Umbilical endometriosis

INTRODUCTION

Endometriosis is a condition in which endometrial tissue is found in a site other than the lining of the uterus.¹ It occurs in 7%–10% of women of reproductive age.¹ It is usually found in other pelvic organs but also occurs in extrapelvic sites such as the pulmonary tract, gastrointestinal tract, urinary tract, and diaphragm, as well as cutaneous regions like the anterior abdominal wall.^2,3

Umbilical endometriosis, a condition in which endometrial-like tissue is found in the umbilical region, is a rare form of endometriosis. It occurs in 0.5%–1% of all cases of endometriosis.²

It may be primary or secondary. Primary umbilical endometriosis (Villar’s nodule) is believed to result from metaplasia of urachal remnants.⁴ Other causes include genetic predisposition, migration of endometrial cells through the abdominal cavity or lymphatic system, and immunogenic problems. It occurs in the absence of surgery.⁴

Secondary umbilical endometriosis, on the other hand, occurs after surgery.⁴ It is due to the iatrogenic implantation of endometrial cells.Umbilical endometrial-like tissue undergoes hormonal stimulation like normal endometrial tissue and may cause pain, swelling, and bleeding, which are usually cyclical.

Dysmenorrhea and dyspareunia may also occur.

Umbilical endometriosis usually presents as nodules, which are usually 0.5–6 cm in diameter.⁵ Its clinical appearance and symptomatology, however, vary with the depth and calcification of the tumor.⁵ Symptoms may therefore not be present, and the diagnosis may be difficult. Management is usually surgical but may also involve hormonal administration, such as combined oral contraceptive pill administration and gonadotropin releasing hormone analogues. High-frequency ultrasound may also be used in its management.^6,7

We present a case of umbilical endometriosis managed in our facility. To the best of our knowledge, this is the first reported case from our environment. We hope that this will raise awareness of this rare condition among our healthcare workers.

CASE REPORT

A 24-year-old Para 0+1, unmarried student of the local university, presented with a 3-year history of an umbilical mass which was painful and bled cyclically during her menstruation.

The mass had progressively increased in size. There was no associated history of dysmenorrhea, dyspareunia, or pelvic pain. Her menstrual cycle remained normal with a 4-day flow in a regular 28-day cycle. She was presently not menstruating, and there was no family history of endometriosis.

Four years earlier, she had a laparotomy for a ruptured tubal ectopic pregnancy and an uneventful recovery.

On examination, she was not pale, was afebrile, and was anicteric. There was a healthy Pfannenstiel scar and a bluish-black umbilical mass which was about 4 cm × 4 cm in size [Figure 1]. The mass was soft, slightly tender, and not freely mobile. A pelvic examination revealed a normal vulva and vagina and a normal-sized, non-tender, anteverted uterus. There were no adnexal masses. However, though there was no tenderness over the pouch of Douglas, there was thickening of the uterosacral ligaments. A rectal examination revealed no abnormalities.

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Figure 1:

Umbilical mass.

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A full blood count and pelvic ultrasound scan were normal.

An assessment of umbilical endometriosis was made. She was counseled and consented to excision of the umbilical mass in the operation theatre. At surgery, a 4 cm × 4 cm, soft, bluish-black mass was found to be attached to the anterior rectus sheath. The mass was completely excised through an elliptical transverse incision [Figure 2].

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Figure 2:

Excision of the mass.

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The histological findings confirmed umbilical endometriosis. Macroscopy revealed an ellipsoid, firm tissue partly covered by negroid skin, measuring 70 cm × 4.5 cm. The skin was hyperpigmented and polyploid. A cut section showed an ovoid, hemorrhagic, well-circumscribed mass measuring 5.0 cm × 3.5 cm. Microscopy further revealed benign-looking endometrial-type glands mixed with their stroma and lymphocytic aggregates. No malignancy was seen [Figure 3].

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Figure 3:

Histology pictures. Hematoxylin and eosin (40X).

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At follow-up, a week after discharge, the wound had completely healed.

She was happy with its appearance and had no complaints. She subsequently lost to follow-up.

DISCUSSION

Umbilical endometriosis is a rare, usually benign condition that may be primary or secondary in origin.⁸ Though very rare, of all cases of extrapelvic endometriosis, abdominal wall endometriosis is the most common, and umbilical endometriosis is the most frequent anterior wall endometriosis.⁹

Our patient was a 24-year-old, which is within the reproductive age group. This is the age of occurrence of umbilical endometriosis.² It usually presents as a bluish-black nodule, as in this patient, and common symptoms are pain, swelling, and bleeding during menses.¹⁰ These occurred in this patient. However, depending on the depth of involvement of aberrant endometrial tissue, the appearance and symptomatology may differ.⁵ There may be no symptoms; therefore, a high index of suspicion is required in its diagnosis.Its differential diagnosis includes keloids, lipomas, hernias, melanomas and sarcomas, hematomas, and lymphomas.¹⁰

Our patient had a laparotomy for a ruptured ectopic pregnancy 4 years prior. However, the lesion was in the umbilical region, which was far from the Pfannenstiel scar used for the surgery. This suggests primary umbilical endometriosis, though thickened uterosacral ligaments were felt during the vaginal examination and may suggest the presence of pelvic endometriosis. Primary umbilical endometriosis frequently coexists with pelvic endometriosis.⁵

The treatment of umbilical endometriosis is radical surgery with a wide excision of the lesion, including healthy-looking margins.⁵ This was done for this patient and is associated with less risk of recurrence.⁵

Medical treatment methods with various hormones like combined oral contraceptive pills, progestin, and gonadotropin-releasing hormone analogs also exist, but the literature is scanty on their long-term efficacy. A high-frequency ultrasound can also be used in its management.^6,7 However, this is not available in our center.

CONCLUSION

Umbilical endometriosis is a rare, relatively benign condition. A high index of suspicion is necessary in patients who bleed from abnormal sites. This is more suggestive of umbilical endometriosis if cyclical bleeding from other sites occurs during menstruation, as was the situation with our patient.