Journal of Medicine in the Tropics

CASE REPORT
Year
: 2020  |  Volume : 22  |  Issue : 2  |  Page : 153--155

Familial imperforate hymen in an adolescent girl: presenting as acute lower abdominal pains and distention − a case report and review of literature


Chidiebere Nwakamma Ononuju1, Yakubu Ahmed Ashuku2, Lucky Lohnan Changkat1, Uzoma Emmanuel Chinaka1, Elizabeth Chioma Ezeaku1, Blaise Ogedi Okwaraoha1,  
1 Departments of Obstetrics and Gynaecology, Dalhatu Araf Specialist Hospital (DASH), Lafia, Nigeria
2 Department of Surgery Dalhatu Araf Specialist Hospital Lafia, Nigeria

Correspondence Address:
Dr. Chidiebere Nwakamma Ononuju
Departments of Obstetrics and Gynaecology, Dalhatu Araf Specialist Hospital, Lafia
Nigeria

Abstract

Imperforate hymen often is not diagnosed until an adolescent girl presents with complaints of primary amenorrhea and cyclical pelvic pains. We report the case of a 15-year old girl with a family history of imperforate hymen that presented with complaints of progressive lower abdominal pains and distention, and primary amenorrhea. A diagnosis of Imperforate hymen was made by history, findings at physical examination and trans-abdominal ultrasonography. She subsequently had hymenotomy for hematocolpometra. Her postoperative recovery was normal and her menstruation became regular.



How to cite this article:
Ononuju CN, Ashuku YA, Changkat LL, Chinaka UE, Ezeaku EC, Okwaraoha BO. Familial imperforate hymen in an adolescent girl: presenting as acute lower abdominal pains and distention − a case report and review of literature.J Med Trop 2020;22:153-155


How to cite this URL:
Ononuju CN, Ashuku YA, Changkat LL, Chinaka UE, Ezeaku EC, Okwaraoha BO. Familial imperforate hymen in an adolescent girl: presenting as acute lower abdominal pains and distention − a case report and review of literature. J Med Trop [serial online] 2020 [cited 2021 Mar 2 ];22:153-155
Available from: https://www.jmedtropics.org/text.asp?2020/22/2/153/294820


Full Text



 INTRODUCTION



Imperforate hymen is an uncommon obstructive anomaly of the female reproductive tract, which usually presents with intermittent and cyclical abdominal pain.[1] Imperforate hymen has an incidence of 1 in 1000 and represents a persistent portion of the urogenital membrane.[2] Imperforate hymens mostly occur in a sporadic manner, although rare familial cases do occur and have been reported,[1],[2],[3] like in our patient. The majority of the cases of the imperforate hymen are diagnosed at the onset of symptoms at puberty.[1],[3] Imperforate hymen should be suspected in any adolescent girl with a positive family history of imperforate hymen, and who may present with complaints of primary amenorrhoea, cyclic abdominal or pelvic pain, back pain, constipation, or urinary retention secondary to mass effect from the vaginal distention.[1],[3],[4] In this article, we present a case of the familial imperforate hymen in a pubertal girl and highlight the psychosocial burden it exerts on both the patient and parents alike.

 CASE REPORT



We present the case of a 15-year-old secondary school student who presented at the Gynaecology emergency unit of Dalhatu Araf Specialist Hospital Lafia, Nigeria on the 30th of July 2018 accompanied by her anxious parents with complaints of progressive lower abdominal pain and distention of 4 days duration. She also reported a pattern of cyclical crampy pain in her lower abdomen, which lasted for 7 days every month over the past 6 months. The pain had become more severe during the previous 3 months and the size of her abdomen had gradually increased. She has never menstruated and wasn’t sexually active. She denied having nausea, vomiting, fever, vaginal discharge, constipation or urinary symptoms. Her mother had a similar complaint at age 17 and subsequently underwent surgical intervention for imperforate hymen in a general hospital. Her medical history was normal. Her parents were very worried that she was having a similar condition that her mother had as a teenager.

Physical examination revealed an anxious healthy looking girl. Her breast buds and pubic hair were well developed. There was a smooth, globular, mobile, tender abdominal mass, about 20 weeks size extending from the pelvis. One can get above the abdominal mass but not below it. Pelvic examination revealed a bulging bluish hymen from the vaginal vestibule that became more prominent when she was asked to cough, otherwise, the external genitalia was essentially normal. The rectal examination revealed a distended vagina bulging into the anterior rectal wall and the abdominal mass was highly suggestive of being the uterus.

An abdomen-pelvic ultrasound showed an enlarged uterus and distended vagina with a homogenous, hypoechoic fluid collection. There was a scanty peritoneal fluid; the ovaries and kidneys were normal. The urine pregnancy test was negative. The results of the complete blood count, urinalysis, and serum electrolytes, urea and creatinine tests were all within normal limits.

A diagnosis of familial imperforate hymen with haematocolpometra was made.

The General Surgery and Paediatric team on-call also reviewed her. The clinical findings and diagnosis were explained to the patient and her parents and appropriate emotional support was given to the family. Informed consent was obtained and she subsequently underwent hymenotomy under general anaesthesia. She was put in a lithotomy position, her bladder was drained with a Foley two-way catheter, and a cruciate incision was made on the central part of the hymenal bulging membrane. Approximately, 950 ml of viscous altered blood was drained. The hymenal orifice was enlarged to allow further egress of menstrual flow and to prevent the obstructing membrane from reforming. The urethral catheter was removed and the patient was discharged home the next day. The Paediatric and Gynecology teams also counseled the family and highlighted that imperforate hymen may occur in her children although she had undergone treatment for it. She was seen at the Gynaecology outpatient clinic on two separate appointments, at the first and third months post-surgery. She was completely symptom-free and had normal ultrasound findings. She had started having regular menstrual cycles.

 DISCUSSION



Imperforate hymen occurs mostly in a sporadic manner, although rare familial cases have been documented.[1],[2] The aetiopathogenesis of imperforate hymen is largely unknown, although the recessive and dominant modes of inheritance have been postulated.[3] However, up to date no genetic markers or mutations have been proven as aetiological factors.[1],[3]

Imperforate hymen is the most common cause of obstruction of the lower female genital tract.[2],[5] It has an incidence of 1 in 1000 and represents a persistent portion of the urogenital membrane.[2] It occurs when the primitive streak abnormally invades the urogenital portion of the cloacal membrane.[2] The normal perforation of the hymen occurs late in fetal life.[2],[3]

Majority of the patients with imperforate hymen present with symptoms at puberty,[1],[3],[6] like our patient who presented with complaints of cyclical lower abdominal pain and distention, and primary amenorrhea at the age of 15. It can also present as urinary outflow obstruction, obstipation, tenesmus, oedema of the lower limbs and back pain.[5],[7] These symptoms are a direct result of obstructed vaginal outflow leading to haematocolpometra, which exerts mechanical effects on the urethra, bladder, intestines, or pelvic blood vessels that can result in urinary retention, obstipation or oedema of the legs.[8] Irritation of the sacral plexus or nerve roots can cause lower back pain.[9]

Imperforate hymen and its varied symptoms can be a source of intense anxiety and fear to both parents and the child alike[2] as was seen in the index patient. Emotional comfort should be provided and adequate information given to the company. The diagnosis of the imperforate hymen was made by history, findings at physical examination and trans-abdominal ultrasonography.

Surgical intervention is the cornerstone of the management of imperforate hymen.[2],[10] Hymenotomy that was done for our patient in the theatre under general anaesthesia using the cruciate incision relieved the outflow obstruction and allowed the haematocolpometra to be drained. Other types of incisions like the X shaped, T shaped, cyclical and simple vertical incisions can also be used.[3] Our patient had no post-surgical complications. Urethral injuries, excessive bleeding, scarring and stenosis of the vaginal opening[10] have been documented in some patients following a hymenotomy. Less invasive treatments for an imperforate hymen include the use of CO2 laser or a Foley catheter.[11],[12] These aforesaid treatment modalities are not done in our center.

Familial imperforate hymen is very rare. Healthcare providers should be aware that there have been reports of familial occurrence of hymenal abnormalities and thus alert young women that their daughters may have a similar abnormality.

The attending physician managing these patients should evaluate their female offspring at birth, as well as other female relatives of the affected patients at an early age as this will ensure early diagnosis, monitoring, and treatment of asymptomatic cases.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Lim YH, Ng SP, Jamil MA. Imperforate hymen: report of an unusual familialoccurrence. J Obstet Gynaecol Res 2003;29:399-401.
2Dvora B. Pediatric and adolescent gynecology. In: Decherney AH, Nathan L, Laufer N, Roman AS editors. Current Diagnosis & Treatment: Obstetrics and Gynecology, 11th edition. USA: Mc Graw Hill Lange; 2013. P. 589.
3Sakalkale R, Samarakkody U. Familial occurrence of imperforate hymen. J Paediatr Adolesc Gynecol 2005;18:427-9.
4Dane C et al. Imperforate hymen-a rare cause of abdominal pain: two cases andreview of the literature. J Pediatr Adolesc Gynecol 2007;20:245-7. [PubMed]
5Doyle JC. Imperforate hymen: with and without hematocolpos. A review of the literature and a report of twenty cases. Cal West Med 1942;56:242-7. [PubMed]
6Uzoma OI, Anolue F. Imperforate hymen: a rare cause of acute abdominal pain inperipubertal girls. A case report and literature review. Web med central Obstetrics and Gynaecology 2015;6:WMC004802
7Anselm OO, Ezegwui UH. Imperforate hymen presenting as acute urinaryretention in a 14-year-old Nigerian girl. J Surg Tech Case Rep 2010;2:84-86. DOI: 10. 4103 / 2006-8808. 73623
8Mwenda AS. Imperforate Hymen − a rare cause of acute abdominal pain andtenesmus: case report and review of the literature. Pan Afr Med J 2013;15:28. Published 2013 May 21. DOI:10.11604/pamj.2013.15.28.2251
9Lardenoije C, Aardenburg R, Mertens H. Imperforate hymen: a cause of abdominalpain in female adolescents. BMJ Case Rep 2009;2009:bcr08.2008. 0722. DOI: 10. 1136 /bcr.08.2008.0722
10Letts M, Haasbeek J. Hematocolpos as a cause of back pain in premenarchaladolescents. J Pediatr Orthop 1990;10:731. [PubMed]
11Chang JW, Yang LY, Wang HH et al. Acute urinary retention as the presentationof imperforate hymen. J Chin Med Assoc 2007;70:559-61 [PubMed]
12Friedman M, Gac D, Peretz B. Management of imperforate hymen with thecarbon dioxide laser. Obstet Gynecol 1989;74:270-2 [PubMed]