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Introduction: A male child with congenital insensitivity to pain (CIP) due to a novel de novo L369P mutation in the SCN11A gene was found to have significant bilateral hip flexion contractures, followed by severe heterotopic ossification after contraction release. This is the first report to describe a patient with this specific mutation and subsequent clinical course. Case Report: A male child with CIP due to de novo L369P mutation in the SCN11A gene was found to have significant bilateral hip flexion contractures. The patient underwent bilateral hip contracture releases to improve his standing ability after failure of conservative treatment. In the coming months he developed significant heterotopic ossification that eventually bridged from the left pelvis to the left femur. Conclusion: Heterotrophic ossification (HO) in patients with CIP is a rare and poorly understood clinical manifestation. Our report describes a rare and aggressive manifestation of HO in a patient with CIP.

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Spinal cord injury is one of the leading causes of morbidity and mortality among young adults in many countries including the United States. Difficulty in the regeneration of neurons is one of the main obstacles that leave spinal cord injury patients with permanent paralysis in most instances. Recent research has found that preventing acute and subacute secondary cellular damages to the neurons and supporting glial cells can help slow the progression of spinal cord injury pathogenesis, in part by reactivating endogenous regenerative proteins including Noggin that are normally present during spinal cord development. Noggin is a complex protein and natural inhibitor of the multifunctional bone morphogenetic proteins, and its expression is high during spinal cord development and after induction of spinal cord injury. In this review article, we first discuss the change in expression of Noggin during pathogenesis in spinal cord injury. Second, we discuss the current research knowledge about the neuroprotective role of Noggin in preclinical models of spinal cord injury. Lastly, we explain the gap in the knowledge for the use of Noggin in the treatment of spinal cord injury. The results from extensive in vitro and in vivo research have revealed that the therapeutic efficacy of Noggin treatment remains debatable due to its neuroprotective effects observed only in early phases of spinal cord injury but little to no effect on altering pathogenesis and functional recovery observed in the chronic phase of spinal cord injury. Furthermore, clinical information regarding the role of Noggin in the alleviation of progression of pathogenesis, its therapeutic efficacy, bioavailability, and safety in human spinal cord injury is still lacking and therefore needs further investigation.

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Klippel-Trenaunnay Syndrome is a rare disease characterized by a clinical triad of capillary malformation, soft tissue and bony hypertrophy, and atypical varicosity. This syndrome ranges from asymptomatic disease to life-threatening bleeding, embolism, and deformities. Management includes early diagnosis, prevention, and treatment of complications. We present a case of a 43-year-old male presenting with pain, swelling and deformity of the right leg for 30 years. On examination, diffusely enlarged tender right limb with several dark patchy discolorations, multiple tortuous vessels were found. Right leg X-ray showed heterotrophic ossification and distortion of ankle joint. Due to chronic severe pain, recurrent infection, contracture and flexion deformity of right leg, the patient underwent above knee amputation. This case focuses on the variable presentation and multiple problems faced by patients with Klippel-Trenaununay Syndrome as they get diagnosed late and shows the importance of high index of suspicion for early diagnosis and prevention of complications.

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Heterotrophic ossification (HO) is a reactive disease presenting the formation of mature lamellar bone in soft tissues. It is known to occur following surgery, soft tissue injury, or central nervous system anomalies. However, a definite cause has not yet been clearly addressed. During the process of approach, reduction, and fixation while conducting surgeries, partial injury of soft tissue is inevitable. Additionally, secondary injuries may be caused during the active and passive range of motion exercises that should be done for the recovery of joint motion after surgery. The authors experienced cases of HO that may occur during surgery and rehabilitation after surgery. The authors recognized that special care is required for patients complaining of severe pain during the early stage of rehabilitation immediately after surgery. This study aimed to reaffirm the principles of fracture treatment by reviewing the cases and to investigate the occurrence of HO after fracture surgery.

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OBJECTIVE: To evaluate the mid-term effectiveness of arthrolysis and hinged external fixation for the treatment of stiff elbow caused by heterotrophic ossification. METHODS: Between January 2014 and December 2017, 11 patients with stiff elbow caused by heterotrophic ossification were admitted. There were 9 males and 2 females with an average age of 32 years (range, 14-48 years), and left side in 6 cases and right side in 5 cases. The cause of stiff elbow included humerus fracture in 5 cases, ulna fracture in 2 cases, fracture of capitulum radii in 1 case, dislocation of capitulum radii in 1 case, terrible triad of the elbow in 1 case, and soft tissue injury in 1 case. The disease duration ranged from 7 to 18 months (mean, 11 months). Preoperative active range of motion of elbow was (19.6±17.5)° and Mayo score was 34.1±9.7. All patients received the treatment of arthrolysis and debridement of heterotrophic ossification lesion combining hinged external fixator, and active and passive rehabilitation with the help of hinged external fixator. The hinged external fixators were removed after 2 months. RESULTS: All patients were followed up 13-36 months (mean, 19.1 months). All incisions healed by first intention, and no complication of infection or nerve lesion occurred postoperatively. At last follow-up, the results of X-ray films showed that no heterotrophic ossification recurred. The active range of motion of elbow was (116.4±16.6)° and Mayo score was 93.2±7.8, showing significant differences when compared with preoperative ones ( t=17.508, P=0.000; t=16.618, P=0.000). CONCLUSION: The application of arthrolysis and debridement of heterotrophic ossification lesion combining hinged external fixator can improve the elbow's range of motion significantly and obtain a good mid-term effectiveness.

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BACKGROUND: Femoral head fractures are an uncommon but severe injury. These high-energy injuries typically occur in association with traumatic hip dislocations. Initial treatment includes urgent concentric reduction; however, controversy exists regarding specific fracture management. The well-known complications of avascular necrosis (AVN), posttraumatic arthritis (PTA), and heterotrophic ossification can leave patients with a significant functional loss of their affected hip. The purpose of this study is to evaluate the clinical and radiographic outcomes of femoral head fractures. METHODS: A retrospective review was performed at our institution assessing all patients who presented from 2007 to 2015 with a femoral head fracture associated with a hip dislocation and at least 6 months of clinical and radiographic follow-up. Twenty-two patients met our inclusion criteria. There were 15 males and 7 females with an average age of 36 years (range: 17-55). The average follow-up time was 18 months (range: 6-102). Fractures were classified according to the Pipkin classification. The Thompson and Epstein score was used to determine functional outcomes. RESULTS: There were five, Pipkin I, 3 Pipkin II, 0 Pipkin III, and 14 Pipkin IV, femoral head fractures. Sixteen patients were successfully closed reduced in the emergency department (ED) and six patients required open reduction after failed reduction in the ED. Four patients (18%) were successfully treated with closed reduction alone and 18 patients (82%) required operative intervention. Of those undergoing operative intervention, one patient underwent excision of the femoral head fragment, seven underwent open reduction internal fixation (ORIF) of the femoral head, nine underwent ORIF of the acetabulum, and one underwent ORIF of the femoral head and the acetabulum. Nine patients (41%) had an uneventful postoperative course. Two patients (9%) developed AVN, both requiring total hip arthroplasty (THA). Five patients (23%) developed PTA, two eventually requiring a THA. Two patients (9%) had sciatic nerve palsy. One patient (5%) developed a postoperative infection and four patients (18%) developed heterotrophic ossification (HO), none requiring operative treatment. Two patients (9%) had persistent anterolateral (AL) thigh numbness. Overall functional results were excellent in six patients (27%), good in six (27%), fair in seven (32%), and poor in three patients (14%). Four patients (18%) required a THA. CONCLUSION: Femoral head fractures are a rare injury with well-known complications. Early diagnosis and concentric reduction are the prerequisites for successful treatment. This study adds to the growing literature on femoral head fractures associated with hip dislocations in efforts to define treatment plans and to guide patient expectations. HOW TO CITE THIS ARTICLE: Del Core MA, Gross B, Ahn J, et al. Clinical and Radiographic Outcomes of Femoral Head Fractures Associated with Traumatic Hip Dislocations. Strategies Trauma Limb Reconstr 2019;14(1):6-10.

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Objective: To evaluate the mid-term effectiveness of arthrolysis and hinged external fixation for the treatment of stiff elbow caused by heterotrophic ossification. Methods: Between January 2014 and December 2017, 11 patients with stiff elbow caused by heterotrophic ossification were admitted. There were 9 males and 2 females with an average age of 32 years (range, 14-48 years), and left side in 6 cases and right side in 5 cases. The cause of stiff elbow included humerus fracture in 5 cases, ulna fracture in 2 cases, fracture of capitulum radii in 1 case, dislocation of capitulum radii in 1 case, terrible triad of the elbow in 1 case, and soft tissue injury in 1 case. The disease duration ranged from 7 to 18 months (mean, 11 months). Preoperative active range of motion of elbow was (19.6±17.5)° and Mayo score was 34.1±9.7. All patients received the treatment of arthrolysis and debridement of heterotrophic ossification lesion combining hinged external fixator, and active and passive rehabilitation with the help of hinged external fixator. The hinged external fixators were removed after 2 months. Results: All patients were followed up 13-36 months (mean, 19.1 months). All incisions healed by first intention, and no complication of infection or nerve lesion occurred postoperatively. At last follow-up, the results of X-ray films showed that no heterotrophic ossification recurred. The active range of motion of elbow was (116.4±16.6)° and Mayo score was 93.2±7.8, showing significant differences when compared with preoperative ones ( t=17.508, P=0.000; t=16.618, P=0.000). Conclusion: The application of arthrolysis and debridement of heterotrophic ossification lesion combining hinged external fixator can improve the elbow's range of motion significantly and obtain a good mid-term effectiveness.

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OBJECTIVE The objective of this study was to identify the risk factors of anterior bone loss (ABL) in cervical disc arthroplasty (CDA) and the subsequent effect of this phenomenon. METHODS The authors performed a retrospective radiological review of 185 patients with a minimum 5-year follow-up after CDA (using Bryan, Discocerv, Mobi-C, or Baguera C). Postoperative radiographs were examined and compared to the initial postoperative films to determine the percentage of ABL. The relationship of ABL to potential risk factors was analyzed. RESULTS Complete radiological assessment was available in 145 patients with 193 CDRs and 383 endplates (average age 45 years, range 25-65 years, 54% women). ABL was identified in 63.7% of CDRs (48.7% mild, 11.9% moderate, 3.1% severe). Age (p = 0.770), sex (p = 0.200), postoperative alignment (p = 0.330), midflexion point (p = 0.509), maximal flexion (p = 0.080), and extension (p = 0.717) did not relate to ABL. There was no significant difference in the rate of severe ABL between implants. Multilevel surgery conferred an increased risk of any and severe ABL (p = 0.013 for both). The upper endplate, defined as superior to the CDA, was more commonly involved (p = 0.008), but there was no significant difference whether the endplate was between or not between implants (p = 0.226). The development of ABL did not affect the long-term range of movement (ROM) of the CDA, but did increase the overall risk of autofusion. ABL was not associated with pain or functional deficits. No patients required a reoperation or revision of their implant during the course of this study, and there were no cases of progressive ABL beyond the first year. CONCLUSIONS ABL is common in all implant types assessed, although most is mild. Age, sex, postoperative alignment, ROM, and midflexion point do not relate to this phenomenon. However, the greater the number of levels operated, the higher the risk of developing ABL. The development of ABL has no long-term effect on the mechanical functioning of the disc or necessity for revision surgery, although it may increase the rate of autofusion.

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Heterotrophic ossification (HO) is a reactive disease that is defined as the formation of mature lamellar bone in the soft tissue but it is rarely associated with loss of motion and stiffness. The reported incidence of HO after total knee arthroplasty (TKA) ranges from 1% to 42%. We experienced a case of HO adversely affecting the ambulation of the patient as a result of the severe ankylosis of the knee after TKA. We report this case with a review of the relevant literature.

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