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双阴茎:一例罕见异常的磁共振成像评估病例报告。

Diphallia: a case report of a rare anomaly evaluated by magnetic resonance imaging.

机构信息

WEBIMAGEM Telerradiologia, São Paulo, São Paulo, Brazil.

出版信息

Turk J Pediatr. 2021;63(5):917-921. doi: 10.24953/turkjped.2021.05.021.

Abstract

BACKGROUND

Diphallus, also known as penile duplication, is a rare malformation, seen once in every 5 to 6 million births. Newborns showing this condition present higher mortality rates due to malformations and infections. The underlying etiology of this malformation is uncertain, but it is thought to be associated with trauma, drug use, or infections that may affect fetal the mesoderm between the 23rd and 25th day of pregnancy. Our objective is to describe this rare malformation - diphallus - through magnetic resonance imaging, as well as additional findings.

CASE

A Three-month-old male patient with a 33-week ultrasound demonstrating genital malformation presented to our clinic. At birth, the physical examination revealed diphallia and imperforated anus. Surgical procedures were carried out right after birth to correct the anus malformation. The child did not present any alteration in skin color, and no signs of pain were shown in the abdomen, pelvis, and penises palpations. Urination was observed only through the right penis. Magnetic resonance imaging (MRI) showed two penile structures, each one presenting developed with corpus cavernosum. The penis located on the right showed a complete urethral path in the corpus spongiosum to the vesical floor while the penis located on the left was bigger and did not present a urethral path.

CONCLUSIONS

Penile duplication is a rare condition that is often, associated with other malformations, especially anorectal. To fully understand the extension of congenital anomalies and to determine the optimal surgical approach, MRI yields detailed imaging of the entire pelvic region, providing a thorough anatomical frame of reference, and should be routinely incorporated into presurgical evaluation.

摘要

背景

双阴茎又称阴茎重复畸形,是一种罕见的畸形,每 500 万至 600 万例新生儿中可见一例。患有这种疾病的新生儿由于畸形和感染,死亡率更高。这种畸形的潜在病因尚不确定,但据认为与创伤、药物使用或感染有关,这些因素可能会影响妊娠第 23 至 25 天的中胚层。我们的目的是通过磁共振成像以及其他发现来描述这种罕见的畸形——双阴茎。

病例

一名三个月大的男性婴儿,在 33 周的超声检查中显示生殖器畸形,到我们的诊所就诊。出生时,体格检查发现双阴茎和肛门闭锁。出生后立即进行了手术以矫正肛门畸形。患儿的皮肤颜色没有改变,腹部、骨盆和阴茎触诊时也没有疼痛迹象。仅通过右侧阴茎观察到排尿。磁共振成像(MRI)显示两个阴茎结构,每个结构都有发育良好的海绵体。位于右侧的阴茎在海绵体中有一条完整的尿道通向膀胱底,而位于左侧的阴茎较大,没有尿道。

结论

阴茎重复畸形是一种罕见的疾病,常与其他畸形,特别是肛门直肠畸形有关。为了全面了解先天性异常的程度,并确定最佳的手术方法,MRI 可对整个骨盆区域进行详细成像,提供全面的解剖参考框架,应常规纳入术前评估。

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