A Cavernous Malformation in a Child: When Is It Symptomatic? When Is It Surgical?
A Cavernous Malformation in a Child: When Is It Symptomatic? When Is It Surgical?
Abstract & Commentary
By Dara Jamieson, MD, Associate Professor of Clinical Neurology, Weill Cornell Medical College. Dr. Jamieson reports she is a retained consultant for Boehringer Ingelheim, Merck, and Ortho-McNeil, and is on the speaker's bureau for Boehringer Ingelheim and Merck.
Synopsis: Cavernous malformations are found in the brain and spinal cord of children but the correlation between the symptoms at diagnosis and the lesion should be considered prior to referral for surgical resection.
Sources: Xia C, Zhang R, Mao Y, et al. Pediatric cavernous malformation in the central nervous system: report of 66 cases. Pediatr Neurosurg 2009;45:105-113; Acciarri N, Galassi E, Giulioni M, et al. Cavernous malformations of the central nervous system in the pediatric age group. Pediatr Neurosurg 2009;45:81-104.
Cavernous malformations (CM) are common symptomatic, or incidentally discovered, intraparenchymal lesions in adults but they are less frequently diagnosed in children. However, two recently published studies of children with CMs illustrate that these potentially symptomatic cerebrovascular lesions can present with a variety of neurological complaints in children and can be successfully treated with microsurgery.
Xia and colleagues retrospectively reviewed clinical data and treatment outcome for 66 patients younger than age 18 with CMs who were treated by neurosurgeons in a Chinese hospital from 1998 to 2007. The mean age at diagnosis of the CM was 11.6 years (range from 15 months to 17.8 years); 60% of the children were boys. The diagnosis of CM was made after presentation with seizures (48%), headache (46%), intracranial hemorrhage (20%), and focal neurological deficits (12%). Multiple CMs were found in seven children. The majority of CMs were supratentorial (83%); they were less commonly infratentorial (9%) or in both locations (6%). One child had an intramedullary spinal cord CM. Microsurgical resection was performed on 62 of 66 children, with generally beneficial results. When 46 children were followed after surgery, the majority improved and only one boy needed continued seizure prophylaxis. The three children who did not undergo surgical resection (two with headache and one with seizures) had spontaneous disappearance of symptoms and stable intracerebral lesions on follow-up MRI scanning. The authors noted the controversies surrounding the treatment of CMs, but opined that for symptomatic solitary CM, the treatment of choice is complete microsurgical excision. Treatment of multiple CMs deserves more cautious consideration.
Acciarri and colleagues published a retrospective study of a series of 42 children with symptomatic CMs (35 supratentorial, five infratentorial, and two spinal) who were operated on between 1975 and 2005 in an Italian hospital; clinical follow-up ranged from 12 to 192 months. Seizures, present in 28 children, were the most common neurological symptom leading to diagnosis. While recurrent headache was present in 11 children, it was the only symptom in two children. Surgical treatment produced excellent or good results in 69% of the children. Unchanged neurological deficits were observed in 24% of cases, while morbidity from surgical procedures was 7%. No child died from surgery. Surgical risk was greatest with deep, critically located lesions. In addition to a detailed analysis of the patients in the surgical series, the paper by Acciarri and colleagues provided a careful and detailed review of the medical literature on CMs, providing almost 200 references.
Commentary
Intracerebral and spinal cavernous malformations, which may be present in up to 1% of the adult population, can be found in up to 0.5% of children, with reported bimodal peaks for symptomatic CM in toddlers and young teens. The lesions can be sporadic, when they are generally singular; or familial, when they are often multiple. In both of these studies, CMs were more common in the left hemisphere and in the frontal and temporal lobes in children. The Italian authors noted that besides the familial form of the disease, which is often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for central nervous systems tumors.
As with adults, the most common presentation for CMs in children is a seizure disorder. While these series of pediatric CMs published by neurosurgeons indicate improved outcome after surgical resection, these children may have had medically intractable seizures prior to neurosurgical referral. These studies do not indicate how successfully children with seizures, presumably related to CMs, can be medically treated with medication prior to consideration of resection of a single lesion in eloquent cortex. Resection of a symptomatic CM, especially in a deep cortical location, should be considered only after seizures cannot be controlled medically.
Headaches are the second most common symptom noted at diagnosis of CM, occurring in almost half the children in the Chinese neurosurgical paper. A CM may present with headache in association with hemorrhage or obstructive hydrocephalus causing increased intracranial pressure. Isolated headache was less common in the Italian children, reflecting the prevalence noted in the literature. The Chinese paper did not indicate whether the headaches were isolated or associated with other symptoms. As primary headache is common in children and an incidental intraparenchymal CM may be picked up on screening brain imaging, the causal relationship between CM and isolated headache is suspect. Therefore, neurosurgical resection in the case of isolated headache may not be indicated.
Cavernous malformations are found in the brain and spinal cord of children but the correlation between the symptoms at diagnosis and the lesion should be considered prior to referral for surgical resection.Subscribe Now for Access
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