Mueller - Weiss Syndrome: An uncommon cause of midfoot pain

Kumar VK1, Renuka Mohan2

1. Asst. Professor of Orthopaedics

2. Professor of Orthopaedics

 

Gokulam Medical College Venjaramoodu Trivandrum, Kerala India

 

 


Introduction

Spontaneous osteonecrosis of the navicular in the adult is known as Mueller-Weiss syndrome. It is a rare disease characterised by a collapse of the lateral portion of the tarsal navicular combined with a medial protrusion of the talar head and peri-navicular osteoarthritis. ( Figure 1, 2) this article, we describe a case of spontaneous osteonecrosis of navicular in a 43-year-old female. The chronic midfoot pain over the dorsomedial aspect of the right foot was resolved after complete removal of the necrotic bone from the navicular, followed by autologous bone grafting of the resultant defect.

 

Case report

A 43 year old female patient presented with pain in the right foot of 2 years duration. The pain was dull aching and insidious in onset. There was no history of trauma. She was not diabetic. There were no constitutional symptoms or history suggestive of any inflammatory arthritis. Over a period the pain has become severe affecting her normal gait.

Examination did not reveal any swelling or deformity of foot. There was severe tenderness over the dorsomedial aspect of the foot in the region of navicular. There was no neurovascular deficit.

Radiological findings revealed sclerotic collapsed navicular with dorsal and lateral extrusion of the fragmented bone.

This idiopathic osteonecrosis of navicular bone was treated with excision of the sclerotic bone and medial column arthrodesis with tricortical iliac graft. To improve the biomechanics of the foot the subtalar joint was also arthrodesed. (Figure 3, 4)

Discussion

Spontaneous osteonecrosis of the navicular in the adult is a rare entity and is known as Mueller-Weiss syndrome. This syndrome is distinct from the osteochondrosis of the tarsal navicular bone that occurs in children (Khler disease). There is little English literature available on this syndrome. In 1927, Mueller described a condition of navicular bone, occurring in adults that he thought was a chronic deformation related to compression from adjacent bones. The radiographic characteristics described in this condition included medial and/or dorsal protrusion of portions of or the entire navicular bone and a comma-shaped deformity due to collapse of the lateral portion of the bone. Bilateral distribution of different severities and pathologic fractures were additional findings in this disease. In 1929, Weiss described adults who had similar abnormalities and proposed osteonecrosis as the primary causative factor. Subsequently, further descriptions of the same disorder in 30 patients, predominantly female patients with changes characterised by increased radio density and fragmentation of the navicular bone have appeared and a variety of names, including bipartite navicular bone and osteopathia deformans, have been used.

The radiographic abnormalities of primary osteonecrosis of the tarsal navicular bone (Mueller-Weiss syndrome) appear to be characteristic. Initial alterations include a loss of volume in the lateral aspect of the tarsal navicular accompanied by an increase inradiodensity of this portion of the bone. The tarsal navicular assumes a comma like shape because of lateral compression. Subsequently, dorsal protrusion and fragmentation of the bone may become evident.

As per the available literature early diagnosed cases of Mueller weiss syndrome (MRI) are best treated with percutaneous decompression of the navicular bone. Late cases require excision of the bone and medial column arthrodesis.

 

References

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Correspondence should be sent to:

[email protected]

 

Cite this article as:

Kumar VK, Renuka Mohan. Mueller - Weiss Syndrome: An uncommon cause of midfoot pain. Kerala Journal of Orthopaedics. 2011;24:40-42

 

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ISSN: 2249 – 2348. Published by: Kerala Orthopaedic Association

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