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Cranial CT of Neurofibromatosis

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Cranial CT of Neurofibromatosis 311 Cranial CT of Neurofibromatosis Charles G. Jacobi The results of computed tomography (CT) of the orbits and brain of 29 patients with Raymundo T. Go neurofibromatosis were reviewed to determine the contribution of CT scanning to the Richard A. Beren diagnosis of this disorder. In the presence of orbital symptoms, CT confirmed the presence of suspected lesions such as optic nerve gliomas, demonstrated asympto­ matic and atypical lesions, and displayed concomitant intracranial involvement of the optic chiasm. CT of the brain revealed a multiplicity of abnormalities such as astrocy­ tomas, intracerebral calcification, hydrocephalus, and congenital lesions. Neurofibromatosis is an inherited disorder resulting in hamartomatous or neoplastic changes in the derivatives of the primary germ layers th at may affect any organ system. Some authors categorize neurofibromatosis into central, peripheral, or mi xed types according to tumor site. The in cidence of central nervous system tumors in 223 patients with neurofibromatosis was 6 times that of the general population [1]. Multiplicity is the predominant characteristic of central nervous system lesions accompanying neurofibromatosis. Li chtenstein [2] presented an overall schema of these lesions and stated that they represent " foci of hyperplasia and neoplasia of the supportive derivatives of the primitive ectoderm ." Some of the more commonly encountered central nervous system tumors include optic gli omas, acoustic schwannomas, meningiomas, piloid astrocytomas, and ependymomas. The plain radiographic find ings of this disease have been comprehensively reviewed [3-5], but we know of only one report dealing with crani al computed tomographic (CT) findings [6]. The purpose of this investigation was to revi ew our experience with cranial CT in patients with neurofibromatosis to determine the contribution of CT scanning in the diag nosis of this disorder. Received July 16, 1979; accepted after revi­ Materials and Methods sion February 7, 1980. From 1973 to 1978, 148 patients with the diagnosis of neurofibromatosis were seen at , All authors: Departm ent of Radiology, Univer­ University of Iowa Hospitals and Clinics. Of these, cranial CT was requested only for those sity of Iowa Hospitals and Clinics, Iowa City, IA 29 patients with appropri ate symptoms. There were 29 examinations of the brain and 16 52242. Address reprint requests to C. G. Jacoby. examinations of the orbits. This article appears in July / August 1980 AJNR We reviewed the medical records of patients undergoing CT and confirmed the diagnosis and September 1980 AJR. of neurofibromatosis using the criterion of six or more cafe au lait spots, each greater than AJNR 1 :311-315, July / AU9ust 1980 0195-6108/80/ 0104-0311 $00.00 1.5 cm in diameter [1). One case (case 21) did not have cutaneous pigmentation but was © American Roentgen Ray Society included because of characteristic radiographic findings and a facial plexiform neuroma. 312 JACOBY ET AL. AJNR: 1. July / Augus1 1980 The most common symptoms (table 1) were visual loss (eight TABLE 1: Predominant Symptoms of 29 Patients with cases) and seizu res (seven cases). Th e other 14 cases demon­ Neurofibromatosis strated a variety of symptoms including hearing loss, endocrine CT Fin dings (No. Patients) disturbance, hemiparesis, ataxia, depression, proptosis, and pe­ Symptom ripheral neuropath y. Abnormal Norm al An EM I Mark 1 scanner, later updated to the EMil 005 configu­ Vi sual lOSS 7 1 ration , was used. One examination was performed with an 80 x 80 Seizures 5 2 matrix and th e remainder were performed with th e 160 x 160 Hearing loss 2 2 matri x. Intravenous contrast enhancement was performed in 21 Endocrine disturbance, rule out hypo- 1 examinati ons. th alami c mass Hemiparesis 1 0 We recorded the gross and histologic descriptions of the lesions Proptosis 3 0 th at had been biopsied and noted the results of contributory radio­ Ataxia . 2 0 graphic examinations (t able 2). We did not evaluate these pati en ts Other 0 2 for macrocranium, a finding reported in up to 75% of children with Total 21 8 neurofibromatosis [7, 8]. Results the larger capacity of the temporal fossa. A subcutaneous Ei ght patients had normal CT examinations. CT of the soft-tissue density extended from th e left ear to the left orbit. oth er 2 1 pati ents (72%) showed one or more abnormality of The left globe was displaced inferiorly. A diffuse soH-tissue the orbits or brain (table 2). The average age of patients mass th at enhanced slightly after contrast infusion was with abnormal examinations was 12 years (range, 2'12 -61). present in the left retrobulbar area and obliterated the optic A family history of neurofibromatosis was found in nine nerve. Several operations have been performed to debulk pati ents and subcutaneous nodules were demonstrated in the facial tumor and to decompress the orbit; the histologic 14 pati ents. reports have shown plexiform neurofibroma in both loca­ tions. Orbital Lesions Pl ain skull films were also available for review in seven patients with orbital abnorm alities. One examination was Of th e 10 patients with demonstrated orbital abnormali­ norm al (case 2). Four cases showed enl argement of the ti es, six had discrete optic nerve lesions consistent with optic canal (cases 1, 4, 5, and 9); case 8 demonstrated a gli oma (cases 1 , 3, 5-7, and 9). One case (case 1) was calcifi ed orbital mass and case 21 confirmed the sphenoidal confirmed by biopsy. Exploration of the opposite optic nerve dysplasia. in this patient showed tumoral swelling th at was not detected by CT. The optic chiasm was normal by CT, pneumoen­ c hephalography, and operative inspection. In three of the Intracranial Abnormalities six cases of opti c gli oma (cases 5-7), a soft-tissue mass of We found intracranial lesions in 16 patients (cases 5-20). the opti c c hi asm was demonstrated by CT (fig . 1). CT in one In case 12, CT showed bilateral enhancing masses in the case (case 2) showed a diffuse density of the retrobulbar cerebellopontine angle cisterns, and pl ain skull examination ti ssues with scleral contrast enhancement, findings not typ­ demonstrated bilateral erosion of the internal auditory ca­ ical of optic gli oma. After biopsy and orbital exenterati on, nals. Although not confirmed histologically, the diagnosis of th e histologic evaluation was malignant schwannoma (fig. bilateral acoustic neurinoma is highly probable. CT identified 2) soft-tissue masses in the region of th e optic chiasm in seven CT in another case (case 4) demonstrated a diffuse retro­ patients (cases 5-7, 10, 11, 18, and 20). Biopsy results of bulbar mass with th e additional finding of globe enl arge­ case 11 indicated a gangiogli oma (fig. 5). ment. A plexiform neurofibroma that had infiltrated the globe CT in six cases demonstrated intraaxial masses. Four was found at operation. showed attenuation less than that of brain tissue (cases 9, A densely calc ified mass along th e optic nerve in case 8 14, 16, and 19) and two showed contrast enhancement was correctly diagnosed as a meningioma of th e optic nerve (cases 11 and 15). Three cases were biopsied revealing low sheath (fig . 3 A). During resection of this lesion, an en plaque grade astrocytoma (cases 14-1 6). meningioma of th e frontal lobe was discovered whic h, even Abnormalities of the ventricular system were seen on in retrospect, was not evident on CT. CT in thi s case also three examinations. Two demonstrated hydrocephalus and revealed several sites of punctate calcification with no as­ one demonstrated agenesis of the corpus callosum and a sociated contrast enhancement (fig. 3B). These areas were midline frontal cystic mass having the appearance of an asymptomatic and were not bi opsied. arachnoid cyst. We did not encounter the characteristic CT in one case (case 21 ) demonstrated extensive unilat­ calvarial lucency in th e lambdoid suture [4]. eral abnormalities of the skull and extracrani al soft tissues (fig. 4). The left greater wing of the sphenoid was dysplastic, resulting in the absence of th e posterior orbital wall and Discussion enlargement of the middle cranial fossa. The ipsil ateral sylvian fissL're was wide, suggesting th at th e volume of brain When evaluating patients with neurofibromatosis who de­ tissue was n ') t increased but was merely accommodating velop visual loss or proptosis, we found CT of the orbits AJNR: 1 , July / August 1980 CT OF NEUROFIBROMATOSIS 313 TABLE 2: CT Abnormalities in Neurofibromatosis Age (yrs). Case No. Symptoms Gender CT Findings Other Stu dies Confirmation 1 3 12, F Vi sual loss R optic nerve mass Rad: enlarged R opti c OP: R optic nerve mass, canal; PEG: neg neg. c hiasm, L opti c nerve swelling ; BX: astrocytoma 1 2 2 12 , F Visual loss Infiltrating L orbital mass Rad : neg Mali gnant schwan noma 3 3 112 , F Vi sual loss L optic nerve mass PEG: neg chiasm 4 17, F Proptosis Infiltrating R retrobulbar Rad: R orbital enlargement Pl exiform neu rofibroma mass; enlarged R globe with infiltrati on of choroid layer of the eye 5 3 , F Proptosis L optic nerve mass; R optic Rad: enlarged Rand L op- nerve thickening; chias- ti c canals mal mass 6 9, F Vi sual loss R optic nerve mass; chias- mal mass 7 17, F Primary R optic nerve mass; chi as- amenorrh ea mal mass 8 20, M Vi sual loss Calcified R optic nerve Rad : calcified R orbital BX: men ingioma mass; R cerebell ar calci- mass fi cati on; L choroid plexus calcificati on at foramen of Munro 9 6 1, F Vi sual loss R opti c nerve thickening: Rad: enlarged R
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