Pediatr Radiol DOI 10.1007/s00247-015-3383-z
CASE REPORT
Tenosynovial chondromatosis of the flexor hallucis longus in a 17-year-old girl Nichelle I. Winters 1 & A. Brian Thomson 2 & Raina R. Flores 3 & Martin I. Jordanov 4
Received: 12 January 2015 / Revised: 27 March 2015 / Accepted: 6 May 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Tenosynovial chondromatosis is a benign chondrogenic metaplasia of extra-articular synovial tissue. The most common locations for tenosynovial chondromatosis to develop are the hands and feet. The condition has rarely been reported in children. We present a case of tenosynovial chondromatosis of the flexor hallucis longus in a 17-year-old girl. The presentation was unusual not only due to the location and young age of the patient but also the absence of any palpable mass on physical exam and complete lack of calcification of the cartilage bodies. Initial diagnosis was made by MRI. The patient underwent tenosynovectomy with an excellent postoperative recovery at 6-month follow-up. Histopathology confirmed the diagnosis of tenosynovial chondromatosis.
Keywords Adolescent . Flexor hallucis longus . Magnetic resonance imaging . Synovial chondromatosis . Tenosynovial
Introduction Tenosynovial chondromatosis is a rare condition in which there is a metaplasia of synovial cells into chondroblastic tissue. Cartilage bodies generated by the transformed synovium may reside within the synovium itself or free float within the tendon sheath. Accumulation of cartilage bodies often leads to a palpable mass, which is the most common presenting complaint. There may or may not be associated soft-tissue swelling or pain. Typically, the cartilage bodies ossify to some degree (~90% of cases); rarely, ossification may be completely absent with a resulting normal appearance on plain radiographs [1, 2]. The hands and feet are most commonly affected and the condition is typically localized to a single tendon sheath though multifocal disease has been reported [1–5]. The peak incidence of tenosynovial chondromatosis is in the third to fifth decades of life; children are rarely affected [1, 2, 6]. We present a case of tenosynovial chondromatosis of the flexor hallucis longus in a teenage girl.
Case report * Nichelle I. Winters [email protected] 1
Vanderbilt University School of Medicine, 2215 Garland Ave. (Light Hall), Nashville, TN 37232, USA
2
Department of Orthopaedic Surgery, Vanderbilt University Medical Center, Nashville, TN, USA
3
Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA
4
Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, Nashville, TN, USA
A healthy 17-year-old girl presented with a 1- to 2-year history of intermittent left ankle swelling associated with mild discomfort. At the time of presentation, the swelling and discomfort of the ankle had acutely worsened during a 5-day period, which she associated with a change in footwear. She did not recall any injury to the area. She had no history of rheumatological disease. On physical exam, she had ill-defined softtissue swelling posterior to the left medial malleolus without any bony tenderness or palpable mass. She had full range of motion without elicited pain. Radiographs of the left ankle did not demonstrate any bony abnormalities though a soft-tissue density was visible obscuring a portion of Kager’s (a.k.a. pre-
Pediatr Radiol
Fig. 1 A lateral radiograph of the 17-year-old girl’s left ankle demonstrates a lobulated soft-tissue density encroaching into the anterior aspect of Kager’s fat pad (arrow) but no ossified bodies
calcaneal/pre-achilles) fat pad (Fig. 1). Due to the rather acute and marked exacerbation of her swelling and discomfort, she was referred for MRI. The MRI, performed without intravenous gadolinium, revealed distension of the flexor hallucis longus tendon sheath with fluid that contained innumerable cartilage bodies measuring from 1 to 4 mm in diameter (Fig. 2). None of the cartilage bodies were ossified explaining why they were not visible on the previously acquired radiographs. The flexor hallucis longus tendon itself appeared normal. There was no involvement of the joint spaces of the ankle or hindfoot with either effusion or cartilage bodies. Based on the findings on the MRI and prior radiographs, the girl was diagnosed with tenosynovial chondromatosis of the flexor hallucis longus tendon sheath. The girl underwent open tenosynovectomy of the flexor hallucis longus tendon sheath. At surgery, there was gross inflammation and scarring of the tenosynovium as well as multiple small cartilaginous bodies, all roughly the same size (Fig. 3). Surgical pathology of the resected flexor hallucis Fig. 2 Selected sagittal fatsaturated proton density-weighted image with TE=45, TR=2,633 (a) and axial fat-saturated T2weighted image with TE=90 and TR=5,167 (b) of the 17-year-old girl’s left ankle demonstrate marked distention of the flexor hallucis longus tendon sheath with fluid. The fluid contains innumerable small filling defects, all roughly the same size (arrows). Note the lack of joint effusions in the tibiotalar and subtalar joints
Fig. 3 Intraoperative photograph of some of the retrieved cartilaginous bodies
lungus tendon sheath demonstrated nodules rich in chondroid matrix with reactive chondrocytes (Fig. 4) confirming the diagnosis of tenosynovial chondromatosis. The patient has done well postoperatively. At her 6-month postoperative visit, she was symptom free with full range of motion.
Discussion Tenosynovial chondromatosis is quite rare in children though the condition has been reported in a patient as young as 7 years [1]. The condition affects both sexes at near equivalent rates [1, 2]. Symptoms may include a slowly growing non-tender mass (most common), swelling, pain or restriction in range of motion. A long history of symptoms, sometimes lasting as long as a decade, may precede eventual presentation and diagnosis [1, 2, 5]. Up to 90% of patients demonstrate some degree of calcification or ossification of the cartilage nodules
Pediatr Radiol
Fig. 4 Histological section of a discrete nodule composed of reactive chondrocytes within a chondroid matrix. Prominent histiocytes are noted along the surface of the chondroid nodule (H&E, 10X)
on radiographs (osteochondromatosis). A ring and arc pattern characteristic of chondroid calcification is most frequently observed [1]. MRI typically shows fluid distending the involved tendon sheath with innumerable small loose bodies, all roughly the same size. The differential diagnosis for tenosynovial chondromatosis in a child varies depending on the imaging modality used to assess the condition. Radiographically, if ossified loose bodies are discernible, the differential diagnosis includes bizarre parosteal osteochondromatous proliferation (Nora lesion), extraskeletal chondroma or chondrosarcoma, and synovial sarcoma. If ossified loose bodies are not evident radiographically but soft-tissue distension of a tendon sheath is appreciable, primary differential considerations include tenosynovitis due to inflammatory arthritis or infection, tenosynovial chondromatosis and tenosynovial giant cell tumor. In this particular case, the differential diagnosis for the radiographic finding of a lobulated soft-tissue density encroaching on the anterior aspect of Kager’s fat pad includes a ganglion cyst arising from the tibiotalar or posterior subtalar joint, or an accessory soleus muscle. On MRI, the differential diagnosis narrows considerably as this imaging modality provides exquisite soft-tissue anatomical detail and allows for precise localization of the abnormalities to within or outside the tendon sheath. Differential considerations for a fluid-distended tendon sheath filled with innumerable small loose bodies should include tenosynovial chondromatosis and rice bodies associated with juvenile idiopathic arthritis (JIA)/rheumatoid arthritis (RA) or chronic tuberculous or fungal infection. Use of intravenous gadolinium may be helpful in identifying
inflammatory changes, as seen in JIA or infection. In addition, if JIA/RA were suspected, inflammatory markers can be helpful laboratory studies. In this particular case, rheumatological disease was not clinically suspected and the diagnosis was made with imaging and pathology studies alone. To our knowledge, there have only been three prior reported cases of tenosynovial chondromatosis involving the flexor hallucis longus tendon sheath. All three patients were male and the ages at presentation were 23, 29 and 41 years [1, 4, 7]. In the case of the 23-year-old man, the primary site of involvement was the tarsal tunnel though there was continuity with the sheaths of the flexor digitorum longus and the flexor hallucis longus tendons. This patient had experienced occasional pain at the site for 13 years. Initial diagnosis was made after radiographs demonstrated multiple ossified bodies. Subsequent imaging included both CT and MRI prior to surgical excision [4]. The 29-year-old man had a 6-month history of worsening ankle pain. Plain radiographs demonstrated multiple ossified bodies and MRI confirmed the diagnosis of tenosynovial chondromatosis [7]. In our case, the young age of the patient, lack of notable physical exam findings and lack of radiographic findings demonstrate the potential difficulty in diagnosing this disorder. Familiarity with the mild clinical nature of the condition and the gamut of potential imaging findings is essential for clinicians and consulting radiologists alike to establish the diagnosis in a timely fashion and pursue appropriate treatment for the patient. Tenosynovectomy is the mainstay of therapy through either an arthroscopic or open approach. Unfortunately, there is a high degree of local recurrence with some patients requiring multiple surgical interventions with a chronic disease course [2]. Recurrence can occur within months or many years later [2, 3]. Thus, close follow-up is essential after surgical treatment.
Conflicts of interest None
References 1.
Walker EA, Murphey MD, Fetsch JF (2011) Imaging characteristics of tenosynovial and bursal chondromatosis. Skeletal Radiol 40:317– 325 2. Fetsch JF, Vinh TN, Remotti F et al (2003) 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 27:1260–1268 3. DeBenedetti MJ, Schwinn CP (1979) Tenosynovial chondromatosis in the hand. J Bone Joint Surg Am 61:898–903 4. Sugimoto K, Iwai M, Kawate K et al (2003) Tenosynovial osteochondromatosis of the tarsal tunnel. Skeletal Radiol 32:99–102
Pediatr Radiol 5.
6.
Mata BA, Eward WC, Brigman BE (2013) Pancarpal synovial and tenosynovial chondromatosis in a 65-year-old man: a highly unusual presentation of a common condition. Am J Orthop 42:E60–E63 Kim HK, Zbojniewicz AM, Merrow AC et al (2011) MR findings of synovial disease in children and young adults: Part 1. Pediatr Radiol 41:495–511
7.
Oakley J, Yewlett A, Makwana N (2010) Tenosynovial osteochondromatosis of the flexor hallucis longus tendon. Foot Ankle Surg 16:148–150
CASE REPORT
Tenosynovial chondromatosis of the flexor hallucis longus in a 17-year-old girl Nichelle I. Winters 1 & A. Brian Thomson 2 & Raina R. Flores 3 & Martin I. Jordanov 4
Received: 12 January 2015 / Revised: 27 March 2015 / Accepted: 6 May 2015 # Springer-Verlag Berlin Heidelberg 2015
Abstract Tenosynovial chondromatosis is a benign chondrogenic metaplasia of extra-articular synovial tissue. The most common locations for tenosynovial chondromatosis to develop are the hands and feet. The condition has rarely been reported in children. We present a case of tenosynovial chondromatosis of the flexor hallucis longus in a 17-year-old girl. The presentation was unusual not only due to the location and young age of the patient but also the absence of any palpable mass on physical exam and complete lack of calcification of the cartilage bodies. Initial diagnosis was made by MRI. The patient underwent tenosynovectomy with an excellent postoperative recovery at 6-month follow-up. Histopathology confirmed the diagnosis of tenosynovial chondromatosis.
Keywords Adolescent . Flexor hallucis longus . Magnetic resonance imaging . Synovial chondromatosis . Tenosynovial
Introduction Tenosynovial chondromatosis is a rare condition in which there is a metaplasia of synovial cells into chondroblastic tissue. Cartilage bodies generated by the transformed synovium may reside within the synovium itself or free float within the tendon sheath. Accumulation of cartilage bodies often leads to a palpable mass, which is the most common presenting complaint. There may or may not be associated soft-tissue swelling or pain. Typically, the cartilage bodies ossify to some degree (~90% of cases); rarely, ossification may be completely absent with a resulting normal appearance on plain radiographs [1, 2]. The hands and feet are most commonly affected and the condition is typically localized to a single tendon sheath though multifocal disease has been reported [1–5]. The peak incidence of tenosynovial chondromatosis is in the third to fifth decades of life; children are rarely affected [1, 2, 6]. We present a case of tenosynovial chondromatosis of the flexor hallucis longus in a teenage girl.
Case report * Nichelle I. Winters [email protected] 1
Vanderbilt University School of Medicine, 2215 Garland Ave. (Light Hall), Nashville, TN 37232, USA
2
Department of Orthopaedic Surgery, Vanderbilt University Medical Center, Nashville, TN, USA
3
Department of Pathology, Microbiology and Immunology, Vanderbilt University Medical Center, Nashville, TN, USA
4
Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, Nashville, TN, USA
A healthy 17-year-old girl presented with a 1- to 2-year history of intermittent left ankle swelling associated with mild discomfort. At the time of presentation, the swelling and discomfort of the ankle had acutely worsened during a 5-day period, which she associated with a change in footwear. She did not recall any injury to the area. She had no history of rheumatological disease. On physical exam, she had ill-defined softtissue swelling posterior to the left medial malleolus without any bony tenderness or palpable mass. She had full range of motion without elicited pain. Radiographs of the left ankle did not demonstrate any bony abnormalities though a soft-tissue density was visible obscuring a portion of Kager’s (a.k.a. pre-
Pediatr Radiol
Fig. 1 A lateral radiograph of the 17-year-old girl’s left ankle demonstrates a lobulated soft-tissue density encroaching into the anterior aspect of Kager’s fat pad (arrow) but no ossified bodies
calcaneal/pre-achilles) fat pad (Fig. 1). Due to the rather acute and marked exacerbation of her swelling and discomfort, she was referred for MRI. The MRI, performed without intravenous gadolinium, revealed distension of the flexor hallucis longus tendon sheath with fluid that contained innumerable cartilage bodies measuring from 1 to 4 mm in diameter (Fig. 2). None of the cartilage bodies were ossified explaining why they were not visible on the previously acquired radiographs. The flexor hallucis longus tendon itself appeared normal. There was no involvement of the joint spaces of the ankle or hindfoot with either effusion or cartilage bodies. Based on the findings on the MRI and prior radiographs, the girl was diagnosed with tenosynovial chondromatosis of the flexor hallucis longus tendon sheath. The girl underwent open tenosynovectomy of the flexor hallucis longus tendon sheath. At surgery, there was gross inflammation and scarring of the tenosynovium as well as multiple small cartilaginous bodies, all roughly the same size (Fig. 3). Surgical pathology of the resected flexor hallucis Fig. 2 Selected sagittal fatsaturated proton density-weighted image with TE=45, TR=2,633 (a) and axial fat-saturated T2weighted image with TE=90 and TR=5,167 (b) of the 17-year-old girl’s left ankle demonstrate marked distention of the flexor hallucis longus tendon sheath with fluid. The fluid contains innumerable small filling defects, all roughly the same size (arrows). Note the lack of joint effusions in the tibiotalar and subtalar joints
Fig. 3 Intraoperative photograph of some of the retrieved cartilaginous bodies
lungus tendon sheath demonstrated nodules rich in chondroid matrix with reactive chondrocytes (Fig. 4) confirming the diagnosis of tenosynovial chondromatosis. The patient has done well postoperatively. At her 6-month postoperative visit, she was symptom free with full range of motion.
Discussion Tenosynovial chondromatosis is quite rare in children though the condition has been reported in a patient as young as 7 years [1]. The condition affects both sexes at near equivalent rates [1, 2]. Symptoms may include a slowly growing non-tender mass (most common), swelling, pain or restriction in range of motion. A long history of symptoms, sometimes lasting as long as a decade, may precede eventual presentation and diagnosis [1, 2, 5]. Up to 90% of patients demonstrate some degree of calcification or ossification of the cartilage nodules
Pediatr Radiol
Fig. 4 Histological section of a discrete nodule composed of reactive chondrocytes within a chondroid matrix. Prominent histiocytes are noted along the surface of the chondroid nodule (H&E, 10X)
on radiographs (osteochondromatosis). A ring and arc pattern characteristic of chondroid calcification is most frequently observed [1]. MRI typically shows fluid distending the involved tendon sheath with innumerable small loose bodies, all roughly the same size. The differential diagnosis for tenosynovial chondromatosis in a child varies depending on the imaging modality used to assess the condition. Radiographically, if ossified loose bodies are discernible, the differential diagnosis includes bizarre parosteal osteochondromatous proliferation (Nora lesion), extraskeletal chondroma or chondrosarcoma, and synovial sarcoma. If ossified loose bodies are not evident radiographically but soft-tissue distension of a tendon sheath is appreciable, primary differential considerations include tenosynovitis due to inflammatory arthritis or infection, tenosynovial chondromatosis and tenosynovial giant cell tumor. In this particular case, the differential diagnosis for the radiographic finding of a lobulated soft-tissue density encroaching on the anterior aspect of Kager’s fat pad includes a ganglion cyst arising from the tibiotalar or posterior subtalar joint, or an accessory soleus muscle. On MRI, the differential diagnosis narrows considerably as this imaging modality provides exquisite soft-tissue anatomical detail and allows for precise localization of the abnormalities to within or outside the tendon sheath. Differential considerations for a fluid-distended tendon sheath filled with innumerable small loose bodies should include tenosynovial chondromatosis and rice bodies associated with juvenile idiopathic arthritis (JIA)/rheumatoid arthritis (RA) or chronic tuberculous or fungal infection. Use of intravenous gadolinium may be helpful in identifying
inflammatory changes, as seen in JIA or infection. In addition, if JIA/RA were suspected, inflammatory markers can be helpful laboratory studies. In this particular case, rheumatological disease was not clinically suspected and the diagnosis was made with imaging and pathology studies alone. To our knowledge, there have only been three prior reported cases of tenosynovial chondromatosis involving the flexor hallucis longus tendon sheath. All three patients were male and the ages at presentation were 23, 29 and 41 years [1, 4, 7]. In the case of the 23-year-old man, the primary site of involvement was the tarsal tunnel though there was continuity with the sheaths of the flexor digitorum longus and the flexor hallucis longus tendons. This patient had experienced occasional pain at the site for 13 years. Initial diagnosis was made after radiographs demonstrated multiple ossified bodies. Subsequent imaging included both CT and MRI prior to surgical excision [4]. The 29-year-old man had a 6-month history of worsening ankle pain. Plain radiographs demonstrated multiple ossified bodies and MRI confirmed the diagnosis of tenosynovial chondromatosis [7]. In our case, the young age of the patient, lack of notable physical exam findings and lack of radiographic findings demonstrate the potential difficulty in diagnosing this disorder. Familiarity with the mild clinical nature of the condition and the gamut of potential imaging findings is essential for clinicians and consulting radiologists alike to establish the diagnosis in a timely fashion and pursue appropriate treatment for the patient. Tenosynovectomy is the mainstay of therapy through either an arthroscopic or open approach. Unfortunately, there is a high degree of local recurrence with some patients requiring multiple surgical interventions with a chronic disease course [2]. Recurrence can occur within months or many years later [2, 3]. Thus, close follow-up is essential after surgical treatment.
Conflicts of interest None
References 1.
Walker EA, Murphey MD, Fetsch JF (2011) Imaging characteristics of tenosynovial and bursal chondromatosis. Skeletal Radiol 40:317– 325 2. Fetsch JF, Vinh TN, Remotti F et al (2003) 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 27:1260–1268 3. DeBenedetti MJ, Schwinn CP (1979) Tenosynovial chondromatosis in the hand. J Bone Joint Surg Am 61:898–903 4. Sugimoto K, Iwai M, Kawate K et al (2003) Tenosynovial osteochondromatosis of the tarsal tunnel. Skeletal Radiol 32:99–102
Pediatr Radiol 5.
6.
Mata BA, Eward WC, Brigman BE (2013) Pancarpal synovial and tenosynovial chondromatosis in a 65-year-old man: a highly unusual presentation of a common condition. Am J Orthop 42:E60–E63 Kim HK, Zbojniewicz AM, Merrow AC et al (2011) MR findings of synovial disease in children and young adults: Part 1. Pediatr Radiol 41:495–511
7.
Oakley J, Yewlett A, Makwana N (2010) Tenosynovial osteochondromatosis of the flexor hallucis longus tendon. Foot Ankle Surg 16:148–150
