CASE 29

By: Dr. Khalid Abdullah AL-Ismail MD

HISTORY: 25-year-old female presented with mild deformity to R/O tumor right ring finger.

What are the findings?

Your differential diagnosis and most likely diagnosis?

Do you need to biopsy this abnormality?

Name 3 associated disorder.

FINDINGS:

Plain radiographs:

Irregular hyperostotic changes of the ulnar cortex of the ring finger and the ulnar aspect of the middle finger resembling melted wax dripping down one side of a candle. Evidence of medullary canal encroachment is noted most likely due to endosteal involvement.

NM:

Marked increase in radionuclide uptake is seen on bone scan.

MRI:

Reveals hypointensity on all pulse sequences.

DDX:

myositis ossificans, osteoma and parosteal osteosarcoma.

Biopsy:

Might be required in small number of cases especially with symptomatic and aggressive like appearance.

DIFFERENTIAL DIAGNOSIS:

It may co-exist with:

• Osteopoikilosis.

• Osteopathia striata.

• Vascular/lymphatic tumors or malformations.

• Soft-tissue ossifications at the site of the joint are common.

• Synovial osteochondromatosis.

• Fibrolipomas.

• Desmoid tumors.

• Multicentric fibromatosis.

• Minimal change nephrotic syndrome.

• Hypophosphatemic rickets.

DIAGNOSIS:

Melorheostosis.

PEARLS AND DISCUSSION:

Melorheostosis is a rare, non-inheritable bone dysplasia characterized by its classic radiographic feature of flowing hyperostosis resembling dripping candle wax, generally on one side of the bone.

Melorheostosis usually affects one limb, more often the long bones of the upper and lower extremity, and rarely the axial skeleton. The short bones of the hand and foot are commonly involved. Both sexes affected equally.

The condition originally was described by Leri and Joanny in 1922. Its etiology remains idiopathic, and treatment in most instances limited to the symptomatic cases.

The diagnosis is usually made in late childhood. Pain, stiffness, deformation of a limb are the main clinical manifestations. The skin may be erythematous and sclerotic.

Treatment of this chronic and sometimes debilitating condition consists of surgical soft-tissue procedures and even, in very rare severe cases, amputation.

FURTHER READING:

1. Vibhor Wadhwa,a Avneesh Chhabra,a Jonathan D. Samet Melorheostosis mimicking synovial osteochondromatosis. Ann Saudi Med 2014; 34(6): 547-550

2. Long HT, Li KH, Zhu Y. Case report: severe melorheostosis involving the ipsilateral extremities. Clin Orthop Relat Res. 2009 Oct;467(10):2738-43. doi: 10.1007/s11999-009-0890-y. Epub 2009 May 21.

3. Ingen-Housz-Oro S, Chigot V, Hamel- Teillac D, Brunelle F, De Prost Y. Melorheostosis associated with arteriovenous malformation of the ear. Ann Dermatol Venereol. 2001 Sep;128(8-9):915-8.