Propranolol Treatment for a Giant Infantile Brain Cavernoma

Abstract

A term infant with Crouzon syndrome underwent a computed tomography (CT) study to rule out the presence of hydrocephalus. Computed tomography revealed hydrocephalus, a large cerebrospinal fluid cyst in the posterior fossa and hypoplastic cerebellar hemispheres with a winged appearance, consisting of Dandy-Walker malformation (Figure 1). The patient underwent a cystoperitoneal shunt insertion to alleviate pressure, and a craniofacial reconstructive procedure was performed at a later time.Crouzon syndrome is a genetic disorder characterized by cranial synostosis, exophthalmos, and midface hypoplasia. The birth prevalence has been calculated to be 16.5 cases per 1 000 000 live births and constitutes approximately 4.8% of all cases of craniosynostosis. 1 Patients with Crouzon syndrome often develop hydrocephalus that requires treatment. Dandy-Walker malformation is a congenital disorder that is characterized by cerebellar dysgenesis, cystic dilation of the fourth ventricle, and large posterior fossa with elevation of the dural sinuses and tentorium. 2 Although Dandy-Walker malformation is usually associated with several other disorders, to our best knowledge this is the first reported case of a patient with Crouzon syndrome. Physicians should be aware of this potential coexistence, given that both disorders have been associated with mental retardation and may act synergistically.

Strategy for Identifying Repurposed Drugs for the Treatment of Cerebral Cavernous Malformation

“…The seven compounds selected from our automated analysis and validated by our secondary screen include compounds from classes previously connected to CCM disease, as well as compounds without any previously described association with the disease (Supplementary Table 2, Supplementary Fig. 3B–H) 47–50 .…”

Preliminary Experiences in Treating Infantile Hemangioma With Propranolol

“…17,18 Clinical experiences in treating infantile hemangioma in a recent study show that propranolol is more effective than corticosteroids, especially for severe and large hemangiomas. 9,19,20 All other treatments have their limits in cases of orbital and nasal hemangiomas, which may lead to severe cosmetic, functional, and psychologic impairments. Propranolol was the first choice because excisional surgery leaves scars and intralesional steroids have a high risk of nasal ulceration and ophthalmic artery occlusion.…”

Letter by Filippidis et al Regarding Article, “Evaluating Strategies for the Treatment of Cerebral Cavernous Malformations”

“…Other candidate drugs, like the ␤-adrenergic receptor blocker, propranolol, demonstrate promising results concerning CCMs and infantile capillary hemangiomas. 2,3 In our current communication, we postulate that oral propranolol administration could provide a novel, alternative approach for the treatment of adults and children with familial cavernous malformation cases of the central nervous system.…”

“…The lesion demonstrated a significant shrinkage after 10 days of propranolol administration. 3 Promising data arise also from the management of cutaneous or nasal capillary infantile hemangiomas with propranolol using the same dosage scheme. 2 Leute-Labreze et al 2 observed the inhibition of growth of capillary infantile hemangiomas, approaching the total remission target by administering 2 to 3 mg propranolol per day by mouth divided into 2 to 3 doses after appropriate cardiology consultation without any side effects or any associated morbidity and mortality.…”