September 01, 2022
Years published: 2022
NORD gratefully acknowledges Anoush Calikyan, MD Candidate, Frank H. Netter MD School of Medicine, Quinnipiac University, Megan Leubner, MD Candidate, Frank H. Netter MD School of Medicine, Quinnipiac University and Tommaso Greco, MD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Università Cattolica del Sacro Cuore for the preparation of this report.
Mueller-Weiss disease (MWD) is a rare foot condition characterized by a misshaped navicular bone and subsequent early-onset degenerative arthritis of the neighboring joints. It typically presents as longstanding subtle midfoot pain without a history of injury and progresses to severe, debilitating pain that worsens with weight-bearing activity. Most patients are females in their 40s to 60s but there are reported cases in males and patients as young as 18. MWD may be present in only one foot or both, and it is not unusual for one side to be more painful than the other. The exact cause of MWD is unknown, but it has traditionally been thought to be due to spontaneous bone cell death (osteonecrosis) of the navicular bone. The navicular bone is commonly described as “comma shaped” on X-ray and may be fragmented in later stages of disease. No gold standard treatment currently exists for MWD, but conservative and surgical options have been described in case reports and case series. Surgical treatment options should be considered if conservative therapy is unsuccessful after six months, and the decision about whether to operate should be based on patient discomfort rather than stage of deformity.
Mueller-Weiss disease was originally described by Schmidt in 1925. W. Mueller, in 1927, was the first to suggest that this condition was caused by compression on the tarsus or an abnormality present at birth (congenital). In 1929, the radiologist, K. Weiss was the first to propose that the disease was due to decreased blood flow (ischemic process) based on radiographic findings. Thus, the condition was named for Mueller and Weiss for being the first to describe the cause of the disease. Mueller-Weiss disease is often misdiagnosed and therefore, not treated properly. Early recognition and treatment of this disease may lead to improved outcomes by prevention of extensive degenerative changes.
Patients usually present with chronic, atraumatic mid and hindfoot pain without prior trauma that worsens with weight-bearing activity. The pain may be constant or intermittent, but typically worsens over time. Patients also complain of difficulty walking, foot swelling, joint stiffness and limitation of movement, and positional foot deformities including a paradoxical varus hindfoot deformity. In later stages of disease, it is common for MWD patients to have a positive heel raise test on physical exam, indicating posterior tibial tendon dysfunction. Patients may also present with arthritis and anterior knee pain.
The exact cause of MWD is unknown. The most popular theory is that MWD is caused by decreased blood supply to the navicular bone of the foot. Decreased blood supply has many potential underlying causes including nutritional deficiency, smoking, alcohol, corticosteroid use, rheumatologic disorders, hematologic disorders and metabolic disorders. Other theories suggest a multifactorial cause that includes chronic pressure on the navicular bone, suboptimal ossification of the navicular bone during development, congenital causes, dysplasia and primary or secondary osteonecrosis of the navicular bone. Some medical experts think that osteonecrosis of the bone begins due to undiagnosed navicular stress fractures, eventually leading to MWD. MWD does not appear to run in families and there is no evidence of a genetic cause.
The incidence and prevalence of MWD is currently unknown. It typically affects female adults more than male adults at a ratio of approximately 6:1. MWD most typically affects adults ages 40-60 but has been reported in a patient as young as 18 years old. Younger individuals tend to have unilateral deformities, while older individuals tend to have deformities of both feet.
Kohler disease is similar to MWD in that it is also a disorder caused by decreased blood flow to the navicular bone of the foot and causes pain, limping and swelling of the foot. However, it is distinct because it occurs only in childhood, typically in children 3-7 years of age and resolves over time as the child grows. The navicular bone regains its density, size and structure, but symptoms rarely last up to two years. MWD has not been reported in children and will not resolve without intervention. (For more information on Kohler disease search for “Kohler” in the Rare Disease Database.)
The diagnosis of MWD involves clinical findings, X-ray (radiologic) evidence and pathologic confirmation of the disease. Clinical findings are nonspecific and must be distinguished from those experienced due to trauma, metabolic bone disease, neoplastic bone tumor, secondary osteonecrosis and a previously undiagnosed stress fracture. The appearance of MWD on physical exam may be mistaken for acquired flat feet deformity. Suspicion of MWD can be confirmed with radiographic findings that show wedging and compression of the navicular bone, a comma shaped navicular bone and joint space narrowing.
It is important to note that hindfoot varus is a sign that must be present to diagnose MWD, although its appearance may be subtle or masked due to the other foot deformities also present in MWD. A common misdiagnosis of MWD is acquired flat feet deformity (valgus pes planus); the varus position of the hindfoot in MWD, combined with the comma-shaped navicular bone, are the hallmark signs of this condition.
MWD can be diagnosed with weight-bearing X-rays of both feet in multiple views. Radiologic evidence of MWD may include: a “comma shaped” or fragmented navicular bone, dorsolateral subluxation of the talus resulting in hindfoot varus, collapse of the medial arch and signs of degenerative arthritis in neighboring joints. MWD can be staged (Maceira’s classification) based on severity of morphological changes observed on the lateral weight-bearing view, from minimal changes (stage 1) to advanced disease characterized by extrusion of the navicular and direct articulation of the cuneiform and talus bones (stage 5).
Clinical Testing and Work-Up
Blood laboratory screening tests should be performed to rule out associated diagnoses that may be contributing to the clinical picture, such as evidence of rheumatological disease, neoplasm and disorders of metabolism.
A positive bone scan, CT or MRI may demonstrate evidence of MWD prior to any changes that may be visible on physical examination or X-ray. MRI imaging is likely to show signs of osteonecrosis, effusion in adjacent joints and bone marrow edema (findings consistent with inflammation and bone death).
There is no evidence of correlation between the patient’s reported pain and stage. Therefore, patient discomfort, rather than severity of deformity, is the basis for when to seek surgical treatment.
Weight-bearing CT scans are considered the gold standard for planning surgical reconstruction because it allows a complex, dynamic deformity to be imaged in 3D while in a functional, load-bearing position.
During surgery, excised bone fragments may be sent to pathology for analysis. Evidence of osteonecrosis is consistent with MWD, but the absence of such changes is also common.
No standard therapy or treatment exists for MWD. Current knowledge of managing this condition is based on results reported in individual case reports and small case series. Both conservative and surgical options have been described.
Conservative measures include reducing activities that require weight bearing, non-steroidal anti-inflammatory medications, rest, elevation and ice. Custom orthotics that simultaneously support the medial arch and correct the hindfoot varus may provide some patients with significant pain relief while also correcting alignment of the knees and hips. Guided joint injections with lidocaine and steroids may provide moderate pain relief to patients for weeks to months.
Identifying MWD in its early stages is helpful because progression of the disease may be slowed through both conservative and surgical measures that aim to reduce pain, retain joint integrity and preserve ideal lower extremity biomechanics. Correcting the alignment and biomechanics of the foot is essential for preventing undue wear and tear on more proximal joints, such as the knees and hips.
The definitive treatment to halt the progression of MWD is surgery. Since there is no established correlation between pain and stage of disease, and no standard surgical intervention exists, the decision about whether to operate is based primarily on the pain and disruption to daily activities as reported by the patient.
Numerous surgical procedures have been used. Almost all reported interventions include debridement of loose bone fragments, internal fixation of the navicular bone and various types of joint fusions (talonavicular, talonavicular-cuneiform, triple and association of triple with naviculo-cuneiform fusion). The exact surgical procedure performed will be specific to the radiographic findings in each patient.
Joint fusions in the midfoot and hindfoot have many disadvantages including loss of motion impacting alignment and adjacent joint function. However, fusions may be necessary to achieve pain relief and stability if MWD is in the advanced stage. If MWD is diagnosed prior to significant joint destruction, joint-preserving surgery may be an option. There have been numerous case reports of restoration of the hindfoot varus through calcaneal osteotomies resulting in significant pain relief and preservation of the talonavicular joint function.
Orthopedic foot and ankle specialist surgeons, radiologists, physical medicine and rehabilitation specialists (“physiotherapists”) and physical therapists all play an important role in the proper diagnosis and management of MWD.
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