CASE REPORT


https://doi.org/10.5005/jp-journals-10040-1279
Journal of Foot and Ankle Surgery (Asia-Pacific)
Volume 11 | Issue 1 | Year 2024

Recurrent Synovial Chondromatosis of the Fifth Metatarsophalangeal joint of the Foot: A Case Report


Vijay Kumar Digge1, Sai Krishna MLV2https://orcid.org/0000-0001-7993-9069, Anmol Anand3, Sumit Kumar4

1–4Department of Orthopaedics, All India Institute of Medical Sciences (AIIMS), Delhi, India

Corresponding Author: Sai Krishna MLV, Department of Orthopaedics, All India Institute of Medical Sciences (AIIMS), Delhi, India, Phone: +91 9894337104, e-mail: krishna.mlv.sai@gmail.com

Received: 01 September 2022; Accepted: 17 December 2022; Published on: 30 December 2023

ABSTRACT

Background: Primary synovial chondromatosis is a disease occurring in normal joints and is characterized by the formation of hyaline cartilage in the subintimal layer of the synovium and loose body formation eventually leading to degenerative arthritis. Though it mostly affects large joints, it can rarely involve small joints of the foot as well. The treatment is excision of the loose bodies along with complete synovectomy because partial synovectomy leads to recurrence. The disorder is rarely associated with malignant transformation as well and hence the excised tissue should always be sent for histopathological examination.

Case description: A 15-year-old girl presented to us with a painful swelling over the fifth metatarsophalangeal joint of the foot. She had a past history of similar complaints two years ago for which she underwent surgical excision of the same. She presented to us again with recurrence, for which she again underwent surgical excision and the biopsy was sent for histopathological examination which was suggestive of metaplastic calcification with no evidence of malignant transformation.

Conclusion: The treatment for synovial chondromatosis is synovectomy and loose body removal either as an arthroscopic or open procedure, and incomplete synovectomy has been attributed to higher recurrence rates. Malignant transformation though rare has also been described and should always be suspected in a recurrent disease that has rapid growth and bone destruction and requires histopathological examination.

How to cite this article: Digge VK, MLV SK, Anand A, et al. Recurrent Synovial Chondromatosis of the Fifth Metatarsophalangeal joint of the Foot: A Case Report. J Foot Ankle Surg (Asia-Pacific) 2024;11(1):33–37.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient’s parents/legal guardians for publication of the case report details and related images.

Keywords: Foot, Metatarsophalangeal joint, Recurrence, Synovial chondromatosis

INTRODUCTION

Synovial chondromatosis or synovial chondromatosis though first described by Leanacc in 1833, was first quoted in literature by Henderson and Jones in 1923 and had an incidence of 1:100000 with a male predominance in their 3rd–5th decades.1,2 Synovial chondromatosis can be classified either as a primary or secondary disease. Primary synovial chondromatosis is a disease occurring in normal joints, whereas secondary occurs in degenerative joints. The etiopathogenesis of the synovial chondromatosis is still not lucid and can be either metaplastic or benign neoplastic.2 Though the disorder affects a single joint and presents as a monoarticular disease at times; it can also affect multiple joints and present as a polyarticular disease in 5% of patients. Of all the joints, the most commonly affected joint is the knee joint, followed by the hip, elbow, and shoulder.1 Synovial chondromatosis of the smaller joints like the foot and ankle is extremely rare and of the handful of cases reported in the literature, the affected joints are ankle, subtalar, intertarsal, tarsometatarsal, and metatarsophalangeal joints.2-6 In this case report, we present a primary recurrent synovial chondromatosis of the fifth metatarsophalangeal joint.

CASE DESCRIPTION

A girl in her teens presented to us with a painful swelling over the fifth metatarsophalangeal joint for the past 8 months. She had a similar history of painful swelling 3 years back at the same site, which was excised, and a healed scar was present on the lateral aspect of the fifth metatarsophalangeal joint. After the primary surgery, she was fine for 2 years, after which she developed recurrent swelling at the same site. Because of the ongoing pandemic, she presented to us 8 months after the onset of swelling. The swelling was firm, painful to touch and on the dorsal side of the fifth web space measuring 5 × 5 cm with normal overlying skin (Fig. 1). There was no history of fever, weight loss, or any other constitutional symptoms.

Fig. 1: Preoperative images showing swelling in the web space between the fourth and fifth digit

The girl’s serum calcium and phosphorus were within normal limits. The X-ray of the foot (anteroposterior and lateral view) (Fig. 2) was suggestive of multiple oval ossified lesions at the fifth metatarsophalangeal joint. The magnetic resonance imaging (MRI) of the foot (Fig. 3); coronal, sagittal, and axial T1 and T2 images, showing irregularly shaped lesion hypo intense in all sequences and seen in the fourth and fifth interphalangeal region.

Fig. 2: X-ray of the foot (anteroposterior and lateral view) was suggestive of multiple oval ossified lesions at the fifth metatarsophalangeal joint

Fig. 3A to F: Coronal, sagittal, and axial MRI T1 and T2 images, showing irregularly shaped lesion hypo intense in all sequences and seen in the fourth and fifth interphalangeal region

The patient was positioned supine, and under regional anesthesia, a Y-shaped incision (Fig. 4) was used around the circumference of the swelling in the fifth web space. The swelling was removed in total along with synovectomy was done of the fifth metatarsophalangeal joint. The wound was closed and a below-knee plaster was applied. The plaster was removed at 2 weeks and the sutures were also removed. Later full weight-bearing mobilization was started with no restriction on any of the activities. The excised material was sent for histopathological examination, which was suggestive of calcification and metaplastic bone formation (Fig. 5).

Figs 1A and B: Intraoperative picture during excision of the swelling

Figs 5A and D: (A) Hematoxylin and eosin (H&E) stained section showing lobular dystrophic calcification in subcutaneous tissue (H&E x40); (B) There is histiocytic cell proliferation with foreign body giant cell reaction (arrows) (H&E x200); (C and D) Mature bone formation is seen in the calcified tissue (H&E x40 and H&E x100)

At 1-year follow-up the girl showed no recurrence and had no complaints at the surgical site (Figs. 6 and 7) and was able to carry out her daily activities and schooling. However, we plan to keep the patient in regular follow-up, keeping in mind the likelihood of a recurrence and thus its early detection.

Figs 6A and B: Healed skin after surgical excision of the swelling

Fig. 7: Postoperative radiograph of the patient

DISCUSSION

Synovial chondromatosis affects synovium-lined structures, either intra-articular synovium or extra-articular synovium-lined structures. The disorder is characterized by the formation of hyaline cartilage in the subintimal layer of the synovium and loose body formation. The disorder is most commonly seen in the intra-articular synovial lined structures like joints, and of all the joints, large joints like the knee joint are most commonly affected. The extra-articular synovium-lined structures, which are also affected but not as common as intra-articular synovium, are bursae and tendon sheaths and are called tenosynovial chondromatosis. The symptoms are because of inflammation like joint pain and swelling, and loose bodies like locking and instability, which eventually leads to degenerative arthritis.1,4

The pathology is characterized by subsynovial hyaline cartilage formation, either metaplastic or neoplastic in origin, along with synovial hyperplasia and eventually leading to the formation of loose bodies, either cartilaginous or osteocartilaginous. The metaplastic origin is attributed to the presence of high levels of bone morphogenic proteins in the synovial tissue and loose bodies.2 But the cytological studies have shown it to be of benign neoplastic in origin, which involves chromosomal abnormalities, and dysregulation of mesenchymal cell differentiation.4

Microscopically the cartilage cells have hyperchromatic nuclei, binucleated cells similar to other cartilaginous tumors with a degree of cellularity and nuclear atypia equivalent to that of low-grade chondrosarcoma. In macroscopy, synovial chondromatosis is characterized as a soft tissue mass containing cartilaginous or osteocartilaginous loose bodies or nodules.1,5-9

Milgram has described three stages of progression of the disease; in the first stage, the cartilaginous bodies are attached to the synovium; as the disease advances in the second stage, the cartilaginous bodies breach the synovium and are released into the joint as loose bodies. In the final stage or the third stage, the disease is inactive with free loose bodies in the joint, which eventually leads to secondary degenerative arthritis.1,2

Primary synovial chondromatosis can present in three forms, intra-articular synovial chondromatosis, wherein multiple intra-articular loose bodies are seen; giant synovial chondromatosis, wherein a single large intra-articular loose body, is seen and extra-articular synovial chondromatosis or tenosynovial chondromatosis. Secondary synovial chondromatosis occurs in a preexisting degenerative joint pathology like degenerative arthritis, inflammatory arthritis, osteonecrosis, or osteochondral fractures, and the differentiating feature, when compared with primary synovial chondromatosis, is the presence of fewer variable sized bodies in secondary osteochondromatosis.1-5

The treatment of choice is the surgical excision of the loose bodies and synovectomy, either arthroscopically or open approach. The recurrence rate of the disease is 3–23% and is due to incomplete excision of the pathological tissue, with recurrence rates of almost 50% when only loose bodies are removed without synovectomy. Synovial chondrosarcoma can occur in preexisting synovial chondromatosis but is a rare occurrence and the differentiating features are rapidly enlarging recurrent swelling.2-8

Synovial chondromatosis to chondrosarcoma transformation is a rare phenomenon, and the symptoms that can be suggestive of such transformation of recurrence of swelling, rapid increase in the size of the swelling, bony invasion on X-ray, or medullary invasion on MRI. The confirmation of such transformation requires a histological diagnosis of synovial chondromatosis before the diagnosis of chondrosarcoma at the same anatomical site or the diagnosis of both conditions in the same resected specimen.7,8

Primary synovial chondromatosis affects large joints and rarely involves smaller joints of the foot. In this case report, we presented such a rare presentation of a recurrent synovial chondromatosis of the fifth metatarsophalangeal joint. Biopsy should always be done before the excision of the swelling to rule out any malignant transformation, and in our study, this was our drawback as we proceeded directly with excisional biopsy. The excision biopsy specimen should always be sent for histopathology to rule out malignant transformation and for diagnosis confirmation.

CONCLUSION

The diagnosis of synovial chondromatosis or synovial chondromatosis requires a histopathological examination of the excised tissue. The treatment for synovial chondromatosis is synovectomy and loose body removal either as an arthroscopic or open procedure, and incomplete synovectomy has been attributed to higher recurrence rates. Malignant transformation though rare, has also been described and should always be suspected in a recurrent disease that has rapid growth and bone destruction and requires histopathological examination.

ORCID

Sai Krishna MLV https://orcid.org/0000-0001-7993-9069

REFERENCES

1. McKenzie G, Raby N, Ritchie D. A pictorial review of primary synovial osteochondromatosis. Eur Radiol 2008;18(11):2662–2669. DOI: 10.1007/s00330-008-1024-8

2. Chiang CH, Jou IM, Wang PH, et al. Synovial osteochondromatosis of the second metatarsophalangeal joint: a case report. J Foot Ankle Surg 2011;50(4):458–461. DOI: 10.1053/j.jfas.2011.03.015

3. Galat DD, Ackerman DB, Spoon D, et al. Synovial chondromatosis of the foot and ankle. Foot Ankle Int 2008;29(3):312–317. DOI: 10.3113/FAI.2008.0312

4. Brucher N, Faruch-Bilfeld M, Molinier F, et al. Primary synovial osteochondromatosis of the first interphalangeal joint of the foot: a case report. Diagn Interv Imaging 2014;95(4):451–453. DOI: 10.1016/j.diii.2013.10.003

5. Kim JB, Song IS, Shin SY, et al. Primary synovial chondromatosis of the talonavicular joint: a case report. Foot Ankle Surg 2016;22(4):e25–e28. DOI: 10.1016/j.fas.2016.08.003

6. Matsuyama T, Suzuki T, Yoshida Y, et al. Synovial osteochondromatosis of the metatarsophalangeal joint of the great toe. Gan No Rinsho 1986;32(9):1051–1055. PMID: 3747157.

7. McCarthy C, Anderson WJ, Vlychou M, et al. Primary synovial chondromatosis: a reassessment of malignant potential in 155 cases. Skeletal Radiol 2016;45(6):755–762. DOI: 10.1007/s00256-016-2353-3

8. Anract P, Katabi M, Forest M, et al. Synovial chondromatosis and chondrosarcoma. A study of the relationship between these two diseases. Rev Chir Orthop Reparatrice Appar Mot 1996;82(3):216–224. PMID: 9005459.

9. Fetsch JF, Vinh TN, Remotti F, et al. Tenosynovial (extraarticular) chondromatosis: an analysis of 37 cases of an underrecognized clinicopathologic entity with a strong predilection for the hands and feet and a high local recurrence rate. Am J Surg Pathol 2003;27(9):1260–1268. DOI: 10.1097/00000478-200309000-00010

________________________
© The Author(s). 2024 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted use, distribution, and non-commercial reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.