Račaitė Julita, Šumkovskaja Alina, Arlauskienė Audronė, Pilypienė Ingrida, Landsbergytė-Bukauskienė Elena
Vilnius University, Faculty of Medicine, Vilnius, Lithuania.
Clinic of Obstetrics and Gynaecology, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius, Lithuania.
Acta Med Litu. 2018;25(2):95-100. doi: 10.6001/actamedica.v25i2.3762.
A congenital cystic adenomatoid malformation (CCAM) is a foetal pulmonary development abnormality caused by airway dysgenesis that is characterized by cystic or adenomatous lesions in the terminal bronchioles. The size of the mass, the degree of the mediastinal shift, and the presence of hydrops and polyhydramnios can all affect the severity of a case. Treatment can be initiated at early stages by applying prenatal and postnatal methods. Because CCAM is a rare pathology that is often only accidentally diagnosed during routine ultrasounds, we would like to share our case report to enrich the literature on this pathology and to present a case successfully treated at our hospital.
A patient with her first multiple pregnancy was seen for prenatal care and her first ultrasound at 17 weeks of gestation. One of the twins was diagnosed with a congenital cystic adenomatoid malformation of the left lung. At 20 weeks of gestation, an enlarged left lung with small cysts, a compressed right lung, a compressed and displaced heart, and oligohydramnios were observed. At 28 weeks of gestation, a fetoplacental circulation disorder appeared. At 32 weeks of gestation, due the unstable condition of the affected foetus, the twins were delivered via a C-section. The treatment of the newborn included antibiotics, caffeine citrate, and breathing therapy.
CCAM are often diagnosed by accident when performing routine pregnancy ultrasound examinations. CT is the most reliable X-ray-based examination method for confirming a diagnosis. When CCAM is suspected in the foetus, amniocentesis and cariotype identification are performed, but chromosomal anomalies related to CCAM are often not identified. Currently, the best treatment results have been achieved by applying combined prenatal therapy and early surgical treatment.
先天性囊性腺瘤样畸形（CCAM）是一种由气道发育异常引起的胎儿肺部发育异常，其特征是终末细支气管出现囊性或腺瘤样病变。肿块大小、纵隔移位程度、是否存在水肿及羊水过多均会影响病情严重程度。可通过产前和产后方法在早期进行治疗。由于CCAM是一种罕见的病理情况，常在常规超声检查时偶然发现，我们希望分享我们的病例报告，以丰富关于该病理情况的文献，并展示我院成功治疗的一例病例。
一名首次怀孕的孕妇在妊娠17周时前来进行产前检查并接受首次超声检查。双胞胎中的一个被诊断为左肺先天性囊性腺瘤样畸形。妊娠20周时，观察到左肺增大伴小囊肿、右肺受压、心脏受压并移位以及羊水过少。妊娠28周时，出现胎儿-胎盘循环障碍。妊娠32周时，由于受影响胎儿情况不稳定，双胞胎通过剖宫产分娩。新生儿的治疗包括使用抗生素、枸橼酸咖啡因和呼吸治疗。
CCAM常在进行常规妊娠超声检查时偶然被诊断出来。CT是基于X射线的最可靠的确诊检查方法。当怀疑胎儿患有CCAM时，会进行羊水穿刺和核型鉴定，但与CCAM相关的染色体异常往往无法识别。目前，通过联合产前治疗和早期手术治疗取得了最佳治疗效果。
