TL;DR: Chiari malformation appears to be an acquired and progressive condition that develops in the first months of life, because of a disproportion between hindbrain growth and an abnormally small posterior fossa, a consequence of the premature fusion of lambdoid and cranial base sutures.
Abstract: Chiari malformation (CM) is a frequent finding in multisutural and syndromic craniosynostosis, occurring in 70% of patients with Crouzon’s syndrome, 75% with oxycephaly, 50% with Pfeiffer’s syndrome and 100% with the Kleeblattschadel deformity. The pathogenesis of this condition and rationale for treatment are still controversial. Since its first description in 1972, several factors have been cited to play a role in inducing CM. In the light of recent publications, the roles of premature fusion of cranial vault and cranial base sutures, of congenital anomalies of the cerebellum and brain stem, of raised intracranial pressure, of venous hypertension and of hydrocephalus are reviewed. Evaluation and management of CM are also discussed. Chiari malformation appears to be an acquired and progressive condition that develops in the first months of life, because of a disproportion between hindbrain growth and an abnormally small posterior fossa, a consequence of the premature fusion of lambdoid and cranial base sutures. Venous hypertension caused by stenosis of the jugular foramen can also be present in these patients, resulting in intracranial hypertension and/or hydrocephalus. Careful MRI evaluation is recommended for the forms of craniosynostosis at a high risk of developing hindbrain herniation. The selection of posterior cranial vault expansion as the first surgical procedure is advocated. In selected cases, treatment of the posterior cranial deformity by occipital vault remodelling and treatment of the Chiari-like deformity by suboccipital decompression can be carried out using the same surgical procedure.
TL;DR: Nine endoscopically treated patients with the diagnosis of sagittal suture synostosis were compared with nine patients treated by using the Marchac remodeling techniques, and the results showed that the estimated blood loss ranged from 5 cc to 150 cc, with blood transfusion required in only one patient.
Abstract: Twelve patients between 0.4 and 7.8 months of age were treated by an endoscopic approach to strip craniectomy. Nine patients had sagittal suture involvement. Two patients had a single unilateral lambdoid suture synostosis, and one patient had a combination of a right coronal synostosis and a metopic synostosis. Postoperatively, all patients were placed in cranial remodeling helmets and the results showed that the estimated blood loss ranged from 5 cc to 150 cc, with blood transfusion required in only one patient. All patients were discharged from the hospital by day 2, and all patients had an improvement in their cranial head shape. The specific technique of using the endoscope to aid in performing a strip craniectomy will be discussed. Nine endoscopically treated patients with the diagnosis of sagittal suture synostosis were compared with nine patients treated by using the Marchac remodeling techniques. The mean operative time (1.6 hours versus 3.5 hours), estimated blood loss (43 cc versus 168 cc), hospital costs ($11,671 versus $36,685), and length of stay (1.16 days versus 5.1 days) were less by using the endoscopic technique. All nine patients treated by using the Marchac technique required a blood transfusion, whereas only one patient was transfused in the endoscopically treated group.
TL;DR: The authors propose that the high incidence of individuals with CTH who have Crouzon's syndrome is related to the premature synostosis of the lambdoid suture in the first 24 months of age.
Abstract: � The incidence of chronic tonsillar herniation (CTH) was evaluated with magnetic resonance imaging in 44 patients with Crouzon’s syndrome and 51 with Apert’s syndrome; the incidence was 72.7% in Crouzon’s syndrome and 1.9% in Apert’s syndrome. All the patients with Crouzon’s syndrome and progressive hydrocephalus had CTH, but of 32 individuals with Crouzon’s syndrome and CTH, only 15 had progressive hydrocephalus. Five patients with Apert’s syndrome were treated for progressive hydrocephalus; none had CTH. The patterns of suture closure in these two groups of patients were studied, and significant differences in coronal, sagittal, and lambdoid sutures were found between patients with Crouzon’s and Apert’s syndromes. In Crouzon’s syndrome, significant differences in the pattern of lambdoid suture closure were found between the groups with and without CTH; in the group with CTH, the lambdoid closure appeared earlier. The authors propose that the high incidence of individuals with CTH who have Crouzon’s syndrome is related to the premature synostosis of the lambdoid suture in the first 24 months of age.
TL;DR: Observations regarding growth in craniosynostosis are illustrated with clinical material in this report.
Abstract: Skull growth after single suture closure was described in 1851 by Virchow, who noted that growth in the plane perpendicular to a fused suture was restricted. However, this observation failed to predict compensatory growth patterns that produce many of the deformities recognized as features of individual syndromes. The deformities resulting from premature closure of a coronal, sagittal, metopic, or lambdoid suture can be predicted on the basis of the following observations: 1) cranial vault bones that are prematurely fused secondary to single suture closure act as a single bone plate with decreased growth potential; 2) asymmetrical bone deposition occurs mainly at perimeter sutures, with increased bone deposition directed away from the bone plate; 3) sutures adjacent to the prematurely fused suture compensate in growth more than those sutures not contiguous with the closed suture; and 4) enhanced symmetrical bone deposition occurs along both sides of a non-perimeter suture that is a continuation of the prematurely closed suture. These observations regarding growth in craniosynostosis are illustrated with clinical material in this report.
TL;DR: A detailed review confirms many of the findings previously described but suggests that the pathology of the condition is still not fully understood.
Abstract: Tne cases of growing fractures seen in the last 10 years are presented. Six of the patients sustained their injury within the first six months of life. The defects formed rapidly, several within two or three months after injury. There was enlargement of the defect in only one case after the date of discovery. Although the defects involved the parietal bone most commonly, in four out of 10 the lesion crossed either the coronal or the lambdoid suture. The edges of the defects were usually thickened; in some areas they were saucer-shaped but in two cases there was erosion of the outer table of the skull at a distance from the margin of the defect, the erosion being related to an extracranial fluid-filled cavity in continuity with a porencephalic cyst. The ipsilateral ventricle was usually dilated and in a number of cases was associated with a porencephalic cyst. In no case was a "leptomeningeal cyst" found beneath the defect at operation. A detailed review confirms many of the findings previously described but suggests that the pathology of the condition is still not fully understood. Computed tomography, undertaken in one case, appears to be the examination of choice. Further light may be thrown on the pathogenesis of this condition by the use of intracystic, intrathecal, and intraventricular water-soluble contrast media.