Ken Harris, K. Davies, S. Dumont, Brian M. Stephenson
Reproduced with the permission of The Lancet. Visit their website for some interesting medical information and a free membership.
Summary: A 64-year-old woman with Dercum's disease(adiposis dolorosa) attended the gynaecological clinic in February, 1996, with a 4-week history of lower left-sided abdominal and pelvic pain.
Subject(s): Tumors--Diagnosis; Abdomen--Diseases
A pain in the groin.
(Case Report)
A 64-year-old woman with Dercum's disease (adiposis dolorosa) attended the gynaecological clinic in February, 1996, with a 4-week history of lower left-sided abdominal and pelvic pain. An ultrasound scan showed uterine fibroids and she underwent hysterectomy and bilateral salpingo-oophorectomy. This did not relieve her symptoms and in July, 1996, she consulted her general practitioner with worsening pain. There was an obvious sausage-shaped lump in the left iliac fossa. She was reassured that this was another lipoma. 4 months later she was still in pain and had frequent bowel motions. A colonoscopy was normal and she was again reassured, as she was not keen to have the lipoma removed. She came back 3 months later with increasing discomfort, and at this time an ultrasound scan, requested to assess the size and position of the lump, failed to identify it. A computed tomograph scan was reported as showing a large abdominal wall lipoma (figure). She was offered surgical excision again, but declined. Despite further reassurance her symptoms got worse.
She then agreed to an exploratory operation where a Spigelian hernia with incarcerated omentum extending down towards the femoral canal was found. The omentum was excised and a large defect (5 cm) closed with prolene mesh. When last seen in April, 1997, she was without symptoms.
Dercum's disease, first described in 1892, is an unusual progressive syndrome of unknown aetiology characterised by multiple painful lipomas most often affecting obese postmenopausal women.1The onset is insidious and progresses to multiple painful lipomas often unresponsive to analgesics. Traditional management has been largely unsatisfactory, relying on weight reduction and surgical excision of particularly troublesome lesions.2More recently, Petersen and Kastrup3 have described a technique involving intravenous lignocaine to treat the pain with some benefit although several infusions were necessary and oral mexiletene was given as a prophylaxis against cardiac side effects. Spangen4 has suggested that Spigelian hernias may be more common than originally thought. The hernial defect can occur at any point along the transversus abdominus aponeurosis (Spigelian fascia) although the vast majority occur at or just cranial to a line connecting the two anterior superior iliac spines. The hernial sac pierces the transversus abdominus and the internal oblique muscle and then may expand between the obliques; the hernia rarely penetrates the dense external oblique aponeurosis. Spigelian hernias are also more frequent in middle-aged women. The pain is often non-specific and its classical exacerbation with a Valsalva manoeuvre rarely manifest. As diagnostic signs are often lacking it has meant that Spigelion hernias are notoriously difficult to detect in all but thin patients. The role of imaging remains uncertain as ultrasound and computed tomograph scans are unreliable5 and the accuracy of magnetic resonance imaging yet to be ascertained. Surgical repair is however strongly advised as the neck of the sac is usually narrow and the risk of strangulation high. We suggest persistently painful groin "lipomas" are explored surgically irrespective of another ready explanation for the clinical findings. The use of large doses of lignocaine should really not be considered as a first-line therapy in this condition in all but those centres with experience in its use, as the therapeutic window is narrow and the adverse effects, especially the risk of epileptiform seizures, carry a considerable morbidity. Furthermore, as the popularity of local infiltration with various anaesthetic or analgesic agents increases, it is important to remember that the omentum is really no more than a very vascular lipoma.
References
1 Palmer ED. Dercum's disease: adiposis dolorosa. Am Fam Phys1981; 24: 155-57.
2 Nahir AH, Schapira O, Scharf Y. Justa-articular adiposis dolorosa--a neglected disease. Isr J Med Sci1983; 19: 858-59.
3 Petersen P, Kastrup J. Dercum's disease (adiposis dolorosa). Treatment of the severe pain with intravenous lidocaine. Pain 1987; 28: 77-80.
4 Spangen L. Spigelian hernia.World J Surg 1989; 13: 573-80.
5 Balthazar EJ, Subramanyam BR, Megibow A. Spigelian hernia: CT and ultrasonography diagnosis.Gastrointest Radiol 1984; 9: 81-84.
© 1997 Lancet Ltd.
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