Umbilical endometriosis: A rare diagnosis in plastic … · Umbilical endometriosis: A rare...

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Can J Plast Surg Vol 18 No 4 Winter 2010 147 147 Umbilical endometriosis: A rare diagnosis in plastic and reconstructive surgery A Daniel Malebranche MSc, Kevin Bush MD FRCSC University of British Columbia and Vancouver General Hospital, Vancouver, British Columbia Correspondence: Dr Kevin Bush, Faculty of Plastic Surgery, University of British Columbia, 808–1200 Burrard Street, Vancouver, British Columbia V6Z 2C7. Telephone 604-806-3676, fax 604-806-0926, e-mail [email protected] E ndometriosis is a disease characterized by the presence of uter- ine tissue outside the endometrium and myometrium (1). It can clinically manifest with dysmenorrhea, dyspareunia, cycli- cal or noncyclical pelvic pain and subfertility. Occurring in 8% to 15% of menstruating women (2), it most commonly pres- ents in the ovaries, uterine ligaments, rectovaginal septum and peritoneum (3). Endometriosis can less commonly pres- ent in other sites such as the intestines, appendix, lungs and kidneys (4). The cutaneous variant of endometriosis is much rarer and has been reported to account for approximately 1% of all cases (5). Cutaneous endometriosis is most prevalent at sites of surgical scars such as those from caesarean sections, episiotomies, hysterectomies, laparoscopies and other abdom- inal surgeries (2-4,6,7). According to Friedman and Rico (6), umbilical endometriosis with no previous abdominal or pelvic surgery is even less common. Patients with cutaneous endometriosis typically present to the gynecologist and the dermatologist. If they have a history of pelvic or abdominal surgery, then they can also present to the general surgeon. There have been only 81 published reports of ‘umbilical endometriosis’ in the world between 1953 and 2008. Although patients with umbilical endometriosis infre- quently present to the plastic surgeon, we present such a case and suggest that it be kept on the plastic surgeon’s differential diagnosis for umbilical lesions. CASE PRESENTATION A 35-year-old nulligravid woman presented with a six-month history of a firm, cyclically swelling lesion developing in her periumbilical region. Measuring approximately 2.5 cm, it extended into the deeper tissues and was fixed to the under- lying abdominal wall (Figure 1). The patient had none of the signs characteristic of pelvic endometriosis except dysmenor- rhea and a one-year history of infertility. There was no dys- pareunia, intermenstrual bleeding or postcoital spotting. Her medical history including surgery and review of systems was unremarkable. The patient was not taking any medication and had a calculated body mass index of 24 kg/m 2 . She took Depo-Provera (Pfizer Inc, USA) from 2001 to 2004, and an oral contraceptive from 1988 to 2000. Biopsy of the lesion revealed umbilical endometriosis (grade IV). Follow-up laparoscopy revealed superficial endo- metriosis on the bladder, posterior uterus, both ovaries and the sigmoid colon. Endometriomas were also found in both ovaries. Both fallopian tubes were patent to instillation of methylene blue. Monopolar cautery with coagulating current was applied to all areas of endometriosis on the bladder, posterior uterus and superficial ovaries. Ovarian endometriomas and sigmoid endo- metriosis were not treated. Surgical excision of the umbilicus and reconstruction was performed using a purse-string suture technique (Figure 2). DISCUSSION Endometriosis infrequently presents on the skin, especially in the absence of pelvic or abdominal surgical procedures (6). The disease commonly presents with dysmenorrhea, dyspareunia, cyclical or noncyclical pelvic pain and subfertility (5); however, CASE REPORT ©2010 Pulsus Group Inc. All rights reserved AD Malebranche, K Bush. Umbilical endometriosis: A rare diagnosis in plastic and reconstructive surgery. Can J Plast Surg 2010;18(4):147-148. Umbilical endometriosis infrequently presents to the plastic surgeon. As such, the diagnosis is difficult to make because it is often overlooked. The current report presents a 35-year-old nulligravid woman with a six-month history of a firm, cyclically swelling lesion in her umbilical region. None of the signs characteristic of pelvic endometriosis except dysmenorrhea and a one-year history of infertility were present. Biopsy of the lesion revealed umbilical endometriosis (grade IV), and laparoscopy uncovered extensive disease. Monopolar cautery with coagulating current umbilical excision and reconstruction with a purse-string suture was used. The results under- score the importance of a broad differential diagnosis for an umbilical lesion in a middle-age woman. They also highlight the importance of early recognition and appropriate surgical intervention to minimize morbidity and mortality associated with umbilical endometriosis. Key Words: Plastic surgery; Reconstruction; Umbilical endometriosis Endométriose ombilicale : Diagnostic rare en chirurgie plastique et de reconstruction L’endométriose ombilicale est une entité rare en chirurgie plastique. À ce titre, le diagnostic est difficile à poser parce qu’il passe souvent inaperçu. Le présent rapport présente le cas d’une nullipare de 35 ans présentant une lésion ferme, cycliquement œdémateuse depuis six mois à la région ombilicale. La patiente ne présentait aucun des signes caractéristiques de l’endométriose pelvienne, à l’exception de dysménorrhée et d’antécédents d’infertilité depuis un an. La biopsie de la lésion a révélé une endométriose ombilicale (de grade IV) et la laparoscopie a dévoilé une maladie étendue. L’approche a été une cautérisation monopolaire avec excision ombilicale par courant coagulant suivie de reconstruction par suture en bourse. Les résultats rappellent l’importance d’établir un diagnostic différentiel élargi en présence de lésions ombilicales chez une femme d’âge moyen. Ils soulignent en outre l’importance de confirmer le diagnostic et d’appliquer la chirurgie appropriée sans tarder pour réduire la morbidité et la mortalité associées à l’endométriose ombilicale.

Transcript of Umbilical endometriosis: A rare diagnosis in plastic … · Umbilical endometriosis: A rare...

Page 1: Umbilical endometriosis: A rare diagnosis in plastic … · Umbilical endometriosis: A rare diagnosis in plastic and reconstructive surgery A Daniel Malebranche MSc, Kevin Bush MD

Can J Plast Surg Vol 18 No 4 Winter 2010 147147

Umbilical endometriosis: A rare diagnosis in plastic and reconstructive surgery

A Daniel Malebranche MSc, Kevin Bush MD FRCSC

University of British Columbia and Vancouver General Hospital, Vancouver, British ColumbiaCorrespondence: Dr Kevin Bush, Faculty of Plastic Surgery, University of British Columbia, 808–1200 Burrard Street, Vancouver,

British Columbia V6Z 2C7. Telephone 604-806-3676, fax 604-806-0926, e-mail [email protected]

Endometriosis is a disease characterized by the presence of uter-ine tissue outside the endometrium and myometrium (1). It

can clinically manifest with dysmenorrhea, dyspareunia, cycli-cal or noncyclical pelvic pain and subfertility. Occurring in 8% to 15% of menstruating women (2), it most commonly pres-ents in the ovaries, uterine ligaments, rectovaginal septum and peritoneum (3). Endometriosis can less commonly pres-ent in other sites such as the intestines, appendix, lungs and kidneys (4). The cutaneous variant of endometriosis is much rarer and has been reported to account for approximately 1% of all cases (5). Cutaneous endometriosis is most prevalent at sites of surgical scars such as those from caesarean sections, episiotomies, hysterectomies, laparoscopies and other abdom-inal surgeries (2-4,6,7). According to Friedman and Rico (6), umbilical endometriosis with no previous abdominal or pelvic surgery is even less common.

Patients with cutaneous endometriosis typically present to the gynecologist and the dermatologist. If they have a history of pelvic or abdominal surgery, then they can also present to the general surgeon. There have been only 81 published reports of ‘umbilical endometriosis’ in the world between 1953 and 2008. Although patients with umbilical endometriosis infre-quently present to the plastic surgeon, we present such a case and suggest that it be kept on the plastic surgeon’s differential diagnosis for umbilical lesions.

Case presentationA 35-year-old nulligravid woman presented with a six-month history of a firm, cyclically swelling lesion developing in her

periumbilical region. Measuring approximately 2.5 cm, it extended into the deeper tissues and was fixed to the under-lying abdominal wall (Figure 1). The patient had none of the signs characteristic of pelvic endometriosis except dysmenor-rhea and a one-year history of infertility. There was no dys-pareunia, intermenstrual bleeding or postcoital spotting.

Her medical history including surgery and review of systems was unremarkable. The patient was not taking any medication and had a calculated body mass index of 24 kg/m2. She took Depo-Provera (Pfizer Inc, USA) from 2001 to 2004, and an oral contraceptive from 1988 to 2000.

Biopsy of the lesion revealed umbilical endometriosis (grade IV). Follow-up laparoscopy revealed superficial endo-metriosis on the bladder, posterior uterus, both ovaries and the sigmoid colon. Endometriomas were also found in both ovaries. Both fallopian tubes were patent to instillation of methylene blue.

Monopolar cautery with coagulating current was applied to all areas of endometriosis on the bladder, posterior uterus and superficial ovaries. Ovarian endometriomas and sigmoid endo-metriosis were not treated. Surgical excision of the umbilicus and reconstruction was performed using a purse-string suture technique (Figure 2).

DisCussionEndometriosis infrequently presents on the skin, especially in the absence of pelvic or abdominal surgical procedures (6). The disease commonly presents with dysmenorrhea, dyspareunia, cyclical or noncyclical pelvic pain and subfertility (5); however,

CAse report

©2010 Pulsus Group Inc. All rights reserved

aD Malebranche, K Bush. umbilical endometriosis: a rare diagnosis in plastic and reconstructive surgery. Can J plast surg 2010;18(4):147-148.

Umbilical endometriosis infrequently presents to the plastic surgeon. As such, the diagnosis is difficult to make because it is often overlooked. The current report presents a 35-year-old nulligravid woman with a six-month history of a firm, cyclically swelling lesion in her umbilical region. None of the signs characteristic of pelvic endometriosis except dysmenorrhea and a one-year history of infertility were present. Biopsy of the lesion revealed umbilical endometriosis (grade IV), and laparoscopy uncovered extensive disease. Monopolar cautery with coagulating current umbilical excision and reconstruction with a purse-string suture was used. The results under-score the importance of a broad differential diagnosis for an umbilical lesion in a middle-age woman. They also highlight the importance of early recognition and appropriate surgical intervention to minimize morbidity and mortality associated with umbilical endometriosis.

Key Words: Plastic surgery; Reconstruction; Umbilical endometriosis

endométriose ombilicale : Diagnostic rare en chirurgie plastique et de reconstruction

L’endométriose ombilicale est une entité rare en chirurgie plastique. À ce titre, le diagnostic est difficile à poser parce qu’il passe souvent inaperçu. Le présent rapport présente le cas d’une nullipare de 35 ans présentant une lésion ferme, cycliquement œdémateuse depuis six mois à la région ombilicale. La patiente ne présentait aucun des signes caractéristiques de l’endométriose pelvienne, à l’exception de dysménorrhée et d’antécédents d’infertilité depuis un an. La biopsie de la lésion a révélé une endométriose ombilicale (de grade IV) et la laparoscopie a dévoilé une maladie étendue. L’approche a été une cautérisation monopolaire avec excision ombilicale par courant coagulant suivie de reconstruction par suture en bourse. Les résultats rappellent l’importance d’établir un diagnostic différentiel élargi en présence de lésions ombilicales chez une femme d’âge moyen. Ils soulignent en outre l’importance de confirmer le diagnostic et d’appliquer la chirurgie appropriée sans tarder pour réduire la morbidité et la mortalité associées à l’endométriose ombilicale.

Page 2: Umbilical endometriosis: A rare diagnosis in plastic … · Umbilical endometriosis: A rare diagnosis in plastic and reconstructive surgery A Daniel Malebranche MSc, Kevin Bush MD

Malebranche and Bush

Can J Plast Surg Vol 18 No 4 Winter 2010148

these findings may also be absent (8). The patient in the current report presented with a firm umbilical nodule accompanied by dysmenorrhea and a one-year history of subfertility following cessation of oral contraception. Because oral progesterone is commonly used in the treatment of endometriosis, it is not sur-prising that the symptoms did not present until she wanted to conceive and discontinued taking such therapies.

The differential diagnosis for umbilical lesions includes pyogenic granuloma, umbilical polyp, melanocytic nevus, seb-orrheic keratosis, epithelial inclusion cyst, desmoid tumour, hemangioma and granular cell tumour (9). Other differential diagnoses can include umbilical hernia, omphalitis, keloid and foreign body granuloma (5). Malignant lesions such as melan-oma, adenocarcinoma, squamous and basal cell carcinoma, and metastases from the gastrointestinal tract should be ruled out (9). Endometriosis, therefore, is not frequently high on the plastic surgeon’s differential diagnosis for solitary umbilical nodules.

Several theories have postulated how endometrial tissue migrates from the uterus to the skin. According to Steck and Helwig (10), transportation theories assert that the tissue is carried from the uterus to an abnormal location where it prolif-erates such as the cells that shed during menstruation, which are then regurgitated through the fallopian tubes and implanted on peritoneal surfaces; metastasis via lymphatic and or vascular channels; and lesions surrounding surgical scars that result

from transplantation. Local origin theories, by contrast, sug-gest that some degree of pluripotentiality is retained by cells in various locations such as the pelvic organs and peritoneum. More recent research has deemed the transport theory as being more widely accepted. Patients with mechanically abnormal fallopian tubes, for example, have increased rates of endometriosis (1). The patient in the present report did not undergo any previous pelvic or abdominal surgeries; thus ruling out iatrogenic etiologies.

Although the etiology is not completely understood, umbil-ical endometriosis is rare. Surgical specialists including plastic surgeons should be aware of umbilical endometriosis when confronted with umbilical lesions. Surgical history is not needed for the patient to present with umbilical endometriosis. Similarly, the typical features of endometriosis such as dysmen-orrhea, dyspareunia, infertility and cyclic pain do not need to be present at the initial stage. Endometriosis can present after the cessation of oral contraceptive therapies and can incur significant morbidity if left untreated. Electrocautery of pelvic disease with surgical excision of the umbilical nodule is the recommended management approach. Recurrence after suffi-cient excision is rare as is malignant transformation, although cases have been documented (11). Therefore, to prevent unnecessary morbidity and mortality, prompt diagnosis and subsequent surgical treatment of umbilical endometriosis is of paramount importance.

Figure 1) Umbilical endometriosis preoperatively Figure 2) Excised umbilical endometriosis and reconstruction using a purse-string suture technique

reFerenCes1. Clement PB. Pathology of endometriosis. Pathol Annu

1990;25:245-95.2. Scholefield HJ, Sajjad Y, Morgan PR. Cutaneous endometriosis and

its association with caesarean section and gynecological procedures. J Obstet Gynaecol 2002;22:553-4.

3. Luisi S, Gabbanini M, Sollazzi S, et al. Surgical scar endometriosis after cesarean section: A case report. Gynecol Endocrinol 2006;22:284-5.

4. Gaunt A, Heard G, McKain ES, et al. Cesarean scar endometrioma. Lancet 2004;364:368

5. Albrecht LE, Tron V, Rivers JK. Cutaneous endometriosis. Int J Dermatol 1995;34:261-2.

6. Friedman PM, Rico MJ. Cutaneous endometriosis. Dermatol Online J 2000;6:8.

7. Von Stemm AMR, Meigel WN, Scheidel P, et al. Umbilical endometriosis. J Eur Acad Dermatol Venereol 1999;12:30-2.

8. Michowitz M, Baratz M, Stovorosky M. Endometriosis of the umbilicus. Dermatologica 1983;167:326-30.

9. Powell FC, SU WP. Dermatoses of the umbilicus. Int J Dermatol 1988;27:150-6.

10. Steck WD, Helwig EB. Cutaneous endometriosis. JAMA 1965;191:167-70.

11. Scully RW, Richardson GS, Barlow JF. The development of malignancy in endometriosis. Clin Obstet Gynecol 1966;9:384-411.