Hydrometrocolpos presenting with acute urinary retention in a neonate Nwafor NN, Eyo IP

CASE REPORT

Year : 2019 | Volume : 8 | Issue : 2 | Page : 120-122

Hydrometrocolpos presenting with acute urinary retention in a neonate

Nkem Nnenna Nwafor, Itoro Paul Eyo
Department of Radiology, University of Uyo Teaching Hospital, Uyo, Akwa Ibom State, Nigeria

Date of Web Publication

13-Sep-2019

Correspondence Address:
Dr. Nkem Nnenna Nwafor
Department Radiology, University of Uyo Teaching Hospital, Uyo, Akwa Ibom State
Nigeria

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/sjhs.sjhs_42_19

Abstract

Hydrometrocolpos (HMC) is a rare clinical condition resulting in accumulation of fluid in the endometrial and vaginal cavities secondary to vaginal obstruction. Vaginal obstruction can be caused by imperforate hymen, transverse vaginal septum, vaginal atresia, abnormal vaginal opening, and malformation of the urogenital sinus. Imperforate hymen is the most common cause. Time of presentation can be from the neonatal period to mid-adolescent age and can present as an isolated case or part of a syndrome. The usual clinical symptom is suprapubic abdominal mass. Symptoms from mass effect on surrounding organs, such as urinary retention and constipation, as well as complications of chronic fluid accumulation, such as tuboovarian abscess can be the initial presentation. With a high index of suspicion, diagnosis can be made with adequate clinical history, examination, and imaging. HMC should be considered in female neonates and infants presenting with midline suprapubic intra-abdominal mass irrespective of other clinical presentations.

Keywords: Hydrometrocolpos, imaging, tubo-ovarian abscess

How to cite this article:
Nwafor NN, Eyo IP. Hydrometrocolpos presenting with acute urinary retention in a neonate. Saudi J Health Sci 2019;8:120-2

How to cite this URL:
Nwafor NN, Eyo IP. Hydrometrocolpos presenting with acute urinary retention in a neonate. Saudi J Health Sci [serial online] 2019 [cited 2021 Jan 25];8:120-2. Available from: https://www.saudijhealthsci.org/text.asp?2019/8/2/120/264680

Introduction

Hydrometrocolpos (HMC) is a clinical condition where there is collection of fluid consisting of mucous vaginal secretions within the endometrial and vaginal cavities; it may extend to the Fallopian tube ^{More Details}s. It is a rare condition resulting from increased stimulation of the cells of the fetal cervical glands by maternal hormones (estrogen) and retention of the secretions due to congenital vaginal obstruction.^[1] The causes of vaginal obstruction include imperforate hymen, transverse vaginal septum, abnormal vaginal opening, vaginal atresia, and malformations such as failure of recanalization of cloaca and urogenital sinus.^[2],[3] There may be associated uterine or Müllerian duct anomalies and isolated anomalies of the digits such as polydactyl. Other associations include tracheoesophageal fistula, anorectal, vertebral and cardiac anomalies as well as syndromic anomalies such as VACTERL association (vertebral, anal, cardiovascular, tracheoesophageal, renal, and limb anomalies), MURCS association (Müllerian duct aplasia/hypoplasia, renal agenesis/ectopia, and cervicothoracic somite dysgenesis such as Klippel–Feil abnormality, anomalous ribs, or Sprengel deformity), and mesodermal anomalies such as McKusick- Kaufman syndrome ^{More Details}, an autosomal recessive disorder which includes vaginal atresia and secondary HMC, hexadactyly, congenital cardiac anomalies, and hydrops fetalis.^[4],[5]

HMC is a rare finding, with a varying incidence of 1 in 1000–1 in 16,000 females.^[6] The most common cause is an imperforate hymen with an incidence of 0.014%–1%.^[7] Imperforate hymen can present clinically in the neonatal period or at puberty.^[1] The determinants of time of presentation are unknown. Presentation at puberty with suprapubic abdominal mass and cyclical pain in a girl child yet to attain menarche is more common and easier to clinically evaluate. Other bizarre modes of presentations and complications are pelvic infection with tubo-ovarian abscess, obstructive acute renal failure, hematosalpinx, peritonitis, endometriosis, mucometrocolpos, constipation, and recurrent urinary tract infection.^[8],[9] In the neonate, a high index of suspicion is required for accurate diagnosis because of the diverse clinical presentations such as abdominal mass, abdominal distension, features of intestinal obstruction, pulmonary hypoplasia, acute urinary tract obstruction, sepsis, and rarely neonatal ascites. Presentation as early as 2 days has been reported.^[10],[11]

Imaging diagnosis can be made in the prenatal and postnatal period using ultrasonography. Magnetic resonance imaging and computed tomography should be used in the postnatal period when available to make a more accurate diagnosis and to plan the surgical intervention, especially in complex cases where congenital anomalies of the urogenital sinus are involved.^[12] However, a high index of suspicion and a thorough clinical examination are necessary to prevent retrospect diagnosis, avertable complications, and surgeries, especially in resource-poor settings.

The aim of this case study is to increase our suspicion for HMC, especially those secondary to imperforate hymen in neonates with pelvic masses irrespective of the other clinical presentations, as clinical diagnosis, simple imaging, and minor surgical intervention are sufficient to manage the case.

Case Report

A 30-day-old female infant presented with a history of progressively increasing abdominal distension, nonbilious vomiting, and reduced urinary output of 11-day duration. On examination, she was afebrile, anicteric, and restless. A large suprapubic mass and hyperemia of the suprapubic region and proximal thigh were noted. The other organ systems were unremarkable. The attempt at catheterization of the urinary bladder was difficult, blood-stained urine was collected, and the catheter was removed. The vulva was edematous. Pregnancy was uneventful, and there was no prenatal ultrasonography report. A clinical diagnosis of bladder outlet obstruction and suspected intestinal obstruction was made. Abdominal ultrasound, urinalysis, urine microscopy, culture and sensitivity, full blood count, and blood culture were ordered.

Abdominal ultrasound using mindray DC-30 ultrasound with a convex probe (3–8MHz) on pediatric abdomen protocol revealed a large, thin-walled, midline cystic structure measuring 7.4 cm × 7.1 cm × 5.9 cm (164.0 ml) in the suprapubic region posterior to a collapsed, thick-walled urinary bladder, and anterior to the sacrum. The lesion contained hypoechoic fluid with a large hyperechoic dependent portion [Figure 1]a. The uterus was not visualized. The kidneys were normal in size with increased parenchymal echo pattern. There was moderate hydronephrosis bilaterally more marked on the right [Figure 1]b, and a right hydroureter was seen down to the posterior wall of the urinary bladder. Cystic teratoma and duplication cyst were considered. The blood tests were within normal limits.

Figure 1: (a) Large midline cystic structure with a large hyperechoic dependent portion in the suprapubic region. (b) Normal-sized hydronephrotic kidneys more marked on the right

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The infant was booked for exploratory laparotomy for suspected bladder tumor following deteriorating clinical condition, passage of bloody urine, and fleshy tissues in urine. Under general anesthesia, attempt at catheterization revealed absent introitus and bulging hymen. A 0.5 cm cruciate hymenotomy was done, and 700 ml of whitish fluid was drained. Repeat ultrasound 5 days postsurgery revealed a relatively large for age hypoechoic uterus with demonstrable midline echogenic stripe [Figure 2]a, a normal urinary bladder [Figure 2]b, a normal left kidney, and mild hydronephrosis of the right kidney. There is no demonstrable uterine anomaly. The postoperative period was satisfactory.

Figure 2: (a) Postsurgery, normal-sized urinary bladder with regular outline. (b) Uterus after hymenotomy

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Discussion

HMC is rare and presents in the neonatal period in an isolated form with or without uterine anomalies or as part of a syndrome. Imperforate hymen is the most common cause of vaginal obstruction which may present clinically at the two extremes of childhood which are in the neonatal period and at puberty with a bulging hymen on examination.^[1] In the index case, the cause of HMC was imperforate hymen. HMC may be easier to evaluate in the pubertal period where there is cyclical abdominal pain and history of no menarche. In the neonatal period, the clinical picture is not clear-cut, and symptoms from other surrounding organ systems such as urinary and gastrointestinal tracts are more conspicuous with some presenting with congenital anomalies; hence, the diagnosis may be made late or intraoperatively. In the index case, diagnosis was made intraoperatively due to low index of suspicion. Similarly, three of nine cases reported by Ameh et al.^[10] in North Nigeria and all seven cases reported by Okoro et al.^[11] in south-south Nigeria were diagnosed intraoperatively. HMC from vaginal atresia and vaginal septum though very rare has been reported in Southeast Nigeria by Ekenze and Ezegwui,^[13] in a situation like this, HMC will not present with bulging hymen; hence, accurate clinical diagnosis will be more difficult.

Acute urinary obstruction and a possibility of bladder tumor were the presenting symptoms in the index case. Urinary retention has also been recorded in some cases of HMC and in adolescents with hematometrocolpos secondary to imperforate hymen.^[14] It is a complication resulting from obstruction of the lower urinary tract by the retained secretions or collections. It can detour the line of thought of the managing physicians resulting in a subsequent delay in accurate diagnosis as seen in the index case. Resolution of obstructive signs is usually complete within 6 months postintervention.

No other congenital anomaly, uterine anomaly, or syndromic feature was seen in the index patient. Other studies have reported congenital anomalies such as anorectal malformations, imperforate anus, Hirschprung's disease, trachea-esophageal fistula, and congenital uterine anomalies.^{[10],[11],[12],[13]} Surgical complications such as rectovaginal fistula have been recorded in patients who had open surgeries;^[11] our patient had hymenotomy with satisfactory postsurgical state. The volume of fluid drained in this case is the highest reported so far. Follow-up was recommended as failed hymenotomy has been reported in the past.^[15]

A possibility of environmental factor in the Niger delta region of Nigeria has been raised by Okoro et al.^[11] The index case was seen in the Niger delta region; however, there is no upsurge to ascertain such claim in this region; this is the only case we have seen and this may be responsible for our poor index of suspicion and late diagnosis.

Conclusion

HMC should be considered in female neonates and infants presenting with midline suprapubic intraabdominal mass irrespective of other clinical presentations. A high index of suspicion is necessary for accurate prenatal and postnatal diagnosis and prompt management of uncomplicated cases. This will forestall unnecessary surgical interventions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Ash DG, Smith IM. Neonatal hematocolpos. Can Med Assoc J 1965;92:1125-6.
2.	Sidatt M, Ould Sidi Mohamed Wedih A, Ould Boubaccar A, Ould Ely Litime A, Feil A, Ould Moussa A, et al. Hydrocolpos and hydrometrocolpos in newborns. Arch Pediatr 2013;20:176-80.
3.	Bischoff A, Levitt MA, Breech L, Louden E, Peña A. Hydrocolpos in cloacal malformations. J Pediatr Surg 2010;45:1241-5.
4.	Hahn-Pedersen J, Kvist N, Nielsen OH. Hydrometrocolpos: Current views on pathogenesis and management. J Urol 1984;132:537-40.
5.	Kos S, Roth K, Korinth D, Zeilinger G, Eich G. Hydrometrocolpos, postaxial polydactyly, and hypothalamic hamartoma in a patient with confirmed pallister-hall syndrome: A clinical overlap with McKusick-Kaufman syndrome. Pediatr Radiol 2008;38:902-6.
6.	Ayaz UY, Dilli A, Api A. Ultrasonographic diagnosis of congenital hydrometrocolpos in prenatal and newborn period: A case report. Med Ultrason 2011;13:234-6.
7.	Mwenda AS. Imperforate hymen – A rare cause of acute abdominal pain and tenesmus: Case report and review of the literature. Pan Afr Med J 2013;15:28.
8.	Ho JW, Angstetra D, Loong R, Fleming T. Tuboovarian abscess as primary presentation for imperforate hymen. Case Rep Obstet Gynecol 2014;2014:142039.
9.	Awada EE, Ahmed S, Tamer MA, Ahmed ME. Imperforate hymen as an unusual cause of non-urological urine retention – A case report. Afr J Urol 2015;21:72-5.
10.	Ameh EA, Mshelbwala PM, Ameh N. Congenital vaginal obstruction in neonates and infants: Recognition and management. J Pediatr Adolesc Gynecol 2011;24:74-8.
11.	Okoro PE, Obiorah C, Enyindah CE. Experience with neonatal hydrometrocolpos in the Niger delta area of Nigeria: Upsurge or increased recognition? Afr J Paediatr Surg 2016;13:161-5. [PUBMED] [Full text]
12.	Gupta M, Bajwa SS, Gupta M. Diagnostic dilemmas in management of neonatal hydrometrocolpos: A crucial role of magnetic resonance imaging. Int J Health Allied Sci 2014;3:251-4. [Full text]
13.	Ekenze SO, Ezegwui HU. Hydrometrocolpos from a low vaginal atresia: An uncommon cause of neonatal intestinal and urinary obstruction. Afr J Paediatr Surg 2008;5:43-5. [PUBMED] [Full text]
14.	Anselm OO, Ezegwui UH. Imperforate hymen presenting as acute urinary retention in a 14-year-old Nigerian girl. J Surg Tech Case Rep 2010;2:84-6.
15.	Al-Hunaidi O, El-Shazly M, Alkandari M, Ghobashy A, Alhajeri F, Allam A, et al. Acute urine retention caused by hematocolpos after failed hymenotomy in 23 year old female. Urol Case Rep 2015;3:50-1.