A case of fetal macrocystic CPAM in which associated hydrops did not resolve despite fetal therapy resulting in worsened mirror syndrome and early neonatal death

IZUMI Ririko, HIDAKA Nobuhiro, KIDO Saki, KAI Shotaro, NAKANO Takahiro, HACHISUGA Masahiro, KATO Kiyoko

doi:10.3179/jjmu.jjmu.a.126

A 39-year-old mother was referred to us at 20 gestational weeks owing to a large fetal left lung multilocular mass (macrocystic CPAM) and minimal ascites. The overall size of the mass lesion corresponded to a CPAM volume ratio (CVR) of 1.61. Placentomegaly was also noted. On admission at 22 weeks’ gestation, the fetus exhibited skin edema, and hydrops fetalis was diagnosed. The patient demonstrated fairly stable vital signs, but complained of emesis and vomiting that had developed over the last several days. Upon physical examination, she exhibited edema of the face and legs. Moreover, the patient’s laboratory values showed anemia, low platelet counts, and hypoproteinemia. The serum β-hCG concentration was 178,077 mIU/ml. Based on the above results, mirror syndrome was diagnosed. The mother underwent thoracoamniotic shunting at 22⁺⁵ gestational weeks, with successful drainage of the largest cyst. However, a fair-sized mass composed of multiple small cysts persisted, and the CVR was calculated as 1.20. Fetal pleural skin edema became somewhat less severe, but neither fetal ascites nor placentomegaly improved. At 23⁺⁵ gestational weeks, the mother complained of breathing difficulties. Chest X-ray demonstrated bilateral pleural effusion and cardiomegaly. Maternal hypoproteinemia and edema had gradually worsened, and the patient showed a decrease in urinary volume. We determined that continuation of the pregnancy was not ideal, and induced labor under administration of albumin and diuretics. At 24⁺² gestational weeks, a female infant weighing 860 g was delivered and died immediately after birth. The edematous placenta weighed 725 g. After delivery, the mother’s condition improved. Based on this experience, we have re-affirmed that successful drainage of the largest cyst does not necessarily improve the hydropic state of fetuses with multilocular macrocystic CPAM. The fetal and maternal condition should both be treated with caution via physical examination, hematological examination, and chest radiography.

A case of fetal macrocystic CPAM in which associated hydrops did not resolve despite fetal therapy resulting in worsened mirror syndrome and early neonatal death

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