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[Gorlin-Cohen syndrome (frontometaphyseal dysplasia)].

The classical case of a Gorlin-Cohen-Syndrom (= fronto-metaphyseal dysplasis=FMD) is presented. The disease has been mentioned in literature for the first time in 1969 and has been described four times up to now. The characteristic features of this syndrome are very prominent supraorbital ridges with generalized bone dysplasia and joint deformities.

Abnormalities, Multiple↗

Microsurgical reconstruction of the hypoplastic thumb, type IIIB.

If a classically hypoplastic thumb, type IIIA, presenting with a first carpometacarpal joint, is amenable to reconstruction, a type IIIB case without the basal joint, is classically treated by pollicization. The authors concur with this opinion, but found two circumstances in which reconstruction is an option in a type IIIB thumb, either when an adolescent asks for improvement of an unstable but utilized thumb, or when a parent definitely refuses pollicization. In such cases, a free, vascularized metatarsophalangeal joint is the first step of the reconstruction, followed by classic non-microsurgical steps. The authors reviewed five cases, with a mean follow-up of 7.8 years. Hypoplasia of the reconstructed thumb, and a limited pinch, allow this option only with restricted indications.

Hand Deformities, Congenital↗

Repair of avulsion injury of the whole hand with single-stage transfer of five combined tissues: case report.

The authors present a 32-year-old man with an entire left hand avulsion injury. This was repaired in one stage with five free-tissue transfers, a combination of a left hallux wrap-around flap for thumb reconstruction, bilateral second toes for middle and ring finger reconstruction, and bilateral femoral anterolateral flaps for coverage of the injured hand at the palmar and dorsal aspects. All the transfers survived uneventfully. Due to enlargement of the flaps and narrowness of the first web space, three further surgical procedures were subsequently carried out. Over a 2-year follow-up, a satisfactory appearance of the reconstructed hand and functional restoration were obtained.

Accidents, Occupational↗

The extended lateral arm flap: a new modification.

The lateral arm flap has been one of the mainstays in the armamentarium of microvascular reconstructive surgeons, especially in the cover of small to medium-sized defects. Despite its many advantages, its widespread application has been limited by its small skin paddle. As a result of detailed anatomic and dye-injection studies, it is possible to determine the actual extent of the cutaneous vascular supply of this flap, which is derived from the posterior radial collateral artery. This artery is found to extend significantly beyond the elbow and into the radial aspect of the upper forearm and, through clinical illustrations, it can be shown that a much larger flap can be harvested and used in a manner that is more versatile than the conventional lateral arm flap.

Adult↗

[Mesomelic dysplasia: presentation of a case and literature of Werner's syndrome].

The extremely rare Werner syndrome (tibial hemimelia, foot polydactyly, triphalangeal thumbs) is represented by an own case: a 21 year old female has been treated since her birth in the orthopedic department of Homburg/Saar university clinic. First, during several operative sessions, there was the correction of the bilateral completely webbed fingers and the resection of the supernumerary toes. Because of tibial hypoplasia there was need of bilateral operative club-foot-correction, too. Presentation of the course over 20 years with clinical and roentgenological late results. In several publications we find different classifications of the collective name, tibial hemimelia'; the symptom-triade congenital tibia-defect/foot-polydactyly/triphalangeal thumb was of special interest.

Adolescent↗

[Idiopathic multicentric osteolysis and the delimitation of the cranio-carpal-tarsal-osteolysis syndrome].

It is shown in the example of a 36 year old female patient with progressive but mainly carpotarsal osteolysis to analyse the radiological and clinical delimitation to other osteolysis-syndromes. Over 35 years during infancy and adolescence the clinical picture was incorrectly related to the "Freeman-Sheldon-Syndrome" and was twice the cause for fruitless attempts to classify. Although no standardized classification or rather grouping of the osteolysis exist, now with means of systematically radiological diagnostic and by long clinical follow-up of over 35 years a definite enrollment of the case represented, proved to be an extreme rare form of idiopathic multicentric osteolysis with nephropathy. It can be listed now as the 13th case in the literature.

Abnormalities, Multiple↗

[Extension according to Matev in malformations of the hands (author's transl)].

Step-by-step distraction of the metacarpal I according to Matev in traumatic loss of the thumb is demonstrated in a modified form with interposition of a corticospongious bone chip. Subsequently, the article reports on the use of this method in congenital malformations of the hand on the basis of three different examples.--Firstly, monodactyly with extension of a second metacarpal as passive counter-support,--Secondly, symbrachydactyly with rudimentary metacarpal rests I to V with extension of I and then of III and IV, followed by subtotal resection of the second metacarpal stump and phalangisation, and--Thirdly, bilateral severe malformation of the hand through amniotic segmentations and exogenous syndactylies with extension of metacarpal I, ligamental transposition and commissural deepening. The results show that--provided indication is strict on account of the almost always satisfactory substitute grasping actions in congenital malformations of the hand--an effective improvement of the grasping function can be achieved in selected individual cases by means of Matev's method.

Adolescent↗

Management of the acutely burned hand for the nonspecialized clinician.

The hand is the most frequent site of thermal injury, and it is reported that didactic burn care training for physical therapists is severely lacking. This article is written for clinicians who do not routinely manage burned hands, with the purpose of outlining a plan for physical therapy evaluation and treatment of acute hand burns. The plan for management is guided by the clinician asking for questions: 1) What is the depth of burn and what tissues are involved? 2) When did the burn occur? 3) What surfaces of the hand are involved? and 4) What course of management has the physician elected to follow? The therapist can then formulate a plan of management for the acutely burned hand through integration of this information with a prior understanding of wound repair, hand anatomy, and kinesiology.

Bandages↗