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ORIGINAL ARTICLE Table of Contents   
Year : 2016  |  Volume : 13  |  Issue : 4  |  Page : 161-165
Experience with neonatal hydrometrocolpos in the Niger Delta area of Nigeria: Upsurge or increased recognition?


1 Department of Surgery, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria
2 Department of Pathology, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria
3 Department of Obstetrics and Gynaecology, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria

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Date of Web Publication24-Nov-2016
 

   Abstract 


Background: Hydrometrocolpos (HMC) is a clinical condition in which there is a cystic distension of the vagina (hydrocolpos), uterus and sometimes, fallopian tubes (hydrosalpinx) with fluid. This study described our experience with cases of HMC seen in our practice, and highlighted the flare in our practice. Patients and Methods: A retrospective study of cases of HMC managed at the University of Port Harcourt Teaching Hospital between September 2010 and August 2012. Results: There were seven cases; their ages ranged from 2 to 27 days (median 13 days). All the patients had abdominal distension but abdominal mass was obvious only in four. Other features varied depending on the presence of sepsis or other associated anomalies. The diagnosis of HMC was missed in all cases by the referring clinicians. One patient was referred with the diagnosis of tracheoesophageal fistula and esophageal atresia, 2 with bladder outlet obstruction, 1 with intestinal obstruction, 2 with anorectal malformation, and 1 with neonatal sepsis. Ultrasound identified bulky uterus in two cases and upper urinary tract dilatation in 3 patients. Six patients had laparotomy, 1 had hymenotomy only. Postoperative complications were basically wound sepsis and rectovaginal fistula resulted. Conclusion: Diagnosis of HMC should be considered as a differential in newborn girls presenting with lower abdominal mass. Attention to clinical detail is necessary to avoid a misdiagnosis.

Keywords: Hydrometrocolpos, Girls, Diagnosis, Management

How to cite this article:
Okoro PE, Obiorah C, Enyindah C E. Experience with neonatal hydrometrocolpos in the Niger Delta area of Nigeria: Upsurge or increased recognition?. Afr J Paediatr Surg 2016;13:161-5

How to cite this URL:
Okoro PE, Obiorah C, Enyindah C E. Experience with neonatal hydrometrocolpos in the Niger Delta area of Nigeria: Upsurge or increased recognition?. Afr J Paediatr Surg [serial online] 2016 [cited 2017 Jul 21];13:161-5. Available from: http://www.afrjpaedsurg.org/text.asp?2016/13/4/161/194666



   Introduction Top


Hydrometrocolpos (HMC) is a clinical condition in which there is a cystic distension of the vagina (hydrocolpos), uterus and sometimes,  Fallopian tube More Detailss (hydrosalpinx) with fluid. It is thought to arise from accumulation of secretions in the vagina and uterus due to excessive stimulation of the fetal cervical mucous glands by maternal estrogen in the presence of an atretic vagina or imperforate hymen.[1],[2] Such congenital obstructions of the female genital tract have been long recognized.[3] Conversely, neonatal HMC is a rare condition despite the fact that imperforate hymen is the most common congenital anomaly of the female genital tract.[4] This suggests that imperforate hymen on its own does not explain the development of HMC. Imperforate hymen most usually passes unrecognized in neonatal life, being identified later due to associated problems or their complications.[5] The aetiological basis for development of HMC in the neonate is therefore yet unclear. There had not been reported cases of HMC in the Niger Delta region of Nigeria; but we have observed a sudden increase in hospital incidence in the past 2 years. Reports of HMC from other regions of Nigeria have not indicated such high incidence within a relatively short period.[6],[7] We reviewed our experiences with HMC, highlighted the difficulties of diagnosing and managing unusual cases in an environment with limited facilities.


   Patients and Methods Top


Data were retrospectively extracted from the notes of cases of HMC managed by the authors between September 2010 and August 2012. Data obtained were place of abode of mother, age at presentation, presenting features, associated anomalies, investigation findings, findings at surgery, and outcome. Data were subjected to a simple descriptive analysis with the Microsoft Office Excel 2007.


   Results Top


There were seven cases of HMC during the period under review. The ages of patients at presentation ranged from 2 to 27 days (median 13 days). Four of the mothers lived in oil producing communities of Rivers and Bayelsa states of Nigeria. At presentation, all the patients had abdominal distension, and abdominal mass in four. Other features included sepsis or other associated anomalies [Table 1]. The diagnosis of HMC was missed in all cases by the referring clinicians. One patient was referred with the diagnosis of tracheoesophageal fistula and esophageal atresia, 2 with bladder outlet obstruction, 1 with intestinal obstruction, 2 with anorectal malformation, and 1 with neonatal sepsis. Ultrasound identified the abdominal mass as the uterus in two cases and upper urinary tract dilatation in 3 patients. Intravenous urography was not done but plain abdominal X-ray identified distended bowel loops in 4 patients. Six patients had laparotomy, while one had hymenotomy only [Table 2]. The appearances of the pelvic mass were typically, hydrocolpos [Figure 1], [Figure 2], [Figure 3], and uterine didelphys [Figure 3]. The fluid was milky white in all cases and volume estimated 50–250 ml [Figure 4]. A biopsy of the vaginal wall in Case 1 was normal [Figure 5]. Biopsy in the subsequent cases was considered unnecessary. There was one partial wound dehiscence, which healed before discharge. A rectovaginal fistula resulted while we tried to do a vaginoplasty (Case 7), then necessitating a sigmoid colostomy. The fistula has since been repaired and patient is presently waiting for colostomy closure. There were two deaths due to respiratory failure (Case 3) and severe neonatal sepsis (Case 6).
Table 1: The main features in the patients

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Table 2: Operative findings, treatment, and outcome

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Figure 1: Hydrometrocolpos

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Figure 2: Hydrocolpos as seen in Case 4

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Figure 3: HMC as seen in Case 2

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Figure 4: Milky fluid content of HMC

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Figure 5: Normal vaginal wall

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   Discussion Top


Neonatal HMC is known to result from excessive fetal cervical secretion caused by maternal estradiol in the presence of a congenital obstruction of the genital tract.[2],[8] Imperforate hymen being the most common congenital anomaly of the female genital tract, it would be expected that HMC by extension, should be common as well. The rarity of HMC therefore suggests that there are some other factors that determine its manifestation and whether it will manifest in the neonatal period or later at puberty. Seven cases of neonatal HMC in 2 years in an environment where it had been almost unknown even in older children and adults elicit significant interest. The fact that all the referring clinicians did not pick up the right diagnosis suggests that this upsurge may not be due to increased awareness of the condition. It also highlights the low index of suspicion and paucity of awareness of this uncommon condition even among specialists. Difficulty with diagnosis has been identified in many reports even in advanced countries.[9],[10] This apparent low index of suspicion among caregivers in our environment indicates that there may be more cases than are seen in our service. In one report, three of the nine patients were only diagnosed intraoperatively.[5] In the only case reported from southeastern Nigeria, diagnosis was also made at surgery.[6] In our series, only three of the seven cases were diagnosed correctly preoperatively. Low index of suspicion for uncommon conditions and the variable presentations of patients are among the reasons adduced for the common misdiagnosis.[8] In our series, most of the patients presented with some other congenital anomalies or complications that tended to obscure the HMC. This indicates that irrespective of the problems any female neonate may present with, HMC must be specifically looked out for. Whereas an earlier report from Northern Nigeria included 9 patients seen over a period of 15 years, 7 patients seen in 2 years is a call for increased awareness. This is particularly in view of the serious urinary and gastrointestinal complications that arise from the obstructive effects of HMC.[4],[11],[12] Upper urinary tract damage as a complication makes prenatal diagnosis desirous. Whereas none of our cases were diagnosed prenatally, there are increasing reports from other parts of the world of the possibility and usefulness of prenatal diagnosis in ameliorating or forestalling the complications.[13],[14],[15],[16] The typical features of neonatal HMC are a mass in the hypogastrum and a bulging of whitish tissue between the labia and behind the urethra. This hymenal bulge is absent when there is vaginal atresia or a urogenital sinus. Associated anomalies or complications often take the attention of the clinician away from the HMC. This was the situation in three of our patients who had tracheoesophageal fistula and esophageal atresia, persistent cloaca, and imperforate anus, respectively. Two of our patients who had postaxial polydactily could have been cases of the recognized associated syndromes such as Bardet Biedl syndrome, Mckusick- Kaufman syndrome More Details but we have not confirmed this conclusively as one died 2 days postoperatively and the other is still being followed up.[17],[18],[19] A high index of suspicion is required in this environment where majority of newborns do not have the benefit of a scrupulous assessment by a neonatologist soon after birth. In our patients, the ultrasonographer mistook the pelvic mass for the urinary bladder. Imaging features of HMC have been described by researchers to aid recognition of HMC.[20],[21] Two of the cases who had laparotomy and who had imperforate hymen could have benefitted from simple hymenotomy but for lack of clarity of the pathological anatomy before the surgery. In addition, a rectovaginal fistula, which resulted following vaginoplasty, is a mishap that could have been avoided if the pathology were properly delineated. A finger or catheter balloon inserted in the rectum in the course of the dissection could have guided the surgeon and possibly avoided the rectal injury. Proper delineation would certainly give a guide to a better quality of treatment. Some authors have made a case for needle aspiration under reliable ultrasound or magnetic resonance imaging guidance. We think that this mode of treatment may predispose to infection of the HMC cavity, incomplete drainage and recurrence, and bleeding into the cavity. It may only be useful in very ill patients and in circumstances where neonatal anesthesia is considerably risky.

In all the flare, we have experienced of HMC awakens the consciousness of the occurrence of this condition in our region and prepares us to recognize it early and treat appropriately. As we watch the trend in its incidence in our region and other regions with similar circumstances, effort should be made to increase the awareness of the occurrence of HMC among caregivers at the primary and secondary levels of the health-care system.


   Conclusion Top


Neonatal HMC has been encountered in our practice with relative high incidence in the past 2 years. Most of the babies are of mothers who live in the oil-producing communities of the Niger Delta region of Nigeria. The awareness and the experience to properly diagnose and manage it are still limited in our practice and some other regions of the world with similar circumstances. There is the need to create the awareness of this condition among all levels of health-care givers. There is a need for a closer look at the possibility that environmental factors may be contributory to the development of neonatal HMC.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Sawhney S, Gupta R, Berry M, Bhatnagar V. Hydrometrocolpos: Diagnosis and follow-up by ultrasound – A case report. Australas Radiol 1990;34:93-4.  Back to cited text no. 1
    
2.
Wilson DA, Stacy TM, Smith EI. Ultrasound diagnosis of hydrocolpos and hydrometrocolpos. Radiology 1978;128:451-4.  Back to cited text no. 2
    
3.
Chawla S, Bery K, Indra KJ. Abnormalities of urinary tract and skeleton associated with congenital absence of vagina. Br Med J 1966;1:1398-400.  Back to cited text no. 3
    
4.
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5.
Ozturk H, Yazici B, Kucuk A, Senses DA. Congenital imperforate hymen with bilateral hydronephrosis, polydactyly and laryngocele: A rare neonatal presentation. Fetal Pediatr Pathol 2010;29:89-94.  Back to cited text no. 5
    
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Ameh EA, Mshelbwala PM, Ameh N. Congenital vaginal obstruction in neonates and infants: Recognition and management. J Pediatr Adolesc Gynecol 2011;24:74-8.  Back to cited text no. 6
    
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8.
Richard R, Martin K. Surgical management of intersexuality, cloacal malformation, and other abnormalities of the genitalia in girls. In: Alan WJ, Louis KR, Andrew NC, Alan PW, Craig PA, editors. Campbell-Walsh Urology. 9th ed. Philadelphia: Saunders Elsevier; 2007. p. 3830-69.  Back to cited text no. 8
    
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Shaked O, Tepper R, Klein Z, Beyth Y. Hydrometrocolpos – Diagnostic and therapeutic dilemmas. J Pediatr Adolesc Gynecol 2008;21:317-21.  Back to cited text no. 9
    
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Shaw LM, Jones WA, Brereton RJ. Imperforate hymen and vaginal atresia and their associated anomalies. J R Soc Med 1983;76:560-6.  Back to cited text no. 10
    
11.
Johal NS, Bogris S, Mushtaq I. Neonatal imperforate hymen causing obstruction of the urinary tract. Urology 2009;73:750-1.  Back to cited text no. 11
    
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Cherian MP, Al-Sanna'a NA, Ayyat FM. Hydrometrocolpos and acute renal failure: A rare neonatal presentation of Bardet-Biedl syndrome. J Pediatr Urol 2008;4:313-6.  Back to cited text no. 12
    
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Liu YP, Chen CP. Fetal MRI of hydrometrocolpos with septate vagina and uterus didelphys as well as massive urinary ascites due to cloacal malformation. Pediatr Radiol 2009;39:877.  Back to cited text no. 13
    
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Taori K, Krishnan V, Sharbidre KG, Andhare A, Kulkarni BR, Bopche S, et al. Prenatal sonographic diagnosis of fetal persistent urogenital sinus with congenital hydrocolpos. Ultrasound Obstet Gynecol 2010;36:641-3.  Back to cited text no. 14
    
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Yildirim G, Gungorduk K, Aslan H, Sudolmus S, Ark C, Saygin S. Prenatal diagnosis of imperforate hymen with hydrometrocolpos. Arch Gynecol Obstet 2008;278:483-5.  Back to cited text no. 15
    
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El-Messidi A, Fleming NA. Congenital imperforate hymen and its life-threatening consequences in the neonatal period. J Pediatr Adolesc Gynecol 2006;19:99-103.  Back to cited text no. 16
    
17.
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.  Back to cited text no. 17
    
18.
Slavin TP, McCandless SE, Lazebnik N. McKusick-Kaufman syndrome: The difficulty of establishing a prenatal diagnosis of an uncommon disorder. J Clin Ultrasound 2010;38:151-5.  Back to cited text no. 18
    
19.
Toma HS, Tan PL, McKusick VA, Katsanis N, Adams NA. Bardet-Biedl Syndrome in an African-American patient: Should the diagnostic criteria be expanded to include hydrometrocolpos? Ophthalmic Genet 2007;28:95-9.  Back to cited text no. 19
    
20.
Schumacher R, Brzezinska R. Hydrometrocolpos: The Fjord sign. Ultraschall Med 2007;28:518-20.  Back to cited text no. 20
    
21.
Subramanian S. Importance of rectal signal on fetal magnetic resonance imaging in patients having hydrometrocolpos. Taiwan J Obstet Gynecol 2008;47:375.  Back to cited text no. 21
    

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Correspondence Address:
Philemon Ekemenye Okoro
Department of Surgery, Paediatric Surgery Unit, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.194666

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