Abstract: Adamantinoma is a rare, low-grade malignant tumor of the bone which grows slowly and typically occurs in the diaphysis of long bones, particularly in the tibia. Adamantinomas have the potential for local recurrence and may metastasize to the lungs, lymph nodes, or bone. We report a case of a 14-year-old female with a tibial adamantinoma who underwent wide resection with limb salvage and has subsequently been followed up for 18 years. The patient went on to have both a local soft tissue recurrence 5 years after the resection and metastases to both an inguinal lymph node and the right lower lobe of the lung 8 years after that recurrence, all of which have been treated successfully with marginal resections. Unique to this case, the patient was also incidentally found to have chromophobe-type renal cell carcinoma when undergoing a partial nephrectomy to resect a presumed metastasis of her adamantinoma. Genetic testing has not revealed any known genetic predisposition to cancer. PubDate: Sun, 25 Mar 2018 00:00:00 +000
Abstract: Introduction. Patients with spondylocostal dysostosis (SCD) have congenital spine and rib deformities associated with frequently severe thoracic insufficiency and respiratory compromise. The literature is largely composed of case reports and small cohorts, and there is little information regarding adults with this condition. In this report, we describe the natural history of a conservatively treated patient and include quality-of-life issues such as childbearing, athletic participation, and occupational selection. Case Presentation. We present a patient with SCD who was conservatively treated by a single physician from birth for 31 years. Our patient was capable of a reasonably good quality of life through adulthood, including participation in gymnastics and employment. At age 18, she became pregnant and subsequently terminated the pregnancy due to obstetrical concerns for compromised respiration. She has had intermittent respiratory complaints and occasionally experiences dyspnea with exertion, but this only has limited her during certain activities in the past three years. Currently, she takes naproxen for chronic back pain with periodic exacerbations. Discussion. Other cases in the literature have described adult SCD patients who have received nonoperative treatment and achieved a wide range of functional outcomes. This provides some limited evidence to suggest that select patients with SCD may be treated conservatively and achieve a reasonable quality of life. However, close clinical follow-up with these patients is recommended, particularly early on, considering the high rates of infant morbidity and mortality. Chest physiotherapy and early pulmonary care have been associated with favorable outcomes in infancy. Surgery to increase thoracic volume and correct scoliosis has been shown in some cases to improve respiratory function. Treatment depends on the degree of thoracic insufficiency and quality of life. The natural history of SCD remains largely unknown, but some patients are capable of relatively favorable life spans, employment, and participation in athletics. PubDate: Thu, 22 Mar 2018 00:00:00 +000
Abstract: Introduction. The enterobacterial genus Yersinia includes a number of human pathogens. Large-diameter, metal-on-metal prostheses are no longer used because of their high failure rate. Here, we describe the first case of Yersinia enterocolitica infection of a metal-on-metal total hip arthroplasty. Clinical Examination. A metal-on-metal prosthesis failed ten years after implantation. After surgical revision, bacteriological testing revealed the presence of a pathogenic strain of Yersinia enterocolitica. Combination antibiotic therapy resulted in a favorable clinical outcome. Discussion. Three cases of hip arthroplasty infected with Yersinia enterocolitica have been described in the literature. The present case is the first infection of a metal-on-metal total hip arthroplasty. We suggest that the risk of infection is increased by the release of metal wear particles and their influence on the surrounding tissue. Conclusion. When a large-diameter, metal-on-metal total hip arthroplasty fails, the known complications associated with this type of prosthesis should not deter the physician from screening for an infectious process that requires specific treatment. PubDate: Thu, 15 Mar 2018 00:00:00 +000
Abstract: Thoracic outlet syndrome (TOS) is caused by heterogeneous factors that compress the brachial plexus and subclavian artery; tumor is rarely a cause of TOS. Here, we present the case of a 26-year-old man with secondary chondrosarcoma arising from osteochondroma of the left clavicle causing TOS, with a direct compression of the brachial plexus and subclavian artery. Immediately after surgery, the symptoms of TOS reduced. To our knowledge, this is the first case of a secondary chondrosarcoma of the clavicle causing TOS, which is possibly the key symptom for diagnosing malignant transformation of osteochondroma of the clavicle. PubDate: Mon, 12 Mar 2018 09:02:55 +000
Abstract: Objective. The present study was aimed at evaluating the use of mechanical and manual lymphatic therapy as a treatment for lymphedema resulting from orthopedic surgery that became painful after an episode of erysipelas. Case Report. A 70-year-old male patient suffered direct trauma resulting in a compound fracture of the tibia and fibula of the left leg. He was treated with an external fixator for four months followed by plaster cast immobilization for three weeks. He presented with fever and paresthesia in the lower left limb that resulted in an episode of erysipelas, and the patient evolved with painful lymphedema. Treatment using the Godoy and Godoy technique was proposed, including manual and mechanical lymphatic therapy. Water displacement volumetry was used to quantify the leg size reduction. Results. After 10 sessions of therapy, the patient presented a significant reduction in the limb volume and remission of symptoms. Conclusions. The method used may be a promising option for the treatment of posttraumatic edemas with pain. PubDate: Mon, 05 Mar 2018 00:00:00 +000
Abstract: This study reports on a 67-year-old woman with partial Brown-Séquard syndrome due to a recurrent cervical neurenteric cyst at C3 to C4. The myelopathic symptoms reappeared 22 years after a previous shunting operation performed posteriorly with a silicone tube connecting the intradural cervical cyst cavity to the subarachnoid space. We have now succeeded in removing the cyst nearly completely with the anterior approach. The surgical procedure consisted of right vertebral artery exposure at C3 and C4 and a subtotal corpectomy of C3 followed by microdissection of the cyst, duraplasty, and iliac strut graft fusion. Spinal cord monitoring with motor-evoked potential studies helped us safely dissect the cyst wall tightly adhering to the spinal cord. Duraplasty with Gore-Tex patch-grafting in conjunction with postoperative lumbar subarachnoid drainage worked well in preventing a spinal fluid fistula. At two years after surgery, the patient showed a nearly complete return of function without any recurrence of the cyst. PubDate: Sun, 04 Mar 2018 00:00:00 +000
Abstract: Introduction. Hemidiaphragm paralysis secondary to phrenic nerve palsy is a well-recognised medical condition. There are few case reports in the literature documenting resolution of hemidiaphragm paralysis following cervical spine surgery. This case report documents our experience with one such case. Case Presentation. A 64-year-old man was referred to the orthopaedic service with right hemidiaphragm paralysis. He had a previous history of asbestos exposure and polio and was initially seen and investigated by the respiratory physicians. He also reported intermittent neck pain and an MRI scan showed right-sided cervical foraminal stenosis. He underwent posterior right C3/4 and C4/5 foraminotomies, and by three months postoperatively, his hemidiaphragm paralysis had resolved and his shortness of breath had also improved. Conclusion. This report documents a unique case of resolution of hemidiaphragm paralysis following posterior unilateral cervical foraminotomies. PubDate: Sun, 04 Mar 2018 00:00:00 +000
Abstract: We describe a case of lumbar stenosis in which retrospective spectral analysis using dual-layer spectral detector computed tomography (CT) had the ability to expand the evaluable region in the spinal canal. Spinal canal stenosis is a common condition whose symptoms (such as lower back and leg pain with walking) deteriorate the quality of life. Generally, magnetic resonance imaging (MRI) and CT myelography are performed to diagnose canal stenosis. Dual-layer spectral detector CT can yield virtual monochromatic imaging and retrospective on-demand spectral analysis without a prescan setting. Spectral analysis could expand the evaluable region in the spinal canal for increasing the contrast enhancement in the canal. PubDate: Sun, 04 Mar 2018 00:00:00 +000
Abstract: Extensor tendon ruptures due to volar plating in distal radius fractures have mostly been described in relation with technique failures such as screw prominence and drill penetration. We report the case of a 71-year-old female with a C2 distal radius fracture with severe dorsal metaphyseal comminution. The patient underwent surgical treatment with reduction of the large fragments and fixation with a volar locking plate; the small dorsal metaphyseal nonarticular fragments were not reduced. Six months later, the patient developed extensor digitorum communis (EDC) rupture and extensor indicis proprius (EIP) laceration in coincidence with the dorsal comminution turned into a bony spur. The possible association between the extensor tendon injury and the dorsal residual metaphyseal bony spur in the distal radius fractures is unusual but should be taken into account in fracture patterns presenting dorsal comminution. PubDate: Wed, 28 Feb 2018 00:00:00 +000
Abstract: Introduction. The purpose of this report was to describe a very rare case of simultaneous bilateral quadriceps tendon rupture seen in a patient who was diagnosed as having diffuse idiopathic skeletal hyperostosis. Case Presentation. A man aged 64 years presented to the emergency department with bilateral quadriceps tendon rupture. Surgical repair was performed with suture anchors and a stainless steel cable. His legs were immobilized in casts for six weeks. After removal of the casts, physiotherapy was started. Four months after surgery, he was able to walk with 0°–120° range of motion and active extension. He was followed up for 8 years without rerupture or other complications. Conclusion. Bilateral rupture of the quadriceps tendon is a rare condition and generally related to metabolic disorders. Diffuse idiopathic skeletal hyperostosis is a metabolic disorder that causes bilateral quadriceps tendon rupture, and it accounted for the differential diagnosis of the underlying condition. PubDate: Thu, 22 Feb 2018 00:00:00 +000
Abstract: Minimally invasive procedure for the treatment of pectus excavatum as described by Nuss has been used from 1987. The bar initially introduced blindly is now introduced under thoracoscopic control to increase safety of the procedure. It is usually removed two to three years after its insertion in a one-day procedure. Complications of the bar removal are rare but potentially serious. We report the case of a serious complication which occurred immediately after the Nuss bar removal. A 15-year-old boy underwent a Nuss procedure for a severe pectus excavatum without relevant complication. The bar has been removed two years after its insertion in a minimally invasive procedure. Unfortunately, he developed in the immediate postoperative period a hemopneumothorax due to a right middle lobe laceration which required a middle lobectomy by thoracotomy for hemostasis. Lesions of intrathoracic organs are a rare but potentially serious complication of the removal of the Nuss bar. We now propose to perform this procedure under thoracoscopic control to avoid it. In our experience, adhesions between the bar and the pleura are always present, and those with potential risk for bleeding or inducing intrathoracic organ lesions are suppressed prior to the bar removal. PubDate: Thu, 22 Feb 2018 00:00:00 +000
Abstract: Isolated trapezium fracture in combination with thumb carpometacarpal (CMC) joint dislocation is extremely rare, and no treatment consensus exists. Herein, we report 3 successfully treated cases of isolated trapezium fracture with thumb CMC joint dislocation. While good short-term results have been reported in the literature, the possibility of substantial ligament injuries that can lead to future instability of the thumb CMC joint must be noted. In order to obtain an excellent long-term clinical result, we propose the consideration of the anatomical repair of the CMC joint in terms of both bony and ligamentous structures in cases where instability remains after fracture fixation. PubDate: Wed, 21 Feb 2018 00:00:00 +000
Abstract: We report a novel case of a patient who had a draining sinus soon after a total hip arthroplasty that spontaneously resolved. The patient voluntarily discontinued antibiotic suppressive therapy (AST) after 10 years of treatment and paradoxically experienced full resolution of signs of chronic prosthetic joint infection (PJI), including recovery of his left-sided draining sinus tract. Now 8 years after discontinuing AST, the patient has no pain, good function, and no major or minor criteria of joint infection according to the Musculoskeletal Infection Society (MSIS) workgroup. The authors have not identified literature describing a similar resolution of draining sinus tracts from around a prosthetic joint after discontinuing AST. Despite the resolution of this patient’s sinus tract, the authors do not advocate for discontinuing AST in patients with a draining sinus tract. However, in spite of the fact that the MSIS consensus statement suggests that a draining sinus is a sure sign of PJI and that the assumption is that the infection will not go away until explant, this case was different. PubDate: Mon, 19 Feb 2018 06:13:45 +000
Abstract: Posttraumatic proximal radioulnar synostosis (PPRUS) is a severe complication of radial head and neck fractures known to occur after severe injury or operative fixation. Cases of PPRUS occurring after minimally displaced, nonoperatively treated radial neck injuries are, by contrast, extremely rare. Here, we present a pediatric case of PPRUS that developed after a nonoperatively treated minimally displaced radial neck fracture with concomitant olecranon fracture. While more cases are needed to establish the association between this pattern of injury and PPRUS, we recommend that when encountering patients with a minimally displaced radial neck fracture and a concomitant elbow injury, the rare possibility of developing proximal radioulnar synostosis should be considered. PubDate: Mon, 19 Feb 2018 00:00:00 +000
Abstract: Case. We present the case of a 21-year-old man who fell from a roof, sustaining a displaced sacral fracture with pelvic instability. He developed acute respiratory distress syndrome (ARDS) within 24 hours of injury. Placement of the pelvic C-clamp resulted in rapid resolution of pulmonary dysfunction, allowing for definitive internal fixation. Conclusion. The C-clamp is most commonly used to control hemorrhage in unstable posterior pelvic ring injuries. Our case demonstrates a rare use of the C-clamp to stabilize the posterior pelvis in a patient with an unstable sacral fracture and ARDS, to rapidly improve pulmonary function prior to definitive surgery. PubDate: Sun, 18 Feb 2018 00:00:00 +000
Abstract: Symptomatic ossification of the anterior longitudinal ligament (OALL) is rare. However, when the osteophyte enlarges and obstructive symptoms occur, the patient may require surgery. We present a case of pyogenic spondylitis caused by methicillin-resistant Staphylococcus aureus associated with tracheostomy followed by resection of OALL. A 69-year-old woman with OALL complained of dysphagia and suffocation, which was caused by prominent OALL at C4-5. Tracheostomy was performed, followed by osteophytectomy 6 weeks later. Two months after osteophytectomy, she complained of muscle weakness of the extremities, neck pain, and elevated temperature. Magnetic resonance imaging showed an intensity change at the C4-5 vertebrae and an epidural abscess that was causing cord compression requiring urgent decompression. Cultures identified methicillin-resistant Staphylococcus aureus. As osteolytic change and muscle weakness gradually progressed, she underwent anterior and posterior reconstruction with an autograft and instrumentation. Bone union was confirmed at 1 year postoperatively with improvement in neurological status. OALL has potentially the risk of airway obstruction. Therefore, appropriate diagnosis and prompt osteophytectomy are needed in cases of a large prominent ossification that puts the patient at risk of suffocation. However, it is noted that osteophytectomy following urgent tracheostomy carries the possible risk of infection. PubDate: Sun, 18 Feb 2018 00:00:00 +000
Abstract: Purpose. We evaluated improvements in gait after using the Hybrid Assistive Limb (HAL®) exoskeleton robot in a patient with late-onset neurological deterioration of lower extremity function after undergoing thoracic spine surgery for a myelopathy due to ossification of the ligamentum flavum. Case Presentation. A 70-year-old man participated in ten 20 min sessions of HAL intervention, twice weekly for five weeks. The effects of each HAL session were evaluated based on changes in performance on the 10 m walk test (10 MWT), lower limb kinematics quantified from motion capture, and the activation ratio of the gastrocnemius, measured before and after the intervention. Muscle activity was recorded using surface electromyography and synchronized to measured kinematics. The HAL intervention improved gait speed and step length, with an increase in the hip flexion angle during the swing phase and a decrease in the activation ratio of the gastrocnemius. The modified Ashworth scale improved from 1+ to 1 and International Standards for Neurological and Functional Classification of Spinal Cord Injury motor scores from 34 to 49. Conclusion. Intervention using the HAL exoskeleton robot may be an effective method to improve functional ambulation in patients with chronic spinal disorders. PubDate: Thu, 08 Feb 2018 00:00:00 +000
Abstract: Osteochondroma (OC) is the most common benign bone tumor and may occur on any bone in which endochondral ossification develops. Although scapular OC accounts for less than 5% of the cases of solitary OC, OC is the most common lesion among the tumors and tumor-like lesions of the scapula. OC that develops near the medial scapular border easily causes friction with the ribcage; hence, almost half the number of cases of OC associated with marked bursa formation develops in the ventral scapula. We report a case of a 27-year-old female with a painful OC of the ventral scapular surface associated with large bursa formation and pseudowinging of the scapula. After l2 years of follow-up with magnetic resonance imaging, we confirm that the accompanied bursa left at surgery disappears. PubDate: Mon, 05 Feb 2018 00:00:00 +000
Abstract: Osteochondroma is the most common benign bone tumor and is characterized as a cartilage-capped bony stalk. This lesion usually develops from the growth plate of long bones. Most osteochondromas are asymptomatic. Neurovascular compressions or cosmetic issues can occur in specific locations. Malignant transformation is extremely rare, and MRI can help evaluate these lesions. Symptomatic mass and malignancy features are the main surgical indications. Uncommonly, an osteochondroma can develop from flat bones. We present the case of a 25-year-old patient with a right scapula osteochondroma causing an accessory nerve compression. The mass was surgically removed, and the diagnosis was confirmed. The patient fully recovered at the latest 3-year follow-up visit. PubDate: Wed, 31 Jan 2018 14:40:55 +000
Abstract: Percutaneous endoscopic discectomy (PED) for lumbar disc herniation is gaining popularity with the transforaminal (TF) approach preferred because it allows surgery under local anesthesia and preserves the spinal muscles. Although this procedure has some characteristic complications, it is rare for PED to be converted to conventional open surgery due to worsening of symptoms intraoperatively. Here, we report PED via the TF approach that required conversion to open surgery. A 20-year-old man with a large disc herniation at L3/4 developed severe progressive leg pain and muscle weakness of the left leg intraoperatively. Magnetic resonance imaging revealed that the size of the herniation was unchanged and the endoscope did not reach the herniated mass. We converted to open surgery, and the patient’s postoperative course was favorable. We discuss the reasons for failure of the approach and suggest planning for an appropriate foraminoplasty to avoid the potential need for conversion to open surgery. PubDate: Wed, 31 Jan 2018 00:00:00 +000
Abstract: Medial epicondyle entrapment after an acute fracture dislocation of the elbow is a common finding in the pediatric population, but a rare finding in adults. We present a case of an adult patient diagnosed with a traumatic fracture dislocation of the elbow joint with intra-articular entrapment of the medial epicondyle. After initial evaluation, closed reduction was done. Stability testing after reduction showed an unstable joint; thus, open reduction and internal fixation was decided. PubDate: Wed, 31 Jan 2018 00:00:00 +000
Abstract: Traditionally, Charcot arthropathy has been considered an absolute contraindication for total hip arthroplasty (THA). However, some recent reports have shown that good short- to mid-term results can be achieved by improving the durability of the implant. This paper reports the mid- to long-term results of THA in two patients with Charcot hip joints caused by congenital insensivity to pain with anhydrosis. Both patients suffered multiple posterior dislocations in the six months immediately following surgery. However, with the continuous use of a hard abduction brace, one patient was eventually able to walk with a lofstrand cane and the other with the use of one crutch. Although one patient experienced a dislocation five years after surgery, X-rays taken after nine years and five years, respectively, revealed no clinical signs of implant loosening. We conclude that, with careful planning and appropriate precautions, THA may be a viable treatment option for Charcot hip joints caused by congenital insensivity to pain with anhydrosis. PubDate: Wed, 31 Jan 2018 00:00:00 +000
Abstract: We present a rare case of a femoral shaft fracture in a 74-year-old woman with a preexisting untreated bilateral congenital hip dislocation and with concomitant leg length discrepancy. Because of the marked deformity of the upper femur, a derivative of the congenital hip disease, an open reduction and internal fixation was performed, with the use of an anatomic femur plate originally designed for the treatment of periprosthetic fractures. The patient was treated successfully and returned to her previous status of activity. The management of a femoral fracture in the presence of ipsilateral hip disease is a challenging issue. The surgical treatment choice in such cases has to be individualized, taking into consideration several anatomic and medical parameters. PubDate: Wed, 31 Jan 2018 00:00:00 +000
Abstract: Introduction. High tibial osteotomy (HTO) is a frequent and effective treatment for unicompartmental gonarthritis. Only a few articles are focused on the treatment of infected nonunion. Patient and Method. A 50-year-old obese patient was operated on by medial opening-wedge HTO. She developed a painful nonunion treated by hardware removal, allograft, and plate fixation. However, the nonunion persisted. 2 years later, cellulitis appeared with an abscess adjacent to the HTO plate. Despite surgical debridement and antibiotics, septic knee arthritis occurred. In a situation of infected nonunion and septic arthritis with chondrolysis, she was scheduled for a 2-stage total knee replacement (TKR). The infected tibial articular block was first resected and replaced by a cement spacer. After a short interval, the TKR was implanted. After 2 years, the patient walked pain-free with good knee function. Discussion. In the literature, different efficient treatments exist for infected nonunion after HTO, but comprehensive studies are missing for a consensus treatment. Current data are mostly based on case reports, since this pathology is quite rare. Conclusion. In a difficult situation of infected nonunion with septic knee arthritis, we performed a 2-stage knee prosthesis implantation. This led to an early mobilization and fast recovery. PubDate: Wed, 31 Jan 2018 00:00:00 +000
Abstract: We report the case of a 30-year-old Polynesian male with a severe gout flare of multiple joints and simultaneous acute compartment syndrome (ACS) of his right forearm and hand without trauma or other typical causes. He had a long history of gout flares, but none were known to be associated with compartment syndrome. He also had concurrent infections in his right elbow joint and olecranon bursa. A few days prior to this episode of ACS, high pain and swelling occurred in his right upper extremity after a minimal workout with light weights. A similar episode occurred seven months prior and was attributed to a gout flare. Unlike past flares that resolved with colchicine and/or anti-inflammatory medications, his current upper extremity pain/swelling worsened and became severe. Hand and forearm fasciotomies were performed. Workup included general medicine, rheumatology and infectious disease consultations, myriad blood tests, and imaging studies including Doppler ultrasound and CT angiography. Additional clinical history suggested that he had previously unrecognized recurrent exertional compartment syndrome that led to the episode of ACS reported here. Chronic exertional compartment syndrome (CECS) presents a difficult diagnosis when presented with multiple symptoms concurrently. This case provides an example of one such diagnosis. PubDate: Tue, 23 Jan 2018 00:00:00 +000
Abstract: We present the case of a 28-year-old woman with oto-spondylo-megaepiphyseal dysplasia, which is a rare skeletal disorder, who underwent bilateral total hip arthroplasty. Full-scale three-dimensional plaster model of the acetabulum and the femur provided us with a feasible preoperative plan. Pre- and postoperative three-dimensional motion analyses proved a significant improvement in her ambulation and confirmed the efficacy of total hip arthroplasty. In conclusion, full-scale three-dimensional plaster models of the bone and three-dimensional motion analyses were useful for total hip arthroplasty in patients with skeletal dysplasia. PubDate: Tue, 23 Jan 2018 00:00:00 +000
Abstract: Diabetic neuroarthropathy of the knee or Charcot knee (CK) is a lesser-known complication of diabetes mellitus, with a limited number of case reports and small case series published in the literature. The majority of these reports describe the complexities and challenges that arise in these patients undergoing knee arthroplasty procedures. We present two cases of CK, including a rare case of concurrent bilateral disease, and also a review of the current literature. PubDate: Tue, 23 Jan 2018 00:00:00 +000
Abstract: Pelvic Ewing sarcoma is associated with a worse prognosis. Thromboembolic events are relatively common in pediatric patients with cancers including sarcomas. We have presented a case of Ewing sarcoma arising from the left iliac bone with tumor thrombus of inferior vena cava (IVC) which was obtained complete response by both chemotherapy and irradiation. Magnetic resonance imaging (MRI) scan demonstrated that the tumor arising from the left iliac bone extended into the left side of sacral bone, suggesting the difficulty of surgical resection. Computed tomography (CT) revealed the existence of the tumor thrombus of IVC. We performed irradiation (31.2 Gy) and chemotherapy (combination of VCR, Act-D, IFM, and ADR). The tumor was controlled successfully, and the tumor thrombus of IVC has completely vanished. Four years after the treatment, coin lesion in the left upper lung appeared. Suspected of metastasis, segmental resection of the left upper lung was performed. Fourteen years after the surgery, the patient has been remained free of recurrence. It is clinically significant for surgeons to treat pelvic Ewing sarcoma with tumor thrombus. PubDate: Sun, 21 Jan 2018 00:00:00 +000
Abstract: We report a patient who developed persistent knee pain with mechanical symptoms after an uncomplicated patellofemoral arthroplasty. The etiology of his knee pain remained inconclusive following magnetic resonance imaging due to metallic artifact image distortion. With the use of an in-office needle arthroscopy, an immediate and definitive diagnosis was obtained, preventing an unnecessary surgery for a diagnostic arthroscopy. We discovered a lateral meniscus tear, an anterior cruciate ligament tear, and a medial femoral condyle chondral defect for which the patient underwent arthroscopic partial meniscectomy, ligament reconstruction, and osteochondral allograft transplantation, with resolution of his knee pain. PubDate: Sun, 21 Jan 2018 00:00:00 +000
Abstract: Introduction. Klippel–Feil syndrome (KFS) is a congenital anomaly resulting from fusion of cervical vertebral bodies secondary to the dysregulation of signaling pathways during somite development. It is commonly associated with scoliosis and Sprengel deformity. We present a case of KFS with commonly associated abnormalities as well as deformities that have not yet been reported in the literature. Case Presentation. A 3-year-old girl presented for further evaluation of a left upper extremity deformity following a negative genetic workup. Upon physical exam and radiographic imaging, the patient was diagnosed with KFS and associated abnormalities including cervical scoliosis, Sprengel deformity, and congenital deformity of the left upper extremity. Deformities of the left upper extremity include radioulnar synostosis, a four-rayed hand, and absent thenar musculature. The Sprengel deformity was corrected surgically with a Woodward procedure. Discussion. Congenital musculoskeletal deformities can be differentiated based upon spinal and limb embryology. The presence of extraspinal abnormalities not originating from somite differentiation may suggest a severe form of KFS. Important considerations in the workup of the KFS patient include looking for deformities of the shoulder girdle and upper extremities to identify abnormalities for intervention at a young age. PubDate: Thu, 18 Jan 2018 00:00:00 +000
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