competing interests exist Open Access: This is an open-access article distributed under the terms of the Creative Commons Attribution- NonCommercial 4.0 International License (CC BY-NC 4.0) Introduction Congenital cystic adenomatoid malformation (CCAM) is a congenital abnormality of lung development caused by an overgrowth of abnormal lung tissue that forms cysts, afecting ≥1 lung lobes [1]. CCAM originates from embryological abnormalities of terminal bronchus that occur before 35 weeks of gestation. Nearly 25% of CCAM cases are accompanied by other congenital abnormalities [2]. About 7% of cases show symptoms of severe respiratory distress and 96% can be born alive [3] Patients with small lesions are usually asymptomatic until they reach middle age when episodes of recurrent airway infections or chest pain occur [4]. CCAM can be diagnosed antenatally by ultrasonography and postnatally by chest CT-scan or MRI [2]. Case Report A 3-day male baby presented with dyspnea was admitted to the NICU. The baby was delivered by Caesarean section at full term, spontaneously breath, amniotic fuid was clear and odorless. The APGAR scores were 7 and 9 at one and fve minutes. The mother’s prenatal history was unremarkable, but prenatal ultrasound showed the presence of fuid in the fetal lung. The baby showed respiratory distress with decreased breath sound at the right hemithorax. Based on clinical features and laboratory investigations, he was diagnosed with unproven sepsis with increased level of procalcitonin and CRP [5]. The chest X-ray showed a thick-walled multicavity at the left lung and the heart appeared to be pushed to the right (Figure 1). The chest CT showed multiseptate cysts, thin-walled, with the largest size of 3.0 × 4.4 × 3.8 cm almost over the left lung lobes (Figure 2). The baby was on bubble n-CPAP, intravenous fuids, ampicillin, and gentamicin. On the 5 th day, the antibiotics were replaced with ceftazidime and amikacin because of no clinical improvement. On the 13 th day, the respiratory distress worsened, he was intubated and put on a ventilator, and antibiotics were replaced with meropenem. On the 19 th day, he fell in shock. Blood gas analysis revealed a respiratory acidosis. Fluid resuscitation and inotropic medication did not improve the condition, patient passed away on the 20 th day of treatment.