Sumeet Garg

Langerhans cell histiocytosis of the spine in children: Long-term follow-up

BACKGROUND Langerhans cell histiocytosis causes destructive lesions in a childs spine. Few large, long-term studies have evaluated the clinical and radiographic presentation, natural history, outcomes of modern treatment approaches, and maintenance of normal spinal growth and stability after the diagnosis of this disease in children. METHODS Twenty-six children with biopsy-proven Langerhans cell histiocytosis involving the spine were treated at our institution between 1970 and 2003. They had a total of forty-four involved vertebrae (twenty cervical, fourteen thoracic, and ten lumbar). Vertebral body collapse was measured on radiographs and classified as grade I (0% to 50% collapse) or grade II (51% to 100% collapse) and subclassified as A (symmetric collapse) or B (asymmetric collapse). Lesions of the posterior elements of the spine were classified as grade III. Twenty-three children were followed for two years or more (mean, 9.4 years), and the analyses of treatment and long-term outcomes were performed in that group of patients. RESULTS There was a predominance of lesions in the cervical spine (p </= 0.02). Sixteen (62%) of the twenty-six children were found to have multifocal skeletal disease. Cervical and lumbar lesions were more commonly associated with multilevel spinal disease. The extent of the initial collapse seen radiographically was grade IA for twenty vertebrae, IB for three, IIA for ten, IIB for nine, and III for two. Grade-I lesions were more likely to be associated with symmetric collapse than were grade-II lesions. Spinal deformity developed in four children, and two later required spinal fusion. No relationship was observed between the grade of the initial collapse and the subsequent development of spinal deformity. Despite heterogeneous treatment, all patients were alive and well with resolution of all presenting signs and symptoms and no evidence of active disease at the time of the most recent follow-up. CONCLUSIONS We found a particularly high prevalence of lesions in the cervical spine and a high prevalence of multiple skeletal lesions. In contrast to the classic finding of vertebra plana, we found that more severe lesions often led to asymmetric collapse; yet, asymmetric collapse was not found to be associated with the development of subsequent spinal deformity. The natural history of these lesions in the spine in the absence of systemic disease or spinal deformity is such that aggressive surgical management is usually not indicated; only follow-up is necessary to monitor recovery and spinal balance.

The diagnostic accuracy of MRI versus CT imaging for osteoid osteoma in children.

Advanced imaging often is obtained in children suspected of having osteoid osteoma. We hypothesized that magnetic resonance imaging gives a falsely aggressive appearance and that computed tomography is better for identifying osteoid osteoma. This is the first prospective blinded study comparatively assessing these imaging studies in children. Twelve preoperative magnetic resonance imaging scans of confirmed cases of osteoid osteoma were collected. Three radiologists blinded to the diagnosis reviewed these images. Only a clinical history of skeletal pain was given. Lesions were classified as benign-latent, benign-aggressive, or malignant. Radiologists were asked to list their primary diagnosis (with a confidence level from 1–10). Seven of these 12 children also had computed tomography scans that subsequently were reviewed in similar manner. With computed tomography scans, lesions were accurately identified as benign-latent (15/21 readings, 71%) and as osteoid osteoma (14/21 readings) more frequently than with magnetic resonance imaging scans (7/36 readings, 19%). Level of Evidence: Diagnostic study, Level III-1 (study of nonconsecutive patients—no consistently applied reference gold standard). See the Guidelines for Authors for a complete description of levels of evidence.

Modern surgical treatment of primary aneurysmal bone cyst of the spine in children and adolescents.

Aneurysmal bone cyst (ABC) is a benign but locally aggressive tumor-like condition. Treating children with spinal involvement poses special hazards due to the proximity of the lesion to the spinal cord and the need to preserve spinal stability and balance after surgery. Twelve children with ABC treated between 1990 and 2002 at a tertiary pediatric musculoskeletal tumor center using modern surgical techniques and technology were retrospectively reviewed. A surgical technique using a four-step approach of intralesional curettage, high-speed bur, electrocautery, and bone grafting was found to have a significantly reduced rate of recurrence (0/8 cases) compared with traditional intralesional curettage and bone grafting (4/4 cases) (P < 0.002). Short-segment spinal fusion with instrumentation was also done in five cases immediately after excision of the ABC under the same anesthesia and was a nonsignificant marker for reduced rate of recurrence (P < 0.08), likely secondary to wide exposure. Overall, at last follow-up, all 12 patients were alive and well with no evidence of disease and no significant spinal deformity. The four-step approach to treatment of ABC of the spine with appropriate spinal instrumentation and fusion is recommended for successful treatment of this aggressive lesion.

The role of serial casting in early-onset scoliosis (EOS).

Background: Serial casting has demonstrated efficacy for idiopathic early-onset scoliosis (EOS). Results of casting in nonidiopathic (syndromic and congenital) EOS patients have not previously been well described. Methods: A total of 53 patients underwent serial casting for EOS from 2005 to 2010 at a single institution. Deformity was classified as idiopathic or nonidiopathic. Diagnosis, time in cast, number of casts, use of bracing, complications, and outcomes were recorded. Radiographic measures included Cobb angle and thoracic height (T1-T12). Thoracic height velocity was calculated and compared with established norms. Results: A total of 36 patients, 19 idiopathic and 17 nonidiopathic (14 syndromic, 3 congenital), completed cast treatment and had >6-month follow-up and were therefore included. Of those, 17% (6/36) experienced resolution of their deformity, 53% (19/26) are currently in braces, and 31% (11/36) had undergone surgery. Surgery occurred on average at age 5.6 years and was delayed by an average of 2.1 years from time of first cast. A 19% complication was observed. There was no statistical difference in the rate of resolution of deformity between idiopathic (5/19) and nonidiopathic (1/17) patients (P=0.182), although there exists a trend toward greater curve correction in idiopathic patients. Surgery occurred in fewer patients (2/19) in the idiopathic group compared with the nonidiopathic group (9/17) (P=0.006). Significant improvements in Cobb angle was observed in the idiopathic group (12.2 degrees) during casting (P=0.003). Nonidiopathic patients did not maintain the correction gained during casting at the time of final follow-up. T1-T12 height increased across all study patients regardless of etiology during the period of casting at similar velocity to established norms of 1.4 cm/y for this age group. Conclusions: Serial casting offers modest deformity correction in idiopathic deformities compared with nonidiopathic deformities. Thoracic height growth continued throughout the casting period at normal velocity. Serial casting maintained normal longitudinal thoracic growth in all patients with EOS in this cohort. Although many required surgery, the increased thoracic height may have positive implications on ultimate pulmonary function. Level of Evidence: Therapeutic level III.

Tumors and tumor-like conditions of the spine in children.

Abstract Tumors and tumor‐like conditions of the spine, although rare, should be included in the differential diagnosis of infants and children with back pain. Skeletal pathology is more frequently the cause of back pain in children than in adults. Although most tumors and tumor‐like conditions of the spine in children are benign, many require surgery. Children with malignant tumors of the spine (with the exception of leukemia and lymphoma) may require multimodality therapy, including surgery, to achieve long‐term cure. Advances in imaging, surgical technique, surgical technology, and adjuvant therapy have led to improvements in diagnosis and treatment and, thus, outcomes. In many cases, however, early and accurate diagnosis is often possible based on only clinical history, physical examination, and plain radiographic imaging.

Use of the Ponseti method for recurrent clubfoot following posteromedial release

Background: A child with recurrent or incompletely corrected clubfoot after previous extensive soft tissue release is treated frequently with revision surgery. This leads to further scarring, pain and limitations in range of motion. We have utilized the Ponseti method of manipulation and casting and when indicated, tibialis anterior tendon transfer, instead of revision surgery for these cases. Materials and Methods: A retrospective review of all children treated since 2002 (n = 11) at our institution for recurrent or incompletely corrected clubfoot after previous extensive soft tissue release was done. Clinical and operative records were reviewed to determine procedure performed. Ponseti manipulation and casting were done until the clubfoot deformity was passively corrected. Based on the residual equinus and dynamic deformity, heel cord lengthening or tenotomy and tibialis anterior transfer were then done. Clinical outcomes regarding pain, function and activity were reviewed. Results: Eleven children (17 feet) with ages ranging from 1.1 to 8.4 years were treated with this protocol. All were correctable with the Ponseti method with one to eight casts. Casts were applied until the only deformities remaining were either or both hindfoot equinus and dynamic supination. Nine feet required a heel cord procedure for equinus and 15 required tibialis anterior transfer for dynamic supination. Seven children have follow-up greater than one year (average 27.1 months) and have had excellent results. Two patients had persistent hindfoot valgus which required hemiepiphyseodesis of the distal medial tibia. Conclusion: The Ponseti method, followed by tibialis anterior transfer and/or heel cord procedure when indicated, can be successfully used to correct recurrent clubfoot deformity in children treated with previous extensive soft tissue release. Early follow-up has shown correction without revision surgery. This treatment protocol prevents complications of stiffness, pain and difficulty in ambulating associated with multiple soft tissue releases for clubfeet.

An atypical presentation of Langerhans cell histiocytosis of the cervical spine in a child.

Study Design. A case report of a child with Langerhans cell histiocytosis of the posterior elements of the cervical spine treated conservatively and with long-term follow-up is described. Objectives. To describe the unique diagnostic and therapeutic challenges of treating Langerhans cell histiocytosis located atypically in the posterior elements of the spine. Summary of Background Data. Langerhans cell histiocytosis involves the spine in approximately 20% of children with the disease. In nearly 95% of cases, spinal Langerhans cell histiocytosis results in destruction of the vertebral body, sparing the posterior elements. Confident diagnosis of vertebral body lesions is often made based on plain radiographs and MRI; however, the diagnosis of posterior element disease is not so straight forward. The natural history of isolated skeletal Langerhans cell histiocytosis is benign in most cases. Methods. The clinical and radiographic presentation of a child with Langerhans cell histiocytosis involving the posterior elements of C3 is described. A soft tissue mass was observed on magnetic resonance imaging; however, a biopsy confirmed the benign diagnosis of Langerhans cell histiocytosis. No further surgical treatment was needed, and conservative therapy incorporating a short course of cervical bracing and low-grade chemotherapy was begun. Results. Within a year, reconstitution of the posterior elements of C3 was apparent on radiographs. By 9 years after diagnosis, the child had near full reconstitution of the posterior elements of C3 and resolution of anterior pseudosubluxation of C3 on C4 that was present at diagnosis. Although diagnosis of Langerhans cell histiocytosis was made difficult by an atypical location in the posterior elements of the spine, after biopsy, conservative treatment led to excellent vertebral reconstitution. Conclusions. Isolated involvement of the posterior spine in Langerhans cell histiocytosis has not been described in detail previously. The reported case illustrates the diagnostic challenges of isolated posterior spine Langerhans cell histiocytosis. Despite an unusual presentation, posterior spine Langerhans cell histiocytosis should be treated conservatively in a similar manner to anterior lesions.

Management of the pediatric pulseless supracondylar humeral fracture: Is vascular exploration necessary?

BACKGROUND Radically different conclusions exist in the pediatric orthopaedic and vascular literature regarding the management of patients with a pink hand but no palpable radial pulse in association with a supracondylar humeral fracture. METHODS One thousand two hundred and ninety-seven consecutive, operatively treated supracondylar humeral fractures in patients presenting to a level-I pediatric trauma center from January 2003 through December 2007 were studied retrospectively. Clinical records were reviewed to determine vascular and neurological examination findings, Gartland classification, timing of surgery, and postoperative complications. RESULTS One thousand two hundred and sixty-six patients had a documented radial pulse examination at the time of arrival in the emergency room; fifty-four (4%) of those patients lacked a palpable radial pulse. All fifty-four patients had type-3 fractures. Five (9%) of the fifty-four patients underwent open exploration of vascular structures on the basis of clinical findings of a pale hand, sluggish capillary refill, and/or weak or no pulse detected with use of Doppler ultrasound after closed reduction and percutaneous pinning. All five underwent vascular surgery to restore blood flow (two primary repairs, three saphenous vein grafts). Twenty (37%) of the fifty-four patients had a pulse documented with use of Doppler ultrasound and a pink hand after closed reduction and percutaneous pinning, but the radial pulse remained nonpalpable. These patients were observed in the hospital for signs of ischemia; one of the twenty patients required vascular repair after developing a pale hand nine hours after closed reduction and percutaneous pinning, and the other nineteen patients were also observed while they were in the hospital, and they all regained a palpable pulse either prior to discharge or by the time of the first postoperative visit. When compared with the group of patients with type-3 fractures for whom data regarding nerve examination were available, patients with type-3 fractures who lacked a palpable radial pulse had a higher rate of nerve palsy postoperatively (31% versus 9%, p < 0.0001). CONCLUSIONS In this cohort, nearly 10% of patients who presented with a type-3 supracondylar humeral fracture and no palpable radial pulse underwent immediate vascular repair to restore blood flow following closed reduction and percutaneous pinning. However, in our series, the lack of a palpable radial pulse after closed reduction and percutaneous pinning was not an absolute indication to proceed with vascular exploration if clinical findings (i.e., Doppler signal and capillary refill) suggested that the limb was perfused. Careful inpatient monitoring of these patients postoperatively is mandatory to identify late-developing vascular compromise. LEVEL OF EVIDENCE Prognostic level III. See Instructions for Authors for a complete description of levels of evidence.

Clinical characteristics of severe supracondylar humerus fractures in children.

Background: The safety of delayed surgical treatment of severe supracondylar elbow fractures in children remains debated. No large studies have evaluated complications of injury and surgery evaluating only type 3 fractures. Our aim was to review the results of our experience treating children with severe supracondylar elbow fractures at various time points after injury. Methods: All children treated operatively for supracondylar humerus fractures from 2004 to 2007 at a single pediatric trauma center were identified. A total of 1296 children had operative treatment, of which 872 had type 3 fractures. Clinical records were reviewed to identify time to surgery from presentation at our institution. Patients were grouped into 4 cohorts [<6 h (n=325), 6 to 12 h (n=224), 12 to 24 h (n=295), and >24 h (n=28)]. Emergency, operative, inpatient, and outpatient records were reviewed to determine morbidity at presentation as well as operative and postoperative complications. Results: There was no difference in sex, age, or energy mechanism between children in the various time groups. An absent pulse was found in 54 children (6%) at presentation, of which only 5 ultimately required a vascular intervention. Nerve injury occurred in 105 patients (12%). Use of a medial entry pin was not associated with ulnar nerve injury. Increased time from presentation to surgery was not associated with increased morbidity from the injury or treatment complications. In contrast, there was a trend to steady decrease in morbidity and complication rates with increased time to surgery. Conclusions: This is the largest single-center study of severe supracondylar humerus fractures and describes rates of vascular compromise, nerve injury, infection, and other complications of these injuries. Most children with type 3 supracondylar humerus fractures can be treated safely in a delayed manner. Appropriate clinical judgment is imperative to optimize outcomes. Level of Evidence: Level III—retrospective comparative study.

Abnormal scarring with keloid formation after osteochondroma excision in children with multiple hereditary exostoses.

Introduction: Multiple hereditary exostoses (MHE) is an autosomal dominant condition characterized by numerous cartilage-capped exostoses/osteochondromas in areas of actively growing bone. Abnormal scarring with keloid formation after osteochondroma excision in children with MHE has not been previously described. Methods: A retrospective double-cohort study was undertaken to determine if children with MHE have a higher rate of abnormal scarring with keloid formation after osteochondroma excision when compared with those with solitary osteochondroma. In the initial phase, all consecutive children with MHE that underwent excision of osteochondroma with a minimum 2-year postoperative follow-up were identified. A control group of age-matched cases of solitary osteochondroma was subsequently identified. All patients were interviewed for wound healing problems and noncosmetic scarring. All patients with unsatisfactory scars were asked to send pictures and/or were invited for follow-up. Data were statistically analyzed. Results: Eighty-three surgeries were performed in 25 patients with MHE, whereas 25 surgeries were performed in 25 patients with solitary osteochondroma. Twelve keloid scars were noted in 7 patients with MHE, and no keloids were noted in any of the patients in the solitary group. Diagnosis of MHE was a statistically significant risk factor for formation of keloids after surgery (P < 0.05). Discussion: Abnormal scarring with keloid formation after osteochondroma excision in MHE has not been previously reported. Although this study has limited numbers, the results demonstrate a statistically significant correlation between keloid formation and MHE. The risk for abnormal scarring and keloid formation should be discussed with all patients before surgery.