The role of primary bone grafting in complex craniomaxillofacial trauma

The role of craniofacial surgical techniques and immediate bone grafting in the management of complex craniofacial trauma has been reviewed. Four hundred and one patients with complex facial injuries have been treated. Two hundred and forty-one primary bone and cartilage grafts have been performed in 66 patients. Complex facial injuries should be managed by direct exposure, reduction, and fixation of all fractures utilizing interfragmentary wiring. Very comminuted or absent bone is replaced by immediate bone grafting, producing a stable skeleton without the need for external fixation devices. Associated mandibular fractures are managed with rigid internal fixation utilizing A-O technique. Results of immediate bone grafting have been excellent, and complications are rare. All deformities should be corrected, whenever possible, during the initial operation. This one-stage reconstruction of even the most complex facial injuries will prevent severe postoperative traumatic deformity and disability that may be extremely difficult or impossible to correct secondarily.     

Surgical treatment of hemifacial microsomia. (First and second branchial arch syndrome).

A review of 17 patients operated upon for unilateral hemifacial microsomia, and followed for up to 18 years, clarifies several principles of management. The management is facilitated by dysplasia predominance grouping, with the major functional deformity dictating the sequence of repair. Our surgical goals are: (1) provision of adequate upper airway; (2) preservation of normal teeth and adequate alveolar bone to achieve proper dental occlusion; (3) restoration of binaural hearing; (4) external ear construction; (5) acceptable facial symmetry; (6) balanced facial animation; and (7) a child with an acceptable self-image.     

Biologic basis for maxillary osteotomies

Adult Rhesus monkeys were used as experimental models to investigate revascularization and bone healing in different single-stage anterior, posterior and total maxillary osteotomy techniques. Microangiographic and histologic studies demonstrated that intraosseous and intrapulpal circulation to the mobilized maxillary segments were maintained by the experimental flap designs which maintained intact soft tissue; the fragments healed by osseous union within six weeks without immobilization of the mandible. The treatment of many severe dental-facial deformities is difficult and challenging. Functional and stable occlusions with facial balance and harmony have been attained in many adult patients by maxillary osteotomy techniques. The Rhesus monkey serves as an excellent experimental model to develop new biologically sound maxillary surgical orthodontic techniques.     

Smoker's face: an underrated clinical sign?

In a prospective survey of patients attending a general medical outpatient clinic roughly half the current cigarette smokers who had smoked for 10 years or more were identified, using defined criteria, by their facial features alone. These facial features, designated "smoker''s face," were present in three (8%) of those who had smoked cigarettes for 10 years or more in the past and in none of the non-smokers. The association of smoker''s face with current smoking that had continued for 10 years or more was significant (p less than 0.001) and remained after the patient''s age, social class, exposure to sunlight, recent change of weight, and estimated lifetime consumption of cigarettes were controlled for. Smoker''s face may be a helpful indicator in antismoking campaigns.     

Craniofacial deformity in patients with uncorrected congenital muscular torticollis: an assessment from three-dimensional computed tomography imaging

Congenital muscular torticollis is caused by idiopathic fibrosis of the sternocleidomastoid muscle that restricts movement and pulls the head toward the involved side. Deformation of the craniofacial skeleton will develop if the restriction is not released and result in aesthetic and functional problems. The purpose of this study was to use three-dimensional computed tomography imaging for qualitative and quantitative evaluation of the craniofacial deformity in a series of patients with uncorrected congenital muscular torticollis, and to assess age as a precipitating factor for severity of the deformity. A total of 14 patients from 1 month to 24 years of age were included. The skull images were rotated into standard orientation and reconfigured for evaluation of the cranium, endocranial base, and facial skeletal structures. The midlines of cranial base and facial bone, angle of midline deviation, width of each hemicranium and hemiface, and the orbital index were defined and measured. The results showed that the cranium and cranial base deformation took place as early as in infant stage, with the most prominent change occurring in the posterior cranial fossa. Facial bone asymmetry started to appear after 5 years of age, at which time the mandibular and occlusal abnormalities were observed. The deformity of the orbits and maxilla occurred at an older age, characterized by the deviation and decreased vertical height on the affected side. The severity of the observed deformities increased with age. The angle of midline deviation was 2.48 +/- 1.68 degrees in the cranial base and 3.26 +/- 3.28 degrees on the facial bone. Both of the midline deviations were significantly correlated with age. Compared with the contralateral side, the width of the ipsilateral posterior hemicranium was longer (54.36 +/- 6.72 mm versus 50.81 +/- 6.55 mm), and the width of the ipsilateral lower hemiface was shorter (35.30 +/- 7.27 mm versus 43.49 +/- 11.34 mm). Both differences were statistically significant. Measurement of the orbital index demonstrated a significantly flatter orbit on the ipsilateral side (89.48 +/- 0.11 versus 92.74 +/- 0.08). This study showed that the cranium and cranial base deformity occurred early in patients with uncorrected torticollis, while the facial bone deformity occurred in childhood stage. The cranial and facial deformity became more severe with age. Early release of the muscle restriction is advised to prevent craniofacial deformation.     

Total reconstruction of the "end-stage" cleft lip and palate deformity

The purpose of this paper is to present a 5-year experience using a comprehensive surgical approach to reconstruct what we have chosen to call the "end-stage cleft lip and palate deformity." The deformity consists of varying degrees of midface retrusion, malocclusion, nasal deformity, and lip deformity. Most of the patients afflicted had unacceptable upper lip anatomy characterized by tightness and lack of cupid's bow and bulk. All had severe palatal scarring with resulting arch collapse and severe malocclusion. Most had had multiple surgical attempts to improve nasal aesthetics using standard rhinoplasty techniques with little or no improvement. The procedure involves splitting the upper lip with incisions extending into the upper buccal sulcus and rim of the nose allowing wide skeletalization of the maxilla and osteocartilagenous nasal skeleton. LeFort I or II maxillary advancement, nasal reconstruction, and upper lip modification (with Abbé flap if indicated) are done. The jaws are placed in intermaxillary fixation for 6 to 8 weeks. This comprehensive approach has been used in 16 patients, aged 15 to 29 years, with follow-up of up to 5 years. Excellent functional and aesthetic improvement has occurred in all patients, and complications have been minimal.     

Controlled trial of plasma exchange in treatment of Raynaud's syndrome.

Twenty-seven patients with Raynaud''s syndrome had their digital vessel patency assessed by Doppler ultrasound after different thermal stresses. Digital vessel patency rates differed significantly after stresses at 15 degrees C and 45 degrees C. In a randomised controlled trial placebo and heparin had no effect either on patients'' symptoms or on the patency of their digital vessels. Plasma exchange improved both symptoms and vessel patency rates at 15 degrees C and 21 degrees C. Improvement in seven out of eight of these patients has been maintained for six months. Assessing digital vessel patency by Doppler techniques allow continuous, atraumatic, and safe evaluation of the effects of different methods of treatment on the patency of the digital vessels and has helped to indicate that plasma exchange is a useful adjunct in the management of patients with severe Raynaud''s syndrome.     

Emotional and behavioral reactions to facially deformed patients before and after craniofacial surgery

The present experiment investigated whether observers' emotional and behavioral reactions to facially deformed patients could be substantially improved by surgical procedures conducted by well-trained specialists in an experienced multidisciplinary team. Also investigated was the hypothesis that emotional states mediate the effects of physical attractiveness and facial deformity on social interaction. Twenty patients between the ages of 3 months and 17 years were randomly selected from over 2000 patients' files of Kenneth E. Salyer of Dallas, Texas. Patient diagnoses included facial clefts, hypertelorism, Treacher Collins syndrome, and craniofacial dysostoses (Crouzon's and Apert's syndromes). Rigorously standardized photographs of patients taken before and after surgery were shown to 22 "naive" raters ranging in age from 18 to 54 years. Raters were asked to predict their emotional and behavioral responses to the patients. These ratings indicated that observers' behavioral reactions to facially deformed children and adolescents would be more positive following craniofacial surgery. Similarly, the ratings indicated that observers' emotional reactions to these patients would be more positive following surgery. The results are discussed in terms of current sociopsychologic theoretical models for the effects of attractiveness on social interaction. A new model is presented that implicates induced emotional states as a mediating process in explaining the effects of attractiveness and facial deformity on the quality of social interactions. Limitations of the current investigation and directions for future research are also discussed.     

The role of surgery in the management of thyroid cancer.

This is a review of one surgeon''s personal experience with 85 patients with thyroid cancer treated over a 20-year period. The data confirm that for papillary thyroid tumours, with rare exceptions, the prognosis is excellent. Anaplastic lesions, however, are consistently lethal. Follicular carcinoma and medullary carcinoma fall between these extremes. A simple clinical classification is offered as a guide to operative management and a reliable index of prognosis. Patients with clinically apparent, "manifest cancer" have serious, life-threatening disease; many such patients die of their disease. Patients with "neck lumps not yet diagnosed" usually have papillary carcinoma; their prognosis is excellent. Patients whose thyroid tumours fall into the category of "malignant nodule" or "pathologist''s cancer" are particularly fortunate: in this series no such patient has died. The importance of age in relation to thyroid cancer is also confirmed: non of the patients first treated before the age of 40 years has died of cancer. For young patients with favourable disease the author recommends conservative surgical treatment, which avoids cosmetic deformity or functional disability, to be followed by administration of levothyroxine to suppress production of thyroid=stimulating hormone. For patients with "unfavourable" thyroid cancer valuable palliation can often be achieved by a combination of surgery and irradiation. Survival rates for the total series are 76% at 5 years and 60% at 10 years.     

Intraocular lens providing good vision 23 years after placement.

A Ridley Perspex lens was placed in the posterior chamber of a man''s eye 23 years ago; 20/25 vision has been maintained since. This technique, first carried out in 1949, has been abandoned because of the high incidence of lens dislocation and glaucoma, but many modified versions of the lens have been produced. Implantation of intraocular lenses is still considered experimental, though careful selection of cases and meticulous surgical procedure can yield good results.     

The role of SH3BP2 in the pathophysiology of cherubism

Cherubism is a rare bone dysplasia that is characterized by symmetrical bone resorption limited to the jaws. Bone lesions are filled with soft fibrous giant cell-rich tissue that can expand and cause severe facial deformity. The disorder typically begins in children at ages of 2-5 years and the bone resorption and facial swelling continues until puberty; in most cases the lesions regress spontaneously thereafter. Most patients with cherubism have germline mutations in the gene encoding SH3BP2, an adapter protein involved in adaptive and innate immune response signaling. A mouse model carrying a Pro416Arg mutation in SH3BP2 develops osteopenia and expansile lytic lesions in bone and some soft tissue organs. In this review we discuss the genetics of cherubism, the biological functions of SH3BP2 and the analysis of the mouse model. The data suggest that the underlying cause for cherubism is a systemic autoinflammatory response to physiologic challenges despite the localized appearance of bone resorption and fibrous expansion to the jaws in humans.     

Treatment of Paget's Disease of Bone with Mithramycin

We report data from three patients with severe Paget''s disease of bone who were treated with mithramycin.Mithramycin infusion resulted in a fall in plasma calcium, phosphate, alkaline phosphatase, and urinary hydroxyproline excretion. There was an improvement in calcium and phosphorus balance in two of the three subjects studied. A pronounced or complete relief of bone pain occurred in all three.We suggest that mithramycin exerts its beneficial effect in Paget''s disease of bone by stimulating parathyroid hormone release. The parathyroid hormone released has a predominantly anabolic action on bone since its catabolic action is blocked by mithramycin, which inhibits bone resorption.     

Frontal bone periosteal osteomas

Osteomas are the most common tumors of the cranial vault and facial skeleton. Osteomas are usually benign in nature, commonly presenting with symptoms of facial deformity, facial pain, and headaches. Although the frontal sinus is the most frequent location of cranial osteomas, they are also occasionally seen involving only the frontal bone periosteum. This study is a retrospective series investigating the characteristics, management, and outcomes of five patients with frontal bone periosteal osteomas surgically treated with superficial osteotomies with primary closure. Medical charts were reviewed focusing on symptoms, size, radiographic findings, and disease of the periosteal osteomas. The chief complaint primarily involved a palpable deformity, which led to surgical evaluation. Radiographic studies were obtained to evaluate size and location of the frontal osteomas. Microanalysis of the specimens confirmed the presence of mature cancellous and/or cortical bone. Postoperative follow-up revealed no evidence of recurrence or complications. The superficial ostectomy technique with primary closure offers a simple, effective method for removal of frontal bone periosteal osteomas with minimal side effects.     

Rotation advancement of the midface by distraction osteogenesis

A wide variety of disease processes produce alteration of midfacial skeletal growth, resulting in moderate-to-severe midface deficiency presenting as retrusion associated with Angle's class III malocclusion. Le Fort III osteotomies with advancement can provide an excellent tool for correction of this deformity. Recently, the corrective procedure of choice for advancement of midfacial segments has been distraction osteogenesis after osteotomy. Straight linear advancement is the most common choice for corrective movement of the midfacial segment, whether accomplished through acute surgical advancement or through the progressive distraction technique. Unfortunately, linear advancement can produce abnormal configurations, both at the nasal root and lateral orbits, regardless of the technique used. Enophthalmos, caused by orbital enlargement, may limit the advancement necessary to achieve class I occlusion.The authors have extended the utility of the Le Fort III procedure and have improved the final outcome by creating a controlled rotation advancement of the midfacial segment using distraction. The application of an existing internal distraction device is modified to control the movement of the midfacial segment in a rotation advancement path. Included in the series were 10 patients with severe midface retrusion secondary to multiple congenital syndromes, along with cleft lip and palate. The ages of the patients ranged from 6 to 14 years. An internal distraction system was used in all cases. Application of the distractor was substantially modified to simplify both fixation and removal and to produce controlled rotation advancement. The team orthodontist determined the final occlusal relationship. Percutaneous distractor drive rods were removed 4 to 6 weeks after active distraction to increase patient comfort. The distractors and all associated hardware were removed after 12 to 16 weeks of consolidation; follow-up periods ranged from 1 to 3 years.By using the modified distractor application to produce rotation advancement, the contour abnormalities at the nasal root and lateral orbit and the enophthalmos produced by linear advancement were eliminated. Significant improvement in facial contour and class I occlusion was obtained in all cases. Complications consisted of near exposure of the device in one patient. Stability has been excellent, with no relapse reported by the orthodontist.Rotational advancement of facial segments by distraction allows successful early intervention in patients with significant midface retrusion. The abnormal nasal root and lateral orbital configurations produced by direct linear advancement are avoided, and a stable and normalized facial configuration is produced.     

PAGET'S DISEASE—Changes Occurring Following Treatment with Newer Hormonal Agents

From experience in six cases the anabolic steroid hormones, especially long-acting testosterone and estrogen preparations, are the treatment of choice in Paget''s disease, as in postmenopausal osteoporosis. Details of the management of three patients over a period of four years are presented.Roughly 4 per cent of the population, mostly persons over 40, show some evidence of Paget''s disease. Only a small number of them, however, have severe manifestations requiring treatment, such as pain, howing or fracture of the bones, pressure on nerves or heart failure. In rare cases malignant changes occur in the involved bone.Since the cause of Paget''s disease is not known, treatment in the past has been largely empirical. Reifenstein and Albright had advocated the therapeutic use of calcium, vitamin D and ascorbic acid, and, in postmenopausal women, administration of estrogens; but with fractures or immobilization, intake of calcium-containing foods, such as milk, must be restricted to avoid dangerous piling up of calcium and kidney stones, and fluids must be forced. In recent years anabolic steroid hormones, principally oral androgens and estrogens, have been employed by Gordan and others to promote bone repair, lessen bone pain and decrease urinary excretion of calcium. While these hormones probably do not arrest the disease, they seem to stabilize it and bring relief of symptoms.More recently, Albright and Henneman demonstrated that very large doses of corticotropin (ACTH) or cortisone resulted in immediate cessation of bone pain, decrease in urinary excretion of calcium and histologic evidence of regression of the disease process. The large doses required, however, also produce dangerous side effects, such as psychosis and osteoporosis, indicating that such treatment probably should not be continued over long periods.     

Fast neutron treatment as an alternative to radical surgery for malignant tumours of the facial area.

Thirty one patients with very advanced tumours of the maxillary sinus were treated with fast neutrons. Tumour regressed completely in 29 (94%) and subsequently recurred in four (14%). No surgical excision of bone, skin, or nerve was required, and an artificial eye was well tolerated in cases where the eye received the tumour dose and had to be removed. Complications occurred in 10 patients, two of whom had already received radical x ray treatment. The overall duration of neutron treatment was four weeks, and admission to hospital was usually unnecessary. These results compared well with those obtained with surgery. Surgery with curative intent for even moderately advanced tumours of the facial region, particularly the paranasal sinuses, results in deformity, which is often severe and always irreversible. Even so, the cure rate is only about 35%. The high rates of tumour control and the avoidance of severe cosmetic and functional defects after fast neutron treatment make it an alternative to radical surgery in the management of malignant tumours of the facial area.     

Surgical correction of Crouzon syndrome

This study analyzes the results of surgical treatment in 39 patients with the Crouzon syndrome. Early fronto-orbital advancement and craniectomy were universally successful in relieving raised intracranial pressure and in reducing ocular proptosis. However, definitive cosmetic correction was not achieved, and early cranial surgery was not able to prevent the development of midface hypoplasia. Thirty-two midfacial advancements have been performed in 30 patients. Sixteen patients had sufficient follow-up data for more than 2 years postoperatively. In all patients, a satisfactory early postoperative result was achieved. In the long-term follow-up group, 11 patients have maintained a satisfactory appearance, while 5 have developed recurrent deformity. Analysis shows this to be associated with a younger age at operation and continued mandibular growth. Frontofacial advancement in adults achieves good long-term results but is associated with a higher incidence of complications.     

Metyrapone in long-term management of Cushing's disease.

Metyrapone was used in the long-term management of 13 patients with pituitary-dependent bilateral adrenal hyperplasia (Cushing''s disease). The total length of treatment ranged from two to 66 months, with a mean of 21 months. The clinical features of the disease rapidly improved on metyrapone and this improvement was maintained. Although plasma ACTH concentrations rose in all patients, the increase was insufficient to overcome the adrenal blockade induced by the drug. Eight of the 13 patients had additional external pituitary irradiation as definitive treatment of their disease and one underwent a transfrontal hypophysectomy. Radiotherapy cured one patient, and after three years metyrapone was withdrawn. Slight hirsuties was noted in four of the seven women who received the drug for six months or more. A fifth woman had more severe hirsuties and this led to bilateral adrenalectomy. Other than hirsuties, side effects were few and the routine use of metyrapone is recommended as an adjunct to more definitive treatment in all patients who present with Cushing''s syndrome, irrespective of aetiology.     

Inflammatory bowel disease in the West Indies.

Inflammatory bowel disease is generally assumed to be rare among negroes and Indians. Over 10 years 34 cases of ulcerative colitis and 14 cases of Crohn''s disease were seen in one medical and one surgical unit in Port-of-Spain, Trinidad. Twenty-six patients were Negroes, 18 were Indians, three were of mixed race, and one was Caucasian. In many of these patients the disease was extensive and several of those with Crohn''s disease suffered severe complications. The assumption that inflammatory bowel disease is rare among West Indians of African and Indian origin therefore seems to be wrong.     

Colposcopy in a family planning clinic: a future model?

The first year''s experience of a satellite colposcopy clinic in the Glasgow Family Planning Centre was analysed. Establishment of the clinic was supervised by an experienced member of the colposcopy team at the department of gynaecology, Western Infirmary, Glasgow, who trained one of the family planning centre''s staff. Close links were thus maintained with the hospital clinic to which patients were referred for treatment. The policy at the new colposcopy clinic was to study prospectively all women in the hospital catchment area whose cervical smears were reported as abnormal. In 58 of 162 such patients there was at least moderate dyskaryosis and the cytologist''s recommendation had been referral for colposcopy. In 104 cases the changes were either atypia alone or mild dyskaryosis and a repeat smear was recommended within three to 12 months; 18 of these patients had grade II or III cervical intraepithelial neoplasia on biopsy, and relying on repeat smears would have resulted in an 11.7% false negative rate. If an atypical cytological picture is to be an indication for colposcopy clinics attached to family planning centres may have an important role, given satisfactory training and close links with central specialist colposcopy clinics.