Congenital Perineal Groove Defect in Monozygotic Twin Infants: A Literature Review.

IF 0.8 Q4 PEDIATRICS AJP Reports Pub Date : 2021-01-01 Epub Date: 2021-03-31 DOI:10.1055/s-0041-1727145
Mimily Harsono, David Yanishevski, Massroor Pourcyrous
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引用次数: 5

Abstract

Perineal groove is a rare benign congenital anomaly with lesion that resembles perforation of mid-perineum or perineal raphe area. Most reported cases of congenital perineal groove presented as an isolated defect in term or early-term singleton female infants. Thus far, there is no reported case of this anomaly in monozygotic twins. Embryo pathogenesis of this female predominance congenital defect remains controversial. Many clinicians are unfamiliar with this congenital anomaly. This congenital defect tends to get self-resolved at around 2 year of age. Nevertheless, the exposed nonepithelized mucous membrane can carry risk of local infection or irritation with the possibility of requiring early surgical correction. The defect can be infrequently associated with other ano-urogenital malformations that required immediate surgical intervention. Most isolated cases tend to be asymptomatic and self-healed with expectant management. Surgical correction may be considered if not healed after 2 years of age. Early diagnosis at birth is important to avoid misdiagnoses at later age for trauma, dermatitis, sexual abuse, and risk of unnecessary aggressive intervention. Early parental counseling for providing good hygiene and close follow-up is important to prevent infection or inflammation. Presentation of this anomaly in both monozygotic twins may support the hypothesis of potential disruption during embryo morphogenesis stages.

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同卵双胞胎婴儿先天性会阴沟缺损的文献回顾。
会阴沟是一种罕见的良性先天性异常,其病变类似于会阴中部或会阴缝区穿孔。大多数报告的病例先天性会阴沟表现为孤立的缺陷足月或早期单胎女婴。到目前为止,还没有在同卵双胞胎中出现这种异常的报道。这种女性优势先天性缺陷的胚胎发病机制仍有争议。许多临床医生不熟悉这种先天性异常。这种先天性缺陷往往在2岁左右自行解决。然而,暴露的非上皮粘膜可能存在局部感染或刺激的风险,可能需要早期手术矫正。该缺陷很少与其他需要立即手术干预的无泌尿生殖器畸形相关。大多数孤立的病例往往是无症状和自愈与保守的管理。如果2岁后不愈合,可以考虑手术矫正。出生时的早期诊断对于避免日后因创伤、皮炎、性虐待和不必要的积极干预风险而误诊非常重要。早期父母咨询提供良好的卫生和密切的随访对预防感染或炎症很重要。在两个同卵双胞胎中出现这种异常可能支持胚胎形态发生阶段潜在破坏的假设。
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来源期刊
AJP Reports
AJP Reports PEDIATRICS-
CiteScore
2.20
自引率
0.00%
发文量
30
审稿时长
12 weeks
期刊最新文献
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