Progressive postnatal craniosynostosis and increased intracranial pressure.

Since its first description by Virchow in 1851, craniosynostosis has been known as a potentially serious condition resulting in premature fusion of skull sutures. Traditionally, craniosynostosis has been regarded as an event that occurs early in fetal development, resulting in a skull shape at birth that is determined by the suture or sutures involved. In recent years, a different form of craniosynostosis has been observed. Patients initially come to the attention of physicians because they exhibit midface hypoplasia or occasionally hypertelorism. The affected individuals all have a normal skull shape and open sutures in infancy but develop multiple-suture craniosynostosis postnatally, ultimately requiring surgical correction. These cases are significant because, although the patients do not initially display the physical manifestations of craniosynostosis, they frequently develop increased intracranial pressure, which can have devastating consequences. Unless these patients are recognized and vigilant follow-up monitoring is instituted at an early age, permanent impairment can result. A retrospective chart review study was conducted, and patients with multiple-suture craniosynostosis who developed symptoms of increased intracranial pressure were selected. The patients were divided into two groups, namely, those with normal sutures and/or head shape at birth (progressive craniosynostosis) (n = 15) and those with abnormal head shapes at birth (classic syndromic craniosynostosis) (n = 12). Clinical and radiological findings typically used to monitor the development of increased intracranial pressure were reviewed for both groups and compared. In addition, mutational analyses were performed. All patients with progressive postnatal craniosynostosis demonstrated clinical, radiological, or ophthalmological evidence of increased intracranial pressure, requiring skull expansion. Those patients displayed papilledema, anterior fontanelle bulge, and thumbprinting more often than did the patients with classic craniosynostosis. Thirteen of 15 patients were given the clinical diagnosis of Crouzon syndrome, which raises the question of whether such patients represent a subset of patients with this syndrome. Mutational analyses for the patients with progressive craniosynostosis demonstrated that, of 13 patients tested, 11 had mutations in exon 7 or 9 of FGFR2, which is a common site of mutations in Crouzon syndrome. The traditional indications of increased intracranial pressure used to monitor patients with classic craniosynostosis can be used to monitor patients with progressive postnatal craniosynostosis, particularly anterior fontanelle bulge, papilledema, and thumbprinting. It is thought that regular monitoring of these characteristics may lead to earlier diagnosis and allow for surgical intervention before the development of undesirable outcomes. It is important for clinicians to be aware of this group of patients, because any delay in diagnosis and treatment can result in severe consequences for the patients.

Differential diagnosis of the trapezoid-shaped head.

OBJECTIVE: To recognize several conditions that result in a trapezoid head shape and review and contrast their various physical findings. METHODS: A detailed review of all patients seen in the Craniofacial Clinic at the Children's Hospital and Regional Medical Center in Seattle, Washington, over a 10-year period from 1991 to 2001, with the diagnosis of craniosynostosis and plagiocephaly was performed. During this period, 690 patients had a surgical correction of craniosynostosis, and 1537 patients had posterior plagiocephaly. RESULTS AND CONCLUSIONS: The shape of the head when viewed from the vertex position in an axial plane can be a significant diagnostic aid when evaluating a patient with plagiocephaly. Positional molding causes the vast majority of plagiocephaly. This deformational change results in a parallelogram-shaped head. A much more rare cause of plagiocephaly is lambdoid synostosis. With premature fusion of one of the lambdoid sutures, the head takes on a very characteristic trapezoid shape when viewed from the vertex. Unilateral coronal synostosis that occurs on the same side as either posterior positional molding or unilateral lambdoid synostosis will also result in the trapezoid head shape. Furthermore, on the rare occasion when anterior and posterior deformational plagiocephaly occurs on the same side, the trapezoid head shape may be the consequence. The choice of appropriate treatment modality requires systematic evaluation of the patient with a trapezoid-shaped head to determine the etiology of the deformation.

A custom mandibular distraction device for the rat.

In the last several years, the rat model has been used as a reliable means of studying distraction osteogenesis (DO). This model has several benefits, including lower cost, the availability of molecular reagents for the rat, and the ability to perform a large number of operations in a short time. We have recently developed an external device that is custom-made for the rat mandible and secured at the mandibular ramus. The device consists of a Leone (Florence, Italy) jackscrew distraction assembly (0.2 mm per one-quarter turn), 2 Luhr L-shaped 0.8-mm five-hole microplates, and four 0.8-mm x 3-mm microscrews (Stryker-Leibinger Corp., Kalamazoo, MI) for fixation. Additionally, there is a methylmethacrylate block that is placed on the lingual surface of the angle of the mandible to support the posterior screws. Sterile surgical technique was used, and the same two surgeons performed all operations. To date, we have operated on 153 Sprague-Dawley rats. The procedure has been well tolerated by the animals. We had 17 postoperative deaths (11% mortality), largely as a result of anesthetic complications. There were no wound infections or dehiscence. A mandible fracture was noted in 6 rats (4%), which occurred at the time of device placement. There were no device dislodgments. Postmortem examination showed the device to be well positioned in all rats, with good callous formation at the distraction site. The rats surpassed their preoperative weight after an average of 9.2 days. These results compare favorably with those of other models currently in use. We have developed a device that is currently being used in the study of DO in the rat mandible. Our device is small in size, does not require wide undermining for its placement, and has had a low incidence of device dislodgment or infection. This model has shown great reproducibility and thus should be effective in its application for the histologic and biochemical investigation of DO in the rat.

Status of the facilities program Princess Margaret Hospital, Nassau, Bahamas

Princess Margaret Hospital, the Bahamas largest hospital, has provided quality patient care to residents, visitors and tourists for nearly 50 years. In recent years the aging physical plant has deteriorated to the point where the quality of patient care is being threatened. The current administration is committed to improving the hospital facilities as well as the maintenance program to support state-of-the-art health care technology. This consultancy supported by the Pan American Health Organizaation seeks to begin the process of improving the facilities program at Princess Margaret Hospital. Seventy specific recommendations have been developed during a two week survey, in four major categories: safety issues, specific maintenance or repair items, administration of the maintenance program and financing. The overall effectiveness of the maintenance program is reviewed and the qualifications and efficiency of the maintenance staff is discussed. A framework for changing current maintenance practices is developed

Status of the facilities program Princess Margaret Hospital, Nassau, Bahamas

Princess Margaret Hospital, the Bahamas largest hospital, has provided quality patient care to residents, visitors and tourists for nearly 50 years. In recent years the aging physical plant has deteriorated to the point where the quality of patient care is being threatened. The current administration is committed to improving the hospital facilities as well as the maintenance program to support state-of-the-art health care technology. This consultancy supported by the Pan Américan Health Organization seeks to begin the process of improving the facilities program at Princess Margaret Hospital. Seventy specific recommendations have been developed during a two week survey, in four major categories: safety issues, specific maintenance or repair items, administration of the maintenance program and financing. The overall effectiveness of the maintenance program is reviewed and the qualifications and efficiency of the maintenance staff is discussed. A framework for changing current maintenance practices is developed