Rare Presentation of Pediatric Multiple Enchondromatosis Limited to Single Ray or Single Nerve Distribution in the Hand: A Multicenter Case Series.

BACKGROUND: Multiple enchondromas in the pediatric hand is a relatively rare occurrence and the literature regarding its incidence and treatment is sparse. Within this rare subset of patients, we identified a unique cohort in which lesions are confined to multiple bones in a single ray or adjacent rays within a single nerve distribution. We review the clinical and pathologic characteristics and describe the indications for and outcomes of treatment in this unique subset of patients as well as offer conjectures about its occurrence. METHODS: Institutional review board (IRB)-approved retrospective multicenter study between 2010 and 2018 identified subjects with isolated multiple enchondromas and minimum 2-year follow-up. Data analyzed included demographics, lesion quantification and localization, symptoms and/or fracture(s), treatment of lesion(s), complications, recurrence, and presence of malignant transformation. RESULTS: Ten patients were evaluated with average age at presentation of 9 years (range: 4 to 16) and mean clinical follow-up of 6 years (range: 2.8 to 8.6). Five subjects had multiple ray involvement in a single nerve distribution and 5 had single ray involvement with an average of 4 lesions noted per subject (range: 2 to 8). All children in the study had histopathologic-proven enchondromas and underwent operative curettage±bone grafting. Indications for surgical intervention included persistent pain, multiple prior pathologic fractures, impending fracture and deformity. During the study period three subjects experienced pathologic fracture treated successfully with immobilization. Recurrence was noted in 40% at an average of 105 weeks postoperatively (range: 24 to 260) and appears higher than that reported in the literature. No case of malignant transformation was observed during the study period. CONCLUSIONS: A rare subset of pediatric patients with multiple enchondromas of the hand is described with lesions limited to a single ray or single nerve distribution. Further awareness of this unique subset of patients may increase our understanding of the disease and improve patient outcomes. LEVEL OF EVIDENCE: Level IV-therapeutic (case series).

The Costs of Interfacility Transfers for Nonurgent Pediatric Supracondylar Fractures.

BACKGROUND: Unnecessary transfers of nonemergent pediatric musculoskeletal injuries to regional trauma centers can be costly. The severity of fracture displacement in supracondylar humerus fractures dictates the risk of complications, the urgency of transfer and the need for surgical treatment. The purpose of this study is to examine the transfer patterns of Gartland type II pediatric supracondylar humerus fractures to identify strategies for improving patient care, improving health care system efficiency, and reducing costs. We hypothesize that there will be a high rate of unnecessary, emergent transfers resulting in increased cost. METHODS: We retrospectively identified all pediatric patients that underwent treatment for a supracondylar humerus fracture between 2013 and 2018. Patient demographics, injury characteristics, chronological data, and surgical data were collected and analyzed from ambulance run sheets, transferring hospital records, and electronic medical records. Transfer distances were estimated using Google-Maps, while transfer costs were estimated using Internal Revenue Service (IRS) standard mileage rates and the American Ambulance Association Medicare Rate Calculator. A student t test was used to evaluate different treatment groups. RESULTS: Sixty-two patients had available and complete transfer data, of which 44 (71%) patients were safely transferred via private vehicle an average distance of 51.8 miles, and 18 (29%) patients were transferred via ambulance on an average distance of 55.6 miles ( P =0.76). The average transfer time was 4.1 hours by private vehicle, compared with 3.9 hours by ambulance ( P =0.56). The average estimated cost of transportation was $28.23 by private vehicle, compared with $647.83 by ambulance ( P =0.0001). On average, it took 16.1 hours after injury to undergo surgery and 25.7 hours to be discharged from the hospital, without a significant difference in either of these times between groups. There were no preoperative or postoperative neurovascular deficits. CONCLUSION: Patients with isolated Gartland type II supracondylar humerus fractures that are transferred emergently via ambulance are subjected to a significantly greater financial burden with no demonstrable improvement in the quality of their care, since prior research has shown these injuries can safely be treated on an outpatient basis. Potential options to help limit costs could include greater provider education, telemedicine and improved coordination of care. LEVEL OF EVIDENCE: Level III (retrospective comparative study).

Evaluating Perioperative Complications Surrounding Supracondylar Humerus Fractures: Expanding Indications for Outpatient Surgery.

BACKGROUND: Supracondylar humerus (SCH) fractures are one of the most common pediatric orthopaedic injuries. Described using the Wilkins modification of the Gartland Classification system, current practice guidelines give moderate evidence for closed reduction and percutaneous pinning of type 2 and 3 injuries, but little evidence exists regarding the appropriate surgical setting for fixation. The goal of this study was to evaluate the perioperative complication profile of type 3 fractures with maintained metaphyseal contact and determine their suitability for outpatient surgery. METHODS: Skeletally immature patients with type 2 and 3 SCH fractures treated at a single, Level-1 trauma institution from March 2019 to January 2000 were retrospectively reviewed. A total of 1126 subjects were identified. Open, concomitant injuries, incomplete physical examination, initial neurovascular compromise, flexion-type fractures, ecchymosis, skin compromise, and those managed nonoperatively were excluded. Type 3 fractures were categorized as either "3M" versus type "3" ("M" denoting metaphyseal bony contact). Demographic data, neurovascular changes, and postoperative complications were collected. Categorical variables were evaluated using χ2 or Fisher exact tests, and continuous variables analyzed using analysis of variance, with significance defined as a P-value <0.05. RESULTS: A total of 485 patients (189 type 2, 164 type 3M, 132 type 3) met inclusion criteria. Sex and length of stay did not differ among groups. The incidence of neurovascular change between initial presentation and surgical fixation was significantly greater for type 3 fractures compared with other groups (P=0.02). No child in the 3M group had preoperative neurovascular examination changes, compared with 3 patients with type 3 injuries. When directly compared with the 3M group, type 3 fractures had a higher incidence of neurovascular examination changes that trended towards significance (P=0.08). There was no difference in postoperative complication rate between groups (P=0.61). CONCLUSIONS: Our findings demonstrate that Gartland type 3 SCH fractures lacking metaphyseal bony contact, compared with types 3M and 2, are more likely to experience neurovascular examination changes between initial presentation and definitive surgical fixation. Type 3M fractures clinically behaved like type 2 injuries and, accordingly, could be considered for treatment on an outpatient basis. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Pediatric Ganglion Cyst Recurrence: Location Isn't the Only Risk Factor.

INTRODUCTION: Ganglion cysts represent the most common benign soft-tissue masses of the hand and wrist, most are treated nonoperatively, with relatively few local recurrences. Few studies have identified risk factors for recurrence in the pediatric population. The aim of this study is to identify risks of cyst recurrence and to establish if ultrasonographic imaging aids in the prediction of recurrence. METHODS: A single-center retrospective chart review was performed, identifying patients diagnosed with a ganglion cyst of the hand or wrist. Demographic information, cyst characteristics, and ultrasound examination reports were documented. Standard statistical and logistic regression analyses were performed. RESULTS: A total of 132 cysts were identified in 126 patients and the average age was 8.5 years old. The most common location was the dorsal wrist (57/132, 43.2%). There were 14 recurrences [11/14, (79%) dorsal wrist, 3/14 (21%) volar wrist, 0/14 (0%) in nonwrist locations]. The risk of recurrence was significantly greater for dorsal wrist cysts than nonwrist locations (odds ratio=18.1; 95% confidence interval: 1.02, 316.65; P=0.048); there was no statistical difference in recurrence rates between dorsal and volar cysts (P=0.15). Recurrence was noted in older patients (12.2 vs. 8.1 y, P<0.001) and those patients with painful masses (P=0.02). Patients undergoing surgical excision had a higher risk of recurrence than those who did not undergo surgical excision (P<0.001). Cyst volume as measured by ultrasound was performed in 37 cysts, with repeat ultrasounds in 12 cases demonstrating a decreased volume of 0.85 cm at baseline to 0.35 cm with repeat examination (P=0.40). In patients that received at least 1 ultrasound, there were no differences in cyst volume between those that recurred and those that did not (P=0.40). CONCLUSIONS: Risk factors for recurrence in pediatric patients with a ganglion cyst include older age, symptomatic masses, cysts located around the wrist, and those requiring surgical excision. Ultrasound examination of cyst volume did not predict recurrence. LEVEL OF EVIDENCE: Level III-therapeutic.

Upper extremity anomalies in children with femoral and fibular deficiency.

Defects occurring in the femoral-fibular-ulnar developmental field are believed to cause the cluster of anomalies seen with femoral, fibular and ulnar limb deficiencies. Upper limb function must be considered in the management of lower limb deficiencies. The purpose of this study is to determine the frequency and type of upper extremity anomalies found in children with femoral and/or fibular deficiency. A retrospective review of 327 consecutive patients with the diagnosis of femoral and/or fibular deficiency was performed using existing records and radiographs. Characteristics of those with and without upper extremity anomalies were compared. Upper extremity anomalies were identified in 56 patients. They were more common among those with bilateral, compared with unilateral, lower extremity deficiencies (P < 0.0001). Seventy-five upper limbs were involved with 50 ulnar deficiencies, nine congenital transhumeral deficiencies, four congenital shoulder disarticulations, seven cleft hands, two radial head dislocations and one each - radial deficiency, syndactyly and capitate-lunate coalition. Two patients with bilateral upper extremity anomalies had ulnar deficiency on one side and a transverse deficiency on the other. Upper extremity anomalies are found in 17% of children with femoral and/or fibular deficiency, especially with bilateral lower extremity involvement. Ulnar deficiency is the most common type but one-third had other anomalies. The frequent finding of congenital transverse upper extremity deficiencies suggests there may be common embryology.

Severity of ulnar deficiency and its relationship with lower extremity deficiencies.

To assess the characteristics of ulnar deficiency (UD) and their relationship to lower extremity deficiencies, we retrospectively classified 82 limbs with UD in 62 patients, 55% of whom had femoral, fibular, or combined deficiencies. In general, UD severity classification at one level (elbow, ulna, fingers, thumb/first web space) statistically correlated with similar severity at another. Ours is the first study to show that presence of a lower limb deficiency is associated with less severe UD on the basis of elbow, ulnar, and thumb/first web parameters. This is consistent with the embryological timing of proximal upper extremities developing before the lower extremities.

Radiologic Evaluation of the Distal Radius Indices in Early And Late Childhood.

The osseous anatomy of the distal radius is well documented in adults. Three commonly discussed variables are the volar tilt (also known as palmar tilt or palmar inclination), radial inclination, and radial height. These values are not well defined in the growing skeleton. We studied the radiographic measurements of normal distal radius osseous anatomy in children and identified how these values change with age. 372 patients (215 males and 157 females) between the ages of 8 and 16 were included in the study. Normal values of volar tilt, radial inclination, and radial epiphyseal height were defined for each age group. Regression analysis showed that volar tilt increased significantly by increase in age (P <0.001). Radial inclination and radial epiphyseal height both showed significant increase with increase in age (P<0.001). This is the first study to define these radiographic values in children and their change with age.

Radial Polydactyly and the Incidence of Reoperation Using A New Classification System.

BACKGROUND: Polydactyly is one of the more common congenital deformities with an incidence of 0.8 to 1.4 per 1000. Traditionally the Wassel Classification system has been used, which is based on the level of duplication seen on plain radiographs. Although it is helpful in describing the anatomic characteristics, it is somewhat limited with regards to surgical planning and postoperative outcomes. Chung and colleagues, recently proposed a new classification system that categorizes radial polydactyly based on morphologic features that provides helpful information to be used in surgical decision making. We reviewed all radial polydactyly cases that had undergone operative intervention at our center over a 10-year period to investigate if this new classification system correlates with the rate of reoperation. METHODS: A total of 60 thumbs in 54 patients that were treated surgically from 2000 to 2010 at our institution were included in this study. Only patients with a minimum follow-up of 2 years were included. The authors categorized all duplications based on the classification system proposed by Chung and colleagues: type I (Joint Type), type II (Single Epiphyseal Type), type III (Osteochondroma-like Type), and type IV (Hypoplastic Type). Statistical analysis was then used to look at this classification system as it relates to sex, family history, syndrome association, and the need for reoperation. RESULTS: Of the 60 radial polydactyly cases, 37 (62%) were type I; 6 (10%) were type II; 6 (10%) were type III; and 11 (18%) were type IV. Six thumbs underwent reoperation for residual deformity-3 type I, 3 type II, and none of the types III or IV. No statistical significance was found when comparing classification group to sex, family history, syndrome association, laterality, or bilateral involvement. Statistical significance (P<0.05) was found between groups and the need for reoperation. CONCLUSIONS: The new classification system proposed by Chung and colleagues is easy to use and can guide practitioners in their discussions with patients regarding surgical outcomes and possible need for revision surgery.

Thumb hypoplasia.

Hypoplasia of the thumb refers to a spectrum of clinical abnormalities ranging from a slightly small digit to complete absence (or aplasia) of the thumb unit. As a component of radial dysplasia, thumb hypoplasia can be either an isolated entity or seen in conjunction with other elements of radial longitudinal deficiency. Treatment of this condition initially involves identifying and addressing co-morbid anomalies in other body systems. The severity of thumb hypoplasia is then graded so that appropriate treatment methods can be instituted. The goal of treatment is ultimately to provide the child with a stable and functional thumb unit.

Capture and manipulation of magnetically aligned Pf1 with an aqueous polymer gel.

The magnetic alignment of the Pseudomonas bacteriophage Pf1 is captured indefinitely in a gel of the aqueous triblock copolymer Pluronic F-127. In addition to preserving high-resolution liquids NMR spectra for dissolved solutes, the gel prevents the reorientation of the phage allowing mechanical manipulation of the angle between the axis of the phage alignment and the static magnetic field. The residual 2H quadrupolar couplings for several solutes dissolved in this material as a function of the angle Theta between the non-spinning sample tube and the static magnetic field are consistent with the value of P(2)(cosTheta)=(3cos(2)Theta-1)/2. The variable-angle correlation spectrum for these solutes is shown to separate residual quadrupolar effects from isotropic chemical shifts. Finally, the compatibility of Pluronic F-127 with NMR studies of aqueous biological macromolecules is demonstrated in a measurement of residual dipolar couplings in an 15N-labeled nucleic acid.