Macrodystrophia lipomatosa: macrodactyly related to affected nerves, and a review of the literature.

Macrodystrophia lipomatosa (ML) is a rare disease causing congenital local gigantism of part of an extremity mostly through proliferation of fibro-adipose tissue. This study is one of few that specifically describe 8 ML patients with histological confirmation of the diagnosis combined with the 79 histologically confirmed cases already described in the literature. The goals of this study are to describe this malformation by using only histologically confirmed cases, to offer some insight on current opinions on ML by reviewing the histologically confirmed cases described in the literature, and to make the postulation of autonomic nerve dysfunction as the aetiology of ML more plausible.

[The first gender-changing operation from female to male in The Netherlands, 1959/'60]. / De eerste geslachtsveranderende operatie van vrouw naar man in Nederland, 1959/'60.

The first sex-change operation in a transsexual patient, i.e. the construction of a penis (so-called phalloplasty), took place in the Netherlands in Arnhem in 1959-1960. The operative technique had already been described in 1938 and was perfected after World War II. The discussion of this controversial procedure during a staff meeting in Arnhem Municipal Hospital was published at that time in the Nederlands Tijdschrift voor Geneeskunde [Dutch Journal of Medicine] and this resulted in a large number of protesting letters to the editor. Although the operation was carefully planned and proved to be a success, a judicial inquiry was started and questions were even raised in parliament. As a result of all the commotion, the plastic surgeon Woudstra who performed the first phalloplasty never again dared to perform this kind of surgery, but he had opened the way for the formation of the first gender teams to be established in Amsterdam, Groningen and Rotterdam during the 1970s, by which time sex-change operations were fully accepted.

[Formation of a pseudocyst following abdominoplasty]. / Pseudocystevorming na abdominoplastiek.

A 49-year-old woman presented for follow-up examination after an abdominoplasty performed in Germany. She complained of pain in the abdomen and was not satisfied with the final result. The patient presented with a bulging, painful abdomen. The symptoms disappeared for a few weeks after puncture of the seroma, but then returned. Neither haematoma, seroma nor neuroma were found at the first exploration, but a second abdominoplasty revealed a large pseudocyst extending from the epigastric to the supra-pubic area. The pseudocyst was extirpated. Seroma formation is a frequently occurring complication after abdominoplasty, but is ultimately reabsorbed spontaneously. Pseudocyst formation following abdominoplasty is a rare complication, which has been described in the literature only when liposuction was done as well. Liposuction creates multiple channels that result in an open abdominoplasty cavity. Seroma and blood clots provoke an inflammatory response, resulting in a pseudocyst after the formation of a thick capsule. The prevention of seroma and haematoma formation is of essential importance in preventing this rare complication. Sharp dissection, prolonged drainage with high vacuum and a compressive bandage are very important in this connection.

[The anatomy lesson of Dr Nicolaes Tulp by Rembrandt (1632) and the findings during dissection of the forearm of a cadaver: anatomical discrepancies]. / De anatomische les van dr. Nicolaes Tulp door Rembrandt (1632) en de bevindingen bij de dissectie van de onderarm van een kadaver: anatomische discrepanties.

OBJECTIVE: To compare The anatomy lesson of Dr Nicolaes Tulp by Rembrandt (1632) with the findings during dissection of the forearm of a cadaver. DESIGN: Descriptive. METHOD: The left forearm of a 41-year-old white male cadaver was dissected, photographs being taken at every stage of dissection. The anatomical structures in the original painting and during dissection were described and compared. At dissection, several structures were displaced in an attempt to reproduce the anatomical structures as shown in the painting. RESULTS: Dissection revealed four anatomical differences in comparison with Rembrandt's painting: (a) the muscle belly seen at the proximal ulnar side of the forearm in the painting was not seen on dissection; section of the insertion of the M. flexor carpi radialis and transposition of this muscle to the location of the muscle belly created the possibility of lifting up the M. flexor digitorum superficialis and reproducing the muscle belly; (b) in the painting, Tulp lifted only the M. flexor digitorum superficialis, but the amount of muscle tissue found in the forceps was increased in the painting compared with the dissection; (c) the positions of the bellies of the M. flexor digitorum superficialis to the index finger and 5th digit and to the 3rd and 4th digits were found to be reversed; (d) the longitudinal white structure situated in the ulnar part of the 5th digit in the painting was not found on dissection. This may have been an anatomical variant of the ulnar nerve. CONCLUSION: The anatomical characteristics of the painting could not be reproduced by dissection of the forearm of a cadaver.

Long-term follow-up of surgical treatment for thumb duplication.

There are few long-term follow-up reports concerning the treatment of thumb duplication. We reviewed the treatment of 19 of 74 patients treated at our institution between 1956 and 2002. The average follow-up was 22 (range, 7 years to 35 years) years. Satisfactory function was achieved in 18 thumbs and cosmesis in 12 thumbs. Six thumbs were cold-intolerant at this late follow-up. Pinch strength was similar to the contralateral normal thumbs. Collateral ligament repair did not significantly contribute to joint stability. An objective method of postoperative evaluation showed good results in five, fair results in 12 and poor results in two thumbs.

[Treatment of phimosis without circumcision and reconstruction of the prepuce following circumcision]. / Behandeling van fimosis zonder circumcisie en reconstructie van het preputium na circumcisie.

Phimosis of the prepuce can be treated without performing a circumcision. The most common and most effective treatment option is the local application ofcorticosteroid ointment. Should this be insufficiently effective, then the constricted ring can be interrupted with two Z-plasties, which is sufficient to expose the glans penis painlessly. No skin is removed. The intervention may be performed under regional anaesthesia on an outpatient basis. In cases where reconstruction of the prepuce is indicated, this can be accomplished with a distally inverted flap of penile shaft skin combined with the application of free skin grafts.

[Hyperextension trauma of the finger]. / Het hyperextensietrauma van de vinger.

In 2 patients, a man aged 30 and a woman aged 31, a hyperextension trauma of the proximal interphalangeal joint of the right index finger and the left middle finger respectively was diagnosed. The man underwent surgery for volar plate rupture in the acute phase. The woman presented 7 weeks after the trauma with a chronic flexion contracture; she had an avulsion fracture of the second phalanx and was treated conservatively with splints and exercise therapy. The hyperextension trauma of the proximal interphalangeal joint of a finger is one of the most frequently occurring hand traumas. In the more severe cases, there can be substantial damage to the structures ofthe joint capsule, such as a volar plate rupture. In the acute phase, a ruptured volar plate results in pain and dorsal dislocation. In the long term however, two significant late complications can arise: the chronic post-traumatic hyperextension deformity and the flexion contracture. These complications are difficult to treat, but can be prevented by adequate initial treatment. The diagnosis of volar plate rupture is essential to this and can be formulated after a carefully conducted history taking and physical examination usually with simple radiological studies ofthe affected digit. Treatment of a volar plate rupture can be either conservative or surgical and is directed at regaining finger function and preventing complications. For the treatment to be successful, intensive physiotherapy during follow-up is essential.

A hyaluronan-based nerve guide: in vitro cytotoxicity, subcutaneous tissue reactions, and degradation in the rat.

We investigated possible cytotoxic effects, biocompatibility, and degradation of a hyaluronan-based conduit for peripheral nerve repair. We subjected the conduits to an in vitro fibroblast cytotoxicity test and concluded that the conduits were not cytotoxic. Subsequently, we implanted the conduits subcutaneously in rats, in order to investigate tissue reactions and biodegradation. Initially, a fibrin matrix was formed around the material, while the surroundings were relatively quiet. Macrophages (MØ) migrated to the conduits and formed giant cells next to the material after 5 days. The maximum presence of MØ was found after 3-6 weeks. The appearance of MHC class II cells showed a similar pattern. Highest numbers of giants reached a maximum after 6-12 weeks. Angiogenesis was started in the surroundings of the hyaluronan-based conduit within a few days. Massive ingrowth of blood vessels into the biomaterial was found after 6 weeks as well as cellular ingrowth into the lumen of the tube. At that time the tubular structure of the conduit was lost and loose biomaterial fibers were observed. The results show that a hyaluronan-based conduit is not cytotoxic and shows good biocompatibility. Such a conduit may be suitable as a guide in peripheral nerve repair.

Cerebral reorganisation of human hand movement following dynamic immobilisation.

Surgical treatment of a flexor tendon lesion of the hand is followed by a 6-week period of dynamic immobilisation. This is achieved by the elastic strings of a Kleinert splint, enabling only passive and no active flexor movements. After such immobilisation, the appearance of a temporary clumsy hand indicates decreased efficiency of cerebral motor control. Using PET we identified the recruitment of contralateral parietal and cingulate activations specifically related to the suboptimal character of these hand movements. After 6-8 weeks, normalised movement was related with contralateral putamen activation. Activations of the sensorimotor cortex and cerebellum were present during both scanning sessions. Changes in the pattern of cerebral activations reflect functional reorganisation. The shift from cortical to striatal involvement, observed in the group of four patients, generates the concept of unlearned movements being relearned.

Breast cancer and the role of breast size as a contributory factor.

Several studies have been published on the incidence of breast cancer following augmentation mammaplasty, with very different conclusions. A lower incidence of breast cancer than expected was found in a study conducted by Deapen and Brody in women who had undergone augmentation surgery, and an anticarcinogenic effect of silicone implants has been suggested. Before accepting this conclusion it is important to study the relationship between breast size and the risk of developing breast cancer. It can be assumed that on average, women who have their breasts enlarged have smaller breasts, originally, than other women. Therefore, it seemed possible that breast size might be the predominant factor, and not the silicone implant. To test the hypothesis that women with breast cancer have statistically larger breasts than women who are not found to have breast cancer, a questionnaire was sent to each of 232 women who had undergone surgery for breast cancer; 146 of these questionnaires were returned. Body mass index (BMI) and breast size were compared against the corresponding values in a population-based control group. The results showed no statistical difference between the two groups in BMI or breast size. These findings seem to support the hypothesis that silicone breast implants have some kind of anticarcinogenic effect.

Long-term results of syndactyly correction: full-thickness versus split-thickness skin grafts.

In order to compare the long-term results of full-thickness and split-thickness skin grafts after the correction of congenital syndactyly, 27 patients have been investigated after an average follow-up of 21 years. Post-operative functional and cosmetic results have been assessed by patient records, questionnaires and physical examination. The webs that had received split-thickness grafts showed more flexion and extension lags and the overall spreading of the operated fingers was significantly decreased compared to the control fingers. On the other hand, more re-operations because of web creep had to be performed after full-thickness grafts. Hyperpigmentation and hair growth in the grafts was found in most of the full-thickness grafts, while breakdown of the graft was found in some of the split-thickness grafts. Therefore, based on the results of this study, either full- or split-thickness skin grafts can be used when treating of congenital syndactyly.

Functional nerve recovery after bridging a 15 mm gap in rat sciatic nerve with a biodegradable nerve guide.

Recovery of nerve function was evaluated after bridging a 15 mm sciatic nerve gap in 51 rats with a biodegradable poly(DL-lactide-epsilon-caprolactone) nerve guide. Recovery of function was investigated by analysing the footprints, by analysing video recordings of gait, by electrically eliciting the withdrawal reflex, by nerve conduction velocity and by electromyography (EMG). Sensory nerve function recovered as measured by electrostimulation. Motor nerve function partly recovered but electromyograms remained abnormal throughout the study. We conclude that functional reinnervation by regenerating axons occurs after bridging a 15 mm nerve gap with a biodegradable poly(DL-lactide-epsilon-caprolactone) nerve guide, but the walking patterns remain abnormal. Video analysis is a useful tool to record and analyse the walking patterns of rats. Further studies are necessary to investigate the possibility of obtaining selective reinnervation of specific muscles.

[A degradable artificial nerve guide to bridge peripheral nerve defects]. / Een afbreekbare kunststof zenuwgeleider voor de overbrugging van perifere zenuwdefecten.

The standard technique for bridging a peripheral nerve defect is an autologous nerve graft if the nerve ends cannot be sutured. Recent evidence indicates that an alternative procedure-application of a degradable nerve guide-may be feasible. Currently the use of such a degradable nerve guide for the recovery of peripheral nerve defects in the hand is being tested in a multicenter trial. Conventional suturing as well as autologous nerve grafting are accepted methods in the control group within the protocol of the multicenter study. In the first two patients to receive the implant, a 28-year-old man and a 50-year-old women with tendon and nerve injury due to glass cuts, the operation was technically successful. Functional nerve recovery will be assessed in due course.

Cerebral consequences of dynamic immobilisation after primary digital flexor tendon repair.

Current treatment protocols for flexor tendon injuries of the hand generally result in an acceptable function, which can be quantified by objective parameters such as range of motion. The latter does not always match the patients' subjective experiences of persisting dysfunction. This raises the question whether changes in the cerebral control of movement might contribute to the perceived deficit. The main objective of the present positron emission tomography (PET) study was to characterise the cerebral responses in movement-associated areas during simple finger flexion immediately after dynamic immobilisation and after a subsequent 6-week period of active training. Ten subjects with flexor tendon injury participated in the PET study. Electromyography (EMG) recordings were made during finger flexion and extension in an additional subject. The main finding was that the (ventral) putamen contralateral to flexor movement was not activated immediately after release from splinting, while such activation reappeared after a period of training. This indicates a temporary loss of efficient motor control of over-learnt movements. The increase of unwanted co-contractions during flexion in a first EMG session, and not during extension, supports a concept of lost skills.

Bell's palsy before Bell: Evert Jan Thomassen à Thuessink and idiopathic peripheral facial paralysis.

Bell's palsy is the eponym for idiopathic peripheral facial paralysis. It is named after Sir Charles Bell (1774-1842), who, in the first half of the nineteenth century, discovered the function of the facial nerve and attracted the attention of the medical world to facial paralysis. Our knowledge of this condition before Bell's landmark publications is very limited and is based on just a few documents. In 1804 and 1805, Evert Jan Thomassen à Thuessink (1762-1832) published what appears to be the first known extensive study on idiopathic peripheral facial paralysis. His description of this condition was quite accurate. He located several other early descriptions and concluded from this literature that, previously, the condition had usually been confused with other afflictions (such as 'spasmus cynicus', central facial paralysis and trigeminal neuralgia). According to Thomassen à Thuessink, idiopathic peripheral facial paralysis and trigeminal neuralgia were related, being different expressions of the same condition. Thomassen à Thuessink believed that idiopathic peripheral facial paralysis was caused by 'rheumatism' or exposure to cold. Many aetiological theories have since been proposed. Despite this, the cold hypothesis persists even today.

Clinical implications of cerebral reorganisation after primary digital flexor tendon repair.

After flexor tendon injury, most attention is given to the quality of the tendon repair and postoperative early passive dynamic mobilisation. Schemes for active mobilisation have been developed to prevent tendon adhesions and joint stiffness. This paper describes five patients to demonstrate the cerebral consequences of immobilisation allowing only passive movements, which implies a prolonged absence of actual motor commands. At the end of such immobilisation, PET imaging revealed reduced blood flow in specific motor areas, associated with temporary loss of efficient motor control. Effective motor control was regained after active flexion exercises which was reflected in normalised cerebral activations. This suggests that temporary, reversible cerebral dysfunction may affect the outcome of flexor tendon injuries.

Changes in speed of information processing in the brain following tendon repair.

The objective of this study was to measure the "preparation time" that is the speed of information processing in the brain, and discuss the relevance of this parameter in the restoration of hand function following flexor tendon repair. The preparation time of 48 healthy adult participants was measured twice at a 6-week interval and compared with that of 12 patients after flexor tendon repair. There was no difference between the left and right hands of the healthy participants. The correlation between repeated measurements was high, although healthy participants performed 2.6% faster 6 weeks after the first measurement. After 6 weeks of immobilisation, patients showed a significant deterioration with respect to the speed of information processing by the brain on both the injured and uninjured sides compared with healthy participants, who had improved between the first and the second measurements. The results indicate that a period of lack of normal use of the hand leads to a change in cerebral control of hand movements.

Architecture of European plastic surgery.

The architecture of European Plastic Surgery was published in 1996 [Nicolai JPA, Scuderi N. Plastic surgical Europe in an organogram. Eur J Plast Surg 1996; 19: 253-256.] It is the objective of this paper to update information of that article. Continuing medical education (CME), science, training, examination, quality assurance and relations with the European Commission and Parliament all are aspects covered by the organisations to be discussed.