A 14 year old girl presented with severe dysmenorrhea. Her clinical examination findings were normal. She failed to respond to medical treatment with diclofenac and drotaverine. A magnetic resonance imaging scan showed the presence of bicornuate uterus with a single cervix. There was a hemiheatometra on the left side due to noncommunicating horn. However laparoscopy revealed a normal uterus, left lateral wall leiomyoma of the uterine corpus, and pelvic endometriosis.
Severe dysmenorrhea in early teenage is usually due to a mullerian anomaly like a hematometra. Pelvic endometriosis is unusul at this age, but not unknown. Endometriosis can also be present in a case of mullerian anomaly, especially when there is hematometra due to obstruction to the outflow tract. Uterine leiomyoma at this age is very uncommon. Owing to good abdominal wall muscle tone, inability to perform a vaginal examination due to intact hymen and non co-operation of the young girl during examination, a clinical distinction between these conditions is difficult. A case of a young girl with severe dysmenorrhea, diagnosed on magnetic resonance imaging (MRI) to have hemihematometra due to outflow tract obstruction, turned out to be a degenerated leiomyoma of the uterus and pelvic endometriosis.
A 14 year old sexually inactive single girl presented with severe dysmenorrhea for a period of 5 months. She had menarche at the age of 13 years. Her present cycles were every 28-34 days, regular and extremely painful, with moderate flow. She did not respond to oral medication like diclofenac and drotaverine, and had to be hospitalized for parenteral anlgesics during every menstrual flow. Her past surgical and medical history was not contributory. Findings of her general and systemic examination were normal. Abdominal wall tone was good. There was no area of tenderness, guarding or rigidity. Her external genitals were normal and hymen was intact. A rectal examination showed a normal sized uterus. There was no pelvic lump, but some tenderness was felt on the left side of the uterus. She had an MRI scan. It showed a left sided hematometra due to a noncommunicating rudimentary horn of the uterus. (figure 1). There was no evidence of any endometrioma. Counseling was done about the nature of the condition and need for excision of the hemihepatometra, after which the patient and her relatives opted for undergoing a laparoscopy and any additional surgery as required. Her investigations showed that she was fit for anesthesia and surgery. A laparoscopy was performed. It showed a normal sized uterus, a 2 cm diameter leiomyoma anterior to the point of attachment of the left fallopian tube, and the left round ligament passing from the lateral pole of the leiomyoma to the left internal inguinal ring (figure 2). There were endometriotic spots in the anterior wall of the pouch of Douglas (figure 3). The leiomyoma was excised. It was degenerated and required sharp dissection for excision. The endometriotic spots were electrocauterized. The patient had an uneventful recovery. Histopathological examination of the mass excised showed a leiomyoma with hyaline degeneration.
Figure 1. MRI of the pelvis, coronal view, showing a normal uterus (white arrow) and hemihematometra (green arrow).
Figure 2. Laparoscopy showing uterus (U), leiomyoma (L), left fallopian tube (LFT) and left round ligament (LRL).
Figure 3. Laparoscopy showing endometriotic spots in the anterior wall of the pouch of Douglas.
The prevalence of a bicornuate uterus is 0.4% in the general population  It is the result of an incomplete lateral fusion of the two Mullerian ducts. Its association with cervical atresia is rare [2,3] That would cause accumulation of menstrual blood in the obstructed horn, resulting in severe dysmenorrhea. The diagnosis is not suspected because the girl continues to menstruate cyclically, the outflow tract of the communicating horn being patent. Occurrence of endometriosis in teenage girls is uncommon. The incidence of endometriosis in teenage girls is 19-73%.[5,6] The incidence of endometriosis is raised by the presence of mullerian anomalies, especially of the obstructive type.[7,8] Uterine leiomyomas are extremely rare in teenage girls. Co-existence of uterine leiomyoma and pelvic endometriosis in teenage girls is very rare.
The case presented here was rare in that there was a leiomyoma of the uterus and pelvic endometriosis together. Furthermore, the leiomyoma had already undergone degeneration, which made it even rarer. The leiomyoma was removed because it could have grown further if left untreated. It was considered to be partly responsible for the pelvic pain too, as was confirmed by the presence of degeneration in the leiomyoma. Usually MRI is considered to be very accurate in assessment of pelvic masses. Unfortunately the leiomyoma in this case was diagnosed as hemihepatometra. The radiological diagnosis was possibly subject to clinical bias and also to rarity of leiomyoma at that age. The diagnosis was evident during laparoscopy, as the medial end of the fallopian tube was posterior to the uterine swelling, while it would have been at its lateral end in case of a hemihematometra. The round ligament was attached to its outer end, while it could have been at its medial margin anteriorly if it were a hemihematometra. It was also important to look for pelvic endometriosis after identification of a uterine lump, as it can be associated with both mullerian anomalies and uterine leiomyomas.
Avoiding statistical bias, careful attention to reporting MRI images, and application of anatomical knowledge while interpreting findings during laparoscopy and/or laparotomy would make the diagnosis accurate in teenage girls with severe dysmenorrhea and uterine swellings.
I thank Dr Sarika Solanke for laparoscopy images.
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Parulekar SV. Hemihematometra Or Leiomyoma? JPGO 2019. Vol 6, No. 8. Available from: https://www.jpgo.org/2019/08/hemihematometra-or-leiomyoma.html