Headache and Diplopia after Rapid Maxillary Expansion: A Clue to Underdiagnosed Pseudotumor Cerebri Syndrome?

Abstract

Rapid maxillary expansion (RME) is an orthodontic procedure that separates the two maxillary bones at the mid palatine suture level. RME is commonly used in the pediatric age group to reduce and/or eliminate a transverse maxillary deficiency. At our institution we followed up an 11-year-old adolescent who was diagnosed with a class III malocclusion and was treated by RME with the combined use of expansion appliances (i.e., Hyrax) in addition to maxillary protraction devices (i.e., Delaire facemask). Three months after the start of treatment, he complained of headache and double vision, and was admitted to our hospital. A funduscopic examination revealed papilledema. Magnetic resonance imaging of the brain showed intraocular protrusion of the optic nerve head and enlarged perioptic subarachnoid space. Cerebrospinal fluid opening pressure during lumbar puncture was elevated (+370?mm H2O), confirming the diagnosis of the pseudotumor cerebri syndrome (PTCS). Removal of the maxillary expander and facemask led to the complete resolution of clinical symptoms in 1 week. The association of RME and PTCS-related manifestations was first reported by Timms in 1986, but, to the best of our knowledge, further cases have been not described in the medical literature. This overlooks PTCS as a potential RME-associated complication. We suggest that clinicians should carefully consider PTCS in all pediatric patients that complain of headache and/or visual disturbances during a treatment by RME. We also speculate on the possible changes of the cerebral venous circulation during RME, potentially leading to an impaired venous drainage that may cause increased intracranial pressure and PTCS.

Ultrasonographic Evaluation of Intracranial Pressure during Rapid Maxillary Expansion

“…In a case report, it was reported that RME treatment may cause raised intracranial pressure in patients with impaired venous drainage and pseudotumor cerebri syndrome (PTCS) by affecting cerebral venous circulation on MRI. 7 In our current investigation, there was a notable increase in ONSD one minute after screw activations (T1, T5) in comparison to the ONSD values prior to screw activations (T0, T4) (p=0.001). While the precise mechanisms underlying the elevation of ICP are not yet fully understood, we speculate that this phenomenon might be attributed to varying levels of stress and cerebral hemodynamic alterations within the intracranial region.…”

“…6,11,19 Importantly, the strain exerted around various cranial base foramina can lead to the enlargement of adjacent blood vessels. 7 In a study on rabbits by Li et al 6 , it was reported that anatomical stresses on craniofacial structures during RME therapy cause cerebral hemodynamic changes, including increases in CBF and CBV. In a case report, it was reported that RME treatment may cause raised intracranial pressure in patients with impaired venous drainage and pseudotumor cerebri syndrome (PTCS) by affecting cerebral venous circulation on MRI.…”

“…5 Li et al 6 determined that intensive anatomical stresses on craniofacial structures during the expansion process can induce cerebral hemodynamic changes, including increased cerebral blood volume (CBV) and cerebral blood flow (CBF). Romeo et al 7 observed that the use of RME may increase intracranial pressure, impairing venous drainage by affecting cerebral venous circulation on magnetic resonance imaging (MRI).…”

Adult maxillary expansion: CBCT evaluation of skeletal changes and determining an efficiency factor between force-controlled polycyclic slow activation and continuous rapid activation for mini-screw-assisted palatal expansion - MASPE vs MARPE.

“…It is well known that maxillary disjunction generates strains and stresses in the cranial structures, especially in the zygomatic arch (29), as it represents a buttress of great resistance against maxillary expansion with high tension exerted in the orbit of the unpaired sphenoid bone, orbital ssure, and the pterygoid processes (30). Headache and diplopia due to increasing intracranial pressure (pseudotumor cerebri syndrome) have been reported (31). Moreover, in both groups even with rapid activation distance between both optic foramen and both pterygoid canals do not change between T1/T2 (Table 1).…”