Noviello Carmine, Romano Mercedes, Nino Fabiano, Martino Ascanio, Cobellis Giovanni
a Pediatric Surgery Unit , Salesi Children's Hospital, Università Politecnica delle Marche , Ancona , Italy.
Gynecol Endocrinol. 2018 Jan;34(1):56-58. doi: 10.1080/09513590.2017.1332178. Epub 2017 May 31.
The authors present their experience in the management of pediatric patients with Herlyn-Werner-Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation.
All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices).
Six patients were treated in the study period. The mean age was 9 years (2 months-15 years). According to the characteristics of the HWWS, we had 5 patients in group A, 1 in group B, and none in group C. One-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary for a better resection of the septum. At a mean follow-up of 18 months all patients were symptoms free.
Prognosis of this malformation is good in case of early diagnosis and treatment. We suggest that when a renal agenesia is diagnosed, the patient needs an ultrasonographic follow-up of the contralateral kidney but also of the genital tract to find each minimal abnormalities, furthermore, a MRI scanning before the onset of menstruation can be necessary.
作者介绍了他们在治疗患有赫林-韦纳-温德利希综合征（HWWS）的儿科患者方面的经验，同时考虑了畸形的临床分类和解剖特征。
收集了2010年2月至2015年8月在我们儿科外科就诊的所有患者的数据。根据畸形类型，患者分为3组：A组（完全阻塞的半阴道）、B组（不完全阻塞的半阴道）和C组（双宫颈之间有连通）。
在研究期间共治疗了6例患者。平均年龄为9岁（2个月至15岁）。根据HWWS的特征，A组有5例患者，B组有1例患者，C组无患者。所有完全阻塞的病例均进行了一期手术治疗，但有1例需要再次手术以更好地切除隔膜。平均随访18个月时，所有患者均无症状。
这种畸形若能早期诊断和治疗，预后良好。我们建议，当诊断出肾缺如时，患者不仅需要对侧肾脏的超声随访，还需要对生殖道进行超声随访，以发现任何微小异常，此外，在月经初潮前可能需要进行MRI扫描。
