Kinoshita Yuya, Sakamoto Rieko, Hattori Yusuke, Furuie Keishiro, Kuraoka Shohei, Hidaka Yuko, Tamura Hiroshi, Mitsubuchi Hiroshi, Nakamura Kimitoshi
Department of Pediatrics, Graduate School of Medicine Sciences, Kumamoto University, Kumamoto, Japan.
Case Rep Pediatr. 2023 Jan 31;2023:3216232. doi: 10.1155/2023/3216232. eCollection 2023.
Potter syndrome, first reported in 1946 by Edith Potter, refers to fatal cases of bilateral renal aplasia with pulmonary hypoplasia, peculiar facial features, and limb deformities. Presently, patients with oligohydramnios showing similar pathological manifestations due to oligohydramnios caused by conditions other than bilateral renal aplasia have been reported, and are known as the Potter sequence. There are limited studies and unclear guidelines on the safest delivery time and detailed postpartum management for patients with the Potter sequence. We experienced a case of Potter sequence, in which the patient was born by elective cesarean section at gestational age (GA) of 34 weeks. Fetal ultrasound at GA of 26 weeks 4 days showed oligohydramnios, multilocular cystic lesions in the left kidney, and an absent right kidney. Prenatal fetal MRI at GA of 33 weeks and 3 days showed pulmonary hypoplasia, and the ratio of fetal lung volume (FLV) to fetal body weight (FBW) was 0.0135 ml/g. We suspected that the fetal lung could not grow because of persistent oligohydramnios, which leads to a further decline in the ratio of FLV to FBW during pregnancy. We performed a cesarean section at GA of 34 weeks to prevent the exacerbation of the imbalance between lung volume and physique. We struggled to keep her condition stabilized with strict management of her respiratory condition, dialysis, and nutrition. She was discharged from the hospital at 169 days of age. Elective caesarean section in the term of premature birth prevented the progression of pulmonary hypoplasia and made it possible to save her life. Potter sequence is still relatively unknown, and it is necessary for more studies to be conducted in the future.
波特综合征于1946年由伊迪丝·波特首次报道，指双侧肾缺如合并肺发育不全、特殊面部特征及肢体畸形的致命病例。目前，已报道因双侧肾缺如以外的其他情况导致羊水过少而出现类似病理表现的患者，被称为波特序列征。关于波特序列征患者最安全的分娩时间及详细产后管理的研究有限，指南也不明确。我们遇到一例波特序列征病例，该患者在孕34周时通过择期剖宫产出生。孕26周4天时的胎儿超声显示羊水过少、左肾多发囊性病变及右肾缺如。孕33周3天时的产前胎儿磁共振成像显示肺发育不全，胎儿肺体积（FLV）与胎儿体重（FBW）之比为0.0135 ml/g。我们怀疑由于持续性羊水过少，胎儿肺无法生长，这导致孕期FLV与FBW之比进一步下降。我们在孕34周时进行剖宫产以防止肺体积与体格之间的失衡加剧。我们通过严格管理她的呼吸状况、透析及营养来努力维持她的病情稳定。她在169日龄时出院。早产时进行择期剖宫产防止了肺发育不全的进展并使挽救她的生命成为可能。波特序列征仍然相对不为人知，未来有必要进行更多研究。
