Kobayashi Haruka, Baba Tokuro, Kuda Masaaki, Ieiri Satoshi, Takatsuki Mitsuhisa
Department of Digestive and General Surgery, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
Keiaikai Nakagami Hospital, Okinawa, Japan.
Surg Case Rep. 2023 Jul 10;9(1):126. doi: 10.1186/s40792-023-01710-y.
A few cases of small omphalocele with umbilical evagination of the bladder have been reported. However, its embryology is yet to be elucidated. Only a few reports have indicated the existence of urachal anomalies and umbilical cysts related to bladder evagination. The incidence of urachal anomalies at birth is reported to be 1 in 5000-8000 live birth, and urachal aplasia is rare. Herein, we report a rare, novel case of urachal aplasia.
We encountered a small omphalocele with bladder evagination associated with urachal aplasia for which the neonate underwent surgery one day after birth. The patient was a one-day-old boy with a prenatally diagnosed omphalocele. A fetal magnetic resonance image (MRI) scan (25 weeks of gestation) revealed a 30 × 33 mm (approximately 1.3 in.) cystic lesion which was suspected to be an umbilical cyst. The baby was born vaginally at 38 weeks, weighing 2956 g. An omphalocele (hernial orifice diameter, 4 cm × 3 cm) with bladder prolapse was recognized. After sac excision, the prolapsed bladder was resected and closed with two-layer sutures. In order to secure sufficient bladder capacity, we estimated the minimum residual volume as 21 ml after bladder plasty. The remaining bladder capacity was confirmed to be 30 ml by injecting a contrast dye and saline into the bladder. The neonate had no associated cardiac urogenital or skeletal anomalies. Postoperative course was uneventful. The patient was regularly followed up for two years after surgery and underwent umbilicoplasty. He had no trouble with urinary function.
In this case, we experienced extremely rare condition of a small omphalocele with bladder evagination associated with urachal aplasia and reviewed 7 case reports of anomalies similar to those in the present case. Umbilical cord cysts may be an informative indicator of these symptoms in utero. Therefore, ultrasonography scans should be conducted until delivery, despite the spontaneous disappearance of cord cysts.
已有少数小型脐膨出合并膀胱脐部外翻病例的报道。然而，其胚胎学仍有待阐明。仅有少数报道指出存在与膀胱外翻相关的脐尿管异常和脐囊肿。据报道，出生时脐尿管异常的发生率为1/5000 - 8000活产儿，脐尿管发育不全较为罕见。在此，我们报告一例罕见的、新型的脐尿管发育不全病例。
我们遇到一例小型脐膨出合并膀胱外翻且伴有脐尿管发育不全的病例，该新生儿于出生后一天接受了手术。患者为一名1日龄男婴，产前诊断为脐膨出。胎儿磁共振成像（MRI）扫描（妊娠25周）显示一个30×33毫米（约1.3英寸）的囊性病变，怀疑为脐囊肿。婴儿于38周经阴道分娩，体重2956克。发现一个伴有膀胱脱垂的脐膨出（疝孔直径4厘米×3厘米）。切除囊袋后，切除脱垂的膀胱并用两层缝线缝合。为确保足够的膀胱容量，我们在膀胱成形术后估计最小残余容量为21毫升。通过向膀胱内注入造影剂和生理盐水，确认剩余膀胱容量为30毫升。该新生儿无相关心脏、泌尿生殖系统或骨骼异常。术后过程顺利。患者术后定期随访两年并接受了脐成形术。他的排尿功能没有问题。
在本病例中，我们遇到了一种极为罕见的情况，即小型脐膨出合并膀胱外翻且伴有脐尿管发育不全，并回顾了7例与本病例类似异常的报告。脐带囊肿可能是子宫内这些症状的一个有参考价值指标。因此，尽管脐带囊肿会自行消失，但在分娩前仍应进行超声扫描。
