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PubMed1998

[Callus distraction for lengthening of mid-hand and finger stumps in congenital hand abnormalities--personal results and review of the literature].

Distraction-lengthening technique is quite useful in a variety of congenital hand deformities with hypoplastic, or primary normal but secondary shortened (constriction ring syndrome) finger rays. It appears that around the age of two years is the earliest practical time to start distraction; certainly distraction and secondary surgical procedures to improve function should be completed before school entry age whenever possible. Between June 1990 and March 1993, nine distraction lengthening procedures (5 thumbs, 1 index, 3 little fingers) in five patients presenting with congenital hand deformities, were carried out. Although restoring length to the finger, lengthening does not provide normal circumference or, of course, interphalangeal joint motion. Amelioration in function seems to be more important than the esthetic gain. Distraction lengthening tolerates only few errors of indication, operative technique, and/or postoperative management. A high compliance of the patient and her/his parents as well as a close follow-up by an experienced surgeon, are mandatory for a good result. A variety of possible complications have been described. Generally, complication risk increases in cases of simultaneous and multiple level lengthening. Provided adequate operative technique and postoperative care, superficial pin infection and fracture in the region of distraction are the major complications. Contrary to adults, sufficient bone formation by distraction is the rule in children. Therefore, the distraction-lengthening technique is preferred to the distraction-interposition technique in the treatment of congenital hand deformities. The latter should only be used as a salvage procedure in the rare cases of insufficient callus formation. Because of the missing growth potential and reduced joint mobility, distraction lengthening is the therapy of second choice when compared to microvascular second toe transplantation.

Acrocephalosyndactylia↗
PubMedDate not supplied

A case of a four-day-old male with Carpenter's syndrome with transposition of great arteries.

Carpenter's syndrome (acrocephalopolysyndactyly type II) is an autosomal recessive syndrome characterized by peculiar facies, synbrachydactyly on fingers and preaxial polysyndactyly on feet. To our knowledge there are about 40 reported cases of Carpenter's syndrome in the literature. Congenital heart disease is an uncommon entity in Carpenter's syndrome. In the case we present, transposition of great arteries, subpulmonic ventricular septal defect (VSD) and secundum atrial septal defect (ASD) were diagnosed with echocardiographic examination. Therefore, a cardiologic examination should be done in every newly diagnosed case of Carpenter's syndrome for possible heart defect. Early fatality is seen in Carpenter's syndrome cases associated with congenital heart disease. This is particularly important from the genetic counselling point of view.

Acrocephalosyndactylia↗
PubMed1998

[Bone substitute with growth factor. Preliminary clinical cases for cranio- and maxillo-facial indications].

Several biological materials have been analyzed in combination with osteo-inductive growths factors to determine whether such a system can replace bone grafting in surgical practice. Efforts have been aimed at the discovery of the best carriers and delivery systems. We present the results of the surgical treatment of 11 cranio-maxillo-facial defects in 9 patients using a combination of natural coral skeleton (NCS in blocks or granules), human fibrin glue and transforming growth factor beta-1 (TGF-beta 1) as a composite bone substitute. Three patients were initially excluded because of early extrusion of the materials due to a technical error. Clinical and radiological evaluation was performed in all cases, with the patient acting as his own control. Clinical firmness and radiological mineralization occurred in three quarters of cases. New bone formation was confirmed histologically in two of these patients. Clinically the initial results remained stable over a three years follow-up with staged surgical procedures performed on a number of patients. None of the patients suffered any detrimental effect from implantation of the bone substitute. Although the numbers in these series are limited, the association of TGF-beta 1, human fibrin glue and NCS represented an interesting step, although the clinical results could be improved. Important factors in the success of this technique appeared to be stabilisation of the biological materials, quality and asepsis of the surrounding tissue and the dose of growth factor.

Acrocephalosyndactylia↗
PubMed1998

Congenital hand problems. Terminology, etiology, and management.

Although simple "cookbook" treatment plans for most congenital hand problems are not possible, certain guiding principles should be followed. It is an important to identify when to operate as it is to know when not to operate. The timing of surgical interventions as well as the sequence of multistaged interventions play an important role in ultimate function of the hand. It may not always be possible to achieve both satisfactory power grasp and precision handling. Redistributing functional patterns that we have generally employed in our approach to traumatic injuries can be used in planning therapy for congenital malformations of the hand to try to achieve the best possible function with the available functioning anatomic parts.

Acrocephalosyndactylia↗