A case of Mueller–Weiss syndrome in an adult

Clinical Cases 13.08.2024
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 23 years, male
Authors: Hongmin Xu, Erin Brown, Beshoy Youssef, Hamidou Drammeh, Emad Allam
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AI Report

Clinical History

A 23-year-old male presented with bilateral foot pain for several years, left greater than right, with symptoms aggravated by weight-bearing and activity, improved by rest. He has not been able to exercise for about two months. There was no history of trauma.

Imaging Findings

Weight-bearing foot radiographs demonstrated bilateral navicular deformities (comma-shaped) with possible fragmentation (Figures 1a, 1b and 1c). CT scan of the bilateral feet demonstrated a left navicular fracture with nonunion, in addition to confirming the radiographic findings (Figures 2a and 2b).

The patient underwent open surgery for the left navicular fracture, with bone graft placement and screw fixation. Post-surgical radiograph of the left foot showed appropriate internal fixation with the screw in place (Figure 3).

Discussion

Mueller–Weiss syndrome is a rare condition characterised by avascular necrosis of the tarsal navicular bone in the foot. It typically affects adults, with a higher prevalence in females aged between 40 to 60 years [1,2]. The exact cause remains unclear, but it is believed to involve a combination of vascular insufficiency, mechanical stress, and genetic factors [3]. The central third of the navicular is a watershed area between the medial plantar artery and the dorsalis pedis artery, and this area is prone to avascular necrosis and stress fractures [2]. Childhood-onset osteochondrosis of the navicular bone is called Köhler disease [2]. Note that Köhler disease would apply to paediatric patients, not adults.

Patients with Mueller–Weiss syndrome often present with insidious onset of foot pain, localised to the medial aspect of the midfoot. The pain is typically exacerbated by weight-bearing activities and may be associated with swelling. Clinical presentation and imaging studies can help differentiate Mueller–Weiss syndrome from other causes of midfoot pain, such as stress fractures, osteoarthritis, and other inflammatory conditions. While a fracture is seen in this case, the underlying pathology is Mueller–Weiss syndrome with bilateral navicular deformities.

Weight-bearing foot radiographs are the standard imaging method for diagnosis and disease staging. Radiographs may initially appear normal but can later reveal collapse and sclerosis of the navicular bone. A comma-shaped appearance of the navicular can be observed [2]. CT scan allows a more detailed evaluation of the extent of deformity, identifying arthritic changes and potential fractures. MRI can detect early signs of avascular necrosis, bone marrow oedema, and subchondral fracture lines. MRI also helps differentiate Mueller–Weiss syndrome from other conditions by assessing the extent of bone involvement and identifying associated soft tissue abnormalities.

Once the diagnosis of Mueller–Weiss syndrome is confirmed, treatment options can be considered. Non-surgical management, including activity modification, non-steroidal anti-inflammatory drugs (NSAIDs), and orthotic devices, are typically attempted initially [2]. In cases where conservative measures fail or in patients with navicular fracture nonunion, surgical intervention may be necessary, such as navicular bone decompression, internal fixation, bone grafting, or joint fusions [2,4].

The patient is doing well in follow-ups after the open surgery. He is able to walk and exercise with no pain and has gradually returned to normal activities. Understanding the typical clinical presentation and complications, the importance of imaging, and the available treatment options can aid in the accurate management of Mueller–Weiss syndrome.

All patient data have been completely anonymised throughout the entire manuscript and related files.

Differential Diagnosis List

Mueller–Weiss syndrome (bilateral)
Stress fracture
Köhler disease

Final Diagnosis

Mueller–Weiss syndrome (bilateral)

Figures

Standing AP & oblique radiographs of the feet

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Standing AP and oblique radiographs of the feet show bilateral comma-shaped navicular deformities (commas/asterisks) with possible fragmentation.
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Standing AP and oblique radiographs of the feet show bilateral comma-shaped navicular deformities (commas/asterisks) with possible fragmentation.
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Standing AP and oblique radiographs of the feet show bilateral comma-shaped navicular deformities (commas/asterisks) with possible fragmentation.

Axial & oblique axial CT images of the feet

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Axial and oblique axial CT images of the feet show bilateral navicular deformities (asterisks) with left navicular fracture (arrow).
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Axial and oblique axial CT images of the feet show bilateral navicular deformities (asterisks) with left navicular fracture (arrow).

Post-surgical radiograph of the left foot

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Post-surgical radiograph of the left foot shows bone graft placement and screw fixation of the navicular fracture (arrow).