Müller-Weiss Disease: a Topical Review
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FAIXXX10.1177/1071100719877000Foot & Ankle InternationalAhmed et al 877000research-article2019 Topical Review Foot & Ankle International® 2019, Vol. 40(12) 1447 –1457 Müller-Weiss Disease: A Topical Review © The Author(s) 2019 Article reuse guidelines: sagepub.com/journals-permissions DOI:https://doi.org/10.1177/1071100719877000 10.1177/1071100719877000 journals.sagepub.com/home/fai Abdel-Salam Abdel-Aleem Ahmed, MD1 , Mahmoud Ibrahim Kandil, MD1 , Eslam Abdelshafi Tabl, MD1 , and Amr S. Elgazzar, MD1 Level of Evidence: Level V, expert opinion. Keywords: Müller-Weiss disease, Brailsford disease, foot arthrodesis, calcaneal osteotomy Müller-Weiss disease (MWD) is a painful foot condition articulates proximally with the talar head, distally with the characterized by deformity, sclerosis, and fragmentation of cuneiforms, and inferolaterally with the cuboid. Proximally, the navicular. The diseased navicular is characteristically the talocalcaneonavicular articulation is also known as the comma shaped with varying degrees of arthritis present at acetabulum pedis for morphologic similarity to the hip the talonavicular and naviculocuneiform joints.17,19,27 Other joint. It is a ball-socket synovial joint allowing for rotatory names for MWD include Brailsford disease,37 adult tarsal and gliding movements. The talocalcaneonavicular and the scaphoiditis,26 spontaneous adult navicular osteonecrosis,34 naviculocuneiform joints sustain the greatest load transmis- and listhesis navicularis.23 There is substantial controversy sion in comparison to other foot joints.11 regarding the etiology, pathophysiology, and natural history The navicular is the latest tarsal bone to ossify. of MWD, as well as optimal methods of treatment. This Ossification occurs during the period of increased mobility article reviews the historical and current literature regarding and activity in children (at the end of the second year in girls MWD, including the latest surgical interventions that have and at the beginning of the fourth year in boys). With subop- been reported. timal ossification of the navicular, it may sustain higher shearing forces at the level of the lateral cuneiforms.36 Historical Background The navicular is perfused by 2 arteries. The dorsalis pedis artery supplies the dorsal and lateral one-third of the Despite being named after Müller and Weiss, Schmidt was bone while the medial plantar artery supplies the plantar the first to report a similar condition in a patient with pluri- aspect and some of the medial aspect of the bone. The arte- glandular insufficiency syndrome in 1925.23 Walther 28 rial supply has a circumferential pattern resulting in a cen- Müller, a German surgeon, reported a case of MWD in tral osseous zone that has a diminished blood supply that 1927 and attributed it to mechanical collapse from com- may even decrease with age.40 pressive forces. One year later, he proposed a congenital 29 42 Being the keystone of the medial column of the foot, the theory as the cause of the condition. Konrad Weiss, an navicular contributes to the integrity of both the medial lon- Austrian radiologist, presented 2 cases with similar find- gitudinal and the transverse arches of the foot.35 In the case ings. However, based on radiographic signs of increased of dorsolateral fragmentation and collapse of the navicular, bone sclerosis and adjacent joint space narrowing, he attrib- functional malalignment and progressive deformities of the uted the condition to osteonecrosis. Brailsford identified midfoot and the hindfoot occur.23,41 MWD as osteochondritis of the navicular bone or listhesis navicularis.23,31 1Faculty of Medicine, Benha University, Benha, Qalyubia, Egypt Anatomical and Biomechanical Corresponding Author: Considerations Mahmoud Ibrahim Kandil, MD, Faculty of Medicine, Benha University, El-Shaheed Farid Nada St, Benha, Qalyubia, 020, Egypt. The healthy navicular bone is typically boat shaped with Emails: [email protected], Mahmoud.ibrahim@fmed. convex distal and concave proximal articular surfaces. It bu.edu.eg 1448 Foot & Ankle International 40(12) Any lateral shift of the compression forces on the navic- Moreover, the central (not the lateral) middle third of the ular may then lead to further flattening and fragmentation of navicular has a relatively poor blood supply.6,20 Therefore, the navicular, often with the formation of a cleft between osteonecrosis would theoretically start in the central zone in the medial and dorsolateral fragments. This collapse may contrast to the lateral collapse and fragmentation seen with result in secondary lateral talar head shift and hindfoot MWD. varus. In late stages, further collapse and fragmentation Mohiuddin et al27 recently surmised that MWD is a result in direct articulation of the talus with the lateral cune- sequelae of undiagnosed navicular stress fractures with the iforms, creating enough space to permit plantarflexion of hypothesis that the central one-third of the navicular bone is the talar head and consequently paradoxical pes plan- subjected to the maximum shear stresses. Once again, their ovarus.23,43 However, it is not known whether the planus theory is about the middle rather than the lateral third of the deformity in MWD is a cause or a result of the navicular bone, and there are no available reports of MWD compli- collapse and fragmentation.38 cating a diagnosed navicular stress fracture. Relative hypertrophy of the second metatarsal and a The etiology of MWD may be multifactorial and associ- short first ray have been reported to be common findings in ated with uneven loading of a suboptimally ossified navicu- MWD.34 The short first ray could be attributed to primary lar that is at risk for central ischemia due to its vascularity.34 first metatarsal shortening, or it may be secondary to rela- Maceira and Rochera23 believed that 2 prerequisites are tive shortening of the medial column because of internal necessary for MWD: delayed ossification of the navicular rotation of the navicular.23,24 The short first ray may lead to and an abnormal force distribution pattern leading to com- abnormal force distribution with lateralization of stance pression of the lateral part of the bone. Delayed ossification phase compressive forces toward the second ray and lateral of the navicular may be due to a localized or generalized navicular.24 developmental disturbance. Hetsroni et al13 studied the plantar pressure distribution in patients with MWD and found increased midfoot plan- Clinical Presentation tar pressures with reduction in toe pressures. Pain and dis- comfort could be attributed to this abnormal pressure The typical presentation is chronic pain on the dorsum of distribution. the midfoot and/or hindfoot.23,27,39 There may be a delay in the diagnosis, and a stiff deformity is observed at presenta- 26 Epidemiology tion in most cases (Figure 1). Tenderness is usually located over the dorsomedial The true incidence of MWD is unknown. It is more fre- aspect of the midfoot. The medial longitudinal arch may be quently bilateral and demonstrates a female predomi- normal or low depending on the severity of the disease.27,43 nance.45 The female to male ratio was 6:1 in 1 study and 2:1 With regard to hindfoot alignment, a false impression of in another.13,45 Several studies have reported that the age of hindfoot valgus may occur due to prominence of the navic- patients at the time of diagnosis of MWD was over 40 ular on the medial aspect of the foot.34,43 Maceira and years.13,26,44 However, in a large multicenter study of 191 Rochera23 considered fixed hindfoot varus a constant find- cases (101 patients), the age at diagnosis ranged from 13 to ing in MWD. Cases in which this is accompanied by flatten- 91 years, with an average of 47.6 years.23 In the series of 12 ing of the longitudinal arch may be said to demonstrate cases reported by Doyle et al,7 1 patient was 14 years old. “paradoxical pes planovarus.” The paradoxical heel varus Patients with MWD may also have a have a higher body deformity with a concomitant pes planus is considered a mass index (BMI).27 Fornaciari et al10 reported a mean BMI hallmark of MWD.6,43 However, this is controversial and of 29.6 kg/m2 in their study of 10 patients while Hetsroni descriptions of hindfoot position in MWD have included et al13 reported an average BMI of 27 kg/m2. slight heel varus,19 flatfoot without hindfoot varus,45 neutral hindfoot,41 and flatfoot without comment on hindfoot posi- 4,5 12 Etiology and Pathogenesis tion. Haller et al reported 5 cases of MWD with flatfeet and hindfoot valgus. Only few published studies com- Several etiological theories of MWD have been proposed, mented on the subtalar motion, and both reduced including primary osteonecrosis, posttraumatic necrosis, motion7,23,27,43 and normal motion have been described.30,32,39 osteochondritis, congenital theory, and the abnormal evolu- Hetsroni et al13 reported that the subtalar motion was not tion of Köhler’s.23 Despite its description as osteonecrosis significantly limited. of the navicular, most of the histopathological studies do Patients with MWD may complain of anterior knee pain not provide conclusive evidence of osteonecrosis (empty because the tibia is forced into external rotation secondary lacunae).6,17,27,39 Tan et al38 presented a single case report to pes planovarus with posterior positioning of the fibula with histological evidence of osteonecrosis. Other micro- that could theoretically lead to altered knee biomechanics scopic studies, however, have reported normal bone.22,25,46 and patellar maltracking.23,27 Ahmed et al 1449 Figure 1. Plain radiographs of a patient having Müller-Weiss Figure 2. Plain radiographs comparing the foot of 2 different disease (that was accidentally discovered on routine radiographs patients to compare between (A) the flatfoot in Müller-Weiss after trivial trauma) showing the characteristic comma-shaped disease (MWD) and (B) flexible flatfoot. Note that the medial navicular bone with compression of its lateral part in addition to foot prominence in MWD is formed by the navicular and by the the development of talonavicular osteoarthritis.