Central nervous system imaging in Crouzon's syndrome.

Although the need to prevent the secondary effects of craniosynostosis on the central nervous system is fundamental to the practice of craniofacial surgery, the detailed structural anatomy of the central nervous system in the syndromal craniosynostoses has become the subject of recent interest. A clinical and radiographic review of a population of 59 patients with Crouzon's syndrome determined the frequency of central nervous system deformities. Twelve percent of patients had evidence of decreased mental function. Ventriculomegaly on computed tomographic scan was present in 51% and found to be of three grades: mild, moderate, and severe (hydrocephalus). This was nonprogressive in 7 of the 11 patients with follow-up computed tomographic scans. Ten patients underwent surgical release to increase intracranial space; however, 6 of these patients showed no progression in ventricular size. Nonventricular anomalies were found less frequently (14%). Central nervous system findings show fewer nonventricular anomalies than in Apert's syndrome patients, with a corresponding higher mental function. The principal anomaly of ventriculomegaly is not directly related to suture defect and may represent a primary brain abnormality. Recommendations are made for the assessment and management of patients with Crouzon's syndrome with reference to these areas.

Noncraniofacial manifestations of Crouzon's disease.

Although most interest centers on the craniofacial region in Crouzon's disease, noncraniofacial manifestations are important because they may complicate both diagnosis and management. A population of 59 patients with Crouzon's disease was reviewed to determine the frequency of these deformities. Stylohyoid ligament calcification (50%) and cervical spine (40%) and elbow (18%) abnormalities were the most common. Minor hand deformities (10%), other musculoskeletal deformities (7%), and visceral anomalies (7%) were also seen. Acanthosis nigricans was not present in this population. Recommendations are made for the assessment and management of Crouzon's disease with reference to these areas.

Mandibular lengthening by distraction for airway obstruction in Treacher-Collins syndrome.

Mandibular lengthening by distraction was performed in a 6-year-old tracheostomy-dependent Treacher-Collins syndrome patient. Detailed preoperative imaging revealed an occluded retrotongue base pharyngeal airway, which, following mandibular distraction, became patent and permitted tracheostomy removal. Mandibular distraction as a technique must be targeted toward clinical problems--management of upper-airway obstruction may be one such scenario.

Progressive hemifacial atrophy (Romberg's disease): skeletal involvement and treatment.

Progressive hemifacial atrophy (Romberg's disease) manifests variable involvement of the skin, soft tissue and underlying cranio-facial skeleton. Significant bony deformation has been identified in those patients with early onset disease, the result of factors both intrinsic to the disease process and secondary to the abnormal environment in which the skeleton develops (functional matrix). Treatment demands combined osteotomy and augmentation of the skeleton in concert with conventional approaches to soft tissue correction.

An anomaly of the median artery associated with the anterior interosseous nerve syndrome.

The anterior interosseous nerve syndrome is characterized by paralysis of the flexor pollicis longus muscle, the flexor digitorum profundus muscle to the index and middle fingers, and the pronator quadratus muscle. The most common cause is entrapment of the anterior interosseous nerve near its origin from the median nerve by a variety of structures. Compression is most frequently caused by the deep head of the pronator teres muscle, or the fibrous arcade of the flexor digitorum superficialis muscle. Vascular compression has been reported infrequently. A patient with anterior interosseous nerve syndrome was found at operation to have the median artery passing through the anterior interosseous nerve just below the elbow. This artery has not previously been associated with the syndrome. A cadaver dissection confirmed the relationship.

The radial forearm--flexor carpi radialis myocutaneous flap: case report.

A patient sustained a compound fracture of the elbow resulting in a deep soft tissue defect with exposed bone, joint and nerve. The flexor carpi radialis (FCR) muscle was incorporated within the radial forearm island flap to cover the defect. Wrist flexion was not compromised by this procedure. The blood supply and applications of the radial forearm--flexor carpi radialis myocutaneous flap are described.

The very long posterior tibial artery island flap.

Limb salvage procedures utilising tissue which would otherwise be discarded have become a well established practice in the management of trauma. These principles may also be utilised in the treatment of the burn injury. Two patients are presented with severe burns to the lower limbs involving muscle and bone where above knee amputation seemed inevitable. Salvage of the knee joint and conversion to a below knee amputation has been made possible by utilising the remaining skin of the sole of the foot based on a very long posterior tibial neurovascular pedicle ("Fillet of Sole" flap). This has provided full thickness sensate skin over the knee joint and below knee stump and has led to improved functional performance of the patient.

Cephaloceles: classification, pathology, and management.

A cephalocele is defined as a herniation of cranial contents through a defect in the skull. Cephaloceles are classified according to their contents and location. We have reviewed a total of 112 patients with cephaloceles, 51 of whom had sincipital meningoencephaloceles (fronto-ethmoidal meningoencephaloceles). This group is distinctive in its demographic distribution, in the effect on growth of other facial structures, and in the combined craniofacial approach needed to treat them. This review is based on the sincipital encephaloceles with the other cephaloceles included for completeness. Despite many theories, the cause of congenital cephalocele is not known. Preoperative work-up includes 3-dimensional computed tomography scan of the facial skeleton, and surgical management is multidisciplinary in nature. The aim is to remove the lesion before the deformity has time to greatly distort facial growth, which appears to realign itself after surgery. The 50 patients who underwent surgery for fronto-ethmoidal encephalocele all survived with minimal complications.