Endometriosis is a disease in which endometrial glands and stroma implant and grow in areas outside the uterus. Endometriosis thus commonly cause significant morbidity among women of reproductive age group.[17, 18] Endometriosis is diagnosed in women who are of 12–80 years in age, and the average age at diagnosis is said to be approximately 28.[19] Our patient was 46 years. Women who are caucasians appear to be more likely to suffer from endometriosis than African Americans or Asians.[20] There are several types of endometriosis, these include Ovarian, peritoneal, deep infiltrating (DIE) and endometriosis of other locations, which is where umbilical endometriosis belongs.[21] Endometriosis occurring outside the pelvis is a rare phenomenon. Literature data provide information on respiratory endometriosis, pericardial endometriosis and endometriosis in a scar after surgery with laparotomy access. [22, 23]
The commonest locations of endometriosis are the ovaries (up to 88% of all cases), followed by the appendix, intestine, cervix, omentum and skin.[24] 70% of cutaneous endometriosis, more frequently are secondary, which follow previous abdominopelvic surgery, but can appear spontaneously (30%), when they occur in the absence of prior surgery.[24] When there is primary endometriosis, it appears most commonly on the umbilicus, followed by the inguinal region [24, 25, 26] and these are cases in which the lateral abdominal wall is involved.[27, 28] Our patient had primary umbilical endometriosis because she did not have any prior abdominal surgery.
The pathogenic mechanism of cutaneous endometriosis is still in the realm of speculation. It has been thought to arise from iatrogenic implantation for example following previous surgeries or from haematogenous or lymphatic metastasis of endometrial tissues[24, 29]. In the case of our patient, the umbilical endometriosis may have arisen most likely from haematogenous or lymphogenous metastasis of endometrial tissue. Spontaneous endometriosis is said to be associated with more severe pelvic disease than scar endometriosis. There were moderate pelvic adhesion seen in our patient which made the exteriorization of the uterus and the passage of a Foley catheter tourniquet at the Cervico- isthmic junction and at the base of the broad ligament impossible. Umbilical endometriosis could present with symptoms such as swelling at the umbilicus,Umbilical swelling that correlates with the menstrual cycle, and bleeding from the umbilicus which is usually cycle, but some patients are asymptomatic. [24, 29, 30] Our patient presented with a growth at the umbilicus and cyclical bleeding at the umbilicus. More often than not Umbilical endometriosis is associated with pelvic endometriosis with complaint of dysmenorrhea, dyspaurenia, or pain while defaecating.[24] Our patient had pelvic endometriosis as was evidenced by the finding of moderate pelvic adhesion at surgery, as such she also presented with symptoms of dysmenorrhea and deep dyspaurenia. On examination of the abdomen, umbilical endometriosis presents as a rubbery or firm nodule, and it’s size may vary from several millimeters to 6cm. [24, 29, 30] Our patient had a 3cm x 3cm nodule at the umbilicus which was dark brown in colour, with hyperpigmented areas, it was non-tender. Even though umbilical endometriosis can be suspected based on the clinical presentation, but confirming the diagnosis by histopathologic analysis is good clinical practice.[31] It is also vital to differentiate umbilical endometriosis from the other metastatic tumours of the umbilicus which are well known as sister Mary Joseph nodule (SMJN).[26]
The golden standard for the diagnosis of umbilical endometriosis is histopathological examination, however diagnostic tools such as ultrasound, Magnetic Resonance Imaging (MRI) or CT Scan can be helpful. [31] Our patient had an abdominal pelvic ultrasound scan which diagnosed umbilical endometriosis and multiple uterine fibroids. She also had an abdominal CT Scan which diagnosed umbilical endometriosis and multiple leiomyomas. We do not have an MRI in our hospital and it’s also quite expensive in other facilities that has it within our locality as such it was not done. Transcutaneous ultrasound scan ,MRI or CT Scan can be helpful in investigating the relationship of the nodule with the surrounding tissue and also helps to differentiate between other umbilical lesions such as umbilical hernia.[32, 33, 34] Fine needle aspiration cytology can be supplementary, but results have been reported to be inconclusive in as high as 75% of cases.[35] A high level of tumour makers such as CEA and CA 125 may raise the suspicion of concomitant pelvic endometriosis.[8] In our case, the typical presentation along with an USS and CT Scan revealing no invasion in the underlying structures was found sufficient to establish a tentative diagnosis in order to initiate treatment.
When considering a diagnosis of umbilical endometriosis, the following differentials should be taken into account, melanocyte naevus, endosalpingiosis presenting as periumbilical papules, pyogenic/foreign body granuloma, umbilical polyp, seborrheic keratosis, epithelial inclusion cyst, desmoid tumour, haemangioma, keloid, omphalltis, umbilical hernia and granular cell tumour.[32] The following should also be ruled out, primary or secondary neoplasms, such as melanoma or sister Mary Joseph’s nodule. [32, 33, 36, 37, 38] The risk of malignancy in cases of umbilical endometriosis is quite low, only three cases have been reported to be associated with malignancy.
Umbilical endometriosis can be managed surgically or medically but surgical management is the preferred. Our patient had radical Omphalectomy at the same that she had a midline abdominal incision for abdominal myomectomy. At the time of the radical omphalectomy, the margin of 1cm was respected, she also had a repair of the underlying fascia. The literature reports a 13–15% incidence of simultaneous pelvic endometriosis presence.[39] At the time of the laparotomy to perform abdominal myomectomy there were no obvious pelvic endometriotic deposits, however the finding of moderate pelvic adhesions and some red cell pigments that were lying freely in her pelvis, suggests that she also had pelvic endometriosis.
Medical treatment is still a subject of debate. Medical treatment is intended to ameliorate the symptoms by reducing the size of the umbilical nodule, thereby limiting the amount of specimen to be excised and reducing angiogenesis. The overall results from medical treatment of umbilical endometriosis is said to be poor due to the relatively low levels of oestrogen receptors found in cutaneous endometriotic lesions. [34, 40, 41] The following medical agents have been used to treat umbilical endometriosis: Danazol or GnRH analogues, progesterone and oral contraceptives. Our patient was not offered medical treatment; this was because she had multiple uterine fibroids, which were also an indication for abdominal myomectomy. The prognosis of umbilical endometriosis is good if an optimal and complete surgical excision is achieved, like in our case. Our case was uncommon in that it was in association with an enlarged uterus of about 16 week’s equivalent of gestational age, due to multiple uterine fibroids (a total of 13 uterine fibroid nodules were enucleated at surgery). The correlation of the pathogenesis between the endometriosis and the uterine fibroids was unclear. The genetic foundations of both uterine fibroids and endometriosis are yet to be fully and clearly understood, but recent evidence seems to suggest common underpinings.[42] Recent evidence from classic and genetic epidemiology also points to an association of both conditions. [15, 16] Our patient had umbilical endometriosis coexisting with multiple uterine fibroids. Surgery is a treatment option for both umbilical endometriosis and uterine fibroids. The aim of the surgical modality of treatment for umbilical endometriosis is to relieve pain and the cyclical bleeding during menstruation. This is achieved by removing all visible lesions or signs of the disease. [43] The symptoms of uterine fibroids such as heavy menstrual bleeding, pain, pressure and reduced fertility can also be improved by surgery. 44 Our patient had radical omphalectomy and abdominal myomectomy and her symptoms were proven to have been relieved during her follow-up visits.