Cendron Marc
Department of Urology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
Front Pediatr. 2018 Mar 14;6:55. doi: 10.3389/fped.2018.00055. eCollection 2018.
The megameatus intact prepuce (MIP) variant of hypospadias is a rare variant of hypospadias that is diagnosed either early at the time of circumcision or later as the foreskin is retracted. The true incidence of the anomaly is difficult to determine precisely as some patient never come to medical attention but is felt to under 5% of all cases of hypospadias. The purposes of this study are to review the embryology and clinical findings of MIP and then, in light of a personal experience, present a series of patients evaluated for MIP who were treated with a modification of the Mathieu technique.
A PubMed search of all articles in the MIP variant of hypospadias was carried out followed by an exhaustive review of the literature. The charts of all patients evaluated and treated at Boston Children's Hospital by MC between 2007 and 2017 were reviewed retrospectively. The patients were divided into two groups: those who underwent the standard procedure and those who underwent a repair using a modification of the Mathieu procedure using an inframeatal flap.
The embryologic explanation of the MIP variant is not clear but failure of the distal, glanular portion of the urethra to tubularize results in spectrum of abnormality characterized by a deep glanular groove and an abnormal opening of the urethra anywhere from the mid-glans to a subcoronal location. Surgical repair is complicated by a wide distal urethra which may be injured if not properly identified. Overall good outcomes were noted with one patient experiencing a urethra cutaneous fistula in the first group and one patient having a mild glans dehiscence in the second.
The MIP variant of hypospadias is a rare variant of hypospadias that presents as a spectrum of urethral anomaly. Surgical repair may not always be necessary but if surgical repair is carried out, the Mathieu technique modification may offer better anatomic delineation of the urethra and will provide an extra layer of tissue to cover the reconstructed urethra. Low complication rates should be expected with adequate functional outcome such as a normal urinary stream. In addition, criteria for selecting patients for surgical repair are provided.
尿道下裂的巨尿道口完整包皮（MIP）变异型是一种罕见的尿道下裂变异类型，在包皮环切术时早期诊断，或在包皮回缩后较晚诊断。由于一些患者从未就医，该异常的确切发病率难以精确确定，但据认为在所有尿道下裂病例中占比不到5%。本研究的目的是回顾MIP的胚胎学和临床发现，然后结合个人经验，介绍一系列接受改良马蒂厄技术治疗的MIP评估患者。
对尿道下裂MIP变异型的所有文章进行PubMed检索，随后对文献进行详尽回顾。回顾性分析2007年至2017年间在波士顿儿童医院由MC评估和治疗的所有患者的病历。患者分为两组：接受标准手术的患者和接受使用改良马蒂厄手术（采用阴茎内叶皮瓣）修复的患者。
MIP变异型的胚胎学解释尚不清楚，但尿道远端、龟头部分未能形成管状导致一系列异常，其特征为龟头深沟以及尿道从中龟头至冠状沟下任何位置的异常开口。手术修复因远端尿道宽阔而复杂化，如果未正确识别可能会受损。总体结果良好，第一组有1例患者出现尿道皮肤瘘，第二组有1例患者出现轻度龟头裂开。
尿道下裂的MIP变异型是一种罕见的尿道下裂变异类型，表现为一系列尿道异常。手术修复并非总是必要，但如果进行手术修复，改良马蒂厄技术可能能更好地解剖显示尿道，并提供额外一层组织覆盖重建的尿道。预期并发症发生率较低，功能结果良好，如正常尿流。此外，还提供了选择手术修复患者的标准。
