[Lipofibromatosis in the finger of a child]. / Lipofibromatose digitale chez un enfant.

BACKGROUND: Lipofibromatosis is a recently classified form of benign tumor in children; it is of non-specific clinical presentation and is seen mainly on the extremities. We report a case involving the index finger of a child. PATIENTS AND METHODS: An 8-year-old boy consulted for a nodule on the palmar surface of the left index finger that had appeared 6 months earlier. The nodule, which adhered to the deeper skin, measured 1cm in diameter and was bluish and firm on palpation. Histological examination of the resected specimen revealed fatty tissue associated with a proliferation of spindle cells. Immunohistochemical analysis showed that certain of these cells expressed smooth muscle actin, suggesting partial myofibroblastic differentiation. A diagnosis of lipofibromatosis was made. DISCUSSION: Lipofibromatosis is a rare tumor. The main problem in terms of severity is its locally invasive development, which frequently causes problems for resection. Due to the age at onset, its course with deep invasion, the frequency of recurrence and the histological aspect, lipofibromatosis is classed as a form of childhood fibromatosis.

Shrinkage of skin excision specimens: formalin fixation is not the culprit.

BACKGROUND: Discrepancies between cutaneous specimen sizes reported by the dermatosurgeon and the pathologist are important to evaluate because of their legal implications for malignant tumours and the downcoding of surgical acts. OBJECTIVES: The objective of this study was to determine the magnitude of changes in size and the factors influencing the retraction of routine skin excision specimens. METHODS: Three measurements of 82 skin excision specimens--consisting of length and width of the planned surgical excision (in vivo), length, width and depth of the specimens following excision (ex vivo) and of the specimens after formalin fixation (in vitro)--were performed and compared using a nonparametric paired test. Factors (age, sex, type and location of the lesions and initial measures) that could influence the amount of shrinkage were analysed using multiple linear regression models. RESULTS: The mean in vivo to in vitro shrinkage was 16% for length and 18% for width (P<0.001). The shrinkage was significant between in vivo and ex vivo measures (P<0.001), while no difference was observed between ex vivo to in vitro measures. In multivariate analysis, length shrinkage increased significantly with initial length (regression coefficient of 0.24, P=0.001) and limb location (1.25, P=0.048), and decreased significantly with initial width (-0.19, P=0.016). After adjusting for initial width, width shrinkage was neither significantly associated with type of lesion (malignant or not, P=0.20), nor with location (P=0.35). CONCLUSIONS: Shrinkage of skin excision specimens occurred immediately after surgical excision and prior to formalin fixation. Patients' age, sex and type of skin lesion did not influence the amount of shrinkage. Length shrinkage was more important for specimens excised from the extremities and increased with initial length and smaller width.

Physiolysis for correction of clinodactyly with delta phalanx: Early improvement.

Congenital clinodactyly known as "delta phalanx" is a congenital finger deviation caused by a middle phalanx abnormality. Progressive realignment can be achieved with normal growth through physiolysis according to Vickers. The purpose of this investigation was to identify the rate of correction in congenital clinodactyly following epiphyseal bar resection.We studied retrospectively 24 fingers in 14 patients aged from 14 months to 11 years old (y/o), operated on by two surgeons performing epiphyseal bar resection using a modified Vickers' method. Angle measurements were compared pre-operatively, at 1 year and at the final follow-up. The rate of correction was evaluated at each time point. The cohort was divided into two groups: surgery < 6 y/o and ≥ 6 y/o. Statistical analyses were conducted to compare the patient groups.The mean deviation decreased from 33° pre-operatively (20°-80°) to 17.2° (0°-40°) at 1 year after surgery. The deviation at the last follow-up decreased to 10.6° (0°-40°). Seventy percent of the operated fingers were completely corrected (angle ≤ 10°) at the latest follow-up visit. No difference was found regarding the age at surgery. There were two cases of premature fusion of the epiphyseal plates. Regardless of the patient's age, Vickers' procedure is a simple and effective solution for treating delta phalanx with early correction. Our data suggest that physiolysis is a successful surgery in patients up to 11 years old. LEVEL OF EVIDENCE: Level IV, therapeutic case series.

[Surgical management of the Blauth 1 to 3A thumb hypoplasia]. / Conduite à tenir devant une hypoplasie du pouce de type 1 à 3A.

Thumb hypoplasia is a sequence of congenital abnormalities, from minor hypoplastic thumb with a preserved function to complete thumb aplasia. We only discuss in this chapter on the management of the thumb hypoplasia type 1 to 3A according to the modified Blauth's classification. Their common feature is the conservative treatment, in opposition with severe hypoplastic thumbs usually treated by index pollicisation. Whatever is the degree of hypoplasia, patients should be carefully examined for bilaterality, other anomalies and syndrome. Surgical treatment, when indicated, requires systematically widening of the first web space, stabilization of the metacarpophalangeal joint and restoration of opposition and extension. Reconstruction of the infant thumb should be started in the first year of life. Functional result depends on the degree of hypoplasia and differs dramatically whether if the thumb hypoplasia is isolated or associated with a radial club hand.

Developmental biology of the upper limb.

This article aims to provide hand surgeons with current knowledge on the developmental biology of the upper limb. It will review positioning, limb bud emergence and formation of the apical ectodermal ridge. The development of the limb bud is analyzed in its three axes: proximal-distal, anteroposterior and dorsoventral. The signaling center and primary morphogens that initiate and stimulate the development of each axis will be described. For the proximal-distal axis, the apical ectodermal ridge stimulates the production of FGFs in the underlying distal mesoderm. The anteroposterior (or radio-ulnar) differentiation is a function of the zone of polarizing activity via the small Sonic hedgehog protein, which diffuses in a decreasing concentration gradient from the ulnar to the radial side of the bud. This gradient is essential to digit identity and numbers. For the dorsoventral differentiation, the signaling center is the dorsal ectoderm, which secretes WNT7A. Limb segmentation is described in three parts (arm, forearm and hand) along with the formation of the digital rays until finger separation. An example of congenital anomalies is provided for each step. To keep the length of this lecture within reason, the embryogenesis of nerves, blood vessels, muscles and tendons will not be discussed. On the other hand, the singularity of the thumb relative to the other fingers will be described. With a better understanding of developmental biology, surgeons should have better insight into congenital anomalies of the upper limb. This approach is the basis for the new OMT classification used by the IFFSH.

[Vascular tumors and malformations of the limbs]. / Tumeurs et malformations vasculaires des membres.

According to many practitioners, any vascular swelling is a "Hemangioma". This generic term leads to much confusion: some lesions present a spontaneous regression while the others tend to worsen all the life long. The only common feature is the histological pattern. The vast majority of the vascular lesions are benign and the malignant tumors are extremely rare. However, if some vascular lesions have minor consequences and can be easily managed, in many cases a curative treatment cannot be assessed and some complex lesions can threaten life. An International Classification has been established. It allows appreciating the differences between the various entities - malformations and tumors - their evolutive features and helps to decide the most appropriate investigations and treatment. Now, every surgeon should learn and use this classification, to achieve an accurate diagnosis and to improve the management of these vascular anomalies.

Metacarpal lengthening in children: comparison of three different techniques in 15 consecutive cases.

Metacarpal lengthening is a useful procedure to address hand deficiencies in children. In this study, we aimed to compare the results of three different techniques from one consecutive clinical series of hand deficiencies. A total of 15 metacarpal lengthenings have been performed in 12 children aged from 9 to 14 years. The callotasis technique was used in seven cases, the two-stage distraction-graft technique in four cases and the single-stage lengthening in four cases. All the metacarpals healed with bone. The lengthening obtained was a mean of 13 mm (range 8-21 mm), a mean of 22 mm (range 13-32 mm) and a mean of 12 mm (range 9-15 mm), respectively, in the three different techniques. The healing index was longer for callotasis (81 days/cm) compared with the other techniques (41 days/cm and 46 days/cm, respectively). We observed one case of fracture after callotasis and one after distraction-graft. One patient underwent tenolysis of the extensor mechanism after single-stage lengthening. In conclusion, distraction graft and single-stage lengthening may be valuable alternatives to callotasis. LEVEL OF EVIDENCE: IV; therapeutic study; multi-case series.

An experimental model of scalp expansion: a method of optimizing flap size and shape.

An experimental model has been developed for testing and comparing the placement and characteristics of expanders for soft-tissue advancement. Expanders are fixed to model heads and covered with a stockinet sleeve, and the sleeve is impregnated with prevulcanized latex. The resulting stockinet-latex has many of the physical properties of skin and can be manipulated to ensure optimal covering and suture length prior to surgery. The system is rapid and reliable. Several types of scalp flaps were tested using the model. The models can be used in teaching and prior to surgery. Their use ensures an à la carte expansion that minimizes the risk of expansion failure.

Boutonniere deformity in Dupuytren's disease.

The boutonnière deformity in Dupuytren's disease has been found to be due to fibrous contraction of the transverse retinacular ligament which anteriorly displaces the fibres of the extensor apparatus. Complete ablation of this ligament and liberation of the extensor apparatus allows easy extension of the finger when operating on severe cases of Dupuytren's contracture.

Surgical management of the hand in Apert syndrome.

In patients with Apert syndrome, the hands demonstrate many disturbances of soft tissue and bony structures. These include a short thumb with radial clinodactyly, complex syndactyly with a bony fusion involving the index, long and ring fingers, symphalangism and simple syndactyly of the fourth web space. The soft tissue anomalies involve the intrinsic muscles, the extrinsic tendon insertions and the neurovascular bundles. We have reviewed 52 patients who underwent surgical reconstruction of their hands. The aim of this study is to propose a better surgical management in the light of recent publications and to improve our understanding of the syndrome, attempting to reduce the number of procedures and to select the best possible procedures for each patient.

[Ulnar dimelia: imaging modalities and surgical implications]. / "Main miroir": données de l'imagerie et implications thérapeutiques.

PURPOSE: To report 2patients with ulnar dimelia. METHODS: Plain radiographs (2patients), angiography (1patient), MR (2patients), CT (2patients) with 3Dreconstruction (1patient) were performed. RESULTS: Unilateral polydactyly with absence of the thumb, ulnar duplication with absence of radius were observed in both patients. Shoulder dislocation was present in one patient. Vascular anomalities were observed in one patient who underwent angiography but did not interfere with polydactyly surgical treatment and pollicisation. CT with 3D helped in delineating the elbow and shoulder bone abnormalities and thus in planning elbow osteotomies. MR was helpful in imaging muscle anatomy. CONCLUSIONS: Ulnar dimelia or "mirror hand" is a rare congenital disorder of the upper limb. Approximately 70cases have been reported, including 3 with shoulder dislocation. A multimodality imaging approach, exploring the various aspects of the malformation is mandatory to help the surgeon in order to obtain a functional and aesthetic upper limb after complex surgical procedures taking into account the various aspects of the malformation.

Anatomic variations in a cleft hand of an elder cadaveric specimen.

Cleft hand is a rare and complex malformation in which the central part of the hand is missing. Since the nineteenth century, a very small number of dissections in cleft hand have been reported and very few reports describe the soft-tissue abnormalities. We report a case of an elderly cadaveric specimen with bilateral cleft hand. X-ray, CT-scan with 3D reconstruction and dissection were performed on the right hand and forearm. On both the sides, we noted the absence of the index, middle and ring finger. Malformation and degenerative arthrosis at the carpal bones level have been identified. The flexor and extensor tendons of the absent fingers fuse with one another over the end of the remaining carpal bones, forming a radial and an ulnar tendinous plexus and two tendon loops. Data concerning intrinsic muscles, vessels and nerves are entirely recorded. Soft-tissue abnormal distribution is due to the abnormal bony configuration. Correction of the bone abnormalities does not resume the surgical treatment of a cleft hand. Hand surgeons need to be aware of the soft-tissue disorders and their surgical implications.

[Nasolabial musculocutaneous island flap]. / Lambeau nasogénien musculo-cutané en îlot.

The authors describe a musculocutaneous island flap with a superior pedicle raised from the nasolabial sulcus and comprising the levator labii superioris and levator labii superioris alequae nasi. This flap was used in 23 cases to repair defects following resection of skin cancers of the lateral side of the nose, the upper lip and the cheek. The anatomical study of the blood supply of the nasolabial sulcus reveals the existence of a vascular junction within the muscle. The use of this muscle allows an increase in the dimensions, reliability and mobility of previously described flaps.

[Surgical treatment of camptodactyly]. / Traitement chirurgical des camptodactylies.

Almost all anatomical structures of the hand have been held responsible for non-traumatic, non-paralytic flexion deformity of the proximal interphalangeal joint. Together with Millesi and Flatt, we define camptodactyly as a disorder of flexor-extensor equilibrium in the PIP. This definition determined our therapeutic approach. 59 patients with one or several fingers affected by camptodactyly were followed between 1975 and 1989. In 3 cases, the anomaly was associated with another malformation of the hand and 56 cases, it was isolated. 11 children did not receive any active treatment, but were followed and manipulated. 17 (27 fingers) were treated by static or dynamic splints. 31 (43 fingers) were operated according to the following technique: total anterior tenoarthrolysis leading to recession of the flexor apparatus and lengthening of the skin on the palmar surface of the first phalanx by a rotation flap. This operation was preceded and followed by application of a dynamic extension splint onto the operated finger. Of the 20 fingers treated by manipulations and reviewed, 11 had improved, 7 were stable and 2 had deteriorated. Of the 24 fingers treated by splints and reviewed, 14 had improved, 5 were stable and 5 had deteriorated. Of the 30 fingers operated and reviewed, 30 had improved, 7 were stable and 2 had deteriorated. Although recent anatomical studies tend to indicate that anomalies of the lumbrical muscles are frequent in operated camptodactyly, these studies do not take into account the numerous anomalies of these muscles observed in the general population. The therapeutic conclusion resulting from these studies are also unconvincing. The technique which we propose cannot be applied to every case. The milder forms only require orthopaedic treatment. C camptodactyly in adolescents or adults with joint blocking are only slightly improved by surgery and only require corrective osteotomy. In all other cases, especially in young children, anterior tenoarthrolysis of the finger with cutaneous elongation allows reduction or correction of the deformity.

[Median clefts of the hand. Classification and therapeutic indications apropos of 29 cases]. / Fentes médianes de la main. Classification et indications thérapeutiques a propos de 29 cas.

Based on a series of 29 patients with median clefts of the hand (29 hands), the authors define the place of this malformation, its various clinical features and the therapeutic indications. The series consists of 13 boys and 7 girls, 9 bilateral cases and 11 unilateral cases. Involvement of the feet was observed in 9 cases. Several children also presented other malformations. 16 children were operated (22 hands). Two types of technique were used: simple closure of the cleft (Barsky's operation) and transposition of the index finger according to the Snow-Littler or Miura-Komada techniques. All operated patients were reviewed with a follow-up of more than one year and the results were assessed in terms of three criteria: overall use of the hand, thumb-index finger pinch grip, aesthetic appearance. The authors propose a new classification of median clefts of the hand based on examination of this series of 20 children: simple clefts with more or less complete absence of the middle finger, complex clefts with syndactyly (especially I and II), transverse bone polydactyly, extensive clefts with severe aplasia of the radial segment of the hand. Clinical and radiological examination confirm the experimental studies by Ogino. Barsky's operation gives satisfactory results in simple forms with parallel fingers. Translocation of the index finger to the base of the 3rd metacarpal is necessary in the presence of divergent fingers and syndactyly. Median clefts of the hand are very distinct from median aplasia, which is always unilateral, with no familial nature and no involvement of the feet and which can be classified together with brachysyndactyly. Each case must be studied before deciding treatment, as functional adaptation is always remarkable. The least favourable surgical results are observed in forms with abnormal position of the index finger (malrotation syndactyly). Lastly, the Snow-Littler operation is not devoid of complications.

[Surgical treatment of duplication of the thumb. Apropos of 106 cases]. / Traitement chirurgical des duplications du pouce. A propos de 106 dossiers.

The surgical treatment of thumb duplication remains difficult, the sequelae usually observed are aesthetic (ungual dystrophy, hypoplastic pulp, clinodactyly) or functional (joint stiffness or instability). In order to clarify our therapeutic approach, we classify duplications into two types: proximal duplications (Wassel stage IV, V and VI) and distal duplications (Wassel stage I, II and III). Our experience is based on 106 cases operated over the last 15 years (1977). Our indications and operative techniques have progressed since 1988, in particular, we no longer perform Bilhaut-Cloquet operation, and prefer to perform the first operation before the age of one year. Resection of the hypoplastic supernumerary thumb. Preservation of the pulp skin to reconstitute the paronychium and pulp of the remaining thumb. Resection of supplementary joint facets. Meticulous repair of the capsulo-ligamentus apparatus by means of a capsulo-periosteal flap taken from the "sacrificed" thumb. Tendinous reequilibration and reinsertion of thenar muscles. We do not perform systematic subcapital osteotomy. When necessary, this procedure is performed during a complementary operation at the age of 2 to 3 years. Since the introduction of these technical modifications, our functional and aesthetic results have improved. Temporary overcorrection of the pulp is necessary. Parents must be advised that this deliberately hypertrophic appearance becomes normal with growth.

[Fibrolipoma of the median nerve. Review of 10 surgically treated cases with a mean recall of 8 years]. / Fibrolipomes du nerf médian. Revue de dix cas opérés avec un recul moyen de huit ans.

Lipofibromatous hamartoma of the median nerve and its branches is rare, but is the most frequent lesion associated with digital hypertrophy in the upper limb. We present a pediatric series of 8 patients, two of whom had bilateral involvement. Digital enlargement or true macrodactyly was the presenting complaint. Fatty infiltration of the palm was a constant finding and is considered very suggestive of lipofibromatous hamartoma of the median nerve, as confirmed by MRI in cases of uncertain diagnosis. No causal link between the nerve hamartoma and the enlargement of soft tissues in the territory of the nerve has yet been proven; it appears to be a regional disease related to unknown development factors, and other congenital anomalies may be associated in the same territory. Treatment was mostly symptomatic, with carpal tunnel release and superficial removal of epineural proliferation; no interfascicular dissection was performed and we never made any attempt to completely resect the lipofibromatous constituents; no median nerve resection was performed. Debulking of the palm and enlarged digits was associated with nerve decompression, but true macrodactyly required a separate operative procedure and is not the subject of this study. Mean follow-up is 8 years and 5 operated lipofibromas have been reviewed after 13 years. Two-point discrimination was often abnormal, but sensory impairment was stabilized, as well as soft tissue enlargement. However no objective argument can currently support more radical nerve surgery, and the superiority of nerve resection on the course of digital hypertrophy has not been proven.

Ligamentary structure of the base of the nail.

The authors report the results of 115 dissections of the base of the distal phalanx of fingers and toes. In 85% of cases including hypoplastic supernumerary digits, there is a connective ligament-like structure. It is a dorsal expansion of the lateral ligament of the distal inter-phalangeal joint arising from the intermediate phalanx and ending in the matrix and the lunula. This ligament may have a role in biomechanical strains on the nail. It can explain some dystrophic nails associated with some malpositioned joints in fingers or toes.

Anatomical basis of a new naso-labial island flap.

We describe a nasolabial island flap with a proximal base. This anatomical study confirms the interest of a proximal base especially with regard to venous return. As opposed to classical naso-labial flaps with subcutaneous pedicle, it has a vascularization of musculo-cutaneous type which allows a greater degree of mobility and reliability. This flap is an effective therapeutic option in surgery for skin defects of the face.