与肛门直肠畸形相关的泌尿生殖系统畸形

Sameer P. A, Priya Ranganath
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摘要

:肛门直肠畸形是常见的先天性畸形之一,发病率在 1-3000 到 5000 之间。高达 70% 的患者伴有肛门畸形。泌尿生殖系统畸形是最常见的伴发畸形。 本研究的目的是根据克里肯贝克分类法,确定泌尿生殖系统畸形的类型和频率,以及与 ARM 不同变异相关的泌尿生殖系统畸形:研究共纳入 150 名患者。对患儿进行全面体检,然后进行婴儿造影、超声心动图、脊柱超声扫描和 KUB 超声波扫描,以检查不同的相关畸形。如果需要进一步明确,还需进行核磁共振成像检查。根据克里肯贝克分类法对患者进行分类:伴有会阴瘘的 ARM 是常见的 ARM 类型。73%的患者伴有畸形,32%的患者伴有泌尿生殖系统(UG)畸形。70.8%的患者有泌尿系统异常。52.1%的患者有生殖器异常。本研究中最常见的泌尿系统异常类型是输尿管肾积水(HUN)(41.2%)。32.4%的泌尿系统异常患者患有膀胱输尿管反流(VUR)。32.4%的患者有肾脏发育不全,5.9%的患者有神经源性膀胱,5.9%的患者有肾积水,5.9%的患者有异位输尿管,5.9%的患者有交叉融合的异位肾:与不同亚型肛门直肠畸形相关的泌尿生殖系统异常因人而异。了解这些相关畸形有助于外科医生规划治疗方案,从而决定患者的预后和生活质量。
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Urogenital anomalies associated with anorectal malformations
: Anorectal malformations are one of the common congenital anomalies with an incidence of 1-3000 to 5000. Up to 70% of the patients have associated anomalies. Urogenital anomalies are the most common associated anomalies. : This study was done to determine the type and frequency of urogenital anomalies associated with different variants of ARMs according to the Krickenbeck classification.: 150 patients were included in the study. A full physical examination of the child was conducted followed by an infantogram, echocardiogram, spinal ultrasound scan, and KUB ultrasound was done to investigate different associated anomalies. MRI was done if further clarity was needed. The patients were classified according to the Krickenbeck classification.: ARM with perineal fistula was the common type of ARM. 73% had associated anomalies, and 32% of patients had urogenital (UG) anomalies. 70.8% of patients had urinary anomalies. 52.1% of patients had genital anomalies. The most common type of urinary anomaly found in the present study was hydroureteronephrosis (HUN) (41.2%). 32.4% of the patients with urinary anomalies had vesicoureteral reflux (VUR). Renal agenesis was found in 32.4%, neurogenic bladder 5.9%, hydronephrotic kidney 5.9%, ectopic ureter 5.9%, cross fused ectopic kidney 5.9%.: Urogenital anomalies associated with different subtypes of anorectal malformation vary drastically from patient to patient. The knowledge of these associated anomalies will help the surgeons in planning the course of treatment which can determine the prognosis and quality of life of the patients.
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