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Extra genital endometriosis
(supporting documentation)

Endometriosis can be found in almost any tissue in the body apart from the spleen. Symptoms will depend on the site of the disease. Cyclicity of symptoms is usually present, at least in early stages, and may be the only clue which leads to the diagnosis of endometriosis. Treatment will again depend on the site. If complete excision is possible, this is the treatment of choice; when this is not possible, long term medical treatment is necessary. Same principles of medical treatment for pelvic endometriosis will apply for extragenital endometriosis (see section Treatment of Confirmed Disease) (Berqvist 1992; Joseph and Sahn 1996; Jubanyik and Comite1997; Nisolle et al., 2007).

Our knowledge of extragenital endometriosis mainly comes from case series or reports and there are no comparative studies due to relative rarity of this condition. The subject has been reviewed by several authors over the last two decades (Berqvist 1992; Joseph and Sahn 1996; Jubanyik and Comite 1997; Nisolle et al., 2007).

Patients with distant site endometriosis may not be diagnosed for several years due to unfamiliarity of physicians they consult with the diagnosis of endometriosis for their symptoms. Symptoms will depend on the site of the disease. Cyclicity of symptoms is usually present, at least in early stages, and may be the only clue which leads to the diagnosis of endometriosis. Diagnosis is usually made by histological confirmation, this is important to exclude other pathology, particularly malignancy. Additional imaging and endoscopic investigations specific to the location may also be used. Treatment will again depend on the site. In general if complete excision is possible surgery would be the treatment of choice, however when this is not possible long term medical treatment is necessary. Same principles of medical treatment for pelvic endometriosis will apply for extragenital endometriosis (see section Treatment of Confirmed Disease).

Intestinal endometriosis

Bowel endometriosis is reported to be present in 5-40% of patients with pelvic endometriosis. Rectum and sigmoid are the most common sites (up to 95% of cases) and 5-20% of the cases have appendix endometriosis (Jubanyik and Comite 1997). Endometriosis of the small intestine is relatively rare.

Depending on the location some patients with intestinal endometriosis may have no symptoms, but chronic abdominal pelvic pain, dyschezia (pain during defecation during menstrual period), dysmenorrhoea, dyspareunia, tenesmus, constipation or diarrhoea and rectal bleeding are reported by some patients. Diagnosis is usually made at laparoscopy, additional imaging techniques such as MRI, contrast studies or rectosigmoidoscopy may be used. Surgical treatment of rectosigmoid endometriosis is discussed in detail in the Surgical Treatment section. Appendicular endometriosis is usually treated by appendicectomy.

Urinary tract endometriosis

Urinary tract endometriosis is found in 1-4% of women with pelvic endometriosis, 80-90% of these are on the bladder and the rest are ureteral endometriosis. Endometriosis of the kidney is extremely rare (Jubanyik and Comite 1997). Ureteral endometriosis is of particular importance as it may cause obstruction and functional loss of a kidney without causing symptoms (i.e. silent kidney). The majority of ureteral endometriosis lesions are extrinsic, lesions within the wall of the ureters are less common.

The symptoms of bladder endometriosis include cyclical suprapubic pain, dysuria, frequency and haematuria. Ureteral endometriosis is mostly asymptomatic but may cause low back pain, haematuria and recurrent urinary tract infections.

Pelvic and abdominal ultrasonography, computerised tomography or MRI, intravenous urography and cystoscopy with biopsy are helpful investigations used for the diagnosis of bladder endometriosis (Jubanyik and Comite 1997). If rectovaginal endometriosis is diagnosed on physical examination MRI, sonography of the kidney or an intravenous pyelography is of use to diagnose or exclude ureteral obstruction. If ureteral obstruction is diagnosed renography is indicated to diagnose loss of kidney function.

Surgical treatment of bladder endometriosis is usually in the form of excision of the lesion and primary closure of the bladder wall. Ureteral lesions may be excised after stenting the ureter, however in the presence of intrinsic lesions or significant obstruction segmental excision with end-to-end anastomosis or reimplantation may be necessary. When surgery is not possible medical treatment options may also be used.

Abdominal wall and perineal endometriosis

This form of endometriosis is usually the easiest to diagnose and treat. Endometriotic lesions at the site of previous surgical scars, umbilicus or inguinal canal have been reported. These lesions are located within the scar of gynaecological operations, particularly hysterotomy, caesarean sections or episiotomy (Jubanyik and Comite 1997; Nisolle et al., 2007). They appear as dark red-blue or brown, tender nodules. They usually become more painful during menstruation and occasionally there might be cyclical bleeding from these lesions.

Diagnosis is usually by history and clinical examination and treatment is by complete excision of the nodule (Nisolle et al., 2007).

Thoracic endometriosis

Endometriotic lesions of the pleura, lung parenchyma and the diaphragmatic surface may present with pneumothorax, haemothorax, haemoptysis, chest pain and dyspnoea. The symptoms are in general cyclical and tend to start within 24-48 hours after the onset of menstruation (Joseph and Sahn 1996). Women with pleural disease frequently have pelvic endometriosis, it almost always affects the right side (Nisolle et al., 2007), the right to left ratio being 9:1 (Jubanyik and Comite 1997). In contrast, the lung parenchyma is a bilateral disease. This pattern is probably due to pleural/diaphragmatic lesions being secondary to transabdominal and transdiaphragmatic migration while lung lesions being due to lymphovascular embolisation (Nisolle et al., 2007).

Diagnosis may be based on history, chest X-ray, computerised tomography or MRI but additional investigations to confirm diagnosis or exclude other pathology include thoracoscopy, thoracotomy for pleural/diaphragmatic disease and bronchoscopy for pulmonary disease. However, the latter group have limited diagnostic value due to inaccessibility of pulmonary lesions at bronchoscopy or localised nature of pleural lesions.

Medical, surgical or combination treatment options are used. Immediate treatment of pneumothorax or haemothorax is by insertion of a chest tube drain. Hormonal treatment is known to be effective in a significant proportion of the patients. In cases of recurrent pneumothorax or haemothorax chemical pleurodesis, pleural abrasion or pleurectomy may be helpful. Persistent haemoptysis due to parenchymal lesions may be treated by lobectomy, segmentectomy or rarely tracheobronchoscpic laser ablation (Nisolle et al., 2007).

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