Congenital Cystic Adenomatoid Malformation: A Case Report

Abstract

Introduction: Congenital Pulmonary Airway Malformation (CPAM), known as Congenital Cystic Adenomatoid Malformation (CCAM) is a rare abnormality with prevalence of 1: 25000-35000. The lung lesion appears prenatal as a cyst in the chest and made up of nonfunctional lung tissue that continues to grow. After retrieving publication/database, to our knowledge, this is the first case of CPAM in Indonesia. Diagnosis of CCAM is either pre/post natal, using ultrasonography of pregnant mother or MRI. The neonatal outcome depends on factors: hydropsfetalis, microcystic and lesion size. Mortality rate of baby ranges from 9-49%. CCAM complication: recurrent infections, pneumothorax and malignancy. Definitive therapy is surgical excision.Case report:A-23 month old male baby diagnosed with CCAM during prenatal period. The mother was in 17 weeks pregnancy, underwent ultrasonography and revealed CCAM. The abnormality was on lower right lung. Mediastinal shift developed during 20week of gestation and required intrauterine thoracentesis. The baby suffered from hydropsfetalis. Thoracentesis performed three times: 20, 22, 26 week gestation. The outcome of treatment was good, the hydrops fetalis improved. However, the baby was born pretermly in 35week and underwent NICU for 50 days. Throughout the period, twice surgery were performed and pathology confirmed CCAM. Both surgeries were not performed as lobectomy but cyst elimination. Post natal complication: recurrent lung infections and pneumothorax. Surgical excision lobectomy was finally performed when 23month old. The baby is now healthy and no lung infection or pneumothorax reported during last 3 months.Discussion: CCAM is a congenital lung abnormality and the diagnosed was made since intrauterine period. Prenatal care will be more specific to take care of the development of the cyst, its progression, complications, etc. In this case, worsening of the cyst led to fetal cardiological emergency. Fetal bradycardia reported once prenatal care visits. Intrauterine thoracentesis were performed. Outcome of treatment was good, also for hydropsfetalis improvement. In CCAM, hydropsfetalis correlate to the worse outcome and higher mortality rate. In this case, hydropsfetalis was cured. Surgical excision is stated to be the definitive therapy for CCAM but unfortunately the surgery lobectomy was not treatment chosen in this case. Instead, the first surgeries performed just to cut the cysts. Lung infections happened several times. This can be due to the remaining dead space of the affected lung that is pro-for bacterial growth.Conclusion:Prenatal diagnose of CCAM is important, it might need invasive treatment. Hydropsfetalis not always ends up with mortality. Surgical excision is the best treatment.

Ultrasound Differential Diagnosis and Prognosis of Right Main Bronchus Atresia in Fetus: Clinical Case

“…Частота случаев секвестрации составляет от 0,15 до 1,8% в общей популяции [11]. Интралобарный тип порока обнаруживается в 3 раза чаще, чем экстралобарный [11,12]. Интралобарная секвестрация легкого обычно встречается в задней базальной области, чаще в левой нижней доле легкого, экстралобарный тип -рядом со средостением или диафрагмой [11].…”

“…Интралобарная секвестрация легкого обычно встречается в задней базальной области, чаще в левой нижней доле легкого, экстралобарный тип -рядом со средостением или диафрагмой [11]. Кровоснабжение интралобарного легочного секвестра происходит от нисходящей аорты, венозный отток -через легочные вены [11,12]. Возможно сочетание интралобарной секвестрации с КАПРЛ [11].…”