Congenital Anomalies of the Kidney and Urinary Tract in Patients with Hirschsprung Disease.

Muniba Mehmood, Jamshed Akhtar, Naima Zamir
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Abstract

Objective: To determine the frequency and types of congenital anomalies of the kidney and urinary tract (CAKUT) in patients with Hirschsprung disease.

Study design:  An observational cross-sectional study. Place and Duration of the Study: Department of Paediatric Surgery, National Institute of Child Health, Karachi, Pakistan, from June to December 2022.

Methodology: All patients with biopsy-proven Hirschsprung disease were included. Ultrasound was done to find out the anatomical and structural anomalies of the kidney and urinary tract. Functional assessment was done by renal function tests and MAG3 radioisotope scan where indicated. Fisher's exact test was applied to find out the association. A p ≤0.05 was taken as significant.

Results: Out of a total of 83, 15 (18.0%) patients had CAKUT. The mean age of the study population was 5.5 ± 2.3 years. It included 61 (73.5%) males and 22 (26.5%) females. No significant association was found between the type of Hirschsprung disease and CAKUT (p = 0.7). The gender distribution between CAKUT patients was also insignificant (p = 0.7). Renal hypoplasia was the most common anomaly found in six patients followed by hydronephrosis due to pelvi-ureteric junction obstruction in four children. All of these children were asymptomatic. Five male patients had undescended testis.

Conclusion: Nearly a fifth of the children with Hirschsprung disease had CAKUT of whom renal dysplasia was the most common anomaly. There was no gender predilection and patients were asymptomatic regarding the urinary system. A routine ultrasound abdomen is a good screening investigation for identifying CAKUT.

Key words: Hirschsprung disease, Kidney and urinary tract, Congenital diseases of the urinary tract, Congenital anomalies.

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赫氏普隆病患者的肾脏和泌尿道先天性异常。
研究目的研究设计: 观察性横断面研究。研究地点和时间研究地点和时间:巴基斯坦卡拉奇国立儿童健康研究所小儿外科部,2022年6月至12月:方法:纳入所有经活检证实患有赫氏胃肠病的患者。通过超声波检查发现肾脏和泌尿道的解剖和结构异常。功能评估通过肾功能检查和 MAG3 放射性同位素扫描(如有必要)进行。采用费舍尔精确检验找出相关性。结果:在83名患者中,15名(18.0%)患有CAKUT。研究对象的平均年龄为(5.5 ± 2.3)岁。其中男性 61 人(73.5%),女性 22 人(26.5%)。赫氏胃肠病的类型与 CAKUT 之间无明显关联(p = 0.7)。CAKUT患者的性别分布也不明显(p = 0.7)。肾发育不全是最常见的异常情况,有 6 名患儿,其次是肾盂输尿管连接处梗阻导致的肾积水,有 4 名患儿。所有这些患儿均无症状。五名男性患者的睾丸未降:结论:近五分之一的赫氏普隆病患儿患有 CAKUT,其中肾发育不良是最常见的异常。没有性别偏好,患者在泌尿系统方面没有任何症状。常规腹部超声波检查是识别CAKUT的良好筛查方法:赫氏病 肾脏和泌尿系统 泌尿系统先天性疾病 先天性畸形
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