Mueller-Weiss Syndrome: What to Know
Mallory-Weiss Tear
Severe and prolonged vomiting can result in tears in the lining of the esophagus. The esophagus is the tube that connects your throat to your stomach.
Mallory-Weiss syndrome (MWS) is a condition marked by a tear in the mucous membrane, or inner lining, where the esophagus meets the stomach.
Most tears heal within 72 hours without treatment, but Mallory-Weiss tears can cause significant bleeding. Depending on the severity of the tear, surgery may be necessary to repair the damage.
The most common cause of MWS is severe or prolonged vomiting. While this type of vomiting can occur with stomach illness, it also frequently occurs due to chronic alcohol use or bulimia.
Other conditions can result in a tear of the esophagus as well. These include:
- trauma to the chest or abdomen
- severe or prolonged hiccups
- intense coughing
- heavy lifting or straining
- gastritis, which is an inflammation of the lining of the stomach
- hiatal hernia, which occurs when part of your stomach pushes through part of your diaphragm
- convulsions
Receiving cardiopulmonary resuscitation (CPR) can also lead to a tear of the esophagus.
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MWS is more common in males than in females. It occurs more often in people with alcoholism. According to the National Organization for Rare Disorders, people between the ages of 40 and 60 are more likely to develop this condition. However, there are cases of Mallory-Weiss tears in children and young adults.
There are a few predisposing factors that can put you at risk of Mallory-Weiss tears. These can include:
- heavy alcohol consumption
- bulimia nervosa diagnosis
- persistent acid reflux
- severe vomiting
Having a hiatal hernia is currently up for debate as to whether it’s a risk factor for Mallory-Weiss tears. While many who have a diagnosis of MWS have reported having hernias, it’s still unclear whether there’s a proven link.
MWS doesn’t always produce symptoms. This is more common in mild cases when tears of the esophagus produce only a small amount of bleeding and heal quickly without treatment.
In most cases, however, symptoms will develop. These may include:
- abdominal pain
- vomiting up blood, which is called hematemesis
- involuntary retching
- bloody or black stools
Vomiting blood
Blood in the vomit will usually be dark and clotted and may look like coffee grounds. Occasionally it can be red, which indicates it’s fresh.
Blood that appears in the stool will be dark and look like tar, unless you have a large bleed, in which case it will be red.
If you have these symptoms, seek immediate emergency care. In some cases, blood loss from MWS can be substantial and life threatening.
There are other health problems that may produce similar symptoms.
Symptoms associated with MWS may also occur with the following disorders:
- Zollinger-Ellison syndrome, which is a rare disorder in which small tumors create excess stomach acids that lead to chronic ulcers
- chronic erosive gastritis, which is inflammation of the stomach lining that causes ulcer-like lesions
- perforation of the esophagus
- peptic ulcer
- Boerhaave’s syndrome, which is a rupture of the esophagus due to vomiting
It’s worth noting that MWS is a rare condition, and can only be diagnosed by a doctor. If you exhibit symptoms it’s important to see your physician immediately in order to get a proper diagnosis.
Your doctor will ask you about any medical issues, including daily alcohol intake and recent illnesses, to identify the underlying cause of your symptoms.
If your symptoms indicate active bleeding in the esophagus, your doctor may do what’s called an esophagogastroduodenoscopy (EGD).
You’ll need to take a sedative and a painkiller to prevent discomfort during this procedure. Your doctor will insert a small, flexible tube with a camera attached to it, called an endoscope, down your esophagus and into the stomach. This can help your doctor see your esophagus and identify the location of the tear.
Your doctor will likely also order a complete blood count (CBC) to confirm the number of red blood cells. Your red blood cell count may be low if you have bleeding in the esophagus.
Your doctor will be able to determine if you have MWS based on the findings from these tests.
According to the National Organization for Rare Disorders, the bleeding that results from tears in the esophagus will stop on its own in about 80 to 90 percent of MWS cases.
Healing typically occurs in a few days and doesn’t require treatment. But if the bleeding doesn’t stop, you may need one of the following treatments.
Endoscopic therapy
You may need endoscopic therapy if the bleeding doesn’t stop on its own. The doctor performing the EGD can do this therapy. Endoscopic options include:
- injection therapy, or sclerotherapy, which delivers medication to the tear to close off the blood vessel and stop the bleeding
- coagulation therapy, which delivers heat to seal off the torn vessel
Extensive blood loss may require the use of transfusions to replace lost blood.
Surgical and other options
Sometimes, endoscopic therapy isn’t enough to stop the bleeding. So doctors use other ways of stopping it, such as laparoscopic surgery to sew the tear.
If you can’t undergo surgery, your doctor may use arteriography to identify the bleeding vessel and plug it to stop the bleeding.
Medication
Medications to reduce stomach acid production, such as famotidine (Pepcid) or lansoprazole (Prevacid), may also be necessary. However, the effectiveness of these medications is still under debate.
Mueller-Weiss Syndrome: What to Know
Mueller-Weiss syndrome is a rare condition that causes severe arch pain on the inside of your mid- and hindfoot. It’s mostly found in people ages 40-60, and it’s much more common in women. It’s a progressive disease, meaning it gets worse over time, and it usually affects both of your feet. It causes flatfoot, which happens when your arch collapses. It can also cause your feet to be disfigured or bent out of shape.
What Causes Mueller-Weiss Syndrome?
Experts don’t know its exact cause. But they think it may be caused by things like:
- A forceful squeeze or compression on the cluster of bones in your midfoot (known as the lesser tarsus)
- A birth defect (congenital)
- The loss of blood supply to your navicular bone (ischemia)
Your navicular bone is a half-moon or boat-shaped bone in the middle of your foot. If it suddenly loses its blood supply, your bone tissue starts to die, which then causes your joint to erode and your bone to collapse over time.
Your navicular bone also happens to be the last bone to fully form (known as ossification) in your foot during childhood. If this bone was slow to form, you might be at risk for Mueller-Weiss syndrome. Research also suggests you might be at higher risk if you’re obese or overweight.
What Are the Symptoms of Mueller-Weiss Syndrome?
- Severe midfoot pain
- Foot swelling
- Tenderness on top of your feet
- Arch pain
- Trouble walking
- Your navicular bone becomes comma or hourglass shaped.
But it might take months or years before you notice any symptoms.
How Is It Diagnosed?
Because Mueller-Weiss syndrome is uncommon, it’s often under- or misdiagnosed. That’s because doctors might be slow to identify flatfoot issues. If you have foot pain, especially in your arch or around your midfoot, let your doctor know.
They may refer you to a podiatrist (foot and ankle expert) or an orthopedist (doctor who specializes in bones). They’ll take your detailed medical history and examine your feet.
They may run a few imaging tests on both of your feet. Those may include:
This will help them get a clear picture of what’s going on. Based on the amount of tissue death and erosion you may have in your navicular bone and its surrounding area, they’ll stage your condition and symptoms as mild (stage 1), moderate (stage 2-3), or severe (stage 4-5).
What Are the Treatment Options?
For now, there isn’t one best treatment for Mueller-Weiss syndrome. If your condition and symptoms are in the early stages, your doctor may prescribe nonsurgical therapies to help improve your quality of life.
These may include:
- Anti-inflammatory medications (NSAIDs)
- Braces that cover and support your foot and ankle (ankle-foot orthosis)
- A temporary foot cast to stop you from moving your foot too much
- Custom insoles or shoes for pain relief and arch support (orthotics)
- Less physical activity
If these therapies don’t ease your pain, or your symptoms get worse over 6 months, your doctor might consider surgery. It’s often recommended if your condition is past stage 2.
The surgery might include a combination of:
Talonavicular cuneiform arthrodesis. This procedure is done to ease your foot pain and restore some arch support.
Bone grafting. For this procedure, your doctor will take a piece of bone from your hips, legs, or ribs and use it to repair your damaged foot bones. In some cases, they might use bone from a donor.
There are other types of surgeries for this condition, such as joint fusion, but they are less common. You may need physical therapy before and after your surgery to help you recover.
What’s the Outlook?
Over-the-counter pain medications, custom shoes, and foot braces might help ease your pain, take any pressure or stress off your feet, and improve your quality of life. If these therapies don’t help, talk to your doctor to find out if surgery might be an option for you.
Show Sources
Mayo Clinic: “Avascular Necrosis (Osteonecrosis).”
Johns Hopkins Medicine: “Bone Grafting.”
Indian Journal of Musculoskeletal Radiology: “Mueller Weiss syndrome, a less elucidated and unusual cause of midfoot pain: A case report.”
World Journal of Orthopedics: “Müller-Weiss disease: Four case reports.”
Foot and Ankle Clinics: “Management of Muller-Weiss Disease.”
Foot and Ankle Online Journal: “Case study of idiopathic degeneration of the talonavicular joint.”
American Journal of Roentgenology: “Imaging of Mueller-Weiss Syndrome: A Review of Clinical Presentations and Imaging Spectrum.”
Mueller-Weiss Syndrome
August 2016, VOLUME 207
NUMBER 2
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August 2016, Volume 207, Number 2
Residents’ Section
Review
Imaging of Mueller-Weiss Syndrome: A Review of Clinical Presentations and Imaging Spectrum
Mohammad Samim 1 , Hicham A. Moukaddam 1, 2 and Edward Smitaman 3
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+ Affiliations:
1 Department of Radiology, Yale University School of Medicine, New Haven, CT 06520-8042.
2 Present address: 1606 Leist Ave., Lima, OH 45805.
3 Department of Radiology, University of California San Diego, San Diego, CA.
Citation: American Journal of Roentgenology. 2016;207: W8-W18. 10.2214/AJR.15.15843
OBJECTIVE. Mueller-Weiss syndrome is a complex condition of the adult tarsal navicular characterized by progressive fragmentation leading to mid- and hindfoot pain and deformity. Since its first descriptions in the early 20th century, controversy has persisted regarding its pathogenesis.
CONCLUSION. This article reviews the literature and discusses the anatomy, epidemiology, causes, clinical and radiologic findings, and treatment of Mueller-Weiss syndrome, and thus permits a better understanding of this disease and its management.
Mueller-Weiss syndrome, also known as Brailsford disease [1], is a rare disease that historically refers to spontaneous adult-onset tarsal navicular osteonecrosis. Although this entity was initially described by Schmidt in 1925, it was subsequently named after W. Müller, who postulated that this condition was either secondary to compressive force acting on the tarsus or a congenital defect [2, 3], and K. Weiss, who recognized radiographic findings similar to those of lunate necrosis and attributed this navicular deformity to an ischemic process [4]. Mueller-Weiss syndrome is a separate entity from Koehler phenomenon, which is a childhood osteochondrosis of the navicular thought to reflect either an ossification variant or, perhaps, osteonecrosis [5, 6]. Mueller-Weiss syndrome is uncommonly encountered and, as such, may be misdiagnosed and improperly treated [7]. This article presents a review of the literature regarding Mueller-Weiss syndrome, including anatomy, epidemiology, pathophysiology, clinical and radiologic findings, and treatment, thus allowing earlier recognition and improved treatment of this disease.
The navicular (from the Latin navicularis, which means pertaining to a boat or boat-shaped) bone has two major oblique axes oriented in the dorsoplantar and lateromedial directions [8]. Its concave posterior cartilaginous surface articulates with the talar head. Anteriorly, it has a convex nephroid appearance ( Fig. 1 ) with two crests creating three articular surfaces (medial, middle, and lateral), each articulating with a corresponding cuneiform bone. These three anterior articular surfaces converge plantarly to form the transverse tarsal arch [8]. The navicular bone is also considered the keystone of the medial column of the foot. Thus, the navicular contributes to the integrity of both the transverse and medial longitudinal arches of the foot [9, 10].
Fig. 1A —22-year-old man with normal navicular anatomy.
A, Frontal weight-bearing radiograph of foot shows normal concave posterior surface of navicular bone articulating with talar head. Anteriorly, navicular has convex nephroid appearance with two crests creating three articular surfaces for cuneiform bones (refer to panel D). Note cyma line, S-shaped line composed of well-aligned talonavicular and calcaneocuboid joints (dotted yellow line). Area of relative decreased vascularization (striated black and white oval) resides in central portion of navicular, between dorsalis pedis and medial plantar arteries (portions of which are drawn in red).
Fig. 1B —22-year-old man with normal navicular anatomy.
B, Oblique weight-bearing radiograph of foot depicts one (arrow) of two crests at convex articular surface of navicular.
Fig. 1C —22-year-old man with normal navicular anatomy.
C, Lateral weight-bearing radiograph of foot shows Meary-Tomeno axis (dashed black line), angle formed between longitudinal axes of talus and first metatarsal, which is 0° and normal in this foot. Navicular is obliquely oriented in dorsoplantar or coronal plane (dashed white line). Note that cyma line (dotted yellow line) can also be seen on lateral view of foot.
Fig. 1D —22-year-old man with normal navicular anatomy.
D, Volume-rendered 3D image shows two crests (arrows) at convex articular surface of navicular, creating three articular surfaces for cuneiform bones. Navicular is also obliquely oriented in mediolateral or short-axis plane (dashed line) of foot.
The navicular has an osseous prominence or tuberosity medially, which can be variable in size, and when it is unfused it is known as an accessory navicular bone, os naviculare, or os tibiale externum. The talocalcaneonavicular joint—also known as the acetabulum pedis because of its morphologic similarity to the hip joint [11]—is a ball-in-socket synovial joint with rotating and gliding movements. The naviculocuneiform and cuboideonavicular articulations are also synovial joints capable of gliding function [10]. Several studies have suggested that the talonavicular and naviculocuneiform joints bear the greatest stress and load transmission, compared with other joints, in the foot [12–14]. As such, these joints and osseous structures are stabilized by numerous ligaments, including the dorsal and plantar cuneonavicular ligaments, bifurcate ligament, spring ligamentous complex, and cuboideonavicular ligaments [8, 10] (a summary of these ligaments is provided in Table 1).
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TABLE 1: Perinavicular Ligaments and Anatomy
The dorsalis pedis and medial plantar arteries provide blood supply to the navicular in a circumferential and centripetal fashion—the former supplies the dorsal and lateral one-third of the navicular, and the latter nourishes the plantar aspect of the navicular [10, 15]—creating a centripetal vascular perfusion arch [7]. The navicular tuberosity is supplied by the anastomosis of these two arteries [8]. As a result of this vascular configuration, there is a paucity of vascularization centrally [10, 16] ( Fig. 1 ); this finding increases with age [17] and may partly explain the prevalence of stress fractures and osteonecrosis in this navicular region [16, 18–20].
| Epidemiology |
|---|
Mueller-Weiss syndrome usually affects adults between 40 and 60 years of age, is more common in women (up to 70% of cases in one study) [7], and is frequently bilateral [10, 21]. Although 277 cases of Mueller-Weiss syndrome have been reported in the literature to our knowledge [21–38], the true frequency of this disease is unknown and likely underestimated, because it may be misattributed to osteoarthrosis of the perinavicular joints [7, 39].
In the largest published series to date (n = 191) by Maceira and Rochera [7], the highest frequency of Mueller-Weiss syndrome was observed after major struggles (e.g., crop failures, war, post-war poverty, and international isolation) in Spain during the first half of the 20th century, suggesting environmental or nutritional predispositions leading to a generalized developmental disturbance. In their study, Maceira and Rochera highlighted skeletal signs of extrinsic stress and developmental disturbance that are common in Mueller-Weiss syndrome: growth arrest lines, dental enamel hypoplasia, and—most importantly, because it is a prerequisite for Mueller-Weiss syndrome—delayed ossification of the navicular. However, other authors did not find an association between Mueller-Weiss syndrome and conditions of extreme environmental stress (i.e., poverty) [33], and no history of malnourishment, chronic disease, or the presence of growth arrest lines were observed in the cases used for this article.
| Pathogenesis |
|---|
The cause of Mueller-Weiss syndrome is unknown, but several theories have been proposed, including primary or secondary osteonecrosis [7, 21, 27, 40–42], osteochondrosis [43], trauma or altered biomechanics [44], congenital causes [7, 45], dysplasia [46], and migration of an accessory cuboid [45]. Regardless of the exact cause, the pathogenesis of Mueller-Weiss syndrome is probably multifactorial and related to chronic loading on a suboptimally ossified navicular—a bone that is predisposed to central ischemia owing to its centripetal vascular perfusion arch [7, 47, 48].
In Mueller-Weiss syndrome, the chronic loading forces are unevenly distributed, preferentially causing compression of the lateral half of the navicular. Any biomechanical precondition—such as, subtalar varus, first ray brachymetatarsia, and mild forms of club-foot [7]—that leads to lateral displacement of the net compressive force on the navicular and lateral incompetence of the acetabulum pedis may contribute to the development of Mueller-Weiss syndrome.
Suboptimal ossification of the navicular is the second precondition to the development of Mueller-Weiss syndrome. The navicular is the last tarsal bone to ossify, during a time of increased mobility in children (i.e., at the end of the second year in girls and at the beginning of the fourth year in boys) [49], and therefore may be at increased risk of shearing injury at the level of the lateral cuneiforms. Other extrinsic factors that not only contribute to suboptimal navicular ossification but also compromise its vascular perfusion are diabetes, nutritional deficiency, smoking, alcohol, corticosteroid use, and various rheumatologic, hematologic, and metabolic disorders [25, 50–52]. In a recent study, Harmouche et al. [12] observed decreased anteroposterior dimension of the navicular in patients with type 2 diabetes, highlighting the influence of abnormal chronic loading and vascularity in this cohort of patients with likely some degree of neuroarthropathy and vascular compromise.
| Clinical Findings |
|---|
Most patients present with chronic midand hindfoot pain in the fourth or fifth decades [7, 10], as the navicular collapses and secondary osteoarthrosis develops. With further navicular collapse, pes planovarus develops, and the tibia is forced into external rotation, which may lead to altered biomechanics and osteoarthrosis of the knee [7, 10, 47].
Physical findings include swelling and tenderness over dorsomedial aspect of the midfoot and heel varus, but a prominent navicular tuberosity may give the false clinical impression of hindfoot valgus, or pseudohindfoot valgus. The medial longitudinal arch can be markedly flattened depending on the severity of the disease [10]. This combination of pseudohindfoot valgus and pes planus deformity is also known as paradoxical pes planovarus [7, 10, 47] ( Fig. 2 ).
Fig. 2A —36-year-old man with foot pain and Mueller-Weiss syndrome.
A, Long-axis CT image of foot shows collapse and sclerosis of lateral half of navicular (arrow) and peritalar (i.e., medial) subluxation of navicular tuberosity (star), creating navicular with comma-shaped appearance and pseudohindfoot valgus.
Fig. 2B —36-year-old man with foot pain and Mueller-Weiss syndrome.
B, Long-axis CT image of foot (superior to image in A) shows extrusion of dorsolateral fragment of fragmented navicular (arrow), or “listhesis navicularis.”
| Radiologic Findings and Staging |
|---|
Weight-bearing radiographs in the frontal and lateral projections remain the test of choice in the evaluation of Mueller-Weiss syndrome, and its radiologic findings can be grouped by anatomic location for didactic purposes [7, 10, 22, 24, 47].
Ankle and Hindfoot
As the lateral half of the navicular collapses, there is peritalar (i.e., medial) subluxation of the talar head, resulting in hindfoot varus with loss of the cyma line (an S-shaped line that is seen on frontal and lateral radiographs and is composed of well-aligned talonavicular and calcaneocuboid joints). On the lateral view, there is an impression of a capacious sinus tarsi as the talocalcaneal angle (the angle between the talar and calcaneal longitudinal axes on a weight-bearing lateral radiograph; a normal angle is between 25° and 40°) decreases. There is also retroposition of the fibula as the tibia externally rotates.
Midfoot
As the lateral half of the navicular collapses, it becomes sclerotic and comma- or hourglass-shaped in appearance. There is peritalar subluxation of the remaining medial aspect of the navicular bone; this, along with the aforementioned lateral subluxation of the talar head, leads to creation of a talocuneiform (i.e., talus and cuneiforms) articulation ( Fig. 3 ), osteoarthrosis of the talonavicular and naviculocuneiform joints, and medial subluxation of the cuboid with respect to the calcaneus (the cuboid sign).
Fig. 3 —Schematic progression of Mueller-Weiss syndrome. Long-axis diagram of portions of mid- and hindfoot shows gradual collapse and, ultimately, fragmentation of lateral aspect of navicular (arrows). With progressive compression and fragmentation of navicular, there is lateral subluxation of talar head (arrowheads) that results in subtalar varus, pseudohindfoot valgus (stars), and talocuneiform neoarticulation (shaded yellow area). Adapted from Foot and Ankle Clinics, vol. 9, Maceira E, Rochera R, Müller-Weiss disease: clinical and biomechanical features, 105–125, copyright 2004, with permission from Elsevier [7]
Forefoot
As the plantar arch fails, the metatarsals assume a parallel orientation, and hypertrophic changes of the second metatarsal develop because of altered transmission of compressive forces through the second, rather than the first, metatarsal and tarsometatarsal joints.
Maceira and Rochera [7] also provided a descriptive staging system, composed of five degrees of deformity, for Mueller-Weiss syndrome as assessed on a weight-bearing lateral radiograph (Table 2 and Fig. 4 ). The degree of deformity is determined by the appearance of the navicular and the Meary-Tomeno angle (the angle formed between the longitudinal axes of the talus and first metatarsal). In the normal foot, these axes are aligned. Additionally, this line passes through the axes of the navicular and medial or first cuneiform ( Fig. 1 ). A Meary-Tomeno angle greater than 4° convex downward is indicative of pes planus [10]. The radiologic findings—according to Maceira and Rochera’s staging system—of mild (stage 1), moderate (stages 2 and 3), and advanced disease (stages 4 and 5)—are summarized in Table 2. Although familiarity with their radiologic staging system may help understand Mueller-Weiss syndrome, Maceira and Rochera highlight that the transition between increasing degrees of deformity may not be seen with long-term follow-up and that the severity of clinical symptoms may not correspond with the radiologic stage.
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TABLE 2: Five Radiologic Stages of Mueller-Weiss Syndrome
Fig. 4 —Stages of Mueller-Weiss syndrome. Stage 1: normal, but subtle subtalar varus deformity may be present due to lateral displacement of talar head, causing it to overlap with anterior calcaneal process (crosshatch). Solid line denotes normal Meary-Tomeno axis. Stages 2 through 4: dorsal, neutral, and plantar intersection and thus, angulation of Meary-Tomeno lines (dashed lines), respectively. Note splitting of navicular in stage 3. Stage 5: talocuneiform neoarticulation and extrusion of fragmented navicular, or “listhesis navicularis” (arrow). Blue shaded areas denote navicular bone. Adapted from Foot and Ankle Clinics, vol. 9, Maceira E, Rochera R, Müller-Weiss disease: clinical and biomechanical features, 105–125, copyright 2004, with permission from Elsevier [7]
Nevertheless, using Maceira and Rochera’s [7] staging system coupled with an understanding of the findings by anatomic location, a sequence of events caused by a net lateral compression force acting on a suboptimally ossified navicular can be appreciated with both radiographs and cross-sectional imaging ( Figs. 5 and 6A – 6F ): first, lateral compression of the navicular; second, lateral subluxation of the talar head (i.e., acetabular protrusion at the lateral aspect of the acetabulum pedis); third, subtalar varus; fourth, fragmentation of the navicular into large medial and small lateral or dorsolateral fragments with extrusion (“listhesis navicularis”); fifth, exposure of the lateral, and sometimes the intermediate, cuneiforms with talocuneiform neoarticulation; and sixth, plantar flexion of the talus leading to paradoxical pes planovarus. Cross-sectional imaging can glean additional information. CT can assess bony arrangement for preoperative planning, and MRI can detect bone marrow edema and early osteoarthritic changes and also exclude other differential considerations, such as stress fractures or infection [10].
Fig. 5A —43-year-old man with chronic midfoot pain and stage 3 (moderate) Mueller-Weiss syndrome.
A, Frontal radiograph of foot shows comma-shaped, sclerotic, and split navicular (arrow), disruption of cyma line (dotted yellow lines), and lateral subluxation of talar head (arrowhead).
Fig. 5B —43-year-old man with chronic midfoot pain and stage 3 (moderate) Mueller-Weiss syndrome.
B, Lateral radiograph of foot shows neutral Meary-Tomeno angle (dashed line) and compressed and split navicular (arrow).
Fig. 5C —43-year-old man with chronic midfoot pain and stage 3 (moderate) Mueller-Weiss syndrome.
C, Long-axis (C) and T2-weighted fat-suppressed (D) images show marrow edema (arrows, C and D) in split navicular, beginnings of neoarticulation between laterally subluxed talar head and lateral cuneiform (arrowhead, D), and degenerative changes at talonavicular joint and early talocuneiform neoarticulation.
Fig. 5D —43-year-old man with chronic midfoot pain and stage 3 (moderate) Mueller-Weiss syndrome.
D, Long-axis (C) and T2-weighted fat-suppressed (D) images show marrow edema (arrows, C and D) in split navicular, beginnings of neoarticulation between laterally subluxed talar head and lateral cuneiform (arrowhead, D), and degenerative changes at talonavicular joint and early talocuneiform neoarticulation.
Fig. 5E —43-year-old man with chronic midfoot pain and stage 3 (moderate) Mueller-Weiss syndrome.
E, Sagittal T2-weighted fat-suppressed image shows split navicular and some marrow edema in more plantar navicular fragment (arrows).
Fig. 6A —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
A, Frontal (A) and oblique (B) radiographs of foot show comma-shaped fragmented navicular (large arrows, A and B) with extrusion, or “listhesis navicularis.” There is also lateral subluxation of talar head with talocuneiform neoarticulation (arrowhead, A), loss of cyma line (dotted yellow lines, A), pseudohindfoot valgus (stars, A and B), medial subluxation of cuboid with respect to calcaneus, creating cuboid sign (small arrow, A), and osteoarthrosis of perinavicular joints.
Fig. 6B —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
B, Frontal (A) and oblique (B) radiographs of foot show comma-shaped fragmented navicular (large arrows, A and B) with extrusion, or “listhesis navicularis.” There is also lateral subluxation of talar head with talocuneiform neoarticulation (arrowhead, A), loss of cyma line (dotted yellow lines, A), pseudohindfoot valgus (stars, A and B), medial subluxation of cuboid with respect to calcaneus, creating cuboid sign (small arrow, A), and osteoarthrosis of perinavicular joints.
Fig. 6C —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
C, Lateral radiograph of foot shows fragmented navicular (arrow), slight plantar angulation of Meary-Tomeno lines (dashed lines), and capacious sinus tarsi (oval) owing to subtalar varus.
Fig. 6D —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
D, Long-axis T1-weighted (D) and T2-weighted (E) fat-suppressed images confirm previous radiographic findings: fragmented navicular (arrow, D), talocuneiform neoarticulation (arrowheads, D and E), pseudohindfoot valgus (stars, D and E), and chondral loss with mild to moderate amount of subchondral marrow edema involving perinavicular joints indicating osteoarthrosis.
Fig. 6E —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
E, Long-axis T1-weighted (D) and T2-weighted (E) fat-suppressed images confirm previous radiographic findings: fragmented navicular (arrow, D), talocuneiform neoarticulation (arrowheads, D and E), pseudohindfoot valgus (stars, D and E), and chondral loss with mild to moderate amount of subchondral marrow edema involving perinavicular joints indicating osteoarthrosis.
Fig. 6F —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
F, Sagittal T2-weighted fat-suppressed image confirms fragmented navicular (arrow) and perinavicular osteoarthrosis.
Fig. 6G —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
G, Subsequent frontal (G), oblique (H), and lateral (I) postoperative images of foot show talonaviculocuneiform arthrodesis using medial and dorsal side plates with associated screws. Navicular (arrows) and perinavicular joints have been reduced into adequate alignment. Notice bone graft harvest site in calcaneal body, which is filled in with methyl methacrylate.
Fig. 6H —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
H, Subsequent frontal (G), oblique (H), and lateral (I) postoperative images of foot show talonaviculocuneiform arthrodesis using medial and dorsal side plates with associated screws. Navicular (arrows) and perinavicular joints have been reduced into adequate alignment. Notice bone graft harvest site in calcaneal body, which is filled in with methyl methacrylate.
Fig. 6I —40-year-old woman with chronic foot pain and stage 5 (advanced) Mueller-Weiss syndrome.
I, Subsequent frontal (G), oblique (H), and lateral (I) postoperative images of foot show talonaviculocuneiform arthrodesis using medial and dorsal side plates with associated screws. Navicular (arrows) and perinavicular joints have been reduced into adequate alignment. Notice bone graft harvest site in calcaneal body, which is filled in with methyl methacrylate.
With progressive lateral collapse of the tarsal navicular and distortion of the midfoot anatomy, the perinavicular ligaments may undergo degeneration, but the precise soft-tissue pathoanatomy of Mueller-Weiss syndrome has not been explained in the literature. This reflects the emphasis placed on the osseous changes in diagnosing and staging Mueller-Weiss syndrome. However, it can be surmised that there is failure at the lateral aspect—lateral calcaneonavicular component of the bifurcate ligament and lateral calcaneonavicular capsule—of the acetabulum pedis [53], because Mueller-Weiss syndrome is the clinical expression of acetabular protrusion at the lateral aspect of the acetabulum pedis [7]. Future research on the static and dynamic soft-tissue stabilizers involved in Mueller-Weiss syndrome may provide further insight and improved treatment of this disease.
Initial treatment is conservative and includes antiinflammatories, custom orthotics, short-leg casts, and activity restriction. The use of orthotics may effectively decrease pain and improve function [25].
Surgical treatment may be indicated if symptoms persist or after failure of conservative management. Currently, there is no standard surgical procedure. Although most authors consider surgery on the basis of symptom severity, rather than the degree of the deformity, the majority of surgical cases are stage 3 (moderate in degree) or worse [10, 25, 28]. Multiple surgical techniques have been described in the literature, all sharing two common goals [1]: relief of pain by fusing symptomatic degenerative joints and [2] restoration of the plantar and medial longitudinal arches by correcting the Meary-Tomeno line [7, 10, 25, 28]. Talonaviculocuneiform arthrodesis with bone grafting satisfies these two goals and provides the most satisfactory results [10, 32, 54] ( Figs. 6G – 6I ). There are other less commonly performed procedures for Mueller-Weiss syndrome, but each has its weakness. Percutaneous core decompression of the navicular can be performed in the early stages of the disease [28], but most patients have already developed degenerative changes and simple core decompression is less likely to be of benefit. Isolated talonavicular arthrodesis has a high risk of pseudoarthosis because the naviculocuneiform joint is not repaired [32, 54]. Finally, triple arthrodesis (i.e., fusion of the subtalar, talonavicular, and calcaneocuboid joints) [55] fails to address naviculocuneiform osteoarthrosis.
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Mueller-Weiss syndrome is likely a multifactorial disease of the tarsal navicular related to chronic lateral overloading on a suboptimally ossified bone that is predisposed to central ischemia. Mueller-Weiss syndrome can result in disability and deformity; however, a better understanding of this disease and its radiologic manifestations may allow earlier diagnosis and improved future management.
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