Case
She was a 30-year-old nulliparous woman with a history of laparoscopic resection of a urachal cyst one year prior. Pathology confirmed the diagnosis. She presented to our department with a three-month history of an umbilical nodule that became painful and darkened during her menstrual periods. Her cycles were regular, and she had no known history of pelvic endometriosis. On physical examination, a firm, hyperpigmented 2 cm nodule was observed within the umbilicus. The lesion was painful, irreducible upon palpation, and showed no signs of infection ( Fig. 1 ). Fig. 1 Shows hyperpigmented 2 cm nodule within the umbilicus with no signs of infection (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) Fig. 1
Shows hyperpigmented 2 cm nodule within the umbilicus with no signs of infection (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Abdominal ultrasound revealed a hypoechoic, non-vascularized subcutaneous mass in the umbilical region, measuring 20 mm ∗ 25 mm. CT scan of the abdominopelvic region showed no pelvic endometriotic lesions, but it revealed a small umbilical tissue nodule measuring 23 mm ∗ 25 mm ( Fig. 2 ). Fig. 2 Shows an umbilical nodule measuring 23 ∗ 25 mm (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) Fig. 2
Shows an umbilical nodule measuring 23 ∗ 25 mm (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Given the cyclical pattern of umbilical pain and bleeding aligning with the patient's menstrual periods, a diagnosis of umbilical endometriosis was considered. As a result, surgical excision was planned.
Under general anesthesia, the umbilical mass was excised with a 1 cm margin, requiring full-thickness removal of the umbilicus. Given the aesthetic importance of the umbilicus, immediate reconstruction was performed using a modified purse-string technique with deep anchoring sutures to the linea alba ( Fig. 3 ). Fig. 3 Intraoperative view after tightening of the outer and inner purse-string sutures and placement of the outer fixation suture (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) Fig. 3
Intraoperative view after tightening of the outer and inner purse-string sutures and placement of the outer fixation suture (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
The postoperative period was uneventful. The patient reported minimal pain and was discharged on postoperative day 1. Histopathological analysis confirmed the diagnosis of endometriosis with no malignant features ( Fig. 4 ). Fig. 4 (A) High-power view highlighting the endometrial glands lined by columnar epithelium and surrounding stromal cells (H&E, ×200), (B) low-power view showing ectopic endometrial glands and stroma embedded in fibrous tissue (H&E, ×100). Fig. 4
(A) High-power view highlighting the endometrial glands lined by columnar epithelium and surrounding stromal cells (H&E, ×200), (B) low-power view showing ectopic endometrial glands and stroma embedded in fibrous tissue (H&E, ×100).
She was referred to a gynecologist for further evaluation and started on hormonal therapy using GnRH agonists, along with non-steroidal anti-inflammatory drugs (NSAIDs) to manage symptoms and lower the risk of recurrence.
Follow-up evaluations at 3 and 12 months postoperatively revealed that the reconstructed umbilicus retained a satisfactory cosmetic outcome, with no signs of symptom recurrence or lesion regrowth. The patient was very pleased with the cosmetic result of the umbilical reconstruction ( Fig. 5 ). Fig. 5 Aesthetically pleasing result 1 year after operation with natural depth of the reconstructed new navel crater (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.) Fig. 5
Aesthetically pleasing result 1 year after operation with natural depth of the reconstructed new navel crater (red arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Author
Sebai Amine: conceptualization, data curation, redaction.
Zaiem Aida: conceptualization, data curation, redaction.
Atri Souhaib: conceptualization, data curation, redaction, project manager.
Ben Mahmoud Ahmed: resources, visualization.
Khadhar Aida: Supervision, validation, visualization.
Kacem Montassar: Supervision, validation, visualization.
Consent
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Ethical
Our institution (Medicine Faculty of Tunis, Department of surgery la Rabta hospital) require no ethics approval for case reports reporting on a single case.
Research
Not applicable. This is not a research study. It is report about one case.
Guarantor
Sebai Amine.
Zaiem Aida.
Conclusion
This case underlines the importance of recognizing umbilical endometriosis as a differential diagnosis of umbilical masses, particularly in women of reproductive age with cyclical symptoms. Complete excision followed by immediate umbilical reconstruction is a viable approach, offering excellent cosmetic and functional outcomes.
Discussion
Endometriosis is defined as the presence of functional endometrial glands and stroma outside the uterine cavity [ 5 ]. Among its rare forms, cutaneous endometriosis is notable for most frequently affecting the umbilicus, making it the most common site of extra-pelvic skin involvement [ 6 ]. First described by Rokitansky in 1860, umbilical endometriosis refers specifically to ectopic endometrial tissue found within the skin of the umbilical region [ 5 ]. Although benign, this condition is rare, with the average age of onset reported between 28 and 33 years [ 7 ].
Pathophysiologically, endometriosis is a chronic and likely multifactorial condition, the precise mechanisms of which remain only partially understood. To explain its origin, several theories have been proposed. Most notably, Sampson's theory suggests that retrograde menstruation allows viable endometrial cells to flow backward through the fallopian tubes, implanting onto pelvic organs and peritoneal surfaces. In contrast, Meyer's theory posits a metaplastic transformation of coelomic epithelium into endometrial cells, influenced by factors such as infection, toxins, or hormonal changes [ 8 ].
Additionally, the lymphatic and vascular metastasis theory suggests that endometrial cells can disseminate through the bloodstream or lymphatic system. This mechanism offers a plausible explanation for distant spread, including to the umbilicus, retroperitoneal space, and lower extremities, due to the anatomical connectivity of these systems [ 9 ].
In the case presented, the patient had undergone surgical excision of a urachal cyst one year prior. This previous intervention may have facilitated the migration and implantation of endometrial cells at the umbilical scar site, thus supporting a iatrogenic pathway of spread.
Clinically, umbilical endometriosis most commonly presents with a painful umbilical nodule, often accompanied by swelling and a cyclic pattern of symptoms related to menstruation. Lesions may appear hyperpigmented and dome-shaped, although bleeding during menstruation is observed in less than half of cases [ 10 ]. Pain likely results from inflammatory responses and hormonal fluctuations, and while some patients are asymptomatic, lesion sizes can range from 0.5 to 3 cm [ 11 ]. It is worth noting that cases have also been described in postmenopausal women, during pregnancy, and in association with uterine fibroids. Although umbilical nodules may raise suspicion for malignancy, the vast majority remain benign.
In our patient's case, she presented with a 2 cm firm, hyperpigmented umbilical nodule that became painful during menstruation. The lesion was consistent with typical clinical findings of umbilical endometriosis.
Diagnosis relies heavily on clinical history and physical examination, yet the differential diagnosis is broad. It includes conditions such as umbilical hernia, pyogenic granuloma, keloid, melanoma, and both primary and metastatic carcinomas, such as Sister Mary Joseph's nodule. Importantly, umbilical endometriosis should be considered even in the absence of pelvic endometriosis [ 12 ].
Imaging studies, including ultrasound, CT, and MRI, are valuable for assessing the depth and extent of lesions and ruling out other conditions [ 13 ]. However, these modalities are not specific for endometriosis. Therefore, histological examination remains the gold standard for diagnosis, confirming the presence of endometrial glands and stroma, often accompanied by hemosiderin deposits and inflammatory changes. Immunohistochemical staining (e.g., CD10) can support diagnosis, especially in gland-poor samples [ 14 ]. Although rare, malignant transformation has been reported and must be ruled out in atypical or recurrent cases [ 15 ].
The treatment of choice is wide surgical excision, ensuring removal of the lesion with clear margins and including any affected peritoneal tissue. When necessary, umbilical reconstruction is performed to restore aesthetics and reduce recurrence risk [ 16 ].
Previous reconstructive techniques, such as the inverted C—V flap, lunchbox approach, and conchal cartilage graft, showed some short-term success, but were not suitable for immediate reconstruction. The “iris” technique, while applicable for immediate repair, is associated with additional scarring [ 17 ].
Umbilical flap techniques use excess umbilical skin and include both single- and double-flap reconstructions. Single-flap methods include triangular island flaps closed in a V—Y fashion, and semicircular flaps shaped into a cone using a wedge excision. Two-flap techniques involve paired flaps such as lateral rhomboids to form an inverted pouch, or a combination of “lazy-M” and “Omega” flaps. Another approach uses a transverse incision to unfold the umbilical skin into two flaps placed into a defect after scar and fat removal. These methods suit cases with redundant umbilical tissue [ 18 ].
By contrast, the purse-string technique has emerged in the literature as a promising method for immediate umbilical reconstruction, offering long-term shape retention. This approach utilizes existing incisions, thereby minimizing scarring and forming a permanent ring structure on the abdominal wall. It is especially beneficial in thin patients, where creating a three-dimensional contour is challenging due to taut skin [ 19 ]. Despite this, in our experience and as reported, aesthetically and clinically satisfactory outcomes were achieved.
Furthermore, pelvic laparoscopic evaluation is recommended to detect other potential endometriotic sites, particularly in symptomatic patients or those with suspected pelvic disease [ 20 ].
Adjunctive hormonal therapy, including oral contraceptives or gonadotropin-releasing hormone agonists, may be used either preoperatively to reduce lesion size or postoperatively to manage residual symptoms and lower recurrence risk. Nevertheless, supporting evidence for long-term benefit is limited [ 21 ]. In our patient, hormonal treatment was initiated postoperatively due to persistent pain, although its effectiveness over time remains uncertain.
Finally, regardless of the chosen treatment modality, patients should be informed about the risk of recurrence and the potential for malignant transformation. Regular long-term follow-up is essential. In cases of recurrence, repeat histological analysis is critical to exclude malignancy and guide further management.
Introduction
Endometriosis is a chronic gynecological condition marked by the presence of endometrial-like tissue outside the uterus. It affects around 10 % of women at reproductive age [ 1 ].
The disease can be classified as primary, occurring spontaneously, or secondary, following surgical procedures. While it primarily affects pelvic organs, extrapelvic involvement occurs in about 12 % of cases, with the umbilicus being a rare site [ 2 ]. Umbilical endometriosis, or Villar's nodule, accounts for 0.5 %–1 % of extragenital cases [ 3 ].
This report presents a case of a secondary umbilical endometriosis and outlines its clinical features, diagnosis, and surgical management with umbilical reconstruction.
This work has been reported in line with the SCARE criteria [ 4 ].
Coi Statement
All authors declare that they have no conflicts of interest.
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