Surgical management of umbilical endometrioma within an umbilical hernia

A nulliparous woman in her 40s presented with umbilical pain and bleeding with menses. Her medical history was significant for hypertension, body mass index (BMI) of 37 kg/m 2 , uterine fibroids, abnormal uterine bleeding and dyschezia. Surgical history was significant for two abdominal myomectomy procedures through a Pfannenstiel incision with breach of the endometrium in both surgeries. Pathology revealed fragments of benign leiomyomata with areas of increased cellularity. Examination revealed a protuberant abdomen with a 3 cm blood-filled cyst at the umbilicus consistent with umbilical endometriosis (see figure 1 ). Bimanual examination showed a small uterus and no adnexal masses or nodularity on the uterosacral ligaments, rectovaginal septum, parametrium or cul-de-sac.

The patient was discharged home the same day of surgery. Surgical pathology revealed cystic endometriosis within subcutaneous tissue, consistent with umbilical endometrioma. Her postoperative course was complicated by a surgical site infection. On postoperative day 33, the patient began a course of oral antibiotic therapy. Medical management failed initially. She was then treated successfully inpatient with intravenous vancomycin and piperacillin-tazobactam therapy and ultrasound-guided percutaneous drainage. Deep wound culture grew coagulase-negative Staphylococcus and rare Corynebacterium jeikeium sensitive to vancomycin. At the time of this writing, the patient has had a 2-year recurrence-free interval.

Before referral for surgical management, the patient attempted medical therapy with medroxyprogesterone acetate without relief and was not a candidate for combined oral contraceptives due to hypertension and elevated BMI. The patient, therefore, underwent umbilectomy, excision of umbilical endometriosis, lysis of adhesions and umbilical hernia repair under general anaesthesia. A circumferential incision was made around the umbilicus using a scalpel. This was taken down to the subcutaneous tissue using electrocautery. The endometrioma was densely adherent to the umbilical skin as expected, as well as the hernia sac, the omental fat contents of the umbilical hernia sac and the anterior rectus sheath (see figure 3 ). This lesion was hence excised en masse with a wide margin, resulting in a large defect in the anterior abdominal wall (see figure 4 ). The defect was closed primarily using synthetic mesh and the skin edges were approximated using staples, leaving a subcutaneous drain (see figure 5 ). The specimen was sent to pathology for analysis.

Endometriosis affects about 5% of women of reproductive age and was first coined by Sampson as the presence of endometrial glands and stroma outside the endometrium and myometrium. 1 4 It usually affects the pelvis, including the ovaries, uterosacral ligaments and the pouch of Douglas. Extrapelvic endometriosis is less prevalent and symptomatology varies based on site, making the exact prevalence challenging to estimate. 5 Extrapelvic lesions comprise about 12% of lesions, and abdominal wall endometriosis (AWE) is the most common extrapelvic site. 2 Umbilical endometriosis accounts for about 30–40% of AWE and 0.5–1.0% of all endometriosis and can appear either as a primary lesion in the absence of surgery, also known as Villar’s node after Villar first described it in 1886, or as a secondary lesion arising from subsequent scar tissue after abdominal procedures. 2 5 6 Patients typically present with a red, purple or black umbilical nodule lesion at the umbilicus and swelling colour/consistency change (83.5%), pain (83%) or bleeding (50.9%) from the umbilicus. 7 Diagnosis is primarily made clinically, though MRI can aid in evaluation. Endometriomas appear homogeneously hyperintense on T1-weighted imaging. 8 Histological findings generally include the presence of endometrial glands and stroma. 8 Management can include hormonal or surgical therapy. Surgical therapy is typically recommended and involves wide local excision of umbilical endometriosis. Resection of umbilical endometriosis has many advantages; it has been well studied, clearly establishes a diagnosis of endometriosis and rules out malignancy. Surgical management boasts low recurrence rates, decreases risk of malignant transformation and is the most common treatment modality for umbilical endometriosis in the literature. 19 14 Conversely, medical management has had varying degrees of success. Hormonal options reported in the literature include oral contraceptives, progestins and gonadotropin-releasing hormone analogues. 10 Small studies have demonstrated symptomatic control through oral contraceptive pills, while others have seen incomplete response, which suggests it may not be curative. 5 13 15 Furthermore, there are no sound studies that compare medical to surgical management. 2 11 15 Timely diagnosis and effective treatment can be crucial to improving quality of life and outcomes. In this case, we report a secondary umbilical endometrioma arising from an umbilical hernia in a patient with prior abdominal myomectomy that failed medical management and was successfully treated surgically with excision and simultaneous hernia repair.

We present a case of full thickness umbilical endometriosis presenting in an umbilical hernia sac in a patient who had had two prior myomectomy procedures through a Pfannenstiel incision with breaching of the endometrium. Having failed medical management, the lesion was successfully surgically removed with a 2-year recurrence-free interval to date. The surgery, however, was complicated by a surgical site infection necessitating readmission. Umbilical endometriosis accounts for 0.5–1.0% of all endometriosis and can appear either as a primary lesion in the absence of surgery, also known as Villar’s node, or as a secondary lesion arising from subsequent scar tissue after abdominal procedures. 2 5 6 Abdominal wall endometriosis is reported to have rates of pelvic endometriosis ranging from 13% to 34%, but the exact rate is unknown since the peritoneum is not routinely evaluated during the workup or management of those cases. 16 17 Several theories explaining the pathogenesis of endometriosis exist, including both local implantation via refluxed endometrial cells entering the abdominal cavity through the tubes 1 and dissemination through lymphovascular channels. 18 Although overall a rare condition, patients with obstetric surgical history such as caesarean sections are at risk for umbilical endometriomas. 519 21 It is therefore conceivable that a breach of the endometrium during her two myomectomy procedures may have caused seeding of the umbilical hernia in our patient. Umbilical endometrioma must be differentiated from ventral hernia, suture granulomas, abscess, cyst or lipoma. The initial management is therefore often surgical, especially if there is no known history of pelvic endometriosis. When the diagnosis is suspected, on the other hand, some investigators have attempted medical management for those tumours. 2 22 However, medical management has been infrequently reported, and the studies that investigated medical management have had small sample sizes and did not include long-term follow-up. Additionally, there is not much data specifically comparing medical and surgical management outcomes, which begs the question of whether the role of hormonal therapy should be reserved for postoperative long-term symptom control. 2 5 11 15 Surgical management with wide local excision is recommended, although it lacks long-term efficacy and safety outcome data. 2 11 15 Although our patient has had no recurrence over a 2-year follow-up, the recurrence rate after local resection is reported to be about 10%. 5 The typical excisional technique is the circumferential or ‘stump’ technique, similar to our approach. 2 3 Most resections do not involve entry into the peritoneum, and reconstruction will typically depend on the size of the defect and preference for umbilicoplasty. 23 26 A superior approach for umbilicoplasty has not been identified, and long-term complication rates have yet to be determined and compared. 23 26 Umbilicoplasty methods reported include a four-flap umbilicoplasty, 23 bilateral square teeth flaps, 25 purse string suture technique 24 and a v-y flap technique. 26 The simplest techniques are the purse string method, 24 which does not require defatting of the subcutaneous tissue, and the four-flap technique. 23 Techniques that involve defatting of the subcutaneous tissue include the v-y flap 26 and square teeth 25 techniques, which had low complication rates with adequate depth after healing in all but one reported case ( table 1 ). We did not perform an umbilicoplasty, resulting in a poor inferior cosmetic result. Does not require remaining umbilical tissue Good aesthetic outcome Good for midline vertical incisions Simple to perform Not as aesthetically pleasing Can cause stenosis of the umbilicus with a shallow scar Good for reconstruction Mostly studied in those with the absence of the umbilicus following the repair of a large incisional and umbilical hernia The anterior abdominal wall defect size having resected the entire tumour en masse was 8–10 cm. Therefore, a mesh repair was indicated. 27 The hernia was repaired using synthetic mesh in accordance with Hernia Surge guidance. 28 Postoperative course was complicated by a surgical site infection. Fortunately, this infection resolved with medical management and did not necessitate more invasive management such as surgical debridement or mesh removal, and is doing very well without any long-term complications. The patient was at risk for surgical site infection given her elevated BMI. 28 29 In summary, umbilical endometriosis should enter into the differential diagnosis of umbilical tumours, and the workup should include a search for pelvic endometriosis. Surgical excision in this reported case had a 2-year recurrence-free interval but was complicated by a surgical site infection. Several umbilicoplasty techniques exist for optimal cosmesis. We agree with the European Society of Human Reproduction and Embryology guidelines which state that surgical management is preferred for extrapelvic endometriosis as it removes the symptomatic mass and provides a histopathological diagnosis. 30 Medical management may have a role in the treatment of umbilical endometriosis although further studies in cases of endometriosis in a hernia are warranted, as it may be futile to attempt medical therapy, prolonging time to surgery. The role of medical management after surgery to prevent recurrence also deserves further study especially in the presence of pelvic endometriosis. Recognise the prevalence of extrapelvic endometriosis in reproductive age women. Umbilical endometriosis usually presents with catamenial symptoms and can exist within an umbilical hernia. After complete surgical excision, individualised approaches to umbilical reconstruction based on patient and disease characteristics may be warranted.

MRI showed an umbilical endometrioma (see figure 2 ).