Diffuse abdominal lipomatosis.
ABSTRACT We report a 22-year male who developed progressive distension of abdomen, clinically diagnosed as ascites. A diagnosis of abdominal lipomatosis was made on the basis of CT evidence of excessive fatty tissue in abdominal cavity which was confirmed on laparotomy.
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ABSTRACT: Peritoneal lipomatosis is a rare disease in childhood with only two cases previously described in children. We report a further case of a 12-year-old boy diagnosed with peritoneal lipomatosis. His main symptoms were abdominal pain, alternating bowel habit, abdominal distension, and melaena. His diagnostic work up included an abdominal MRI, wireless capsule endoscopy and single-balloon enteroscopy. Peritoneal lipomatosis although rare can be diagnosed in childhood. It is a benign clinical entity with variable manifestations.Case reports in gastrointestinal medicine. 01/2013; 2013:496419.
JAPI • VOL. 51 • JUNE 2003621
Diffuse Abdominal Lipomatosis
AH Zargar,* BA Laway,* SR Masoodi,* MH Bhat,* MI Bashir,* AI Wani,* NA Wani**
We report a 22-year male who developed progressive distension of abdomen, clinically diagnosed as
ascites. A diagnosis of abdominal lipomatosis was made on the basis of CT evidence of excessive fatty
tissue in abdominal cavity which was confirmed on laparotomy.
ipomatosis represents a distinct
clinicopathological entity characterized by
massive development of non-encapsulated
lipomas in subcutaneous tissues. Lipomatosis
of the face, head and neck, upper and lower
extremities, trunk and abdominal cavity and
pelvis have all been reported.1
MA, 22-year male presented with slowly
progressive painless distension of abdomen of
5 years duration. There was no associated
history of constitutional symptoms, altered
bowel habits, alcohol consumption or symptoms
suggestive of chronic liver disease. The patient
gave history of repeated negative abdominal
paracentesis in the past. Examination revealed
emaciated young male with marfanoid habitus,
weight 52 kg, body mass index 17.9 kg/m2 and
waist to hip ratio of 1.0. Patient was
* Dept. of Endocrinology; ** Dept. of General Surgery Sher-I-Kashmir
Institute of Medical Sciences, Srinagar, Kashmir, India.
Received : 19.08.2002; Accepted 23.04.2003
omentum (low attenuation value -92.9 HU) displacing bowel
loops posteriorly (Fig. 1). There was no well circumscribed
mass lesion of free fluid in the abdomen. Fine needle aspiration
cytology of omental fat revealed adipose cells only.
Antegrade urography didn’t reveal any obstruction of ureters
or bladder. The patient was subjected to laparatomy.
Abdominal cavity was found to be occupied from diaphragm
to pelvis by a huge mass of fat originating from right side of
retroperitoneum. Bowel loops as well as right ureter were
displaced to left side. Liver did not show any gross features
of fatty infiltration. About 15 kg of solid fat was removed by
excising along the right side of the lateral margians (Fig. 2).
Colon and right ureter were kept back at their anatomical
position. The patient had an uneventful post-surgery
recovery. Histopathological examination confirmed the
diagnosis of benign lipomatosis.
Lipomatosis can affect a multitude of anatomic sites,
although a predilection for the lower extremities exists in most
Fig. 1 : CT scan abdomen showing diffuse lipomatosis of abdominal cavity.
normotensive and general physical examination was
unremarkable. Abdomen was distended (abdominal girth 89
cms) with few visible veins and bilateral indirect inguinal
hernias. Percussion note was dull all over abdomen and there
was no shifting dullness; however, a doubtful fluid thrill was
palpable. Abdominal paracentesis was negative. Routine
investigations including hemoglobin, complete blood counts,
blood glucose, blood urea nitrogen, serum creatinine,
transaminases, proteins, uric acid, and glucose tolerance test
were in normal range. Lipidogram revealed a total cholesterol
of 154 mg/dl, HDL 58 mg/dl, LDL 78 mg/dl and serum
triglycerides 90 mg/dl. Electrocardiograph, x-ray of chest
and urine analysis were normal. Ultrasonography of abdomen
revealed mild hepatosplenomegaly with increased
echogenecity and no free fluid in abdomen. Computed
tomography of abdomen showed diffuse fatty infiltration of
622JAPI • VOL. 51 • JUNE 2003
cases. Multiple symmetrical lipomatosis (MSL) is a rare
condition of the fatty tissue affecting mostly white men
between 25 and 60 years of age. It may occur sporadically or
in families. MSL is characterized by collection of large non-
encapsulated lipomas mainly located in the subcutaneous
tissues of the cervical, deltoid, thoracic, abdominal and pelvic
areas.2 The disease is often associated with dyslipidemia
(hypertriglyceridemia and paradoxically high HDL), impaired
glucose tolerance, hyperuricemia, macrocytic anemia and
peripheral neuropathy.2,3 Central nervous system
abnormalities and neuropathy (sensory, motor, autonomic)
have been reported in this disorder.1 MSL may primarily
involve nape of the neck and supraclavicular and deltoid
regions resulting in the bull-necked appearance (Madelung
collar) or may extend over whole body, giving the appearance
of simple obesity.1
The cause of MSL is not known. Isolated adipocytes
have increased lipoprotein lipase activity and a defect in
adrenergic lipolysis.1 Lipolytic responses to cyclic AMP is
intact, suggesting an abnormality at the hormone receptor/
adenylate cyclase unit. Some authors have suggested that
the defective lipolysis is due to a disorder in the mitochondria
of brown fat whose distribution is similar to the pecular pattern
of lipomas in MSL.2 Alcohol consumption is common and
may cause coexisting folate deficiency, macrocytic anemia
and abnormal liver functions. Alcohol could both promote
development of lipomas through changes in the number and
function of beta adrenergic receptors because of its lipogenic
and antilipolytic action.2
Mediastino-abdominal lipomatosis is a variant of MSL
characterized by massive enlargement of abdomen
(psuedoascites) due to intraperitoneal and retroperitoneal
fat, exertional dyspnea due to compression of airways by
lipomas of mediastinum, and abnormal glucose tolerance
or diabetes mellitus.4 Intra-abdominal lipomatous tissue
obtained during laparoscopy from four patients in one
series documented a reduced lipolytic response to β-
adrenergic stimulation.4 Thus, fat deposition in abdominal
and mediastinal areas could be casually related to defective
lipid mobilization in lipomatocytes.4 Lipomatosis may
involve small intestine, colon or arise in the mesentery.5
Pelvic lipomatosis has been reported more commonly
in males. Symptoms include bladder dysfunction,
constipation, vague abdominal discomfort or edema of the
lower extremities.6 Bilateral ureteral obstruction may cause
hydronephrosis and renal failure. Magnetic resonance
imaging or computerized tomography defines the
Fig. 2 : Photograph showing specimen of fat excised from the
compressing fat in pelvic space enveloping the pelvic viscera.
Epidural lipomatosis causing spinal cord compression is seen
with chronic glucocorticoid therapy and may also occur in
Cushing’s syndrome.1 There is no treatment except surgical
removal of lipomas that cause compression of viscera or for
1.Garg A. The lipodystrophies and other rare disorders of
adipose tissue. In: Harrison’s Principles of Internal Medicine.
15th Ed. Braunwald E, et al (Eds.). McGraw Hill, New York,
2.Lomartive N, Ciocca F, DiStanislao C, Bologna G, Giuliani M.
Multiple symmetrical lipomatosis (MSL): A clinical case and
review of the literature. Ann Ital Chir 1999;70:259-63.
3.Enzi G. Multiple symmetric lipomatosis: An updated clinical
report. Medicine 1984;53:56.
4. Enzi G, Digito M, Marin R, Carraro R, Baritussio A, Manzato
E. Mediastino-abdominal lipomatosis: deep accumulation of
fat mimicking a respiratory disease and ascites. Clinical
aspects and metabolic studies in vitro. Q J Med 1984;53:453-
5. Tatsuguchi A, Fukuda Y, Moriyama T, Yamanaka N.
Lipomatosis of the small intestine and colon associated with
intussusception in the ileocaecal region. Gastrointestinal
6. Hyenes CF. Pelvic lipomatosis: A review of its diagnosis and
management. J Urol 1991;146:267.