Bülent Erol

Percutaneous intramedullary decompression, curettage, and grafting with medical-grade calcium sulfate pellets for unicameral bone cysts in children: a new minimally invasive technique.

Several treatment options exist for unicameral bone cysts (UBCs), including observation, steroid injection, bone marrow injection, and curettage and bone grafting. These are all associated with high recurrence rates, persistence, and occasional complications. Newer techniques have been described, most with variable success and only short follow-up reported. Because of these factors, a new minimally invasive percutaneous technique was developed for the treatment of UBCs in children. Twenty-eight children with UBCs who underwent percutaneous intramedullary decompression, curettage, and grafting with medical-grade calcium sulfate (MGCS) pellets by the senior author (J.P.D.) between April 2000 and April 2003 were analyzed as part of a pediatric musculoskeletal tumor registry at a large tertiary childrens hospital. Four patients were lost to follow-up, and the remaining 24 patients had an average follow-up of 21.9 months (range 4-48 months). Twelve patients were followed for at least 24 months. Six of the 24 children had received previous treatment of their UBC, most often at an outside institution. Follow-up was performed through clinical evaluation and radiographic review. Postoperative radiographs at most recent follow-up showed complete healing, defined as more than 95% opacification, in 22 of 24 patients (91.7%). One patient (4.2%) demonstrated partial healing, defined as 80% to 95% opacification. One patient had less than 80% radiographic healing (4.2%). All 24 patients returned to full activities and were asymptomatic at most recent follow-up. The only complication noted was a superficial suture abscess that occurred in one patient; this resolved with local treatment measures. The new minimally invasive technique of percutaneous intramedullary decompression, curettage, and grafting with MGCS pellets demonstrates favorable results with low complication and recurrence rates compared with conventional techniques. The role of intramedullary decompression as a part of this percutaneous technique is discussed.

Molecular analysis of congenital scoliosis: a candidate gene approach.

The etiology of congenital scoliosis is largely unknown. The severe vertebral disorder, spondylocostal dysostosis type 1, is associated with a homozygous delta-like 3 (DLL3) mutation. Scoliosis has been observed in a heterozygous DLL3 carrier, raising the possibility of its involvement in congenital scoliosis. We present the first molecular study of congenital scoliosis by analysis of the candidate gene DLL3 and demonstrate one novel missense variant. However, no novel or previously described mutations are present in our cohort, indicating that DLL3 mutations may not be a major cause of congenital scoliosis. Additionally, we have evaluated patients with congenital scoliosis not diagnosed with a known syndrome and identified a significant number of associated renal and cardiac anomalies and familial incidence of idiopathic scoliosis in this group.

Congenital scoliosis and vertebral malformations: characterization of segmental defects for genetic analysis.

The developmental and genetic etiology of most congenital vertebral malformation disorders remains unknown. The objective of this study was to evaluate and classify congenital vertebral defect cases into groupings based on developmental etiology for clinical genetic studies. This classification is intended to be distinct from but complementary to traditional groupings based on spinal curvature or progression. In the first step of this analysis, the authors identified 84 cases of vertebral segmentation disorders by radiologic screening and prospectively recruited 39 of these patients into a clinical genetic study. Next, the authors quantified the extent of contiguous defects and organized cases by craniocaudal localization. Finally, the authors used available clinical association data to identify syndromic and nonsyndromic subcategories, and identified a high rate of orthopaedic and neurologic associations in nonsyndromic patients. This type of analysis has identified subgroups of patients with multiple, contiguous segmental defects and orthopaedic associations that are particularly suitable for further genetic analysis.

Reconstruction of an Intercalary Defect with Bone Transport after Resection of Ewing???s Sarcoma

We report a 13-year-old girl with Ewings sarcoma of the tibia who was treated with multiagent chemotherapy, followed by local control tumor surgery consisting of wide resection of the tumor and bone transport with distraction osteogenesis for reconstruction. The bone defect created by resection was 13 cm long and was replaced by bone transport using a monolateral external fixator. Evaluation of the resected specimen revealed wide tumor-free margins with 100% chemonecrosis. A planned Harmon-type autogenous bone grafting between the middle and proximal segments of the tibia (docking site) was done primarily after docking occurred, and a solid union was obtained by 23 months after resection. The bone healing index (treatment index) was 54 days/1 cm distraction, which is indicative of slow healing. Clinical evaluation of the affected extremity using the Musculoskeletal Tumor Society rating system revealed 80% normal functional capability. Indications for bone transport in reconstruction of bone defects created by wide resection of bone sarcomas are discussed. In retrospect, we have concerns regarding the suitability of this technique in the setting of diaphyseal sarcoma reconstruction in patients with Ewings sarcoma who require aggressive and intense multiagent chemotherapy.

Lower back pain and mass in a 13-year-old girl.

A 13 year-old girl reported lower back pain, particularly on the left side, for approximately 6 months before presenting to our institution. The pain initially was thought to be muscle strain secondary to playing basketball, and plain radiographs were read as normal. However, the pain increased recently with the patient reporting occasional episodes of numbness and tingling in her upper left leg, extending to the midthigh. A week before presentation, a painful mass on the left side of the patient’s lower back was seen. A review of plain radiographs revealed a possible lesion in the lumbar spine. Magnetic resonance imaging (MRI) was done and revealed a paraspinal mass arising from the lumbar vertebral bodies. The patient was referred to our institution for additional evaluation. The patient had no previous major illnesses, injuries, or surgeries and no evidence of urinary retention, urinary incontinence, constipation, diarrhea, or constitutional symptoms, such as weight loss, fatigue, fever, sweats, chills, nausea, and vomiting. The patient was the product of a full-term, uncomplicated pregnancy, and her developmental and family histories were noncontributory. On physical examination, the patient’s gait was normal. A 7.0 × 8.0 cm, firm, immobile, tender soft tissue mass was present to the left of lumbar spine levels L1-L3. There was no erythema or increased warmth over the area of the mass. Neurovascular examination was normal. Her abdomen was soft and nontender without masses or hepatosplenomegaly. There was no evidence of lymphadenopathy. The remainder of the physical examination was normal. Laboratory tests revealed a normal white blood cell count, hemoglobin, platelet count, and complete chemistry panel. Lactic dehydrogenase was elevated at 1309 U/L (normal, 380–640 U/L). Plain radiographs of the chest showed a possible nodule in the right lung. Computed tomography scans of the chest and bone scan of the entire body confirmed the presence of the right lung nodule and showed additional bony lesions in the thoracolumbar spine. The radiograph of the chest, CT, bone scan, and the plain radiographs and MRI scans of the lumbar spine are shown in Figures 1 to 6. Based on the history, physical findings, and imaging studies, what is the differential diagnosis? From the *Medical Student, Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA; †Attending Physician, Marmara University Hospital, Istanbul, Turkey; ‡Clinical Assistant Professor, Division of Oncology, The Children’s Hospital of Philadelphia, Philadelphia, PA; §Director, Anatomic Pathology, Department of Pathology, The Children’s Hospital of Philadelphia, Philadelphia, PA; Attending Physician in Radiology, Department of Radiology, The Children’s Hospital of Philadelphia, Philadelphia, PA; and the ¶Chief, Division of Orthopaedic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, PA. The authors have disclosed that they have no financial interests in or relationships with any commercial companies pertaining to this educational activity. These materials are available on CORRONLINE for continuing medical educational credit. Correspondence to: John P. Dormans, MD, Chief, Division of Orthopaedic Surgery, The Children’s Hospital of Philadelphia, 2nd Floor Wood Center, 34th & Civic Blvd., Philadelphia, PA 19104-4399; Phone: 215-590-1534; Fax: 215-590-1501; E-mail: dormans@email.CHOP.edu. DOI: 10.1097/01.blo.0000128641.53637.c9 CLINICAL ORTHOPAEDICS AND RELATED RESEARCH Number 430, pp. 248–257

Pathologic hip fracture in a 4-year-old boy.

A 4-year-old boy was transferred from another hospital to the authors’ institution for evaluation and treatment of a pathologic fracture of the left femur. The child had a 5-month history of mild limp and pain in his left proximal thigh. He was knocked over by a dog on the day of admission and subsequently was unable to ambulate. The patient was healthy otherwise with no previous injuries, major illnesses, or surgeries. Developmental, social, and family histories were noncontributory. On physical examination, the patient was a healthy appearing boy who reported pain in his left hip region. He was unable to bear weight on his left lower extremity. There was shortening and external rotation of the left lower extremity. The neurovascular examination was normal. Plain radiographs and magnetic resonance imaging (MRI) scans were taken and are shown in Figures 1 through 4. SECTION III REGULAR AND SPECIAL FEATURES

Surgical treatment of active or aggressive aneurysmal bone cysts in children.

The aim of this study was to evaluate functional and radiological results, tumor control, and complications of the surgical treatment of aneurysmal bone cysts (ABCs) in children by extended curettage using a mechanical burr and cauterization, grafting, and internal fixation in specific locations. Sixty-four children [38 males, 26 females, median age=10 years (range, 5–18 years)] with active or aggressive ABCs of long and flat bones were subjected to a median follow-up of 66 months (range, 28–130 months) following surgical treatment. The pathological fracture rate was 72%. Surgical procedures included intralesional extended curettage (92%) or en-bloc resection (8%). Internal fixation was required in 53%. The pathological fractures healed successfully in 8–12 weeks. The median Musculoskeletal Tumor Society (MSTS) score at the last follow-up was 95% (range, 55–100%). Treatment failure (partial healing/recurrence) and complication rates following extended curettage were 7 and 5.2%, respectively. Statistically, the only parameter affecting partial healing and local recurrence was proximity of the lesion to the growth plate (P=0.011). Extended curettage using a mechanical burr and cauterization, grafting, and internal fixation in specific locations can promote healing in most cases of ABC, with low recurrence and complication rates.

Sternal pain in an 11-year-old boy.

An 11-year-old boy had increasing chest pain for 7 months before presenting to our institution. The patient was admitted to another institution for evaluation, where plain radiographs and CT scans were done. The patient reported that the pain did not interfere with his sleep and was not significantly improved or alleviated by several different nonsteroidal antiinflammatory drugs (NSAIDs). At the time of presentation, the patient had pain, tenderness, and swelling over the upper portion of his sternum. He denied constitutional symptoms, such as weight loss, fever, or malaise. The child otherwise was healthy with no previous major illnesses or major injuries. His surgical history was significant only for a tonsillectomy and adenoidectomy 9 years before presentation. He was the product of a fullterm, uncomplicated pregnancy, and his developmental and family histories were noncontributory. On physical examination, the patient was a healthyappearing boy who reported moderate pain over his upper sternum. An ill-defined swelling without increased warmth or erythema was seen at the manubrial portion of the sternum. There was tenderness over the area and the overlying skin was normal. The neurovascular examination of the upper extremities was normal. There was full ROM of the neck and shoulders. The patient’s physical examination otherwise was normal. Plain radiographs and CT scans of the patient’s chest were repeated at our institution before the incisional biopsy and are shown in Figures 1 and 2. Based on the history, physical findings, and imaging studies, what is the differential diagnosis? From the *Medical Student, University of Pennsylvania School of Medicine, Philadelphia, PA; †Attending Physician, Marmara University Hospital, Istanbul, Turkey; ‡Clinical Research Coordinator, Department of Orthopaedic Surgery, Children’s Hospital of Philadelphia, Philadelphia, PA; §Attending Physician in Radiology, Department of Radiology, Children’s Hospital of Philadelphia, Philadelphia, PA; Director, Anatomic Pathology, Department of Pathology, Children’s Hospital of Philadelphia, Philadelphia, PA; and the ¶Chief of Orthopaedic Surgery, Children’s Hospital of Philadelphia, Professor of Orthopaedic Surgery, University of Pennsylvania School of Medicine, Philadelphia, PA. The authors have disclosed that they have no interests in or significant relationships with any commercial companies pertaining to this educational activity. These materials are available on CORRONLINE for continuing medical education credit. Correspondence to: John P. Dormans, MD, Division of Orthopaedic Surgery, The Children’s Hospital of Philadelphia, 2nd Floor Wood Center, 34th & Civic Center Blvd., Philadelphia, PA 19104-4399. Phone: 215-590-1534; Fax: 215-590-1501; E-mail: dormans@email.CHOP.edu. DOI: 10.1097/01.blo.0000093034.56370.cc CLINICAL ORTHOPAEDICS AND RELATED RESEARCH Number 421, pp. 307–313

Limping in a 12-year-old boy.

281 A 12-year-old boy reported a gradual onset of left thigh and knee pain and limping for 18 months before presenting to the authors’ institution. Plain radiographs taken 12 months before presentation revealed a lesion in his left femur. He had been admitted to another institution for evaluation, where magnetic resonance imaging (MRI) and computed tomography (CT) scanning were done. An incisional biopsy was done at the outside institution and revealed reactive bone. After the biopsy specimen was obtained, the extremity was placed in a long leg cast, and the patient used a wheelchair for 1 month. After cast removal, the patient received outpatient rehabilitation for 2 months. A slight clinical improvement was reported during this period, but pain and limping soon returned. The patient otherwise was healthy with only one previous surgery of bilateral myringotomies at age 3. His mother’s pregnancy, and the developmental, and family histories were normal. On presentation to the authors’ institution, the patient described some discomfort in the distal part of his left thigh. There was no swelling, erythema, or tenderness over the area of the well-healed incision from the previous biopsy. There was mild atrophy of his left thigh musculature, but the patient had full active range of motion (ROM) in the left knee. The neurovascular examination was normal. His gait was abnormal with a mild antalgic limp on the left lower extremity. Plain radiographs, MRI scans, and CT scans of the patient’s extremity obtained before the first incisional biopsy are shown in Figures 1 through 6. Based on the history, physical findings, and imaging studies, what is the differential diagnosis? Orthopaedic • Radiology • Pathology Conference CME ARTICLE

Mid-term radiological and functional results of biological reconstructions of extremity-located bone sarcomas in children and young adults.

Biological reconstruction is a useful option for reconstruction following bone sarcoma resection in children. The mid-term functional and radiological outcomes of biological reconstructions after resection of bone sarcomas in children are presented in this study. Eighteen patients [average age 12.5 years (range 4–22 years)] with primary sarcomas of long bones underwent wide surgical resection and biological reconstruction. The bone defects were managed by intercalary (n=14), osteoarticular (n=3) reconstructions and arthrodesis (n=1) with a vascularized fibular graft (VFG). VFG was combined with a massive allograft in seven lower extremity reconstructions. The average follow-up was 45.7 months (range 25–78 months). Graft union and graft hypertrophy was observed in 17 (94.4%) of 18 patients at 12 months. The VFG–allograft osteointegration rate was 100% at 24 months. The average final follow-up Musculoskeletal Tumor Society (MSTS) scores for lower and upper extremity reconstructions were 79.7% (range 66.6–90%) and 80.9% (range 53.3–100%), respectively. Four (22.2%) complications, including nonunion (n=1), implant failure (n=1), infection (n=1) and skin necrosis (n=1), required reoperation. The disease relapsed in three (16.6%) patients. Defect size and VFG length did not correlate with MSTS scores and radiological parameters (P>0.05). Biological reconstruction with VFG can provide permanent stability and progressively increasing functional and radiological results.