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ISSN: 2090-7214
Clinics in Mother and Child Health
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Hydrometrocolpos - A Lower Mesodermal Defects Sequence

Vijaya Lakshmi Bodduluri*

Mediscan Systems, Chennai, Tamil Nadu, India

Corresponding Author:
Vijaya Lakshmi B
Mediscan Systems, Chennai
Tamil Nadu, India
E-mail: [email protected]

Received Date: January 08, 2016 Accepted Date: February 28, 2016 Published Date: March 25, 2016

Citation:Vijaya Lakshmi B (2016) Hydrometrocolpos – A Lower Mesodermal Defects Sequence. Clinics Mother Child Health 13:227. doi:10.4172/2090-7214.1000227

Copyright: © 2016 Vijaya Lakshmi B. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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Hydrometrocolpos

Hydrocolpos is the distension of the vagina with sterile fluid caused by vaginal outlet obstruction. When there is associated distension of the uterus it is known as hydrometrocolpos. It is an uncommon congenital disorder with an incidence of approximately 1 in 16,000 female births [1]. Vaginal obstruction may result from vaginal atresia, a vaginal septum, imperforate hymen, persistent urogenital sinus or a cloacal anomaly, depending on the timing and nature of the embryological process that is disrupted [2].

The mass in hydrometrocolpos arises secondary to vaginal outlet obstruction which results in retained vaginal and cervical secretions, which are secreted in response to circulating maternal estrogens [2]. Also, associated urinary and intestinal fistulae are usually present leading to urine and meconium being retained [3].

Hydrometrocolpos can have a varied presentation. It can be diagnosed in utero or it can present at birth or at puberty. Prenatal diagnosis of hydrometrocolpos can be difficult as a result of its rarity, variable presentation and poor ultrasonography. The earliest gestation at diagnosis is reported as 25 weeks and most cases are diagnosed in the third trimester [4]. It can present in the neonatal period as an abdominal mass. At puberty, when hydrometrocolpos is usually secondary to an imperforate hymen, are presents as a bulge with a bluish discolouration at the vaginal introitus [5].

The prenatal differential diagnosis of hydrometrocolpos includes renal lesions, enteric cysts, ovarian cysts, mesenteric cysts, dilated ureter, loops of bowel, anterior sacral meningocoele, pelvic component of sacrococcygeal teratoma and retroperitoneal cystic lymphatic malformations [6].

Hydrometrocolpos can be isolated or associated with other abnormalities. It can be a part of several syndromes. In McKusick- Kaufman syndrome, a rare autosomal recessive disorder, congenital hydometrocolpos is associated with polydactyly and congenital heart disease [7]. Bardet-Biedl syndrome is an autosomal recessive disorder characterised by hydrometrocolpos, postaxial polydactyly, retinal dystrophy or retinitis pigmentosa, obesity, nephropathy and mental retardation. While McKusick-Kaufman syndrome can be diagnosed in very young children, the diagnosis of Bardet-Biedl syndrome is generally delayed upto teenage years. In Ellis-van Creveld syndrome there are polydactyly, acromelia and cardiac anomalies in addition to hydrometrocolpos.

The abnormalities associated with hydrometrocolpos can be primary or secondary [8]. Primary defects arise due to developmental defects whereas secondary effects are due to pressure from the mass. The secondary effects could be hydronephrosis, pulmonary hypoplasia, intestinal obstruction, respiratory distress and leg swelling. The prognosis for hydrometrocolpos is excellent if it is an isolated abnormality such as an imperforate hymen. The prognosis is worse, with significant morbidity and mortality, when there are associated congenital abnormalities which are present in 17 – 81 % of cases [9].

In our analysis of four autopsy cases of hydrometrocolpos at our centre [10] (Figure 1), we identified a spectrum of abnormalities associated with hydrometrocolpos, both primary and secondary. In all four cases there was a significant association of abnormalities. Most notable of these was the presence of pulmonary hypoplasia in all four cases. The kidneys were abnormal in three of the four cases, two were due to bilateral hydronephrosis and the third had unilateral renal agenesis. The external genitalia were ambiguous in three cases. The anus was imperforate in three cases with a high anorectal anomaly in all three cases and a colovaginal fistula in one. The bladder was distended in all four cases with urethral atresia in one case. There were vertebral/skeletal abnormalities in all four cases with sacral dysgenesis in two cases, sacral hemivertebra in one case and bilateral arachnodactyly in the fourth. This non-random association of abnormalities of the genital tract, renal/urinary system, lower gastrointestinal tract and axial skeleton is suggestive of lower mesodermal defects sequence [11].

clinics-mother-child-health-Autopsy-cut-specimen

Figure 1: Autopsy cut specimen of distended vagina of a 28 week old fetus showing longitudinal (thin arrow) and transverse (thick arrow) vaginal septa which obstructed the vagina and caused hydrometrocolpos. 1 – Uterus didelphys showing the two horns of the uterus.

Lower mesodermal defects sequence, as described by Pauli et al in 1994 involves the recurrent clustering of abnormalities of the renal, urinary, genital, lower gastrointestinal and axial skeletal systems. All these structures derive from the lower, infraumbilical, portion of the intraembryonic mesoderm. Not all abnormalities need to be present in all cases. But all structures involved share a common embryologic origin. The importance of ascertaining the diagnosis is in helping to counsel the bereaving parents. Firstly, it is the result of a single abnormality, namely, abnormality in the development of the lower embryonic mesoderm rather than there being multiple ‘abnormalities’ in the fetus. Secondly, no specific environmental insult has been identified, which means that the condition is not due to something the parents might have done or not done. Lastly, the diagnosis implies little or no risk of recurrence, which helps in reassuring the parents.

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Review summary

  1. Natalia Barnes
    Posted on Sep 07 2016 at 6:04 pm
    The article is highly significant and provides insights into the field in a simple way that can be easily comprehended. Providing image(s) could be helpful for better explanation of this rare disorder.
 

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