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Idiopathic carpal tarsal osteolysis (ICTO)
with additional elbow involvement
Farzanah Ismail & Craig Muller & Ruan Goller &
Zarina I. Lockhat & Savvas Andronikou
Received: 9 April 2011 /Revised: 18 June 2011 /Accepted: 4 July 2011 /Published online: 9 August 2011
# ISS 2011
Idiopathic carpal tarsal osteolysis (ICTO) with additional
ICTO is a rare congenital disorder characterized by bone
resorption affecting mainly the carpal and tarsal bones .
Previously it was known as idiopathic multicentric osteol-
ysis and was first described in 1838 by this term . Tyler
et al. also preferred the term idiopathic multicentric
osteolysis (1976) as this encompassed the wide array of
descriptive terminology used to describe this condition in
the literature . The term idiopathic carpal tarsal osteol-
ysis was first used by Froelich and Coerret in 1937 [1, 4]. It
is also known as multicentric carpal-tarsal osteolysis with
and without nephropathy, and the association of renal
failure was first described by Marie et al. in 1951 [2, 4].
The condition may occur sporadically or is inherited
as an autosomal dominant or recessive condition .
Knowledge about the genetics of this condition is still
evolving. It was divided into five subtypes by Hardegge et
al. in 1985. Type I is inherited as an autosomal dominant
condition and is infrequently associated with renal involve-
ment. Type II is inherited as autosomal recessive with no
renal involvement. Type III is sporadic with frequent renal
involvement . Type IV is also known as Gorhams disease
and is unicentric and caused by haemangiomatosis. Type V
is also known as Winchester syndrome and is associated
with a different clinical presentation, corneal clouding,
contractures, and skin lesions [2, 3].
Patients with ICTO present in early childhood with
progressive acromelic joint pain, limitation of movement,
swelling, and deformity [2, 4]. The onset of disease usually
begins around age 2 or 3. Laboratory findings are normal
with no evidence of inflammatory, metabolic, or auto-
immune diseases [2, 4]. This was the case with our patient.
The radiologic findings are characteristic with osteolysis
of the carpal and tarsal bones and adjacent tubular bones
. The involvement of the adjacent metacarpals is
described as “sucked candy” . Osteolysis of the distal
ulna (more than the radius) causes ulna deviation of the
hand. Similar findings in the feet cause clubfoot deformity
. In this case there was asymmetry of the involvement of
the hands. The changes on the right side were much more
pronounced with the sucked candy appearance of the
proximal metacarpals in contrast to the truncated appear-
ance on the opposite sides. In contrast there was more
The case presentation can be found at doi:10.1007/s00256-011-1233-0.
The authors declare that no funding or grants have been received.
F. Ismail:C. Muller:R. Goller:Z. I. Lockhat:S. Andronikou
University of Pretoria,
Pretoria, South Africa
Z. I. Lockhat
F. Ismail (*)
Department of Radiology, Level 5, Steve Biko Academic
Hospital, University of Pretoria,
Voortrekker Road, Gezina,
Pretoria, South Africa 0002
Skeletal Radiol (2012) 41:619–620
dramatic involvement of the MCP joints on the left than the
right. Both Carmichael and McDonald et al. described cases
with elbow involvement. This was the case with our patient
as well [1, 4].
Our patient was a sporadic presentation without current
renal involvement (renal involvement is frequently associ-
ated with the sporadic type). Bennett et al. have found that
bone involvement precedes renal involvement by 10–
15 years, hence the need for long term follow-up of renal
function . In a patient with sporadic ICTO, Connor et al.
found that the renal lesion was focal segmental glomerular
sclerosis in early and subsequent renal biopsies. This
patient was successfully treated with cyclosporin A.
Proteinuria was the first sign of renal dysfunction .
The diagnosis of ICTO can only be made once other
differential diagnoses such as juvenile idiopathic arthritis
(JIA), trauma, inflammation, avascular necrosis, and hyper-
parathyroidism are excluded . ICTO presents initially
with joint stiffness, which progresses to hypermobility and
a progressive decrease in pain. It stabilizes by age 20–30.
This may aid in the exclusion of JIA, which demonstrates
progressive signs and symptoms .
Treatment of ICTO is mainly supportive and may
include physiotherapy, occupational therapy, and the use
of orthotics and prosthetics.
1. Carmichael KD, Launikitis RA, Kalia A. The orthopaedic and renal
manifestations of idiopathic carpal tarsal osteolysis. J Pediatr
2. Faber MR, Verlaak R, Fiselier TJW, Hamel BCJ, Franssen MJAM,
Gerrits GPJM. Inherited multicentric osteolysis with carpal tarsal
localisation mimicking juvenile idiopathic arthritis. Eur J Pediatr.
3. Tyler T, Rosenbaum HD. Idiopathic multicentric osteolysis. Am J
4. McDonald K, Toms AP, Armon K, Johnson K, Marshall TJ.
Carpal-tarsal osteolysis with elbow involvement. Skeletal
5. Bennett WM, Houghton DC, Beals RC. Nephropathy of idiopathic
multicentric osteolysis. Nephron. 1980;25:134–8.
6. Connor A, Highton J, Hung NA, Dunbar J, MacGinley R, Walker
R. Multicentric carpal-tarsal osteloysis with nephropathy treated
successfully with cyclosporine A: a case report and literature
review. Am J Kidney Dis. 2007;50:649–54.
620Skeletal Radiol (2012) 41:619–620