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Congenital + dislocation

Amanda T Whitaker, James Kasser, Young-Jo Kim
RATIONALE: The sciatic nerve runs a predictable course combining L4-S3 nerve roots through the true pelvis and under the greater sciatic notch. There are reports of bony protuberances from the sacrum and ilium in cases of spinal dysraphism; however advanced imaging, treatment, or outcomes are not described. There are no cases with associated fibular hemimelia in the current literature. PATIENT CONCERNS: This is a 4-year-old girl with tethered cord, acetabular dysplasia with hip subluxation, congenital short femur, anterior cruciate ligament (ACL) deficiency, and fibular hemimelia with her sciatic nerve coursing through the ilium...
March 2018: Medicine (Baltimore)
Olga M Pavlova, Sergey O Ryabykh, Alexander V Burcev, Alexander V Gubin
STUDY DESIGN: Case series, level 4 of evidence. OBJECTIVES: To analyze clinical and radiological features of pathological atlantoaxial displacement (PAAD) in pediatric patients. To compose treatment algorithm for anomaly-related PAAD. SUMMARY OF BACKGROUND DATA: Criteria of different types of PAAD and treatment algorithms have been widely reported in the literature, but difficulty applicable in patients with odontoid abnormalities, C2-C3 block, spina bifida C1 and children...
March 13, 2018: World Neurosurgery
Narayanam Anantha Sai Kiran, Veldurti Ananta Kiran Kumar, Laxminadh Sivaraju, Valluri Anil Kumar, Chintakunta Rajesh Reddy, Amit Agrawal
BACKGROUND: Aberrant medial retropharyngeal prevertebral course of internal carotid arteries (ICAs) is extremely uncommon. In oropharyngeal surgeries like trans oral odontoidectomy (TOO) this unrecognized aberrant retropharyngeal course of ICAs can result in devastating complications secondary to inadvertent injury of ICAs. Authors describe this aberrant course of ICAs in a patient with craniovertebral junction (CVJ) anomaly with dysmorphic C1 lateral mass on one side and discuss in detail various management issues in this complex case...
March 12, 2018: World Neurosurgery
Rana Muhammad Kamran Shabbir, Gökhan Nalbant, Nafees Ahmad, Sajid Malik, Aslıhan Tolun
BACKGROUND: Carbohydrate sulfotransferase 11 (CHST11) is a membrane protein of Golgi that catalyses the transfer of sulfate to position 4 of the N-acetylgalactosamine residues of chondroitin. Chondroitin sulfate is the predominant proteoglycan in cartilage, and its sulfation is important in the developing growth plate of cartilage. A homozygous deletion encompassing part of the gene and the embedded miRNA MIR3922 had been detected in a woman with hand/foot malformation and malignant lymphoproliferative disease...
March 7, 2018: Journal of Medical Genetics
Jason Silvestre, Joseph Upton, Benjamin Chang, David R Steinberg
BACKGROUND: Hand surgery fellowship programs in the United States are predominately sponsored by departments or divisions of orthopaedic surgery or plastic surgery. This study compares the operative experiences of hand surgery fellows graduating from orthopaedic or plastic surgery hand surgery fellowships. METHODS: Operative case logs of 3 cohorts of hand surgery fellows graduating during the academic years of 2012-2013, 2013-2014, and 2014-2015 were analyzed. The median case volumes were compared by specialty via Mann-Whitney U tests...
March 7, 2018: Journal of Bone and Joint Surgery. American Volume
Parveen Sen, Vinay Kumar S, Pramod Bhende, Pukhraj Rishi, Ekta Rishi, Chetan Rao, Dhanashree Ratra, Pradeep Susvar, Sreelakshmi Kummamuri, Sufiyan Shaikh, Lingam Gopal
OBJECTIVE: To study the outcome and complications of sutured scleral fixated intraocular lenses (SSFIOL) in children. DESIGN: Retrospective study. SUBJECTS: A total of 279 eyes of 230 children who underwent SSFIOL at ≤18 years of age in a tertiary eye care centre in India. METHODS: Treatment-naive children having traumatic cataract or subluxated lens underwent a single-sitting lensectomy and pars plana vitrectomy (PPV), along with SSFIOL insertion...
February 2018: Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie
Matthew Sloan, Atul F Kamath
Colonna capsular arthroplasty represents an option for the management of chronic hip dislocation in young patients with dysplasia. In the appropriate patient, modern capsular arthroplasty procedures may provide an opportunity for hip preservation in patients with preserved femoral head cartilage and not appropriate for total hip arthroplasty. Here, we review our experience with surgical dislocation of the hip and capsular arthroplasty in a 27-year-old female with congenital hip dysplasia and chronic superior hip dislocation...
January 2018: Journal of Hip Preservation Surgery
Vrushali C Ponde, Vinit Vinod Bedekar, Ashok N Johari, Shalin K Maheshwari
No abstract text is available yet for this article.
January 2018: Indian Journal of Anaesthesia
Nobuyuki Takahashi, Kousuke Iba, Megumi Hanaka, Yasuhiro Ozasa, Kohei Kanaya, Toshihiko Yamashita
PURPOSE: The Elson technique is a rigid reconstruction method for a hypoplastic or absent sagittal band in the treatment of chronic extensor digitorum communis (EDC) tendon dislocation. We performed a modified procedure based on the Elson technique for reconstruction of the radial sagittal band in case involving the index finger. We investigated the postoperative outcomes of chronic EDC dislocation after treatment with the original and modified Elson technique. METHODS: We examined five fingers of five patients (2 males and 3 females) with a mean age of 41 years...
January 2018: Journal of Orthopaedic Surgery
Narjes Jafari, Karl Golnik, Mansoor Shahriari, Parvaneh Karimzadeh, Sayena Jabbehdari
Purpose: We aimed to present the ophthalmic manifestations of neuro-metabolic disorders. Methods: Patients who were diagnosed with neuro-metabolic disorders in the Neurology Department of Mofid Pediatric Hospital in Tehran, Iran, between 2004 and 2014 were included in this study. Disorders were confirmed using clinical findings, neuroimaging, laboratory data, and genomic analyses. All enrolled patients were assessed for ophthalmological abnormalities. Results: A total of 213 patients with 34 different neuro-metabolic disorders were included...
January 2018: Journal of Ophthalmic & Vision Research
Adam S Martin, M Craig McMains, Andrew G Shacklett, Hisham M Awan
PURPOSE: To provide an updated analysis of the hand surgery section of the Orthopaedic In-Training Examination (OITE) from 2009 to 2015. The goal was to contribute to the existing literature on the analysis of OITE questions, to aid both residents and residency programs in preparation for the OITE and board examination. METHODS: The authors analyzed all OITE questions pertaining to hand surgery between 2009 and 2015. Hand questions were analyzed for category and subcategory of content, cited reference, treatment intervention, and imaging modality used...
February 2, 2018: Journal of Hand Surgery
Mahesh Krishna Pillai, Rajeev Kariyattil, Rajinder Kumar, Venkatesh Govindaraju, Koshy Kochummen
The authors report the case of congenital atlantoaxial dislocation in a 9-month-old female infant, who presented with progressive quadriparesis and respiratory failure. The problems in management due to such an early age of presentation, including atypical clinical presentation, unique radiological findings, limited management options, and variable clinical outcomes discussed. This is the youngest case of non-syndromic congenital atlantoaxial dislocation, reported to date, and is a unique combination of lateral, rotatory, and antero-posterior atlantoaxial dislocation...
January 29, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Philip L Wilson, Sheena R Black, Henry B Ellis, David A Podeszwa
BACKGROUND: Genu valgum, a risk factor for recurrent patellofemoral instability, can be addressed with a varus producing distal femoral osteotomy (DFO). The purpose of this study is to report 3-year clinical and radiographic outcomes on a series of skeletally mature adolescents with traumatic patellofemoral instability and genu valgum who underwent a varus producing DFO. METHODS: Consecutive patients (n=11) who underwent an isolated DFO for recurrent traumatic patellar instability over a 4-year study period (2009 to 2012) were reviewed...
January 10, 2018: Journal of Pediatric Orthopedics
P B Karthik, Jayesh Sardhara, Nachiketa Tiwari, Sanjay Behari
The most common type of congenital C1-2 dislocation is a combined type in which atlanto-axial dislocation (AAD) and basilar invagination (BI) are often associated with a rotational dislocation and coronal tilt. An optimal surgical treatment involves reduction of AAD and BI with simultaneous correction of the rotation and coronal tilt to achieve an optimal cervical canal decompression, sagittal and coronal realignment and bony fusion. The most acceptable technique to facilitate this correction is the C1-C2 distraction technique, which is accomplished by the manual joint manipulation...
January 2018: Neurology India
Pravin Salunke, Madhivanan Karthigeyan, Narasimhaiah Sunil, Vasundhara Rangan
OBJECTIVES: Manifestation of congenital anomalies of cranio-vertebral junction (CVJ) in the later half of life is unusual and intriguing. Coexisting cervical spondylotic changes with multilevel compression, poorer bone quality as well as less smooth post-surgical recuperation make management of elderly Congenital Atlantoaxial Dislocation/ Basilar Invagination (CAAD/BI) challenging. The clinico-radiological presentation, pathogenesis and outcome are analysed here. PATIENTS AND METHODS: Clinico-radiological data of 20 patients of CAAD/BI (with markers of congenital anomalies) presenting after 50 years of age, the challenges faced and outcomes after C1-C2 fusion have been analysed...
December 21, 2017: Clinical Neurology and Neurosurgery
Ata Can, Ilker A Sarikaya, Necip S Yontar, Ayse O Erdogan, Baris Gorgun, Fahri Erdogan
BACKGROUND: We argue that 1-stage bilaterally total hip arthroplasty (THA) could be acceptable in bilateral coxarthrosis because of high-riding developmental dysplasia of the hip (DDH). METHODS: Sixty-nine cases (51 patients) of high-riding DDH in patients who underwent THA from 2010 to 2013 were reviewed. Patients were divided into 2 groups: unilateral (group 1) and 1-stage bilateral surgery (group 2). The clinical measurements were the visual analog scale and Harris Hip Score...
December 8, 2017: Journal of Arthroplasty
Schaida Schirwani, Kath Smith, Meena Balasubramanian
Structural rearrangements of chromosome band 1p31p32 are rare, and their phenotypic consequences remain poorly delineated. Up to 12 patients with learning difficulties, developmental delay, multiple congenital anomalies and microdeletion of the chromosome band 1p31p32 have been described. Inheritance of this deletion has not been reported previously. We describe the inheritance of the 1p32 deletion and discuss the relevance of this deletion to the described phenotype. The differences in clinical and molecular characteristics between the proband and other published reports are reviewed...
April 2018: Clinical Dysmorphology
Pravin Salunke
Introduction: The deformed joints seen in congenital atlantoaxial dislocation (CAAD) are often associated with vascular anomalies. It is important to identify these vascular anomalies and address them appropriately without compromising the manipulation and fusion of C1-C2 joints. The small bones in pediatric age group pose an additional challenge. Materials and Methods: Data of fifty-six children with CAAD operated in the last 4 years was analyzed. A computed tomography angiogram was obtained preoperatively to assess for the course of the third segment of vertebral artery (VA)...
July 2017: Journal of Pediatric Neurosciences
C Glorion
Surgical reduction of congenital hip dislocation is technically challenging. In our practice, surgical reduction is usually reserved for patients who have failed non-operative treatment, which is the first-line strategy. However, primary surgery may be indicated if the dislocation is diagnosed late and can be performed until 8 years of age. The reduction step is crucial. It starts with painstaking exposure of the capsule. Identifying the lower part of the acetabulum is the key to accurate repositioning of the epiphysis...
February 2018: Orthopaedics & Traumatology, Surgery & Research: OTSR
Don K Nakayama
Sir Denis John Wolko Browne (1892-1967), while not the first in the British Isles to devote his entire surgical practice to pediatric surgery, is accepted as "the father of pediatric surgery in the United Kingdom." He made contributions to operations as varied as tonsillectomy, pyloromyotomy, and hypospadias repair, and provided fundamental insights into the proper treatment of club foot, congenital dislocation of the hip, and cryptorchidism. He introduced the transverse laparotomy incision, primary repair of congenital intestinal obstruction, and the end-to-back anastomosis for intestinal atresia, techniques so commonly used that it is difficult to think of pediatric surgical operations done any differently...
October 26, 2017: Journal of Pediatric Surgery
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