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Introduction: radial longitudinal deficiency (RLD) includes bone, musculotendinous and neurovascular abnormalities of multifactorial aetiology of the radial side of the upper extremity. Treatment includes improving the length of the limb, the appearance and functioning of the hand. The aim of this study was to present our experience in a series of patients attended over the past 15 years and to describe the clinical and radiological results., Methodology: a retrospective study of patients with RLD between 2000 and 2016. Variables were analyzed and age associations were identified at the time of surgery, sex, laterality, type of deformity according to the modified Bayne-Klug classification, surgical technique, physis damage, associated diseases, functional and radiological results., Results: 47 cases of 65 met the inclusion criteria. The average age of surgery was 19 months, 61% female. According to classification 60% were type IV, type III 19%, type 0 in 17% and type I in 4%. The intervention was centralization 72.3%, radialization 8.5% and one case of lengthening. Ulna osteotomy was performed in 55.3%. There was damage to the physis in 31%. The postoperative radiological position was neutral in 48.9%., Conclusions: In patients with centralization at 1year, good clinical and radiological correction were observed, however this was lost over time. The management of soft tissues prior to centralization is believed to give better results. The use of intramedullary nail from the ulna to the carpus could be associated with damage to the distal ulna., (Copyright © 2018 SECOT. Publicado por Elsevier España, S.L.U. All rights reserved.)

The Klippel-Feil syndrome is a congenital malformation of the skull flap involving complex cervical vertebrae and organs, characterized by a classic triad: short neck, limitation of movement of the head due to cervical spine fusion and low hairline in occipital region. It results from an error in the axial skeleton segmentation of the embryo; its incidence is estimated at 1/40,000-42,000 births and predominates in females. The aim of this paper is to describe the clinical picture of a patient with Klippel-Feil syndrome and multiple malformations, including tracheoesophageal fistula, bifid thumb and intracranial lipomas/angiolipomas,that have not been previously described in the syndrome, so it is considered an exceptional finding., (Sociedad Argentina de Pediatría.)

Introduction: Arthrogryposis multiplex congenita is a progressive syndrome characterized by deformed and stiff joints, atrophy or absence of muscles, contracture and thickening of the capsular articular and periarticular tissue, and normal psychic status., Material and Methods: A retrospective, descriptive, evidence level HI study was conducted on the surgical management of patients with Arthrogryposis multiplex congenita and congenital adducted thumb between January 2005 and December 2010. Twenty-seven opening procedures were performed in 16 patients with a dorsoradial index finger flap technique. In 80% of patients the dominant hand was operated first; patients included nine males and seven females; the operated hands included 15 right and 12 left hands., Results: Flap design permits better advancement and space deepening, and cosmetic improvement. The mean preoperative first and second intermetacarpal angle was 20.5 degrees on average, with a minimum of 14 degrees and a maximum of 27 degrees. The mean postoperative first and second intermetacarpal angle was 50 degrees, with a minimum of 36 degrees and a maximum of 65 degrees. This was observed at the clinic with active thumb extension as well as proper thumb apposition., Conclusions: Flap design permits better advancement and space deepening, with a better balance between flexors and extensors. All patients had better limb function and cosmetic improvement.

Triphalangeal thumb is often thought to be a rare congenital abnormality; it has an incidence of 1 in 25,000 births. We reviewed 49 triphalangeal thumbs in 28 Mexico City patients at the Diaz Lombardo Hospital from 1974 to 1979, Shriners Hospital from 1979 to 1982, Instituto Nacional de Ortopedia from 1981 to 1982, and at the ABC Hospital from 1971 to 1998. A total of 21 patients (75%) had bilateral deformity. Patients were classified according to Dieter Buck-Gramcko system and Müller teratologic line. All were surgically treated and evaluated with Cheng graduation scale, finding good results in 48 patients (97%), fair results in one (3%), and no poor results.

Pollicization is a single-stage neurovascular pedicle transfer of the index digit to function as a thumb. The objective of this study is to investigate the results of index finger pollicization for correction of congenital deficiency of the first ray in pediatric hand. We have done 6 pollicizations of index fingers in 6 hands (there were 2 right hands, 2 left hands, and 1 bilaterally) in 5 patients (4 boys and 1 girl) who had absent or nonfunctioning thumbs (type III-V of Blauth's classification). Associated anomalies where numerous and included radial club hand, mirror hand and cardiovascular and urologic anomalies. The average time of Kirschner wire extraction was 32 days (30 to 36 days) and to beginning the hand rehabilitation at 5 degrees to 10 degrees day. The average age at pollicization was 5.5 years (range 2 to 8 years), and follow-up averaged 8 years (5 to 11 years). The cosmetic and functional results were excellent, with manual dexterity of prehension and opposition. Pollicization in children can be performed at least 2 years of age, to due of minor risk of neurovascular lesion but without delayed the cortical representation of the pollicized finger.

Werner in 1915, described a patient is characterized by a tibial bilateral aplasia or hypoplasia, polydactyly and absent thumbs. Autosomal dominant inheritance is demonstrated, with variable expressivity. The objective of this work is to describe a child with clinic and radiologic signs of Tibial Hypoplasia with Polydactyly. The genealogic study allowed us to suppose that the gene has a variable expressivity, since in the maternal branch, malformations such as syndactyly of hands, proximal implantation of thumbs and tibiae vara, have been found. The clinic, radiologic, and genetic aspects are discussed.

This case report deals with a nine year old male patient suffering from Holt-Oram Syndrome, an autosomic dominant disease which causes diverse skeletal malformations and several heart deformities. This is a case with no apparent family history of the disease. The outstanding clinical findings include: a defect of the auricular septum (ostium secundum type), a tri-phalanx thumb, hypoplasia of the first metacarpal and a shorter left arm. The skeletal abnormalities did not effect his manual capability nor his development. This case includes: series of skeletal X-rays, cardiovascular examinations, an examination of motor function. Surgical correction should be considered as part of a careful therapeutic plan. The patient's pre- and postsurgical evaluation are considered.

We present a case of a child with important phenotypic abnormalities (retinoblastoma, hypoplasia of the thumbs and genital), as well as craneofacial and evident psychomotor retardation. The chromosomal study showed a interstitial delection of the long arms of a chromosome from D group. We try to correlate karyotypes and phenotype, telling about difficulties that this relation means insisting about the importance of knowing more cases of chromosome 13 delection. We also think that subbands analysis represents an important factor in this correlation.