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Lipofibromatous Hamartoma with

Macrodactyly of Third Digit Left Hand


in Soeharso Orthopaedic Hospital
Surakarta
A Case Report
Hastomo Agung, Tito Sumarwoto

CASE

Case
Female (student) 14 y.o with macrodactyly of
the third digit left hand since born
Complained of clumsiness, tingling and her
third digit gradually increased in last 6 months
No history of trauma, no family history

Local Status (3rd digit)


L: Localized gigantism
Reddish colour at distal part
F: Soft mass, mobile,
Tenderness (-)
Hipoesthesia (+) at distal part
M: ROM still normal

Assesment
Macrodactyly of third digit left hand,
suspect associated with vascular malformation

Plan
Plain x-ray
CT-Scan with contrast

Assesment II
Macrodactyly of third digit left hand, suspect
associated with macrodystrophia lipomatous

Plan
Surgery

Intra-Operative
Zig-zag incision, we found yellow and cordlike mass
(16 cm in length, 30 mm in diameter) that
surrounded and enlarged by fibrofatty tissue
We do the debulking procedure

Microphotograph shows fibroadipose tissue around the lession


(hematoxylin-eosin,original magnification 100x & 50x)

Post-operative Care
Analgetic & antibiotic intravenous treatment
(1st day post-op)
Routine medication every 48 hours, and
removing suture after 14 days post-op
Active ROM exercise after second day post-op

3 Months Post-op
Results:
decrease of the mass size
motoric function and range of motion of the third
fingger was normal
returned to normal activity 3 weeks after surgery
sensoric deficit of third fingger

DISCUSSION

Macrodatyly

Green's Operative Hand Surgery, 6th ed

Lipofibromatous Hamartoma
A rare, fibrofatty benign tumor comprised of
proliferation of mature adipoctyes, mostly within
peripheral nerves forming a palpable mass
> 1/3 of cases associated with macrodactyly
2:1 female to male ratio, within first three decade
Thought to be congenital or developmental in origin

Lipofibromatous Hamartoma
The mass is usually slow-growing and may cause
nerve compression sensory change, pain and
loss of motor function
Differential diagnosis: vascular malformations,
traumatic neuroma, ganglion cysts and lipomas

Only rarely is the correct


diagnosis made preoperatively

American Journal of Orthopaedics 2008

Histologic Findings
Biopsy has been needed as the standard approach
to establish the diagnosis

Microphotograph of the debulked lipofibromatous hamartoma,


shows fibroadipose tissue between nerve fascicles
Green's Operative Hand Surgery, 6th ed

Treatment
Related to macrodactyly debulking
vs no treatment (controversial)
Mostly consider waiting tumor
become symptomatic or cosmetic
problem
Debulking can improve functional
outcome but sensibility deficit (+)

Conclussion
A rare case and preoperative diagnosis is
difficult
Our treatment is to do debulking
procedure,
with consideration: symptomatic mass
with cosmetic problem
Results: decrease the mass size, normal
motoric function and ROM, nevertheless
sensoric deficit according nerve
distribution

Thank You