Congenital arteriovenous malformations: A follow-up of treatment.

Due to the rarity of arteriovenous malformations (AVMs), there is a paucity of information on the outcomes of various treatments. Presently, the mainstays of treatment of an AVM are embolization, surgical excision or a combination of both. A retrospective study of 26 patients with AVMs treated at The Hospital for Sick Children, Toronto, Ontario between 1985 and 1995 was performed. The treatment strategies and patient outcomes were compared in terms of efficacy, complications and the response of patients and their families to their overall treatment. The overall findings showed that embolization alone was effective in controlling symptoms, but may be associated with an increased AVM size. A partial excision of an AVM does not appear to exacerbate recurrence, as has been previously reported. In fact, even after what appears to be a complete excision of the AVM, recurrence may still occur. Complications from a surgical excision of the AVM are more frequent but less devastating than complications from embolization of the lesion. The overall patient or parent satisfaction with treatment was high with respect to improvement in outcome. It is expected that with an increasing understanding of vascular malformations, and the evolution of interventional radiological techniques, complications will decrease and results as a whole will improve.

Compte tenu de leur rareté, on dispose de peu d'informations sur le résultat des divers traitements appliqués aux malformations artérioveineuses (MAV). À l'heure actuelle, le traitement d'une MAV repose sur l'embolisation, l'excision chirurgicale ou sur les deux approches combinées.Les auteurs ont procédé à une étude rétrospective de 26 cas de MAV traités au Hospital for Sick Children de Toronto, Ontario, entre 1985 et 1995. Les stratégies thérapeutiques et les résultats obtenus ont été comparés sur le plan de leur efficacité et de leurs complications, de même que sur le plan de la réponse des jeunes patients et de leurs parents au traitement dans son ensemble.Les résultats globaux ont montré qu'utilisée seule, l'embolisation était efficace pour maîtriser les symptômes, quoique associée à une augmentation de la taille des malformations. L'excision partielle de ce type de malformation ne semble pas exacerber les récurrences, comme on l'avait d'abord cru. En fait, même après une excision soi-disant complète de la MAV, la récurrence reste possible. Les complications de l'excision chirurgicale des MAV sont plus fréquentes mais moins dangereuses que les complications de leur embolisation. La satisfaction globale des jeunes patients ou de leurs parents à l'égard du traitement a été grande en ce qui a trait à l'amélioration du pronostic. On s'attend à ce qu'avec l'approfondissement des connaissances sur ces malformations vasculaires et l'évolution des techniques d'intervention radiologique, les complications diminuent et les résultats s'améliorent dans l'ensemble.

Histologic, histochemical, and ultrastructural analysis of soft tissues from cleft and normal lips.

The cause of cleft lip remains speculative. The nature and extent of pathophysiologic changes in cleft lip muscle are controversial. This study was undertaken to better understand the developmental processes at work. There were two groups of patients. In group 1, 40 fresh tissue specimens were taken from 22 patients who were 2 to 5 months old-their age at the time of their primary cleft lip repair. In group 2, eight control specimens were collected from six children who were seen in the emergency department with lip lacerations. Fresh specimens fixed in neutral buffered formalin were evaluated by the use of hematoxylin and eosin with Luxol fast blue, Bielschowsky, and Masson trichrome stains. Fresh frozen tissue was histochemically assessed by the use of hematoxylin and eosin, modified Gomori trichrome, and adenosine triphosphatase. Ultrastructural analysis was performed on fine sections of glutaraldehyde-fixed tissue. Histologic examination revealed increased endomysial and perimysial collagen in cleft specimens with evidence of muscle-bundle size variation and nonneurogenic atrophy. Insignificant differences were observed between cleft-side and noncleft-side specimens when the means of 200 counts of neural-tissue bundles in the subdermis were compared (p = 0.093). Histochemical examination revealed no typical checkerboard pattern, but a preponderance of type 2 fiber was seen. By means of electron microscopy, increased numbers of subsarcolemmal mitochondria were found in cleft, noncleft, and control specimens. Increased absolute numbers of mitochondria and variations in size, shape, and crystal arrangement were identified. In conclusion, there is no evidence of deficient neural supply in the cleft lip. There is also no evidence of neurogenic muscle atrophy or a metabolic abnormality. There are characteristic myopathic changes. These, in concert with the observed interstitial fibrosis, may have far-reaching implications for growth and function.

Active counterpositioning or orthotic device to treat positional plagiocephaly?

Active counterpositioning and orthotic helmets are the two main nonsurgical management options for positional plagiocephaly. The purpose of this study was to compare these two management regimens. We included a random sample of infants referred between January 1, 1998 and October 31, 1999 to Middlemore Hospital and Auckland Surgical Center, for management of positional plagiocephaly. Two-dimensional head tracings were taken for each infant, every 3 to 12 months. From these tracings, we obtained Cranial Index and Cranial Vault Asymmetry Index. Seventy-nine infants were assessed during an average of 48.2 weeks. Five infants had normal head tracings, and were therefore excluded from the study. Of the 74 infants included in this study, 45 were managed with active counterpositioning, and 29 with orthotic helmets. Average management time for active counterpositioning was 63.7 weeks, and 21.9 weeks for orthotic helmet treatment. For infants managed with active counterpositioning, the average change in Cranial Vault Asymmetry Index was 1.9%. In the orthotic group, average change in Cranial Vault Asymmetry Index was 1.8%. Orthotic helmets have an outcome comparable to that of active counterpositioning, although the management period is approximately three times shorter. Active counterpositioning generally had a slightly better outcome than orthotic management after the management period.

New Zealand Maori family with the pro250arg fibroblast growth factor receptor 3 mutation associated with craniosynostosis.

BACKGROUND: A large New Zealand Maori family has non-syndromic coronal craniosynostosis, which is inherited as an autosomal dominant mutation with variable expression. The aim of the study is to determine whether the family has the pro250arg mutation in the gene for fibroblast growth factor receptor 3 (FGFR3), a mutation found in patients with various types of craniosynostosis. PATIENTS: Fourteen members of a New Zealand Maori family were evaluated, of whom five have coronal synostosis. A family pedigree tracing six generations was recorded. METHODS: Blood samples were drawn for genomic DNA analysis from 14 family members. Polymerase chain reaction, restriction-enzyme digestion and DNA sequencing was performed to identify the pro250arg mutation in FGFR3. RESULTS: Seven family members were heterozygous for the pro250arg mutation in FGFR3. The mutation showed autosomal dominance with reduced penetrance and variable expressivity. CONCLUSION: Our data and those of other investigators suggest that we should begin integrating molecular diagnosis with phenotypic diagnosis of craniosynostoses.

Bioengineering of elastic cartilage with aggregated porcine and human auricular chondrocytes and hydrogels containing alginate, collagen, and kappa-elastin.

Transplantation of isolated chondrocytes has long been acknowledged as a potential method for rebuilding small defects in damaged or deformed cartilages. Recent advances in tissue engineering permit us to focus on production of larger amounts of cartilaginous tissue, such as might be needed for reconstructive surgery of the entire auricle. In this report we describe modification of the basic techniques that lead to production of a large amount of elastic cartilage originated from porcine and human isolated chondrocytes. Small fragments of auricular cartilage were harvested from children undergoing ear reconstruction for microtia or extirpation of preauricular tags and from ears of juvenile pigs. Enzymatically isolated elastic chondrocytes were then agitated in suspension to form the chondronlike aggregates, which were further embedded in molded hydrogel constructs made of alginate and type I collagen augmented with kappa-elastin. The constructs were then implanted in nude mice and harvested 4 and 12 weeks after heterotransplantation. The resulting neocartilage closely resembled native auricular cartilage at the gross, microscopic, and ultrastructural levels. Immunohistochemistry and electron microscopy additionally confirmed that the newly produced cartilage contained the major components of the elastic cartilage-specific matrix, including collagen type II, proteoglycans, and well-assembled elastic fibers.

Burn area color changes after superficial burns in childhood: can they be predicted?

Pigmentation changes after superficial burn injuries are often difficult to predict. We analyzed a sample of patients with burn injuries, looking for clinical indicators of predictable color changes in burn wounds. A sample of 50 children, predominantly those with pigmented skins, who had sustained superficial partial-thickness, (second degree) thermal, scald, or friction burns, were retrospectively grouped. Chemical and electric burns, and those needing skin grafts were excluded. Forty-one patients returned for interview and examination, an average of 63 months after injury (range, 3 to 276 months). All patients had their skin color graded in terms of the Fitzpatrick scale--a numeric scale that combines innate skin color plus reported history of skin response to sun exposure, to assign a value from 1 to 6, where white skin that reddens but does not tan on sun exposure is 1 and heavily pigmented black skin is 6. The burn site, as well as unburned areas of control skin, were tested for reflectance and luminance with use of a well-validated spectrophotometric technique. As an addendum, clinical photographs of a further 50 patients were similarly analyzed. During the first 3 years after injury, burn site color changes were variable. Subsequently, there was cumulative hyperpigmentation at the burn site, provided that the melanocyte-bearing deep dermis had not been destroyed. Hyperpigmentation correlated significantly with skin color, as quantified by the Fitzpatrick scale (p < 0.01), and with time after injury (p < 0.05).

Case report: unilateral combined facial nerve and brachial plexus palsies in a neonate following a midlevel forceps delivery.

A case is described in which the 2.7-kg fetus of a nonobese primigravid patient, delivered by midlevel forceps manipulation sustained neurapraxias of both the marginal mandibular branch of the seventh cranial nerve and the upper trunks of the ipsilateral brachial plexus. The pregnancy was uneventful, but labor was complicated by an occipitoposterior presentation and a prolonged second stage. Examination of the neonate revealed bruising and skin markings consistent with injury by obstetric forceps and the anatomic location of these marks suggested that cervical and mandibular compression from the forceps, rather than traction by the accoucheur, would account for the observed findings. A review of the English language literature over the past 30 years revealed only four cases in which a combination of facial nerve and brachial plexus injuries could both be linked to obstetric instrumentation.

Design and development of a fascioperiosteal flap for use in experimental cranial surgery in a porcine model.

As part of an ongoing program of research on reconstructive techniques applicable to the growing craniofacial skeleton, this study aimed to characterize a pedicled fasciocutaneous flap on the scalp of a porcine model. Twelve juvenile Yorkshire white pigs were used. The anatomy of the porcine scalp was determined by fresh cadaveric dissection, radiocontrast dye injection, and resin casting of the vascular supply. No direct analogue of the human superficial temporal artery, the key to the superficial and deep temporal fascial flaps in humans, could be demonstrated in the pig. Thus, a fascioperiosteal flap, pedicled on an occipital leash of vessels, was designed. After a 14-day surgical delay, this flap, which covered the entire cranial width, from the nuchal crest to the glabella (7-10 cm long x 3-4 cm wide), could be elevated and split into a periosteal and a fascial layer. A cartilage construct placed between these layers could be kept alive for 4 weeks with no evidence of necrosis. The flap would allow mobilization of the construct to distant sites on the head and neck. An extensive literature survey showed a dearth of suitable cranial flap options in animal models. Accordingly, the pig cranium was examined with a view to finding a fascial flap analogous to the human temporoparietal flap, suitable for use in ear reconstruction. Cadaver dissections of the pig scalp, examining both the fascial layers and blood supply, were performed in three pigs; in an additional three pigs, the vascular supply to the scalp and cranium was injected with a radiocontrast gel, and, after dissecting suitable flaps, x-ray exposure was used to demonstrate the microvascular supply of these flaps. In an additional two pigs, the cranial vascular tree was filled with a plastic resin, and the soft tissues were then removed by thermal and chemical digestion. Finally, a group of three pigs were used to test the functional efficacy of a bilayered, occipitally based fascioperiosteal flap, whose design evolved from the foregoing work. Fluoresceine and disulphine blue were used to demonstrate the vascularity of these flaps before and after a surgical delay procedure. Function of these flaps, as carriers of a reconstructive construct, was shown by implanting autologous cartilage for 3 weeks and then demonstrating complete survival of the implant by fine section and histology. No clinically useful equivalent of the superficial temporal artery in humans could be identified in pigs, thus rendering the porcine superficial temporal artery flap of little use. However, a useful flap, comprising the pericranium and the subgaleal fascia, based on the occipital vasculature, could be raised with relative ease.

Exploring the use of the medicinal leech: a clinical risk-benefit analysis.

The medicinal leech, Hirudo medicinalis, has been used with increasing frequency for salvage of compromised pedicled flaps and microvascular free-tissue transfers. The data pertaining to 18 cases in which flap salvage with leeches was attempted were reviewed and contrasted with the data from 108 published cases from other centers. A further analysis isolated and examined 19 cases in which infection, secondary to leech utilization, supervened. Results suggested that successful salvage of tissue with leeches occurs in 70 to 80 percent of cases. In contrast, the infection rate of most series is between 7 and 20 percent and, when a clinically significant infection occurs, the flap salvage rate drops to 30 percent or less. In addition to infection, the risks of leech therapy include blood loss, which may require replacement transfusion, loss of leeches into body orifices and spaces, allergic reactions, and adverse psychological responses. Clinical indications and contraindications, and strategies to reduce the risks of leech usage, are discussed. Conclusions are that leeches are best used early, and in accordance with an accepted institutional protocol. For each case, the potential risks and benefits must be rigorously assessed and honestly applied.

Turribrachycephaly: a technical note.

We describe a technique for early correction of the so-called towering skull deformity, or turribrachycephaly. The technique makes use of the natural elasticity and plasticity of cranial bone, and it is best applied during the first year of life. Surgery consist of routine exposure of the cranial bone via a coronal incision. The frontal bone flap is elevated and removed. On either side, a bony osteotomy is then made, commencing low down in the temporal region and running posteriorly and superiorly toward the occiput. This approach leaves a superior bone flap, which may be left attached to the occipital bone via a flexible posterior hinge region, or completely detached by continuing the osteotomy across the midline. After the osteotomy, the bone flap is elevated up to the sagittal sinus on either side of the midline. The bone flap is not removed, but pushed down, compressing the brain along the craniocaudal axis. The lateral edge of the flap overlaps the temporal bone, and it can be fixed in the desired position by means of simple positional screws or Vicryl sutures. A standard fronto-orbital advancement is performed prior to lowering the skull vault, which permits the brain to be moved down and forward, filling the dead space in the anterior cranial fossa. The frontal bone flap is then shaped appropriately and fixed by means of plates and screws to the advanced fronto-orbital bar. Posteriorly, the frontal lobe is left "floating." To date, we have performed this technique on 5 patients, and we find it both faster and simpler than other techniques. Short-term results in terms of cranial shape are good. In older infants (> 2 years of age), this technique may not prove useful because of the loss of the loss of plasticity of the bone.

Decision making in primary surgical repair of myelomeningoceles.

A 5-year review of 43 consecutive patients presenting to Scottish Rite Children's Medical Center with an open myelomeningocele defect was undertaken. The aim of the present study was to analyze the myelomeningocele defects, dimensions, and area to better define those factors that dictate the need for plastic surgical consultation for wound closure. Of the 43 patients identified, two were excluded because they first presented as older children; the remaining 41 all had their myelomeningoceles repaired within the first 36 hours of life. Of these, 31 underwent repair by the Neurosurgical Service, whereas for 10 patients (24.4%), the Plastic Surgery Service was asked to assist with closure. Comparison showed the mean (+/- standard deviation) area in the referred patients was 27.4 (7.6) cm2 versus 17.6 (7.9) cm2 in the patients not referred for closure (p = 0.002). A trend analysis predicting referral as a function of myelomeningocele area showed that 0 of 10 (0%) with an area of less than 15 cm2, 2 of 13 (15.4%) with an area equal to 15 to 20 cm2, 3 of 7 (42.9%) with an area of 21 to 25 cm2, and 4 of 9 (44%) with an area greater than 25 cm2 were referred (p = 0.001). Data from the interpretation of maximum myelomeningocele dimension also showed statistically significant trends in referral. Using multiple logistic regression, it was found that the odds of referral increased by a factor of 3.3 for every 1 cm increase in maximum dimension.

Amputation neuromas of the great auricular nerve after rhytidectomy.

A 56-year-old woman presented with complaints of a tender nodule in the anterior triangle of her left neck. The nodule, which was easily palpable through the skin, was approximately 1 x 1.5 cm in size and was, at first, thought to be a lymph node. At operation, a large neuroma of the distal terminus of the transected great auricular nerve was found. Significantly, the patient had undergone a full rhytidectomy some 9 years previously, and it appears that the neuroma was a consequence of iatrogenic injury to the nerve at that time.

Successful use of leeches in the treatment of purpura fulminans.

A case of purpura fulminans secondary to pneumococcal septicemia is presented in an 8-month-old girl. The purpuric lesions on the fingers of both hands, as well as on the lower extremities, were treated by the local application of medicinal leeches. There was nearly complete salvage of the threatened tissues and the baby made a complete recovery. The possible mechanisms by which the leeches may have contributed to the clinical salvage are discussed.

Replantation of the avulsed pinna: 100 percent survival with a single arterial anastomosis and substitution of leeches for a venous anastomosis.

We have presented an alternative form of management for the difficult ear replant in which conventional venous outflow cannot be reestablished. Early and aggressive medicinal leeching provided an excellent means of venous decompression until neovascularization had taken place. The primary use of leeches in such small composite replants may be superior to the time-consuming and technically difficult exercise of trying to find and anastomose suitable veins. In selected patients this technique may allow successful replantation of the amputated ear, thereby providing a superior aesthetic result to most forms of secondary reconstruction.

Cytoskeletal actin: the influence of major burns on neutrophil structure and function.

It has been noted that major trauma and burns patients who survive beyond 48 h most frequently succumb to sepsis and multiple organ failure. Furthermore, such patients are usually markedly hypermetabolic and in negative nitrogen balance at the time of their demise. Along with many other systemic and immune dysfunctions, the polymorphonuclear white blood cells in this setting become functionally impaired. Given that the motile white blood cells contain significant proportions of the contractile protein, actin, we speculated that the leucocyte dysfunction might in part be related to the overall systemic catabolism of actin stores. Accordingly, this hypothesis was explored by comparing the functions and cytoskeletal structure of neutrophilic leucocytes from normal control adults and victims of fresh, major thermal injuries. On days 1 and 7 after a burn of > 25 per cent of total body surface area, peripheral blood was drawn from 10 patients (mean age 33 years, mean burn area 44.2 per cent), and seven unburned controls (mean age 35.2 years). Neutrophils isolated from these specimens were tested for stimulated chemotactic rate, efficacy of intracellular killing as determined by superoxide production rate, and the levels of soluble and insoluble intracellular actin. In addition, both light microscopy and scanning electron microscopy were used to visualize the actin cytoskeleton. The results indicated that both chemotactic rate (12 mu/min vs. 38 mu/min--P < 0.05) and superoxide production rate (9 vs 43 mumol/ml10E6 cells--P < 0.05), were significantly reduced in the burn patients by day 7.(ABSTRACT TRUNCATED AT 250 WORDS)

Combination of unilateral polydactyly, syndactyly, and clinodactyly with occipitocervical encephalocele and vertebral fusion.

This report describes a 6-year-old Mexican boy presenting with inter alia, hitherto unrepaired cervical encephalocele and associated unilateral syndactyly. There was also ipsilateral clinodactyly of the thumb and possible polydactyly of the foot. In addition, there was unilateral fusion of the first and second cervical vertebrae and a Chiari type III malformation. Motor and language skills were grossly normal for age, with the exception of mild left hemiparesis affecting the arm more than the leg. Medical history was significant for incidental drainage from the encephalocele as well as occasional high fevers and possible episodes of central nervous system infection. The clinical findings were not consistent with Meckel-Gruber or any other well-recognized syndrome. It is our contention that this case documents a previously unreported constellation of congenital anomalies and, as such, may suggest a teratological insult or a new syndrome. Surgical repair was carried out with meticulous excision of dysplastic neural tissue, relocation of neural tissue within the thecal sac, and coverage of the repair site with a trapezius muscle flap. Skin was closed directly. Postoperative recovery was largely uneventful, with the exception of a seroma in the donor muscle bed.

Hand infections secondary to fish bone injuries.

Hand injuries associated with fish bones and fin spines are not common but can cause morbidity out of proportion to the original injury. This is because such injuries often leave residual fragments of foreign organic matter in the tissues, leading to troublesome secondary infections. This report details 1 year's experience with nine hand infections after fish bone injury in a busy regional hand surgery referral centre. There were five males and four females with an average age of 45 years. Radiographs taken on presentation in five patients revealed a radiolucent foreign body in only two patients. Two patients presented with a flexor tendon sheath infection, three with a pulp space infection, one with infection of the hypothenar space, one with a subungual infection, one with cellulitis of the dorsum of the hand and one with an abscess on the dorsum of the hand. Antibiotics were prescribed for six patients and all patients except the one with cellulitis of his hand underwent surgical débridement. A single surgical procedure was adequate in five patients. One patient with a hypothenar space infection only had the foreign body removed during the third surgical débridement. The two patients with flexor tendon sheath infections required ray amputation. Fish bones cause a number of different infections in the hand. A foreign body should always be sought even if not present on the radiograph. Antibiotic selection should be tailored to eradicate the causative organism.