Ramona R. Fillman

Evidence-Based Analysis of Removal of Orthopaedic Implants in the Pediatric Population

Background: Requested project of the Pediatric Orthopaedic Society of North America Evidenced-Based Medicine Committee. Methods: The English literature was systematically reviewed for scientific evidence supporting or disputing the common practice of elective removal of implants in children. Results: Several case series reported implant removal, but none contained a control group with retained implants. No articles reported long-term outcomes of retained implants in large numbers. Several small series describe complications associated with retained implants without evidence of causation. The existing literature was not amenable to a meta-analysis. By compiling data from the literature, it is possible to calculate a complication rate of 10% for implant removal surgery. The complication rate for removal of implants placed for slipped capital femoral epiphysis is 34%. Articles regarding postmarket implant surveillance and basic science were also reviewed. Conclusions: There is no evidence in the current literature to support or refute the practice of routine implant removal in children.

Protrusio acetabuli in Marfan syndrome. History, diagnosis, and treatment.

Marfan syndrome is an autosomal dominant disorder of connective tissue, with ocular, skeletal, and cardiovascular manifestations. Protrusio acetabuli is a criterion for the diagnosis of Marfan syndrome. Prolonged protrusio acetabuli may result in secondary osteoarthritic changes in the hip joint. Radiographic criteria for protrusio acetabuli include an abnormally positioned acetabular line, a center-edge angle of Wiberg of >40 degrees, and crossing of the teardrop by the ilioischial line. In a skeletally immature patient with Marfan syndrome in whom the triradiate physis of the acetabulum is still open, closure of the triradiate physis can interrupt and decrease the progression of the deformity. In older patients, valgus intertrochanteric osteotomy and eventually total hip arthroplasty are the only methods available for correction of the protrusio acetabuli.

Trevorʼs disease: the clinical manifestations and treatment of dysplasia epiphysealis hemimelica

Trevors disease is a rare abnormality characterized by aberrant osteocartilaginous growth from an epiphysis. We describe six new cases from our institution. A patient with isolated tibial tuberosity involvement is described for the first time. Each patient was classified according to Azouz into localized (three), classical (two) and generalized (one) form. We recommend that parents be counseled regarding the progressive nature of this disorder owing to the chance of worsening deformity with surgical excision alone, and the need to be followed until skeletal maturity.

Cyst index: a nonpredictor of simple bone cyst fracture.

Purpose: Treatment is indicated for simple bone cysts (SBC) with high risk of fracture. The cyst index was described as a method to determine if a cyst is at high risk or low risk of fracture. Study Design: The cyst index of 32 femoral and humeral SBCs was determined by 8 reviewers. The risk group cyst index was compared with whether a fracture took place. Sensitivities, specificities, and positive and negative predictive values were calculated across varying cutoff levels. Intraobserver and interobserver reliability testing for 10 cysts was made. Results: Receiver operator curves demonstrate that no cutoff value gave an acceptable level of both sensitivity and specificity. The mean value for the cyst index was significantly different for different observers (P < 0.05). Conclusions: This study does not validate the cyst index to be an accurate predictor of fracture. The test has low intraobserver and interobserver reliability. Clinical Relevance: The cyst index cannot reliably discriminate between the patients that will fracture and those that will not.

Precordial Doppler evaluation of simple bone cyst injection.

Background: A randomized prospective trial of marrow-versus-steroids treatment of solitary bone cyst was ongoing. Protocol required contrast injection of the cyst. A subpopulation was noted to have large, rapid venous outflow of contrast material (omnipaque). Central embolization and resultant acute respiratory distress syndrome has been reported with bone marrow transplant. Particulate injection can result in bradycardia, low exhaled CO2, decreased SaO2 and voltage changes on electrocardiogram. Methods: Precordial Doppler can measure flow and turbulence centrally after a peripheral cyst injection. Our cases were monitored with precordial Doppler as well as heart rate, ETCO2, blood pressure, electrocardiogram, and pulse oximetry. Results: Five patients/7 injections were noted to have large and rapid outflow veins from solitary bone cyst. The Doppler showed increased signal in all 7 particulate injections (2 steroid injections, 5 bone marrow aspirates and cyst injections.) One marrow injection resulted in transient bradycardia and decreased blood pressure with no sequelae. One developed transient decrease in exhaled CO2. Conclusions: Rapid outflow has been reported in the past but precordial Doppler monitoring of injection into peripheral cysts is not reported. The potential for embolization with serious physiologic effects is a concern. Others have advocated injection of more particulate substances, such as calcium, sulfate, and demineralized bone matrix. This Doppler technique may be valuable for monitoring the potential harmful effects of these injections and lead to a better understanding of failure of cyst healing due to rapid outflow of material. Level of Evidence: Level IV case series with poor reference standard.

Chronic osteomyelitis in children: Shriners Hospital Honolulu experience.

Chronic osteomyelitis incidence and severity in 55 hospitalized Pacific Island children between 1990 and 2002 were compared with the expected incidence and reports in the literature. Of these 55 cases, 87% were from Polynesia/Micronesia. The average length of hospital stay was 104 days. Staphylococcus aureus was cultured in 64% of the cases with 43% of those being methicillin resistant S. aureus. A total of 111 bones were involved. Average antibiotic treatment was 135 days. Each case required an average of 1.3 irrigations/debridements and 45% required a sequestrectomy. Ninety-two percent had elevated erythrocyte sedimentation rate on admission. Sixty-nine percent of the cases involved metaphyseal, diaphyseal and epiphyseal segments of the bone and 29% were multifocal. Results indicate that Pacific Island children have a higher incidence and increased severity of osteomyelitis when compared with non-Pacific Island children in the literature, requiring a high suspicion for multifocal osteomyelitis, extensive bone involvement, S. aureus positive cultures and a longer period of antibiotic treatment.

Protrusio Acetabuli in Marfan Syndrome: Indication for Surgery in Skeletally Immature Marfan Patients

The authors reviewed all patients in Shriners Hospital for Children, Honolulu, diagnosed with hyperlaxity of the joints (131 in total) for Marfan syndrome. The revised diagnostic criteria for Marfan syndrome were used for inclusion in this study. In the patients diagnosed with Marfan syndrome (n = 13), the incidence (n = 9) and indication for surgical intervention (n = 4) of protrusio acetabuli were evaluated by retrospective radiographic review, clinical findings, and family history. For the identification of protrusio acetabuli, the presence of an abnormal acetabular line position (>1 mm medial to the ilioischial line in boys, >3 mm in girls), a center-edge angle of Wiberg of 40 degrees or more, or crossing of the teardrop by the ilioischial line was used. The authors conclude that serial radiographic investigations, reinforced with a well-documented family history of symptomatic protrusio acetabuli, are needed to rule out progression of the deformity. When progression of the deformity is suggested in a skeletally immature Marfan patient, surgical intervention by closure of the triradiate cartilage is indicated, regardless of the absence of hip complaints or limitation of range of motion.

Pedicle rotation in scoliosis: a marker for occult intrathecal abnormalities.

Study Design. The Perdriolle method was used to assess retrospectively radiographic pedicle rotation for association with occult intrathecal abnormalities in patients with scoliosis. Objective. To determine if pedicle rotation can be predictive of underlying intrathecal abnormalities. Summary of Background Data. Scoliosis associated with intrathecal abnormalities is thought to produce less rotation than true idiopathic scoliosis. No supporting evidence was found in the literature. Methods. A consecutive series of patients with a presenting diagnosis of idiopathic scoliosis were reviewed for anteroposterior radiographs and spinal magnetic resonance imaging (MRI). A blinded single examiner evaluated radiographic curve parameters. MRI reports were reviewed for the presence or absence of intrathecal abnormalities. Results. A total of 78 MRIs included 15 intrathecal abnormalities and 63 normals. The abnormal MRI group had more males and apex left curves. Primary curve in the intrathecal abnormality group had a mean of 9.6° of apical vertebrae rotation compared to 17.7° in idiopathic curves (average 37° and 40° Cobb angles, respectively). Of angulation, 1° correlated with 0.21° and 0.34° of rotation in intrathecal abnormality versus no abnormality groups, respectively. Conclusions. Curves with occult intrathecal pathology had significantly less rotation than those without. Pedicle rotation assessment is a useful adjuvant for identifying scoliosis with intrathecal abnormalities.

New finding of protrusio acetabuli in two families with congenital contractural arachnodactyly. A report of seven cases.

Congenital contractural arachnodactyly is an autosomal dominant disorder of connective tissue and is associated with skeletal features similar to those seen with Marfan syndrome1. Congenital contractural arachnodactyly and Marfan syndrome are linked to mutations in the highly homologous but distinct genes FBN2 (5q23-q31) and FBN1 (15q15-q21), which encode fibrillin 2 and fibrillin 1, respectively2. Fibrillins are major components of extracellular matrix microfibrils, which play an essential role in the formation of elastic fiber and the deposition of tropoelastin and which perform anchoring functions in some tissues3. As a consequence of the abnormal microfibrillogenesis that is seen in association with congenital contractural arachnodactyly and Marfan syndrome, complications may be found in the mesodermal tissues (i.e., the ocular tissue, the skeletal, cardiovascular, pulmonary, and muscular systems, and the skin and integumentum). The phenotypic similarity at times makes it clinically difficult to differentiate congenital contractural arachnodactyly from Marfan syndrome. This is illustrated by the observation that Antoine Marfans original patient may actually have had congenital contractural arachnodactyly and not what is known today as Marfan syndrome4. Contractures are seen in both syndromes. However, the contractures in patients with congenital contractural arachnodactyly are congenital and tend to improve spontaneously, whereas the contractures in Marfan syndrome are developmental and may be progressive1. Ectopia lentis was reported to occur in 60% of 160 patients with Marfan syndrome5. In contrast, to our knowledge, there has been only one report of ectopia lentis in association with congenital contractural arachnodactyly, in a patient with bilateral ectopia lentis6. The presence of a congenital heart defect strongly suggests the diagnosis of Marfan syndrome, although mitral valve prolapse7, atrial septal defects, and ventricular septal defects are also occasionally found in patients with congenital contractural arachnodactyly8 …

Transient monoparesis after blade plate removal in a Hutchinson-Gilford progeria syndrome patient: a case report.

Treatment of patients with Hutchinson–Gilford progeria syndrome (HGPS) is based on the abnormalities of accelerated aging that affect the healing processes, combined with a fragile cardiovascular status. A classic HGPS case, of Korean ancestry, previously treated for severe coxa valga with bilateral varus osteotomies using blade plate fixation is presented. Complications over the blade plate area required removal of the hardware, after which the patient showed right-sided hypertonicity – determined to be a cerebrovascular accident. Subsequently, the patient returned almost completely to her presurgical neurologic status. Perioperative planning for HGPS patients should include risks that are typically considered in the planning for geriatric patient care.