Imperforate hymen and leaking hematosalpinx mimicking acute appendicitis: A report of a rare case and a review of literature

Highlights • Cases of imperforate hymen (IH) that present with hemoperitoneum mimicking acute appendicitis are rare.• Diagnosis and management can be a challenge especially in low-resource setting.• Surgical management of both acute appendicitis and IH can be successful achieved during the same operative session.


Introduction
Imperforate hymen (IH), although rare, is the most common obstructive congenital anomaly of the female genital tract, with an incidence rate of approximately 1 in 2000 females [1]. Patients are usually asymptomatic until menarche when menstrual blood starts to collect behind the imperforate hymen, accumulating either in the vagina (hematocolpos) [2], uterus (hematometra) or/and fallopian tubes (hematosalpinx). Hematocolpos gets worse with each menstrual period. If IH is not surgically treated early, it may result in complications such as endometriosis, adhesions, and ultimately infertility.
The classical presentation of imperforate hymen is primary amenorrhea in adolescent girls with a bulging mass in the vagina, associated with cyclic abdominal pain [3]. Atypical presentation can include urinary retention with kidney failure [4], chronic constipation [5], and uterine infections due to static uterine blood. As a result, girls presenting with atypical IH often are initially misdiagnosed, which increases the likelihood of undergoing unnecessary and yet costly laboratory and radiographic studies. The aim of this case report is to present IH mimicking acute appendicitis. We further review the literature on the different unique presentations of imperforate hymen and discuss surgical management options. This work has been reported in line with the SCARE criteria [6]. https

Case
In March 2019, a 12-year-old premenarchal girl was referred to our hospital for the definitive management of acute appendicitis. She presented with a 5-day history of increasing abdominal pain. On admission to our hospital, the abdominal pain was located in the epigastrium and at the right lower quadrant (RLQ). The pain was gradual in onset and progressively worsened. The pain was severe, 8 on a scale of 1-10, sharp, constant but non-radiating. It was associated with constipation but not with anorexia, nausea or vomiting. The patient did not report any history of dysuria or any change in urinary frequency. She had unremarkable gynecological, birth and developmental history.
On examination, the only abnormal sign was an ill-defined nontender mass in the RLQ on deep palpation. The abdomen was not distended and without signs of peritonitis. A perineal physical examination was missed to be carried out by the attending surgeon and the admitting surgical resident. (This was a failure on the surgical team and a very important lesson we learned to avoid in the future). Based on the clinical symptoms of RLQ pain, tender mass and the migratory nature of the abdominal pain, a differential diagnosis of an appendicitis was made. The patient was prepared for an appendectomy. Her lab work was within normal limits: Hb: 12.5 g/dL (normal range: 12.0-16.0 g/dL), WBC: 9400 cells/uL (normal range: 4000-10,000 cells/uL), Platelets: 319,0000 cells/uL (range: 100,000-300,000 cells/uL). Since the patient's presentation was classic for appendicitis, no imaging studies were needed. The patient was hydrated with intravenous (IV) normal saline and was started on broad-spectrum IV antibiotics (metronidazole and ciprofloxacin). Under general anesthesia, a RLQ incision over the McBurney point (located two thirds of the distance between the umbilicus and the inferior superior iliac spine) was made. On entering the peritoneum, an inflamed appearing appendicitis was noted. There was approximately 200 mL of dark blood in the peritoneum. We performed an appendectomy. Blood in the peritoneum was drained and the abdomen was thoroughly irrigated with warm saline solution. During the inspection of the bowel, we noted a ruptured, hemorrhagic right ovarian follicle and a bulky pelvic mass in the lower segment of the uterus. Under general anesthesia, a vaginal examination was conducted, and a slightly bulging imperforate hymen was identified (Fig. 1A). A hymenectomy was performed through a cruciate incision followed up with re-approximation of hymen edges with Vicryl 2-0 sutures (Fig. 1B). More than 500 mL of viscous old blood was drained from the vagina, uterus and the right salpinx. The bleeding right ovarian follicle was sutured. Both ovaries were of normal size on palpation. She was then discharged on postoperative day 4 without pain or vaginal bleeding. A repeat genitourinary examination 3 weeks later still showed a patent hymen.

Discussion
Although imperforate hymen is an anomaly usually diagnosed by a thorough history taking and a physical examination, the presentation mimicking acute appendicitis and a tender mass in the RLQ was atypical. The appendix was secondarily inflamed by the hemoperitoneum. The typical pathophysiology of an obstructed appendiceal lumen resulting in appendiceal dilation and inflammation leading to suppuration and gangrene was absent. This unique presentation led to the initial diagnosis of appendiceal mass with appendicitis leading to an appendectomy. This was a near miss diagnosis had the abdominal cavity not been carefully explored after appendicectomy. We however recognize that a thorough abdominal cavity exploration should have been done before appendicectomy. Nevertheless, this would not have changed sur-gical management. Typically, imperforate hymen presents with primary amenorrhea and a cyclic pattern of lower abdominal/pelvic pain [7,8], with or without associated symptoms such as back pain (38%-40%), urine retention (37%-60%) [2,3], or constipation (27%). Blood accumulation in the vagina and uterus is evident when the distensible membrane bulges between the labia. However, blood flow dynamics for our case were different in that, retrograde blood flow through the right fallopian tube ended in the peritoneum. Consequently, no accumulation of blood to overly distend imperforate hymen (Fig. 1A). The inflamed appendicitis could have been caused by the irritation of blood in the peritoneum.
Summarized in Table 1 is the review of other case reports with both typical and atypical presentation of IH. Presentations include acute urinary retention, uterine infection and septic shock. Sixty percent of the cases presented with expanding abdominal mass and abdominal pain. In approximately 20%, the abdominal pain was cyclic, timing the menstruation period. Furthermore, a bluish bulging imperforate hymen, exaggerated on valsalva was a common finding on perineal examination. The vast majority of the clinical diagnosis was hematocolpometra. The amount of blood evacuated after hymenotomy ranged between 300-2500 mL, consistent with the amount of blood drained from our patient.
The diagnosis of IH can be made from a physical examination alone. Nevertheless, due to non-specific presentations, patients are usually investigated for other causes of abdomen pain, resulting to expensive laboratory testing or radiographic evaluations. Observing the hymen and viewing into the vaginal vault are best accomplished by applying gentle labial traction with the patient in the supine frog-leg or knee-chest position. In the typical presentation of IH with a bulging mass at the vaginal introitus, the speculum examination is not indicated. Failure to do a perineal examination such as in our case, leads to delayed or missed diagnosis. However, if patient or parents refuse genital exam evaluation, imaging studies can greatly help with diagnosis. Ultrasound scan will show an echogenic fluid accumulation in the vagina that can extend to uterus. In low-resources setting like ours, however, imaging modalities may not be available [9,10].
Although the presentation may be non-specific, once diagnosed, the treatment of imperforate hymen is either hymen sparing hymenotomy or non-sparing hymenectomy (excision of the hymenal tissue). The standard surgical procedure is hymenectomy using cruciate, T, plus, or X shaped (at 2-, 4-, 8-, and 10-o'clock positions) incisions and removal of excess hymenal tissue [11]. However, virginity sparing hymenotomy methods that involve application of a simple vertical incision without excising the hymen [2] or placement of a Foley catheter inserted in the vaginal introitus for 2 weeks [12] have also shown good outcome. One major advantage of a X shaped incision is the reduced risk of injury to the urethra but the disadvantage is the increased risk of refusion especially if suturing of the hymeneal edges is not performed, although the overall rate is generally low at 1% [13]. The procedure should be done with a urinary catheter in situ to prevent iatrogenic damage to the urethra. Application of uterine pressure to expel more blood is not advised as this practice is associated with retrograde flow of blood through the salpinx which could cause endometriosis and tubal adhesions. A laparoscopy may be needed for diagnosing intra-abdominal complications such as ruptured salpinx [14]. Finally, needle aspiration of hematocolpos is discouraged due to the risk of infection and pyocolpos formation.
Important and also actionable clinical implication of this case is improving accurate and timely diagnosis of imperforate hymen, particularly in those with atypical presentation. This calls for thorough genitourinary examination in girls of all ages from birth through the onset of menarche. Therefore, by performing examinations and by promptly diagnosing and treating imperforate hymen,  we can prevent the incidence of obstructive symptoms and the associated complications due to delayed care.
In conclusion, this case suggests that imperforate hymen can mimic acute appendicitis. Gynecological history taking and careful external genital and introitus examination should be recommended in premenarchal girls presenting with abdominal pain. Imaging or laboratory studies are usually not indicated for a classical presentation of imperforate hymen. However, if a patient or parent refuses genital examination, imaging studies can greatly help with diagnosis.

Funding
No funding for this study.

Ethical approval
This article was approved by the ethics committee of the Eastern Regional Hospital, Koforidua, Ghana.

Consent
Written informed consent was obtained from the patient's parents as patient is minor, for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.
Author's contribution Paddy Ssentongo: Concept and design of study, data collection, data interpretation and analysis, drafting, revision, approval of final manuscript.
Foster Amponsah: Study design, performed surgery, data collection, revision, approval of final manuscript.
Anna Ssentongo: Data Collection, revision, approval of final manuscript.
Seth Hansen-Garshong: Data collection, performed surgery, revision, approval of final manuscript.
John Oh: Data interpretation, manuscript revision and final approval.

Registration of research studies
Not applicable to our manuscript.

Provenance and peer review
Not commissioned, externally peer-reviewed.

Declaration of Competing Interest
None declared. The authors have no financial, consultative, institutional, and other relationships that might lead to bias or conflict of interest.