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Congenital deformity

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https://www.readbyqxmd.com/read/28343240/childhood-angular-kyphosis-a-plea-for-involvement-of-the-pediatric-neurosurgeon
#1
E Cornips, S Koudijs, J Vles, L van Rhijn
INTRODUCTION: Childhood angular kyphosis is rare, as most children are affected by a mixed kyphotic and scoliotic deformity. Published series involving a mix of kyphosis and kyphoscoliosis, pediatric and adult, congenital and acquired cases are almost exclusively authored by orthopedic surgeons, suggesting that (pediatric) neurosurgeons are not involved. CASE SERIES: We present five cases that illustrate the spectrum of angular kyphosis, and these were treated by a multidisciplinary team including child neurologist, orthopedic surgeon, and pediatric neurosurgeon as complementary partners...
March 25, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28342821/correction-of-axial-deformity-during-lengthening-in-fibular-hypoplasia-hexapodal-versus-monorail-external-fixation
#2
Antoine Chalopin, Loïc Geffroy, Sébastien Pesenti, Antoine Hamel, Franck Launay
INTRODUCTION: Childhood fibular hypoplasia is a rare pathology which may or may not involve limb-length discrepancy and axial deformity in one or more dimensions. The objective of the present study was to compare the quality of the axial correction achieved in lengthening procedures by hexapodal versus monorail external fixators. The hypothesis was that the hexapodal fixator provides more precise correction. MATERIAL AND METHODS: A retrospective multicenter study included 52 children with fibular hypoplasia...
March 22, 2017: Orthopaedics & Traumatology, Surgery & Research: OTSR
https://www.readbyqxmd.com/read/28330575/clinical-management-of-the-homozygous-%C3%AE-thalassemia-with-unusual-mandibular-manifestation-of-hematopoiesis
#3
J A Ruiz-Roca, R E Oñate-Sánchez, I Urrutia-Rodríguez, A Martínez-Izquierdo, D Mengual-Pujante, F J Rodríguez-Lozano
Alpha (α)-thalassemias are the most common genetic disorder of hemoglobin (Hb) synthesis, affecting up to 5% of the world's population. These congenital hemolytic anemias induce extramedullary hematopoiesis, including the liver, spleen, sinuses, and the diploic spaces of the skull. Oral health problems in patients with thalassemias are mostly related to a varied degree of facial deformities, malocclusions, and/or dental arch dimensions. We present a case with a 49-year-old man, diagnosed with homozygous α thalassemia that came to the Faculty of Dentistry at the University of Murcia for a dental treatment...
February 2017: J Stomatol Oral Maxillofac Surg
https://www.readbyqxmd.com/read/28326341/cdc42-is-crucial-for-facial-and-palatal-formation-during-craniofacial-development
#4
Mutsuko Oshima-Nakayama, Atsushi Yamada, Tamaki Kurosawa, Ryo Aizawa, Dai Suzuki, Yoshiro Saito, Hidetoshi Kassai, Yuki Sato, Matsuo Yamamoto, Tatsuo Shirota, Atsu Aiba, Koutaro Maki, Ryutaro Kamijo
Craniofacial deformities with multifactorial etiologies, such as cleft palate and facial dysmorphism, represent some of the most frequent congenital birth defects seen in humans. Their pathogeneses are often related to cranial neural crest (CNC) cells. During CNC cell migration, changes in cell shape and formation, as well as maintenance of subcellular structures, such as filopodia and lamellipodia, are dependent on the complex functions of Rho family small GTPases, which are regulators of actin cytoskeletal organization...
December 2016: Bone Reports
https://www.readbyqxmd.com/read/28325699/spinal-correction-in-patients-with-fukuyama-congenital-muscular-dystrophy
#5
Wataru Saito, Takanori Namba, Gen Inoue, Takayuki Imura, Masayuki Miyagi, Toshiyuki Nakazawa, Eiki Shirasawa, Kentaro Uchida, Masashi Takaso
BACKGROUND: Fukuyama congenital muscular dystrophy (FCMD) is one of the most common congenital progressive muscular dystrophies in Japan. Some patients develop a severe spinal deformity that leads to an unstable sitting position or pain. Since 2008, we have treated FCMD using posterior spinal fusion. This study reports the short-term clinical and radiographic results of posterior spinal correction and fusion in FCMD. METHODS: We retrospectively reviewed 11 consecutive FCMD patients, average age 13 years old, treated with posterior spinal instrumentation and fusion between 2008 and 2015...
March 18, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28325564/the-effect-of-age-at-surgery-and-compensatory-articulation-on-speech-outcome-in-submucous-cleft-palate-patients-treated-with-double-opposing-z-plasty-a-10-year-experience
#6
Rong-Min Baek, Baek-Kyu Kim, Jae Hoon Jeong, Taeseon Ahn, Mikyong Park, Jihyeon Han
BACKGROUND: Submucous cleft palate is a congenital deformity that may present as velopharyngeal insufficiency. The degree of anatomical abnormality varies widely among patients and does not predict severity of symptom. We present our 10-year experience treating submucous cleft palate patients with double opposing z-plasty and explore the effect of age at surgery and compensatory articulation on speech. METHODS: Preoperative assessment included intraoral examination, nasoendoscopy, and perceptual speech evaluation...
March 7, 2017: Journal of Plastic, Reconstructive & Aesthetic Surgery: JPRAS
https://www.readbyqxmd.com/read/28318781/spinal-fusion-in-a-patient-with-fukuyama-congenital-muscular-dystrophy
#7
Kaori Hino, Mitsumasa Fukuda, Tadao Morino, Tadanori Ogata, Masanori Ito, Eiichi Ishii
Many studies have evaluated surgical treatments for spinal deformities in patients with neuromuscular disease. However, few reports have described patients with Fukuyama congenital muscular dystrophy (FCMD). A 13-year-old boy with FCMD was unable to sit for long periods or sleep in the supine position because of progressive scoliosis. His Cobb angle worsened from 27° to 41° in 5months. He underwent standard posterior spinal fusion and pedicle-screw-alone fixation from T5 to S1. Postoperatively, his Cobb angle improved from 41° to 25° without exacerbation for 2years...
March 16, 2017: Brain & Development
https://www.readbyqxmd.com/read/28302228/anaesthetic-management-in-a-child-with-goldenhar-syndrome
#8
Waqas Ahmed Khan, Bushra Salim, Ausaf Ahmed Khan, Shakaib Chughtai
Goldenhar syndrome is a congenital disorder involving deformities of the face. It usually affects one side of the face only and poses significant challenges in the airway management. We herein, report an 8-year boy, known case of Goldenhar syndrome, who presented to our radiology suite for a magnetic resonance imaging (MRI) brain, followed by a computed tomography (CT) scan brain. The boy had various features of Goldenhar syndrome, e.g. cleft palate, absent right eye and ear, right mandibular hypoplasia, micrognathia, and preauricular tags...
March 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28302104/spinal-epidural-lipomatosis-an-easily-ignored-secondary-intraspinal-disorder-in-spinal-kyphotic-deformities
#9
Zhen Zhang, Zhen Liu, Zezhang Zhu, Yong Qiu
BACKGROUND: A previous study reported a high prevalence of spinal epidural lipomatosis (SEL) in patients with Scheuermann kyphosis (SK) and suggested that it may play a role in the pathogenesis of this disease. According to our observation, however, SEL occurs in other spinal kyphotic deformities as well. The aim of this study was to test the hypothesis that SEL commonly occurs in patients with different types of kyphotic deformities as a secondary intraspinal disorder. METHODS: MR images of 16 patients with congenital kyphosis (CK), 40 patients with SK, 13 patients with tuberculotic kyphosis (TK), and 69 age- and sex-matched controls were retrospectively evaluated...
March 16, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28299270/6-0-nylon-versus-6-0-vicryl-rapide-in-chieloplasty
#10
J K Dayashankara Rao, Payal Luthra, Varun Arya, Vijay Siwach, Anil K Sheorain, Megha Gupta
CONTEXT: Facial cosmetic result is one of the most concerning issues for the parents who get their children operated for cleft lip. Moreover, the discomfort associated with the suture removal encourages one to use any new technology that may replace the need for suture placement. The type of suture material used in surgery has been a long-standing debate among surgeons. In this study, we compared rapidly absorbable suture material (Vicryl Rapide™) with nonabsorbable suture material (nylon)...
July 2016: Annals of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28291424/giant-melanotic-neuroectodermal-tumor-of-infancy-melanotic-progonoma-of-the-head-and-neck-report-of-a-malignant-case
#11
Giancarlo Nicosia, Pietro Spennato, Ferdinando Aliberti, Daniele Cascone, Lucia Quaglietta, Maria Elena Errico, Mario Muto, Franco Ionna, Giuseppe Cinalli
Melanotic neuroectodermal tumor of infancy is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, growing rapidly and developing during the 1st year of life. It most commonly arises from the maxilla, cranial vault, and mandible. Occasionally, it exhibits malignant behavior with local lymph nodes involvement. Cases misdiagnosed and left untreated for a long time can present challenges due to the tumor mass and infiltration. In these cases, adjuvant chemotherapy can be extremely helpful before radical excision...
February 24, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28281004/selective-hemivertebrae-resection-in-a-congenital-scoliosis-patient-with-multiple-hemivertebrae-deformities
#12
Yangpu Zhang, Jincai Yang, Lijin Zhou, Aixing Pan, Yong Hai
PURPOSE: To report the surgical experience of selective hemivertebrae resection for a case of congenital scoliosis with multiple hemivertebrae deformities. METHODS: A 14-year-old male presented with progressive rib hump, tilted torso and spine deformity was admitted in our department. No abnormalities were detected in neurological examination and the comprehensive imaging study demonstrated congenital scoliosis of multiple hemivertebrae in T5, T10, L1 and L3. Treatment of the patient commenced with a 10-day skin traction therapy prior to the surgery...
March 9, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28281002/expert-s-comment-concerning-grand-rounds-case-entitled-selective-hemivertebrae-resection-in-a-congenital-scoliosis-patient-with-multiple-hemivertebrae-deformities-by-yangpu-zhang-et-al-eur-spine-j-2017-doi-10-1007-s00586-017-4960-7
#13
https://www.readbyqxmd.com/read/28278134/limited-open-quadriceps-release-for-treatment-of-congenital-dislocation-of-the-knee
#14
Ahmed Omar Youssef
BACKGROUND: Congenital dislocation of the knee (CDK) is a rare condition for which the treatment is difficult and remains controversial. The aim of this case series is to evaluate the results of limited open quadriceps release for treatment of CDK. METHODS: Sixteen patients with CDK were managed at our institute from May 2006 to May 2014, and included 10 boys and 6 girls, with a mean age at presentation of 5 weeks (range, 1 to16 wk). Three patients who had an underlying neuromuscular syndrome were excluded...
April 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28267910/-surgical-management-of-spinal-deformity-in-a-patient-with-escobar-syndrome-review-of-the-literature
#15
M C Hernández-Hernández, J A Canales-Nájera, J S De La Cruz-Álvarez, M E Tena-Zanabria, J Matus-Jiménez
BACKGROUND: The non-lethal variant of the Escobar or multiple pterygium syndrome is an entity of autosomal recessive inheritance linked to the X chromosome; it is characterized by multiple pterygia (hence its name) located mainly in the neck (95%) and armpits (55%), as well as other orthopedic malformations such as a vertical talus, congenital hip dislocation, and congenital scoliosis. OBJECTIVE: To present an optional surgical technique for the management of severe spinal deformities...
July 2016: Acta Ortopédica Mexicana
https://www.readbyqxmd.com/read/28266297/surgical-treatment-of-a-rare-reverse-madelung-deformity-in-11-years-female-patient
#16
Alexandru Ulici, Daniel Catalin Florea, Iulia Tevanov, Dan Zaharie, Madalina Carp
Madelung deformity is an abnormality of the distal part of the forearm due to a growth arrest in the distal radial physis creating an increase of the radial tilt angle associated with a dorsal subluxation of the distal ulna in most cases. It is a rare condition which represents only 1.7% of hand deformities being characterized by the presence of an abnormal structure, Vickers ligament, that tethers the distal radius to the lunate bone. Although it is believed to be a congenital disorder, the symptoms are absent till late childhood...
January 2017: Chirurgia
https://www.readbyqxmd.com/read/28260755/an-anthropometric-study-of-bimalleolar-breadth-and-its-relationship-with-stature-on-bangladeshi-garo-people
#17
Z Ahmed, S Z Sultana, S Mannan, S Choudhury, S Farjan, A Kabir, N Nawshin, E Z Epsi, R S Zisa, M Ismatsara
A cross sectional descriptive study was conducted at different areas of Mymensingh (Haluaghat, Vatikashor and Kachijhuly) from July 2015 to June 2016 on 101 Bangladeshi Garo people. Among them 60 were male and 41 were female. Sample collection was done by nonrandom purposive sampling technique. Mixed ethnicity and any kind of foot deformity resulting either from congenital or physical injury were excluded to construct standard measurement for Garo ethnicity. Bimalleolar breadth was measured using slide calipers...
January 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28258274/early-orthognathic-surgery-a-review
#18
Saleh Alwadei
Management of growing patients with severe developmental jaw abnormalities can be very difficult. Early surgical intervention may be warranted in situations where function (e.g., mastication, swallowing, breathing, or speech) and/or psychological well-being could be negatively affected. Many surgeons and orthodontists are reluctant to recommend a surgical treatment option for growing patients with severe developmental jaw abnormalities because of their age. Specific surgical procedures can be performed during growth to correct developmental jaw abnormalities with predictable results...
March 1, 2017: Journal of Contemporary Dental Practice
https://www.readbyqxmd.com/read/28255137/clinical-spectrum-of-children-receiving-palliative-care-in-malaysian-hospitals
#19
L A Chong, F Khalid, T B Khoo, S H Teh, G L Kuan, A M Aina Mariana, E Alias, C H Chieng, H Razali, G B Ong, S H Zainah, I N C Shukor, J J Wong
INTRODUCTION: Awareness for paediatric palliative care has resulted in the impetus for paediatrician-led palliative care services across Malaysia. However, there is paucity of local data on patients receiving hospital-based paediatric palliative care. We aim to review the clinical spectrum of patients referred to these services. METHODS: An observational study of children aged between 0-18 years receiving palliative care at 13 hospitals between 1st January and 31st December 2014 was carried out...
February 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28252480/delayed-treatment-of-a-neonatal-type-i-monteggia-fracture-dislocation-a-case-report
#20
William R Smith, Scott H Kozin, Dan A Zlotolow
Delayed diagnosis of a Monteggia fracture-dislocation changes a straightforward, treatable injury into a complex problem. Acute neonatal injuries may be missed because of the inability to visualize the unossified skeleton on radiography, interpreted later as 'congenital' dislocations. We report the case of a 14-month-old with a neonatal Monteggia type-I fracture-dislocation secondary to birth trauma, with anterior radial head dislocation and plastic deformation of the ulna. Uniplanar external fixation was used to restore ulnar length and correct angulation, with subsequent radiocapitellar joint closed reduction...
March 1, 2017: Journal of Pediatric Orthopedics. Part B
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