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Congenital deformity of the hand

Mary Claire B Manske, Jairo J Rios Roque, Gabriel Ramos Zelaya, Michelle A James
Recent reports have demonstrated that nearly two-thirds of the world's population do not have access to adequate surgical care, a burden that is borne disproportionately by residents of resource-poor countries. Although the reasons for limited access to surgical care are complex and multi-factorial, among the most substantial barriers is the lack of trained surgical providers. This is particularly true in surgical subspecialties that focus on life-improving, rather than life-saving, treatments, such as pediatric hand and upper extremity surgery, which manages such conditions as congenital malformations, trauma and post-traumatic deformities including burns, and neuromuscular conditions (brachial plexus birth palsy, spinal cord injury, and cerebral palsy)...
2017: Frontiers in Public Health
James R Rodrigue, David Tomich, Aaron Fleishman, Alexandra K Glazier
Vascularized composite allograft (VCA) transplantation has emerged as a groundbreaking surgical intervention to return identity and function following traumatic injury, congenital deformity, or disfigurement. While public attitudes toward traditional organ/tissue donation are favorable, little is known about attitudes toward VCA donation and transplantation. A survey was conducted of 1,485 U.S. residents in August 2016 to assess VCA donation attitudes. Participants also completed the Revised Health Care System Distrust Scale...
April 8, 2017: American Journal of Transplantation
Jason Silvestre, Ines C Lin, Lawrence Scott Levin, Benjamin Chang
BACKGROUND: Efforts to standardize hand surgery training during plastic surgery residency remain challenging. We analyze the variability of operative hand experience at U.S. plastic surgery residency programs. METHODS: Operative case logs of chief residents in accredited U.S. plastic surgery residency programs were analyzed (2011-2015). Trends in fold differences of hand surgery case volume between the 10th and 90th percentiles of residents were assessed graphically...
March 28, 2017: Journal of Surgical Education
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
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
Joseph A Buckwalter V, Apurva S Shah
Background: Poland anomaly is a sporadic, phenotypically variable congenital condition usually characterized by unilateral pectoral muscle agenesis and ipsilateral hand deformity. Methods: A comprehensive review of the medical literature on Poland anomaly was performed using a Medline search. Results: Poland anomaly is a sporadic, phenotypically variable congenital condition usually characterized by unilateral, simple syndactyly with ipsilateral limb hypoplasia and pectoralis muscle agenesis. Operative management of syndactyly in Poland anomaly is determined by the severity of hand involvement and the resulting anatomical dysfunction...
December 2016: Hand: Official Journal of the American Association for Hand Surgery
David A Pettitt, Zeeshaan Arshad, Anuj Mishra, Paul McArthur
INTRODUCTION: Apert Syndrome is a congenital condition characterised by primary craniosynostosis, midfacial malformations and complex symmetrical malformations of the hands and feet. The hands demonstrate one of the most complex collections of congenital upper limb deformities, posing a significant challenge for the paediatric hand surgeon. This study examines the extant literature and current practice of the four UK specialist craniofacial units regarding the management of Apert hands in order to provide a basis for guideline development...
February 2017: Journal of Cranio-maxillo-facial Surgery
Padmanabhan Rengasamy
Cyclophosphamide (CPA) remains one of the most widely prescribed anticancer drugs. It is also used in the treatment of rheumatoid arthritis, childhood nephrotic syndrome and systemic lupus erythematosus. It is a potent immunosuppressive agent. It is commonly used in blood and bone marrow transplantation. With the growing trend among women postponing childbearing, the number of women who are diagnosed with breast cancer is also increasing thus escalating the chances of exposure of the unborn child to antineoplastic drugs...
December 6, 2016: Anti-cancer Agents in Medicinal Chemistry
Tarek Abulezz, Mohamed Talaat, Asem Elsani, Karam Allam
BACKGROUND: Congenital hand anomalies are numerous and markedly variant. Their significance is attributed to the frequent occurrence and their serious social, psychological and functional impacts on patient's life. PATIENTS AND METHODS: This is a follow-up study of 64 patients with hand anomalies of variable severity. All patients were presented to Plastic Surgery Department of Sohag University Hospital in a period of 24 months. RESULTS: This study revealed that failure of differentiation and duplication deformities were the most frequent, with polydactyly was the most common anomaly encountered...
May 2016: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
N Hidaka, T Uemura, H Nakamura
Congenital hypoplasia of the extensor tendon central slip is a rare entity. This article describes the clinical characteristics in a series of 22 fingers in 16 patients (mean age: 10 months), and the outcomes of conservative treatment. Nine of 22 fingers were classified as slender or hypoplastic. Treatment with bracing was successful in 21 digits, resulting in full active extension of the proximal interphalangeal joint at a mean of 8.5 months after treatment. Bracing was unsuccessful in one digit, in which operative treatment resulted in a successful outcome...
November 7, 2016: Journal of Hand Surgery, European Volume
B Hohendorff, J Franke, C K Spies, L P Müller, C Ries
OBJECTIVE: Arthrodesis of the proximal interphalangeal joint of fingers in a functional and pain-free position. INDICATIONS: Primary and secondary osteoarthritis, traumatic joint destruction, posttraumatic malposition, instability, joint destruction due to infection, irreparable extensor and/or flexor tendon lesion, recurrent flexion deformity in Dupuytren's disease, arthritis (e. g., rheumatoid arthritis, psoriatic arthritis), failed resection arthroplasty, failed prosthesis, congenital disorder (e...
October 25, 2016: Operative Orthopädie und Traumatologie
Sara Nader Marta, Roberto Yoshio Kawakami, Claudia Almeida Prado Piccino Sgavioli, Ana Eliza Correa, Guaniara D'Árk de Oliveira El Kadre, Ricardo Sandri Carvalho
Waardenburg syndrome (WS) is an inherited autosomal dominant genetic disorder presenting variable penetrance and expressivity, with an estimated prevalence of 1:42,000. Clinical characteristics of WS include lateral displacement of the internal eye canthus, hyperplasia of the medial portion of the eyebrows, prominent and broad nasal base, congenital deafness, pigmentation of the iris and skin, and white forelock. A 24-year-old male patient, previously diagnosed with WS, was referred to the Special Needs Dental Clinic of Sacred Heart University, Bauru, Brazil...
August 1, 2016: Journal of Contemporary Dental Practice
Salem Samra, Debra Bourne, Joel Beckett, Michael Matthew, J Grant Thomson
BACKGROUND: Polydactyly is the most common congenital hand deformity. There is currently no consensus among pediatricians or hand surgeons regarding optimal management of ulnar (post-axial) type B polydactyly leading to uncertainty in initial treatment decisions for infants with this type of deformity. METHODS: Parents of newborns with type B ulnar polydactyly were enrolled in a prospective, nonrandomized trial from 2010 to 2012. At enrollment they were offered: 1) no treatment, 2) suture ligature, 3) immediate excision under local anesthesia, and 4) delayed excision at four months of age under general anesthesia...
October 2016: Journal of Hand Surgery Asian-Pacific Volume
Goo Hyun Baek, Jihyeung Kim
BACKGROUND: Oligodactyly of the hand is one of the rarest congenital anomalies of upper extremities and defined as the presence of fewer than five fingers on a hand. Although it usually occurs in association with hypoplasia or absence of ulna, it can occur without abnormality of the forearm bones. The purpose of this study is to present clinical features and radiographic characteristics of hand oligodactyly with thumb. METHODS: Five patients of oligodactyly with thumb who showed normal forearm bones, were evaluated...
October 2016: Journal of Hand Surgery Asian-Pacific Volume
F Duteille, C Beneteau, M-V Camut, P Perrot
Congenital deformities of the hand and upper limb include a significant number of clinical situations. Their expression is, as in all congenital diseases, variable. Therefore, we can almost consider that each clinical situation is a bit unique. The difficulty, as any congenital disease, is the fact that the clinical cases are extremely diverse and difficult to classify. So what to do and surgical strategies are often matter of "School". This is even more true that the evaluation of results is very difficult due to low series (poor statistical value) and functional assessment to be partly due to the growth of the child...
October 2016: Annales de Chirurgie Plastique et Esthétique
Ricardo Horta, Ricardo Nascimento, Alvaro Silva, Rui Pinto, Pedro Negrão, Ricardo São-Simão, Jorge Carvalho, Marta Santos Silva, Jose Amarante
Radial club hand may be congenital or acquired; radial deviation of the hand is usually found, associated with palmar flexion-pronation and treatment of severe forms of radial club hand is often difficult. Here we present a case of reconstruction of a severe postraumatic radial club hand with a free fibular osteoseptocutaneous flap and Sauve-Kapandji procedure in a 28-year-old man. The patient had a radial deviation of the wrist and right upper limb shortening as a result of an infected pseudarthrosis of the radius...
October 2016: Microsurgery
Jingjing Li, Garthiga Manickam, Seemun Ray, Chun-do Oh, Hideyo Yasuda, Pierre Moffatt, Monzur Murshed
Sphingomyelin phosphodiesterase 3 (SMPD3), a lipid-metabolizing enzyme present in bone and cartilage, has been identified to be a key regulator of skeletal development. A homozygous loss-of-function mutation called fragilitas ossium (fro) in the Smpd3 gene causes poor bone and cartilage mineralization resulting in severe congenital skeletal deformities. Here we show that Smpd3 expression in ATDC5 chondrogenic cells is downregulated by parathyroid hormone-related peptide through transcription factor SOX9. Furthermore, we show that transgenic expression of Smpd3 in the chondrocytes of fro/fro mice corrects the cartilage but not the bone abnormalities...
September 1, 2016: Molecular and Cellular Biology
G M Weisz, W R Albury, M Matucci-Cerinic, D Lazzeri
This article analyses the nature of the multiple finger anomalies found in portraits by the French Renaissance artistic dynasty, the Clouets. The multiplicity of finger anomalies could be either innocent congenital variants, or pathological and traumatic deformities. In view of the presence of such `beautifying variations' in the works of other Renaissance artists, the authors decided that these features were not the result of an epidemic of deformities, but instead represented a stylistic approach in paintings of this period at the French Court...
September 2016: QJM: Monthly Journal of the Association of Physicians
Mikhail Y Danilkin
Distraction osteogenesis has become the most used method for bone lengthening and deformity correction including the bones of the hand and foot. The principle techniques used for hand bone lengthening in posttraumatic and congenital conditions using available external fixators are based on this method. We present the technical specifications and surgical techniques with the use of a mini-fixator that was specially designed for short tubular bones. The surgical techniques of lengthening and web space skin stock creation for future web-pasty are supplied with the diagrams of their application to phalanges and illustrative clinical cases...
June 2016: Techniques in Hand & Upper Extremity Surgery
Hamza M Alrabai, Sebastian Farr, Werner Girsch
Triphalangeal thumb (TPT) is a congenital condition characterized by the presence of an additional phalanx. Variable degrees of thumb deformity, malalignment, and excessive length are common features. Impairment of hand function has been reported with TPT probably secondary to anomalistic long thumb incompatible with fine hand skills. Abnormal thumb appearance moreover represents a major psychological concern from patient's perspective. Both removal of the extra phalanx and phalangeal reduction osteotomy with resection of the unhealthy extra interphalangeal joint are established methods for correction of the associated excessive thumb length...
June 2016: Techniques in Hand & Upper Extremity Surgery
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