Decreased vascularization of retroperitoneal kaposiform hemangioendothelioma induced by treatment with sirolimus explains relief of symptoms.

Kaposiform hemangioendothelioma (KHE) is a vascular tumor with poor prognosis. We present a child with progressive disability, extreme pain, and autonomic dysfunction due to a retroperitoneal KHE where radiologic characteristics were essential for diagnosis and monitoring of response to therapy. He received sirolimus, and the symptomatology resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the tumor, although the volume was not significantly affected. We suggest that the sirolimus-induced tumor de-vascularization may explain the clinical and coagulopathy improvement.

The Factors Influencing the Refracture of Pediatric Forearms.

PURPOSE: Pediatric forearm fractures are common and usually heal uneventfully. The purpose of this study was to review the refracture rate and to identify trends and risk factors that may lead to a refracture. METHODS: Using current procedure terminology code and subsequent chart review we retrospectively identified 2590 patients who sustained forearm fractures over the past 10 years (2000 to 2010) and were treated at a single, large pediatric orthopaedic practice. RESULTS: We identified 37 patients who met our search criterion which yielded a refracture rate of 1.4%. Average length of immobilization was 72.2 days for initial fractures and 98.2 days for refractures. Average time to refracture after declared healing of initial injury was 128.7 days with 36% of refractures occurring within 6 weeks of clinical clearance. Fractures with ≥ 15 degrees angulation refractured earlier (mean 40 d). Seventy-one percent (71%) of patients with refractures had ≥ 10 degrees residual angulation at the time of union of the initial fracture. There was complete radiographic healing in 72% of patients that subsequently refractured. Forearm fractures that refractured most commonly occurred in the middle third (72%), with 24% in the proximal third and 4% in the distal third. Only 2 of 28 patients required surgical instrumentation of the forearm to achieve union of the refracture. We identified a trend toward longer immobilization and time to clinical clearance following a refracture, 76.4 versus 104.2 days. CONCLUSIONS: Over the past 10 years, our clinical data identifies a 1.4% refracture rate, which is significantly less than the previously published rate of 5%. Fractures with greater residual angulation (> 15 degrees) showed a tendency toward earlier refracture and may warrant longer immobilization. Forearm refractures united in most instances with closed treatment. Our treatment with cast or protective brace immobilization and limitation of activity until complete radiographic union likely influences our improved refracture rates.

Patients with unstable slipped capital femoral epiphysis have antecedent symptoms.

BACKGROUND: The characteristics of patients who sustain unstable slipped capital femoral epiphyses (SCFEs) are not well described compared to their counterparts who sustain stable SCFE. Although patients with unstable slips are usually identified owing to acute symptoms, it is unclear whether these patients have premonitory symptoms that could heighten the awareness of treating physicians to the possibility of an unstable slip and lead to timely diagnosis and treatment. QUESTIONS/PURPOSE: We determined whether most patients experienced pain and limp before developing an unstable SCFE. METHODS: We retrospectively reviewed 582 patients and identified 82 (41 boys, 41 girls; 85 hips) with unstable SCFEs. Patient records were reviewed for sex, age at onset, weight at onset, and presence and location of pain and/or limp before the unstable slip. Boys averaged 13 years of age at the time occurrence and weighed on average in the 77th percentile. Girls averaged 12 years of age at the time of occurrence and weighed on average in the 79th percentile. RESULTS: For all patients, 73 of 82 (88%) had pain in their hips, thighs, or knees for an average of 42 days before sustaining unstable SCFEs. Sex distribution was equal for patients with unstable SCFEs. CONCLUSIONS: Patients who sustained unstable SCFEs had premonitory pain in the limb. Early recognition and an appropriate diagnosis provide a critical opportunity to prevent a morbid unstable SCFE. LEVEL OF EVIDENCE: Level IV, diagnostic study, See Instructions for Authors for a complete description of levels of evidence.

The unstable slipped capital femoral epiphysis: risk factors for osteonecrosis.

BACKGROUND: The incidence of avascular necrosis (AVN) after unstable slipped capital femoral epiphysis (SCFE) varies widely in the literature (10% to 60%), and few studies have examined why certain unstable slips develop osteonecrosis whereas others do not. Our purpose was to determine risk factors for developing AVN after unstable SCFE. METHODS: We reviewed all unstable SCFEs treated primarily at our center. Medical records were reviewed to determine weight-percentile, age, length of prodromal symptoms, and time to treatment. Operative notes were used to classify treatment as either: (group 1) in situ screw fixation, (group 2) purposeful or inadvertent closed reduction and screw fixation, or (group 3) open reduction and internal fixation, and to determine whether or not the joint was decompressed during surgery. Preoperative radiographs were used to measure slip angle and percent translation. For group 2, these were compared with postoperative radiographs to calculate a Deltaslip angle and Deltatranslation. Student t tests and Fisher exact tests were used for statistical analysis. RESULTS: Of the 70 patients in our series, 14 developed AVN (20%). On the basis of treatment, 3 of 16 patients in group 1 (19%), 10 of 38 patients in group 2 (26%), and only 1 of 16 patients in group 3 (6%) developed AVN. Patients who developed osteonecrosis were significantly younger (11.67 y vs. 12.83 y, P=0.04) and had a significantly shorter duration of prodromal symptoms (17.5 d vs. 65.9 d, P=0.03) compared with those who did not develop AVN. None of the other factors were found to be significant. In a subgroup analysis looking solely at group 2 patients, those who developed AVN had a significantly higher initial slip angle compared with those who did not (62 degrees vs. 51 degrees, P=0.03). CONCLUSIONS: In this, the largest reported series of unstable slips treated with internal fixation, AVN seemed more likely to develop in younger patients with a shorter duration of prodromal symptoms. Patients undergoing open reduction may have a lower incidence of AVN, but our limited power precludes definitive conclusions. LEVEL OF EVIDENCE: Level IV (case series).