Osteochondroma of the Rib Mimicking a Mediastinal Mass : Unexpected Menifestation in Hereditary Multiple Exostoses

Sang Kyun Bae,Won Sik Kang,Seung Hoon Yoo,Jeong Hyeon Cho,Kyung Won Park,Bu Hyun Lee,Jung Hun Baek,Jae Ho Chung 영남대학교 의과대학 2012 Yeungnam University Journal of Medicine Vol.29 No.1

Osteochondroma is a common bone tumor but a rare tumor in the rib. It is often asymptomatic and observed incidentally. This is a case report of a 49-year-old woman with an osteochondroma mimicking a mediastinal mass in hereditary multiple exostoses. The chest X-ray and computed tomography(CT) scans revealed the bony density feature of the mass. Surgical excision confirmed that the lesion was an osteochondroma.

Total Hip Arthroplasty for Secondary Coxarthrosis in Patients with Hereditary Multiple Exostoses: Minimum 5-Year Follow-up Results and Surgical Considerations

윤재연,박찬우,박연수,유정준,김희중 대한정형외과학회 2020 Clinics in Orthopedic Surgery Vol.12 No.4

Background: Hereditary multiple exostoses (HME) is an autosomal dominant disorder. The lesion in the proximal femoral metaphysis can bring about hip dysplasia and subsequent degenerative arthritis. Due to its rare prevalence, there have been a few case reports of total hip arthroplasty (THA) for osteoarthritis secondary to HME. The aim of this study was to report mid- to long-term outcomes of THA in HME patients and discuss special considerations that should be taken into account during surgery. Methods: We retrospectively evaluated the clinical and radiological results of THA for osteoarthritis secondary to HME in 11 hips of 9 patients after a minimum follow-up of 5 years (mean, 9.9 years). There were 3 men (3 hips) and 6 women (8 hips), with a mean age of 53.6 years (range, 46.8–58 years) at the index surgery in this study. Harris hip score (HHS) was used for clinical outcome assessment, and radiologically, implant stability, radiolucent lines, liner wear, and any sign of osteolysis or implant loosening were evaluated. Postoperative complications including infection, deep vein thrombosis, and dislocations were also investigated. Results: Cemented stems and cementless cups with the conventional polyethylene liner were used in bilateral hips of a single patient. In the other cases, cementless implants were used with ceramic-on-ceramic bearings. The mean HHS improved from 34.8 preoperatively to 92.5 postoperatively. Polyethylene liner wear and osteolysis were observed in 1 patient with cemented stems. Radiolucent lines were observed in 2 different cases. However, the femoral stems remained stable. There were no surgery-related complications except heterotopic ossification during follow-up. Conclusions: Despite the several surgical considerations, the mid- to long-term clinical and radiological outcomes of THA in HME patients were satisfactory. The abnormal, wide mediolateral diameter of the proximal metaphysis should be considered in selecting and inserting the stem with adequate anteversion. Leg length discrepancy was also common, so teleradiographs should be obtained before surgery. Intraoperative leg length evaluation might be difficult due to the morphologic changes in the proximal femur after mass excision and individual bone length differences.

Osteochondroma of the Rib Mimicking a Mediastinal Mass: Unexpected Menifestation in Hereditary Multiple Exostoses

배상균,강원식,유승훈,조정현,박경원,이부현,백정훈,정재호 영남대학교 의과대학 2012 Yeungnam University Journal of Medicine Vol.29 No.1

Osteochondroma is a common bone tumor but a rare tumor in the rib. It is often asymptomatic and observed incidentally. This is a case report of a 49-year-old woman with an osteochondroma mimicking a mediastinal mass in hereditary multiple exostoses. The chest X-ray and computed tomography (CT) scans revealed the bony density feature of the mass. Surgical excision confirmed that the lesion was an osteochondroma.

Osteochondroma of the Rib Mimicking a Mediastinal Mass: Unexpected Menifestation in Hereditary Multiple Exostoses

Bae, Sang-Kyun,Kang, Won-Sik,Yoo, Seung-Hoon,Cho, Jeong-Hyeon,Park, Kyung-Won,Lee, Bu-Hyun,Baek, Jung-Hun,Chung, Jae-Ho Yeungnam University College of Medicine 2012 Yeungnam University Journal of Medicine Vol.29 No.1

Osteochondroma is a common bone tumor but a rare tumor in the rib. It is often asymptomatic and observed incidentally. This is a case report of a 49-year-old woman with an osteochondroma mimicking a mediastinal mass in hereditary multiple exostoses. The chest X-ray and computed tomography (CT) scans revealed the bony density feature of the mass. Surgical excision confirmed that the lesion was an osteochondroma.

Autologous Fat Grafting as a Last Resort for Unsustainable Pain in a Woman with Multiple Osteochondromas

Vera Lidwina Negenborn,Esther Moerman,Simon Johannes Ham 대한성형외과학회 2017 Archives of Plastic Surgery Vol.44 No.2

Multiple osteochondromas (MO) is characterized by the formation of osteochondromas throughout the entire body. Although the evidence regarding its pathogenesis is well understood, no curative treatment for the disorder is available. Patients can be treated symptomatically by surgical removal of painful osteochondromas. Unfortunately, some patients still suffer from severe pain, even after surgery. We report on a case concerning a 48-year-old woman with a history of MO who presented with persistent pain after surgical removal of a symptomatic osteochondroma of the left scapula and multiple symptomatic osteochondromas of the left foot and trochanteric region. Several interventions to reduce the pain did not have any lasting effect. Subsequently, she was treated with autologous fat grafting (AFG). After each session she was pain-free for at least one year and reported only partial recurrence of the pain. This is the first case report describing AFG for the treatment of pain after both surgical removal of an osteochondroma and symptomatic osteochondromas in a patient suffering MO with promising results. The treatment is more effective and clearly continues to remain active longer than injection therapy or pain medication. Future studies are necessary to confirm our results.

Cervical Myelopathy Caused by Disc Herniation at the Segment of Existing Osteochondroma in a Patient with Hereditary Multiple Exostoses

Ko Ikuta,Kiyoshi Tarukado,Hideyuki Senba,Takahiro Kitamura,Norihiro Komiya,Satoshi Shidahara 대한척추외과학회 2014 Asian Spine Journal Vol.8 No.6

Hereditary multiple exostoses (HME) is a benign hereditary disorder characterized by multiple osteochondromas. Osteochondroma appears occasionally in the spinal column as a part of HME. A 37-year-old man presented with a history of HME and cervical compressive myelopathy caused by intraspinal osteochondroma arising from the lamina of the C5 and disc herniation at the C5–6. He was treated by open-door laminoplasty at the C5 and C6 with excision of the tumor. The neurological symptoms were immediately relieved after surgery. Magnetic resonance images demonstrated a sufficient decompression of the spinal cord with a spontaneous regression of the herniated disc at one year after surgery. There was no recurrence of the tumor and no appearance of kyphosis and segmental instability of the cervical spine on postoperative imaging studies for three years after surgery. The patient could be successfully treated by laminoplasty with excision of the tumor and without removal of the herniated disc.

Osteochondroma of the Distal Clavicle

Dong-Wan Kim,Ki-Cheor Bae,Eun-Seok Son,Chung-Sin Baek,Chul-Hyun Cho 대한견주관절의학회 2018 대한견주관절의학회지 Vol.21 No.3

Hereditary multiple exostosis (HME) is an autosomal dominant disorder manifested by the presence of multiple osteochondromas. Although the lesions are benign in nature, exostoses are often associated with characteristic progressive skeletal deformity and displaying clinical symptoms such as mechanical irritation or impingement. We present the successful arthroscopic resection in a 24-year-old HME male with impingement syndrome and long head tendon tear of the biceps caused by osteochondroma arising from the distal clavicle.

다발성 골연골종증 환자의 삶의 질에 대한 분석

정원주(Won-Ju Jeong),홍남수(Nam-Soo Hong),박일형(Il-Hyung Park),서일(Il Seo),오창욱(Chang-Wug Oh),윤종필(Jong Pil Yoon),경희수(Hee-Soo Kyung),김하정(Ha-Jeong Kim) 대한정형외과학회 2016 대한정형외과학회지 Vol.51 No.2

목적: 다발성 골연골종증 환자들의 동통, 신체적 사회적 기능, 삶의 질을 연구하여 예후, 치료, 장기적 관리에 도움이 고되자 한다. 대상 및 방법: 1993년 3월부터 2014년 6월까지 다발성 골연골종증을 진단받은 45명을 대상으로 하였다. Research and development-36을 통해 신체 기능, 정신 건강, 생활 활력, 사회적 역할, 동통, 전반적인 건강 상태가 환자의 예후에 영향을 미치는지 분석하였다. 관련 인자는 나이, 성별, 체질량지수, 수술 여부, 침범된 관절 변형 발생 여부, 재발 여부, 가족력 유무, 종양의 위치, 병변의 개수로 하였다. 통계는 SAS ver. 9.3 (SAS Inc., Cary, NC, USA)을 사용하고 p값이 0.05 미만일 경우 의미 있는 것으로 해석하였다. 결과: 가족력이 있는 환자군에서 종합 평가, 신체적 기능, 생활 활력, 사회적 활동, 건강 상태가 저조한 결과를 보였고 통계적으로 유의하였다. 또한 동통, 정서적 건강, 전반적인 건강 상태의 평가결과 통계적으로 유의하지는 않았지만 열등한 경향이 있는 것으로 나타났다. 결론: 가족력을 인지할 만큼 중증도가 높은 환자일수록 예후가 불량하였다. 다발성 골연골종이 동통만이 문제가 아니라 사회적, 심리적인 문제를 포함한 삶에 전체적인 영향을 줄 수 있는 질병임을 알 수 있었다. Purpose: The purpose of this study is to help predict the prognosis of multiple osteochondromatosis patients with the investigation of social function, pain, physical function and quality of life of patients. Materials and Methods: Forty-five cases were diagnosed as multiple osteochondromatosis from March 1993 to June 2014. We performed a survey on pain, daily life, school or work life assessment of research and development-36. Forty-five people who responded to the survey completely were enrolled. Variable factors, including physical functioning, role limitations due to physical health, role limitations due to emotional problems, energy/fatigue, emotional well-being, social functioning, pain, and general health state were considered as elements related to quality of life. In addition, we investigated significant factors for multiple osteochondromatosis patients, and analyzed the survey by scoring. Related factors included age (over 18 years and under 18 years), gender, body mass index, operation, joint deformity, recurrence of disease, family history, the number of involved joints and the location of tumor. Statistical analyses were performed using SAS ver. 9.3 (SAS Inc., Cary, NC, USA). p-values of <0.05 were deemed statistically significant. Results: Patients with a family history of multiple osteochondromatosis showed a significantly decreased result of assessment, physical function, vitality of life, social activities, and health state. In addition, there was a tendency of a poor influence in pain, emotional wellbeing, and general health. Conclusion: The results suggest that family history is a significant factor influencing and predicting the quality of life. In other words, the developed patients in the household including patients with severe enough for the rest of the family to know have poor prognosis. Through this study multiple osteochondromatosis is a chronic disease having a profound impact on quality of life.

Autologous Fat Grafting as a Last Resort for Unsustainable Pain in a Woman with Multiple Osteochondromas

Negenborn, Vera Lidwina,Moerman, Esther,Ham, Simon Johannes Korean Society of Plastic and Reconstructive Surge 2017 Archives of Plastic Surgery Vol.44 No.2

Multiple osteochondromas (MO) is characterized by the formation of osteochondromas throughout the entire body. Although the evidence regarding its pathogenesis is well understood, no curative treatment for the disorder is available. Patients can be treated symptomatically by surgical removal of painful osteochondromas. Unfortunately, some patients still suffer from severe pain, even after surgery. We report on a case concerning a 48-year-old woman with a history of MO who presented with persistent pain after surgical removal of a symptomatic osteochondroma of the left scapula and multiple symptomatic osteochondromas of the left foot and trochanteric region. Several interventions to reduce the pain did not have any lasting effect. Subsequently, she was treated with autologous fat grafting (AFG). After each session she was pain-free for at least one year and reported only partial recurrence of the pain. This is the first case report describing AFG for the treatment of pain after both surgical removal of an osteochondroma and symptomatic osteochondromas in a patient suffering MO with promising results. The treatment is more effective and clearly continues to remain active longer than injection therapy or pain medication. Future studies are necessary to confirm our results.

유전성 다발성 외골종 환자의 하지 변형

정성택(Sung-Taek Jung),설종환(Jong-Hwan Seol),박주권(Ju-Kwon Park) 대한정형외과학회 2010 대한정형외과학회지 Vol.45 No.1

목적: 유전성 다발성 외골종 환자에서 발생하는 하지의 변형 및 축성 배열의 변화에 대해 일아 보고자 하였다. 대상 및 방법: 2001년 1월부터 2007년 12월까지 유전성 다발성 외골종으로 진단 받았던 32명 환자를 대상으로 하였고, 진단 당시 연령을 기준으로 A (0-5세: 6명), B(6-10세: 7명), C(1l-15세: 7명), D (16세 이상: 12명)의 4군으로 분류하였다. 방사선 촬영을 역학적 축의 편위 및 축성 배열을 측정하고 환자 연령군에 따른 변화를 비교하였다. 결과: A/B/C/D 각군의 역학적 대퇴 경골간 각의 평균치는 178.5˚/180.3˚/182.5˚/183.5˚ (p<0.05)이었고, 족관절의 수평축과 경골의 역학적축이 이루는 각은 91.9˚/93.5˚/94.2˚/102.9˚ (p<0.05)로 측정되었다. 역학적 축의 외측 편위는 각각 우측 하지에서 1.7 ㎜/6.0㎜/9.6 ㎜/13.4 ㎜ (p<0.05), 죄측 하지에 2.9㎜/7.6 ㎜/12.2 ㎜/15.2 ㎜ (p<0.05)로 측정되었다. 결론: 유전성 다발성 외골종 환자는 고관절 및 슬관절, 족관절의 외반 변형의 경향을 보이며 슬관절 및 족관절은 연령 증가에 따라 외반 변형의 증가 양상을 보이고 있었다. Purpose: This study was done to analyze the alignment and deformity of the lower extremity in hereditary multiple exostoses patients. Materials and Methods: We enrolled 32 patients who were diagnosed as having hereditary multiple exostoses (HME) between January 2001 and December 2007. Based on age at diagnosis, we categorized them into 4 groups, A (0-5 years: 6 patients), B (6-10 years: 7 patients), C (11-15 years: 7 patients) and D (> 16 years: 12 patients). We measured mechanical axis deviation, This included femorotibial mechanical angle (a), inferolateral angle (b), femoral mechanical proximal anatomical angle (c), femoral mechanical distal anatomical angle (d), distal tibia inferolateral angle (e) and femoral neck-shaft angle (f). We analyzed for differences among the groups of different ages. Results: The average femorotibial mechanical angles (a) of Groups A/B/C/D were respectively, 178.5˚/180.3˚/182.5˚/183.5˚ (p<0.05). Distal tibia inferolateral angles (e) were respectively, 91.9˚/93.5˚/94.2˚/102.9˚ (p<0.05). The mechanical axis deviation of groups A, B, C, and D, respectively, were 1.7 ㎜, 6.0 ㎜, 9.6 ㎜, and 13.4 ㎜ (p<0.05) on the right side, and 2.9 ㎜, 7.6 ㎜, 12.2 ㎜, and 15.2 ㎜ (p<0.05) on the left side. Conclusion: Patients with HME have a tendency towards having valgus deformities of the knee and ankle joints, which tend to increase with age.