Benign Symmetric Lipomatosis With Myelomeningocele in an
Adolescent: An Uncommon Association—Case Report
By J. Nounla, U. Rolle, G. Gra¨ fe, and K. Kra¨ ling
Leipzig, Germany
The authors report an unusual localization of symmetric
adipose tumors associated with spinal dysraphism. Initially,
the patient underwent a closure of the lumbosacral myelo-
meningocele. At that time, the tumors were not evident and
remained undiscovered up until puberty. However, during
puberty, the symmetric lipomatous masses grew at the per-
ineal region. Except the patient’s age, all findings and the
clinical picture supported the diagnosis of a benign symmet-
ric lipomatosis (BSL). To the authors’ knowledge, the follow-
ing case has not been described previously.
J Pediatr Surg 36:E13. Copyright
©
2001 by W.B. Saunders
Company.
INDEX WORDS: Benign symmetric lipomatosis, adipose tis-
sue, myelomeningocele.
B
ENIGN SYMMETRIC LIPOMATOSIS (BSL) is a
rare disease, clinically characterized by painless,
nonencapsulated fat deposits with symmetrical distribu-
tion in the body. Usually, it occurs in men; familiarity,
metabolic disturbances, and malignant tumors have been
shown. Surgical removal through either direct excision
or liposuction provides the only palliation.
1-4
Even
though adolescents and lower body parts are rarely af-
fected, we found signs of the disease in a 14-year-old
boy.
CASE REPORT
A 14-year-old boy weighing 60 kg, who presented a history of a
neurosurgically treated myelomeningocele, was admitted to our de-
partment. During his puberty, the mother had noticed a perineal swell-
ing that progressed during the months preceding the treatment. The
physical examination showed 2 fatty, painless masses, nonconfluent,
symmetrical, 15 cm in diameter, and overlaid with coarse skin. How-
ever, the routine laboratory investigations such as serum hepatic en-
zymes, fasting glucose levels, and internal examinations showed nor-
mal values.
The histologic examination after the removal of the benign masses
and the performance of a skin plastic showed noncapsulated, lobulated
fatty tissue composed of mature adipocytes, fairly uniform in size, thus
suggesting histogenesis from white adipose tissue. The cytoplasm was
compressed at the perimeter by a single lipid vacuole. One year
postoperatively, the appearance was satisfactory (Figs 1-3).
DISCUSSION
Adipose tumors are composed of mature fat and rep-
resent by far the most common mesenchymal neoplasm
in adults. They may be found either single or multiple in
association with clinical syndromes or diseases such as
myelomeningoceles.
5
In this case, both diseases usually
occur simultaneously on the same part of the spine. The
later occurrence of adipose tumor involving another
portion of the body is a rare phenomenon. Fatty lesions
hardly occur during the first 2 decades of life. They
account for only 6% of the soft tissue tumors in children
and adolescents, within 94% benign and 6% malig-
nant.
5,6
Symmetrical distribution of adipose tumors, the be-
nign symmetric lipomatosis, which also is known as
Madelung’s disease or Launois-Bensaude disease, was
first described in 1846 by Brodie.
3
His patient showed
massive submental, neck, and posterior lipomatosis.
Typically, fatty deposits enlarge slowly over several
years, but also can undergo rapid growth in a few
months’ time, as in our patient. BSL is a disease rarely
described in the American and English-language litera-
ture, although it seems not to be uncommon in Europe. It
From the Clinic of Pediatric Surgery, Oststrasse, Leipzig, Germany.
Address reprint requests to J. Nounla, Clinic of Pediatric Surgery,
Oststrasse 21-25, 04317, Leipzig, Germany, or K. Kra¨ling, Institute of
Pathology, Liebigstrasse 26, 04103 Leipzig, Germany.
Copyright © 2001 by W.B. Saunders Company
1531-5037/01/3607-0038$35.00/0
doi:10.1053/jpsu.2001.24776
Fig 1. Symmetric fat deposits on the perineum.
1
Journal of Pediatric Surgery,
Vol 36, No 7 (July), 2001: E13