Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery.

PURPOSE: Multiple imaging parameters have been examined to estimate the presence of syrinx and the need for surgery in Chiari I patients (CM1); however, no consistent or definitive criteria have been proposed. The objective of this study was to review existing and identify novel radiological and clinical characteristics of CM1 patients that associate syrinx development and surgical intervention. METHODS: Patients with Chiari I malformation diagnosed on imaging between 0 and 18 years were retrospectively reviewed from January 1, 2007 to February 12, 2020. Participants were included if they had a baseline MRI of the head and spine prior to surgical intervention if required. Forty age-matched controls with cranial imaging were identified for comparison. Imaging parameters and clinical symptoms were recorded. RESULTS: A total of 122 CM1 patients were included in this study. Of the 122 patients, 28 (23%) had syrinx, and 27 (22%) had surgery. The following imaging parameters associated with syrinx and surgical intervention were identified: midbrain length (P < 0.001; P = 0.032), the obex position (P = 0.002; P < 0.001) and medullary kinking (P = 0.041; P < 0.001). Among the clinical features, the presence of overall pain (P = 0.017; P = 0.042), neck pain (P = 0.005; P = 0.027), and sensory dysfunction (P < 0.001) were found to be strongly associated with syrinx and surgery. CONCLUSION: While further investigation is needed, these specific radiological and clinical parameters should be considered when evaluating CM1 patients and may be used to guide further management.

Spinal Syrinx Due to Lipiodol-Induced Arachnoiditis.

We present a case of a progressive symptomatic intramedullary cyst, diagnosed decades after Lipiodol injection. Lipiodol was originally administered intrathecally for the radiologic diagnosis of spinal masses. A link between the lesion and the history of Lipiodol injection was never suspected. Surgical exploration revealed a membrane above the lesion, separating the intradural space in a cranial and caudal compartment. On the level of the cyst, we identified glassy pearls containing a fatty liquid, compatible with Lipiodol deposits. We hypothesize that the syrinx is secondary to the impact of cerebrospinal fluid pulsations on the reactive membrane and that this membrane originated from an arachnoiditis caused by Lipiodol deposits. Lipiodol was indeed abandoned after it was found to cause arachnoiditis and neurologic sequelae. Despite the cessation of its usage, the causal role of Lipiodol in arachnoiditis and spinal cyst formation should still be considered, as symptoms may arise many years after Lipiodol administration.

Currarino's syndrome in twins presenting as neonatal intestinal obstruction--identical presentation in non-identical twins.

We report a case of non-identical twins who presented with identical neonatal intestinal obstruction with features of anorectal stenosis, presacral mass and sacral anomaly consistent with Currarino's syndrome or triad. Plain sacral radiograph, contrast enema and MRI were diagnostic. Initial management involved a defunctioning colostomy followed by a posterior sagittal anorectoplasty with excision of the teratoma ± anterior sacral meningocele and finally closure of colostomy in a staged multidisciplinary approach. The twins' father is also affected with features of Currarino's syndrome but was diagnosed during family screening. Currarino's syndrome presenting with identical neonatal low intestinal obstruction in a non-identical set of twins is rare and interesting. Antenatal diagnosis of Currarino's syndrome is difficult and may prove to be a challenge even in the postnatal period. Sacral spine radiograph, contrast enema and MRI are diagnostic. Management requires high index of suspicion, low threshold for MRI and multidisciplinary staged approach.

[Incidental syringohydromyelography: report of a case]. / Siringohidromielografia incidental: registro de um caso.

The radiological findings of syringomyelography are described in a five years old patient who underwent a percutaneous injection of opaque contrast medium (Lipiodol) into the intraspinal syringomyelic cavity. The clinical picture, the usual diagnostic methods and the place of syringomyelography for the diagnosis of syringomielia are discussed.

Computerized axial tomography in syringomyelia.

The ACTA-scanner, a device for computerized axial tomography, permits cross-sectional radiographic study of the entire human body, including the spine. In the ACTA-scan, the spinal cord appears as a roundish formation surrounded by the less dense subarachnoidal cerebrospinal fluid. The spines of 18 patients with verified (nine cases) or suspected (nine cases) syringomyelia were studied by ACTA-scanning. In seven of the verified and in four of the nonverified cases, some evidence of cord cavitation was shown. The cystic part of a cervical-cord ependymoma was also demonstrated. ACTA-scanning is an easily performed, noninvasive, innocuous procedure. This technic, which complements other radiographic methods of evaluating the spinal cord, is particularly suitable for screening and follow-up study of patients with syringomyelia. The easily accomplished recognition of a possible associated hydrocephalus is an added advantage of ACTA-scanning.