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一例与先天性肾和尿路异常相关的新生儿骨筋膜室综合征的罕见病例。

An unexpected case of neonatal compartment syndrome associated with congenital anomalies of kidney and urinary tract.

作者信息

Seske Laura M, Mastroianni Melissa, Aziz Keith T, Lewallen Laura W

机构信息

Division of Neonatology, Department of Pediatrics, The Johns Hopkins School of Medicine, Chicago, IL, USA.

Department of Plastic and Reconstructive Surgery, The Johns Hopkins University, Baltimore, MD, USA.

出版信息

Case Rep Perinat Med. 2023 Mar 7;12(1):20220020. doi: 10.1515/crpm-2022-0020. eCollection 2023 Jan.

Abstract

OBJECTIVES

Neonatal compartment syndrome (NCS) occurs when increased pressure within the fasciocutaneous compartment decreases capillary perfusion, causing irreversible tissue damage from ischemia. NCS is a rare condition that requires prompt diagnosis and treatment. Diagnosing NCS is highly dependent on the examination, which can be difficult in newborns. Prompt recognition provides the best chance for good outcomes.

CASE PRESENTATION

We present a case of NCS diagnosed and treated based on physical examination findings. Fetal ultrasonography showed bladder distension, bilateral hydroceles, urethra dilation, and abdominal urinary ascites concerning for lower urinary tract obstruction and possible bladder rupture. At 1 h after birth, examination of the infant's left upper extremity showed no spontaneous movement, the hand and forearm appeared dusky, and the hand had a large blister with desquamation. No pulse distal to the antecubital fossa was detected via Doppler ultrasonography. The infant was diagnosed with NCS and underwent urgent fasciotomy. The clinical appearance and perfusion of the left upper extremity gradually improved. At four months of age, the wounds were healed and the patient had full passive range of motion of the left upper extremity. Recovery of active motion is ongoing.

CONCLUSIONS

The presence of blistering and desquamation should provoke suspicion for NCS. Once NCS is diagnosed, prompt intervention is necessary to reduce the risk of poor functional outcomes. This case highlights the need for increased awareness of the risk developing compartment syndrome as part of the rare sequalae in infants with congenital anomalies of kidney and urinary tract.

摘要

目的

当筋膜皮肤间隙内压力升高导致毛细血管灌注减少,引起缺血性不可逆组织损伤时,会发生新生儿间隙综合征(NCS)。NCS是一种罕见疾病,需要及时诊断和治疗。NCS的诊断高度依赖体格检查,而这在新生儿中可能具有挑战性。及时识别可为良好预后提供最佳机会。

病例报告

我们报告一例基于体格检查结果诊断和治疗的NCS病例。胎儿超声检查显示膀胱扩张、双侧鞘膜积液、尿道扩张以及腹腔内尿液性腹水,提示下尿路梗阻和可能的膀胱破裂。出生后1小时,对婴儿左上肢的检查显示无自主活动,手部和前臂呈暗紫色,手部有一个大水泡且伴有脱皮。通过多普勒超声检查未检测到肘前窝远端的脉搏。该婴儿被诊断为NCS并接受了紧急筋膜切开术。左上肢的临床表现和灌注逐渐改善。在4个月大时,伤口愈合,患者左上肢被动活动范围完全正常。主动活动的恢复仍在进行中。

结论

出现水泡和脱皮应引起对NCS的怀疑。一旦诊断为NCS,必须及时干预以降低功能预后不良的风险。本病例强调需要提高对发生间隙综合征风险的认识,这是先天性肾脏和尿路异常婴儿罕见的后遗症之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8176/11616542/486d0d45cd99/j_crpm-2022-0020_fig_001.jpg

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