The authors report the case of a 13-year-old girl with Kikuchi-Fujimoto disease who presented with features of raised intracranial pressure due to subdural effusions. A biopsy revealed dural involvement in the disease process. The authors review the medical literature of this rare disease and highlight the paucity of documented cases with CNS involvement.
Marcelo Volpon Santos, Pasquale Gallo, Fozia Roked, Ina Nicklaus-Wollenteit, and Desiderio Rodrigues
Nitin Mukerji, Julian Cahill, Desiderio Rodrigues, Savithru Prakash, and Roger Strachan
Lumboperitoneal shunting is the standard treatment for pseudotumour cerebri or idiopathic intracranial hypertension. Complications are common, particularly the problem of overdrainage leading to low pressure symptoms. The authors designed a simple experiment using catheters of different lengths that drained at different pressure heads and with different vertical drops to study the flow characteristics in these shunts and determine the optimal catheter placement and length that would reduce the occurrence of low pressure headaches.
The flow rates through catheters of 3 different lengths (60, 83, and 100 cm) with the same internal radius, at 3 different pressure heads (15, 25, and 35 cm H2O to simulate 3 different placements in the lumbar theca), and 3 different vertical drops (10, 20, and 30 cm to simulate the possible effect of siphoning) were measured and the results analyzed.
Application of Poiseuille's law and Bernoulli's principle to the experimental design shows that the volume of flow is directly proportional to the sum of the pressure head and the vertical drop and inversely proportional to the length of the catheter. The flow rate through the standard catheter lengths over the course of 24 hours can be abnormally high. An attempt to predict the optimal catheter length was made.
Although the catheter position in the theca and abdomen cannot be altered significantly and the internal radius of the tube cannot be reduced further without increasing the risk of blockage, the length of the tube can be increased to combat overdrainage. The authors suggest that currently available catheters are too short.
William B. Lo, Kyaw Z. Thant, Jameel Kaderbhai, Nicholas White, Hiroshi Nishikawa, Michael Stephen Dover, Martin Evans, and Desiderio Rodrigues
Children with syndromic, multisuture, and lambdoid craniosynostosis undergoing calvarial surgery often have Chiari malformation type I (CM-I) (or cerebellar tonsillar herniation). The optimal management of this patient group, including the surgical techniques and timing of surgery, remains uncertain. Posterior calvarial distraction (PCD) is an effective method to increase the supratentorial cranial volume and improve raised intracranial pressure in children with complex craniosynostosis. This study investigated the efficacy of PCD in posterior fossa (PF) volume expansion and treatment of CM-I and associated syringomyelia (syrinx) in this group of children.
This retrospective study included patients who were surgically treated between 2006 and 2015. Over 10 years, 16 patients with multisuture synostosis, lambdoid synostosis, or craniosynostosis associated with a confirmed genetic syndrome, and a concurrent CM-I, were included. The mean age at the time of surgery was 5.1 years (range 8 months–18 years). Fourteen patients had pansynostosis and 2 had lambdoid synostosis. Eight had a confirmed syndromic diagnosis (Crouzon in 8, Apert in 4, Pfeiffer in 1, and Saethre-Chotzen in 1). Ten patients had raised intracranial pressure; 4 had syringomyelia.
The average clinical follow-up was 50 months (range 9–116 months). Clinically, 9 patients improved, 7 remained stable, and none deteriorated. The average distraction distance was 23 mm (range 16–28 mm). The PF anterior-posterior (AP) distance/width ratio increased from 0.73 to 0.80 mm (p = 0.0004). Although an osteotomy extending inferior to the torcula (compared with superior) was associated with a larger absolute PF AP distance increase (13 vs 6 mm, p = 0.028), such a difference was not demonstrable when the PF AP distance/width ratio was calculated. Overall, the mean tonsillar herniation improved from 9.3 to 6.0 mm (p = 0.011). Syrinx dimensions also improved in the AP (from 7.9 to 3.1 mm) and superior-inferior (from 203 to 136 mm) dimensions. No patients required further foramen magnum decompression for CM. Of the 16 patients, 2 had subsequent frontoorbital advancement and remodeling, of which 1 was for volume expansion and 1 was for cosmetic purposes. Two patients required CSF shunt insertion after PCD.
Following PCD, PF volume increased as well as supratentorial volume. This morphometric change was observed in osteotomies both inferior and superior to the torcula. The PF volume increase resulted in improvement of cerebellar tonsillar herniation and syrinx. PCD is an efficacious first-line, single-stage treatment for concurrent pansynostosis and lambdoid craniosynostosis, CM-I, and syrinx.