How to Distinguish Marfan Syndrome from Marfanoid Habitus in a Physical Examination—Comparison of External Features in Patients with Marfan Syndrome and Marfanoid Habitus

Marfan Syndrome (MFS) is a systemic disorder caused by mutations in fibrillin-1. The most common cause of mortality in MFS is dissection and rupture of the aorta. Due to a highly variable and age-dependent clinical spectrum, the diagnosis of MFS still remains sophisticated. The aim of the study was to determine if there exist phenotypic features that can play the role of “red flags” in cases of MFS suspicion. The study population included 306 patients (199 children and 107 adults) who were referred to the Department of Pediatric Cardiology due to suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 109 patients and marfanoid habitus in 168 patients. The study excluded 29 patients with other hereditary thoracic aneurysm syndromes. Comparative analysis between patients with Marfan syndrome and marfanoid habitus was performed. Symptoms with high prevalence and high positive likelihood ratio were identified (pectus carinatum, reduced elbow extension, hindfoot deformity, gothic palate, downslanting palpebral fissures, lens subluxation, myopia ≥ 3 dioptres remarkably high stature). The differentiation between patients with MFS and marfanoid body habitus is not possible by only assessing external body features; however, “red flags” could be helpful in the screening phase.

Association of Decreased Physical Activity with Rheumatoid Mid-Hindfoot Deformity/Destruction

Foot/ankle problems remain important issues in rheumatoid arthritis (RA) patients. Although forefoot deformity generally takes a major place in surgical treatment, concomitant mid-hindfoot deformity is also commonly seen. In this situation, it can be easy to overlook that mid-hindfoot deformity can also induce or exacerbate clinical problems behind the forefoot events. Thus, the relationship between mid-hindfoot deformity/destruction and physical activity/ADL was investigated. Radiographic findings of 101 lower limbs (59 patients) were retrospectively evaluated. Alignment parameters in the lower extremity and joint destruction grade (Larsen grade) were measured. The timed-up-and-go (TUG) test, modified health assessment questionnaire (mHAQ), pain, self-reported scores for the foot and ankle (SAFE-Q), and RA disease activity were investigated to assess clinical status. The relationships among these parameters were evaluated. Subtalar joint destruction was correlated with TUG time (r = 0.329), mHAQ score (r = 0.338), and SAFE-Q: social functioning (r = 0.332). TUG time was correlated with the HKA (r = −0.527), talo-1st metatarsal angle (r = 0.64), calcaneal pitch angle (r = −0.433), M1-M5A (r = −0.345), and M2-M5A (r = −0.475). On multivariable linear regression analysis, TUG time had a relatively strong correlation with the talo-1st metatarsal angle (β = 0.452), and was negatively correlated with calcaneal pitch angle (β = −0.326). Ankle joint destruction was also correlated with TUG time (β = 0.214). Development of structural problems or conditions in mid-hindfoot, especially flatfoot deformity, were related with decreased physical activity in RA patients. Wearing an insole (arch support) as a preventative measure and short foot exercise should be considered from the early phase of deformity/destruction in the mid-hindfoot in the management of RA.

Alignment of the hindfoot in total knee arthroplasty: a systematic review of clinical and radiological outcomes

Aims Patients with a deformity of the hindfoot present a particular challenge when performing total knee arthroplasty (TKA). The literature contains little information about the relationship between TKA and hindfoot alignment. This systematic review aimed to determine from both clinical and radiological studies whether TKA would alter a preoperative hindfoot deformity and whether the outcome of TKA is affected by the presence of a postoperative hindfoot deformity. Methods A systematic literature search was performed in the databases PubMed, EMBASE, Cochrane Library, and Web of Science. Search terms consisted of “total knee arthroplasty/replacement” combined with “hindfoot/ankle alignment”. Inclusion criteria were all English language studies analyzing the association between TKA and the alignment of the hindfoot, including the clinical or radiological outcomes. Exclusion criteria consisted of TKA performed with a concomitant extra-articular osteotomy and case reports or expert opinions. An assessment of quality was conducted using the modified Methodological Index for Non-Randomized Studies (MINORS). The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines and registered in the PROSPERO database (CRD42019106980). Results A total of 17 studies were found to be eligible for review. They included six prospective and ten retrospective studies, and one case-control study. The effects of TKA showed a clinical improvement in the hindfoot deformity in three studies, but did not if there was osteoarthritis (OA) of the ankle (one study) or a persistent deformity of the knee (one study). The radiological alignment of the hindfoot corrected in 11 studies, but did not in the presence of a rigid hindfoot varus deformity (in two studies). The effects of a hindfoot deformity on TKA included a clinical association with instability of the knee in one study, and a shift in the radiological weightbearing axis in two studies. The mean MINORS score was 9.4 out of 16 (7 to 12). Conclusion TKA improves both the function and alignment of the hindfoot in patients with a preoperative deformity of the hindfoot. This may not apply if there is a persistent deformity of the knee, a rigid hindfoot varus deformity, or OA of the ankle. Moreover, a persistent deformity of the hindfoot may adversely affect the stability and longevity of a TKA. These findings should be interpreted with caution due to the moderate methodological quality of the studies which were included. Therefore, further prospective studies are needed in order to determine at which stage correction of a hindfoot deformity is required to optimize the outcome of a TKA. Cite this article: Bone Joint J 2021;103-B(1):87–97.

Late Abduction Deformity of the Midfoot Following the “Medial Double” (Diple) Procedure for Hindfoot Valgus Deformity

Category: Hindfoot Introduction/Purpose: There has been considerable interest in recent years in performing a combined talonavicular and subtalar arthrodesis instead of a standard triple arthrodesis for a rigid hindfoot deformity. The ”medial double” (diple) avoids fusing the calcaneocuboid joint with its lateral incision, common bone block graft and high rate of complications. Although the results of the “medial double” are generally satisfactory, there are no reports of late midfoot abduction deformity, although it was predicted by Evans in 1975. Methods: A 79 year-old-male with long-standing skin psoriasis with joint involvement presented with severe bilateral flatfoot deformities. The left hindfoot was arthritic, deformed and painful. Despite a conservative course of orthotics and ankle braces, the pain persisted. Because there was a psoriatic plaque over the lateral side of the foot, it was decided to perform a ”medial double” arthrodesis with screw fixation, a plantarflexing 1st tarsometatarsal joint fusion for medial column stabilization, and a heel cord lengthening Results: Skin and bony healing were uneventful. Over the subsequent three years a progressive abduction deformity developed through the naviculocuneiform joints and the unfused calcaneocuboid joint. The patient had only occasional pain in the midfoot and wore wide shoewear to accommodate the deformity. Conclusion: A late abduction deformity can develop through the midfoot in patients who have the ‘medial double” procedure for hindfoot valgus. The cuboid settles proximally against the short distal calcaneus, and the naviculocuneiform joints sublux laterally. In patients with hindfoot valgus with severe lateral column shortening secondary to calcaneocuboid joint remodeling, it may be better to perform an actual triple arthrodesis with a bone graft in the calcaneocuboid joint instead of a “medial double.” Alternatively, one could add a naviculocuneiform arthrodesis to the ”medial double” arthrodesis to prevent this complication.

The Hindfoot Alignment in Total Knee Arthroplasty: A Systematic Review

Category: Hindfoot Introduction/Purpose: Patients with a hindfoot deformity pose a particular challenge when performing a total knee arthroplasty (TKA). This could be attributed to the lack of insights concerning hindfoot alignment outcome. Our objective was to perform a systematic review of the literature to investigate the influence of TKA on hindfoot alignment and vice-versa. Methods: This systematic review was performed in accordance to PRISMA guidelines; the original protocol is registered on PROSPERO (ID: 106980). The following electronic databases were searched to identify capable studies: Pubmed, EMBASE, Web of Science, Google Scholar, and Cochrane Library. To ensure the quality of the review, a quality assessment was performed according to the MINORS criteria. The mean MINORS score was 9.5 (range 7-12) and concurred with a good quality of the obtained studies. Results: Sixteen identified articles met the review criteria: six prospective cohort studies, nine retrospective studies, and one case-control study. Five articles reporting on clinical outcomes were identified; a pre-operative hindfoot deformity negatively influenced TKP outcome (N=4) or demonstrated no influence (N=1). Thirteen articles reporting on radiographic outcomes were identified. Five studies reported a significant improvement (P < 0.05) of hindfoot alignment after TKA, two studies demonstrated improvement limited to valgus hindfeet, seven studies observed a significant (P < 0.05) correlation between knee and hindfoot alignment, and two studies reported the association between hindfoot OA in patients undergoing TKA. Two studies reporting biomechanical outcomes were identified; these reported a shift of the weighbearing axis after TKA as a result of the remaining hindfoot deformity. Conclusion: TKA can improve the alignment of valgus hindfeet, but improvement was less pronounced in varus hindfeet. Furthermore, a worse clinical outcome is expected after TKA in patients with concomitant ankle OA. A remaining hindfoot deformity shifts the weightbearing axis, which could shorten the survival of the total knee prosthesis.

Subtalar Nonunion Following Tibiotalocalcaneal Fusion Using an Intramedullary Nail: A Case Series of Errant Entry Points

Category: Hindfoot Introduction/Purpose: Tibiotalocalcaneal (TTC) arthrodesis is a procedure used to treat hindfoot deformity and/or arthritis. Retrograde intramedullary nails have been used as a method of fixation. The nails are either straight or have a bend to accommodate valgus alignment of the hindfoot. Studies comparing nail types or analysis of nonunions are lacking in the available literature on the subject despite a reported nonunion rate of up to 20%. The purpose of this study was to report a series of subtalar nonunions that all had an entry point that was too medial on the calcaneus resulting in inadequate purchase of the nail in the calcaneus. Methods: Six cases of subtalar nonunion were retrospectively reviewed. All were referred for second opinion. Evaluation consisted of examination, radiographs and CT scans. Patient demographics, comorbidities and findings common to all cases were recorded. Results: Presenting complaints in all cases were persistent swelling. Non-neuropathic cases all had hindfoot pain. In this series, all the implants were straight nails. Radiographs and CT scans revealed that all cases were done for severe valgus deformity with subtalar subluxation. In each of the cases, the deformity was under corrected and the nail entry point was too medial on the calcaneus which resulted in reaming out the medial wall of the calcaneus, decreasing the amount of fixation obtained with the nail. In each of the cases, the medial wall of the calcaneus was deficient and the nail had no medial containment. Conclusion: Severe valgus deformity with subtalar subluxation is a risk factor for subtalar nonunion when the deformity is under corrected and a straight nail is used. Ensuring that the tibia talus and calcaneus are collinear and that the entry point in the calcaneus is sufficiently lateral are important factors to consider when addressing this type of deformity. Accurate intraoperative imaging is essential to ensure proper positioning of the entry point to avoid reaming out the medial wall of the calcaneus and to ensure adequate purchase of the nail in the calcaneus.