Hydrometrocolpos and Post-axial Polydactyly Complicated With Acute Intestinal Obstruction and Hydroureteronephrosis

Arriola-Montenegro, Liliana; Arriola-Montenegro, José; Pia-Balmaceda, Maria; Celis, Carlos R.; Riva-Moscoso, Adrian; Cabanillas-Lozada, Patricia; Velásquez-Huarcaya, Vladimir

doi:10.7759/cureus.17612

Cited by 3 publications

(3 citation statements)

References 12 publications

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“…The limitation of our case is the inability to perform molecular testing to confirm a possible underlying congenital syndrome despite having HMC in association with polydactyly. MKS, for instance, presents with cardiac anomalies; however, even with a confirmed cardiac defect on imaging, genetic analysis is required for diagnosis (chromosome 20p12 between D20S162 and D20S894 markers) [13]. Similarly, a diagnosis of BBS requires future surveillance by ophthalmologists to detect other commonly associated findings of this syndrome.…”

Section: Discussionmentioning

confidence: 99%

Hydrometrocolpos With Polydactyly in Consanguineous Parents: A Case Report and Review of Literature

Khalil¹,

Syed²,

Elaasser³

et al. 2023

Cureus

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Neonatal hydrometrocolpos (HMC) is a cystic dilatation of a neonate's vagina and uterus occurring secondary to congenital vaginal obstruction, with or without maternal estrogenic stimulation of uterine and cervical glands causing increased secretions during the prenatal and postnatal period. Diagnosis is made using ultrasonography and further confirmed by MRI. HMC in a neonate can rarely present with congenital anomalies such as polydactyly, which may indicate a variety of underlying genetic syndromes. There is a deficit in the literature as to whether the development of HMC in a neonate of consanguineous parents is an isolated finding or solely related to an underlying syndrome. We hope to help bridge this gap by reporting a case of a 12-day-old neonate presenting with hydrometrocolpos and polydactyly, born to consanguineous parents.

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Section: Discussionmentioning

confidence: 99%

Hydrometrocolpos With Polydactyly in Consanguineous Parents: A Case Report and Review of Literature

Khalil¹,

Syed²,

Elaasser³

et al. 2023

Cureus

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“…1 When the compression is severe, the neonate or infants may present with acute urinary retention or intestinal obstruction. 12,13 Acute urinary retention and obstructive uropathy in a patient with HMC is mainly due to compression of the urethra by the distended vagina, which can prevent emptying of the bladder. 9 In addition, patients with HMC may manifest as abdominal distension, lower abdominal pain, discomfort in the pelvis, pain in the lower back, and palpable, tender cystic intraabdominal mass or a vulvoperineal mass.…”

Section: Discussionmentioning

confidence: 99%

Neonatal Hydrometrocolpos Secondary to Imperforate Hymen Presented with Acute Urinary Retention: Case Report

Tegene,

Assefa,

Edris

2023

RRN

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Background: Hydrometrocolpos is a rare clinical condition characterized by the accumulation of fluid or mucus secretions in the vagina and uterus due to distal vaginal obstruction. Imperforate hymen is the most common cause of congenital hydrometrocolpos. The diagnosis of congenital hydrometrocolpos can be made prenatally or postnatally. Imperforate hymen is generally managed by hymenotomy or hymenectomy. Case Presentation: This is a 26-day-old female neonate who was born from a 29-year-old Para −3 mother after 9 months of amenorrhea. ANC follow-up was uneventful except her obstetric ultrasound scan at 36+3 weeks found a fetal lower intra-abdominal mass. Without proper postnatal evaluation, the neonate was discharged home with the mother after 12 hours of vaginal delivery. Currently, the neonate was referred to our hospital with the diagnosis of Acute Urinary retention, 2 o Hydrometrocolpos, after having frequent crying, abdominal distension, and failure to pass urine of 2 days duration. On physical examination, there was a tender, cystic, and mobile mass palpable in the lower abdomen, and up to opening the labia there was a curved membrane covering the vaginal opening. Abdomino-pelvic ultrasound and Magnetic Resonance Imaging confirmed the hydrometrocolpos and imperforate hymen. After obtaining informed consent from the family, a vertical incision and annular hymenotomy were done to drain 200mL of milky fluid. A follow-up ultrasound was done to confirm the resolution of HMC and neonate discharge on the 4th day with improvement. Conclusion: A high index of suspicion and immediate postnatal evaluation of neonates presented with prenatal U/S showing intraabdominal mass will result in early diagnosis and treatment of neonatal hydrometrocolpos before the development of complications.

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“…Frequently, the urinary tract is compressed causing varying degrees of hydronephrosis as in case 1 and 3, as previous reports mentioned [ 11 , 12 , 13 ]; pressure on the bowel to cause constipation, as in case 2, is not common [ 8 , 14 ]. In more severe compression effects, the infants may present with acute urinary retention and intestinal obstruction [ 15 , 16 , 17 ]. HMC can sometimes be part of several syndromes.…”

Section: Discussionmentioning

confidence: 99%

Hydrometrocolpos in Infants: Etiologies and Clinical Presentations

2022

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Hydrometrocolpos (HMC) is a rare condition where fluids or secretions accumulate in the vagina (hydrocolpos) or up to the uterus (hydrometrocolpos). This case series study reports three infants with different etiologies and presentations of HMC and aims to review literature for proper workup upon initial diagnosis. The first neonate antenatally presented with a huge cystic mass. HMC secondary to imperforate hymen was proved, and hymenotomy was performed at 2 days of age. The second participant presented with persistent urogenital sinus and hematopoietic chimerism, possibly due to transfusion from her twin brother via placenta anastomoses. At 2 months of corrected age, she had difficult defecating, and sonogram revealed HMC with normal appearance of uterus and ovaries. Regular follow-ups and surgical reconstruction will be conducted before puberty. The third patient had cloacal malformation and multiple congenital anomalies at birth. Vesicovaginal fistula-related HMC was detected and managed with surgical drainage in the neonate stage. The girl began menstruation with dysmenorrhea at 12 years. The image studies demonstrated hematometrocolpos secondary to left-side hemivaginal septum, uterine didelphy, and ipsilateral renal agenesis, indicating Herlyn–Werner–Wunderlich syndrome. HMC can be diagnosed easily via sonogram. Careful external genitalia examinations help to identify persistent urogenital sinus or cloacal malformation. Occasionally, the HMC may be part of syndrome manifestations or associated with sex chromosome anomalies. Clinicians may conduct surveillance of renal, cardiac, and skeletal systems as well as chromosome study for early diagnosis and management.

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Hydrometrocolpos and Post-axial Polydactyly Complicated With Acute Intestinal Obstruction and Hydroureteronephrosis

Abstract: Arriola-Montenegro et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Cited by 3 publications

References 12 publications

Hydrometrocolpos With Polydactyly in Consanguineous Parents: A Case Report and Review of Literature

Hydrometrocolpos With Polydactyly in Consanguineous Parents: A Case Report and Review of Literature

Neonatal Hydrometrocolpos Secondary to Imperforate Hymen Presented with Acute Urinary Retention: Case Report

Hydrometrocolpos in Infants: Etiologies and Clinical Presentations

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