CHOROID PLEXUS TUMOR IN A CHILD M. Anne St John, FRCPC, FAAP, and Hadley Clarke, FRCSC, FRCS Barbados, West Indies
A 5-month-old infant is reported who presented with fever, nonspecific symptoms, a rapidly enlarging head, and a regression in motor development. Investigations subsequently confirmed the diagnosis of a choroid plexus papilloma. (J NatI Med Assoc. 1992;84:888891.) Key words * choroid plexus tumors * choroid plexus papillomas * intracranial tumors Choroid plexus papillomas are rare lesions that are seen more frequently in children.I-I° They comprise less than 1 % of intracranial tumors, the first case having been documented in 1833 by Guerard et al. I In a recent series reviewed by Humphreys et al,2 52% of 23 children with choroid plexus tumors were under the age of 1 year. These lesions should be suspected in cases of hydrocephalus where there appears to be no obvious cause or neurological deficit.
CASE REPORT A 5-month-old male child was referred by a general practitioner and admitted to the Queen Elizabeth Hospital, St Michael, Barbados, West Indies on July 16, 1988 with a 2-day history of having a large head, drowsiness, fever, irritability, and poor feeding. He was an only child and had been born in the Queen Elizabeth Hospital to a 30-year-old gravida 5 para 4 mother, by a normal vaginal delivery with a birthweight of 2880 kg, head circumference 32 cm, and length 53 cm. The infant had a normal neonatal period. Developmental history From the Departments of Pediatrics and Surgery, Queen Elizabeth Hospital, Barbados, West Indies. Requests for reprints should be addressed to Dr M. Anne St John, Queen Elizabeth Hospital, Martindales Rd, St Michael, Barbados, West Indies. 888
revealed that he was not able to lift his head off the bed nor was he able to sit up. He had received three combined diphtheria, pertussis, and tetanus immunizations. A physical examination revealed a well-nourished, but irritable infant. He was febrile (37.2°C, axillary) with a pulse of 128/minute, weighed 8 kg (75th percentile), had a head circumference of 47 cm (>95th percentile), and his length was 74 cm (97th percentile). Both fontanelles were open and flat. He had a significant degree of head lag and was unable to lift his head off the bed or roll over. He did not follow or focus on objects with his eyes, the latter having a "setting sun" appearance. His fundi revealed Grade 1 papillodema with congested reddened discs. He was hyperreflexic. The rest of his physical examination was normal. Investigations revealed normal complete blood count, serum electrolytes, and blood glucose. A lumbar puncture revealed increased cerebrospinal fluid (CSF) pressure, increased CSF protein (600 mg/L), normal CSF glucose, and a cell count of 87 red cells and 0 white cells, India ink and Gram stain negative. A throat swab grew normal flora, and CSF and blood cultures were negative. A skull radiograph showed widened coronal and sagittal sutures, with thinning of the outer table. On July 22, an axial computerized tomography (CT) scan revealed marked ventricular dilatation, with a large enhancing lesion in the right occipital horn of the lateral ventricle and marked hydrocephalus (Figures 1 and 2). During the next few days, his head circumference increased to 48.5 cm. He was evaluated by the neurosurgeon and then transferred to The Hospital for Sick Children, Toronto, Canada on July 27 for surgery. A carotid angiography investigation did not show a tumor blush. Surgery was performed 48 hours later, and the mass in the right lateral ventricle was excised. A postoperative CT scan revealed that the entire tumor had been removed. Histology reports confirmed a JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 10
CHOROID PLEXUS TUMORS
Figure 1. Unenhanced axial CT scan of the brain showing marked ventricular dilatation. The 4th ventricle is communicating widely with a dilated cisterna magna.
Figure 2. Enhanced axial CT scan of the brain showing marked ventricular dilatation with a large enhancing lesion in the right occipital horn of the lateral ventricle.
benign choroid plexus papilloma. The initial postoperative clinical examination revealed a mild left-sided weakness with a major visual loss, without an ability to fixate or follow light. The wound healed well, and the patient was discharged from the hospital on August 20. At a follow-up visit on September 16, he was observed to have searching ocular movements and bilateral subdural effusions. He was readmitted for insertion of a subdural peritoneal shunt. He and his mother subsequently remained in Canada and returned to Barbados during the first week of December 1989. A few days later, he was admitted to the Queen Elizabeth Hospital pediatric medical ward because of shaking of his left hand for 1 minute. His physical examination was normal except for tonic-clonic movements of his left upper limb. He was unable to follow or fixate on a light. His head circumference was 53.5 cm. He was treated with intravenous phenobarbital, and the seizure movements disappeared. He was continued on phenytoin in maintenance doses. On December 13, an enhanced CT scan (Figure 3) showed dilatation of all ventricles and a subdural effusion in the right occipital region. The patient became febrile. A shunt infection was suspected, and he was treated with intravenous cloxacillin and gentamicin. A new shunt was inserted on the next day. Postoperatively, he again became febrile. Shunt fluid was taken from the reservoir and his
antibiotic therapy was changed to ceftazidime for 1 week. An area of redness was subsequently observed over the shunt site on his scalp, and parenteral cloxacillin treatment was restarted. Within a few days, he became afebrile and was discharged on oral phenytoin and a 10-day course of cloxacillin orally. His developmental follow-up examination in February 1990 revealed that he could say two-word phrases, see objects from his left eye, and was stepping with support and reaching for objects. He had an obvious strabismus and nystagmus. His head circumference was 53 cm (>95% percentile). Phenytoin was discontinued, and he was referred to the Children's Development Centre and an ophthalmology clinic. He was subsequently admitted to the the Queen Elizabeth Hospital on March 20 with a 2-hour history of continuous seizures involving the left upper and lower limbs, and the face. On examination, he was afebrile, drowsy, and responded poorly to pain. His weight was 14 kg, respiratory rate 24/minute, and pulse 144/minute. Significant abnormal findings included tense shunt reservoir pressures and bilateral nystagmus to the left. Investigations included a normal blood count, differential, and electrolytes. An unenhanced CT scan performed (Figure 4) revealed ventricular dilatation with the shunt catheter tip positioned in the right frontal region and a small subdural effusion in the right occipital region. He received a loading dose of phenytoin and was
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 10
889
CHOROID PLEXUS TUMORS
....
Figure 3. Enhanced CT scan of the bra'in showing dilatation of the ventricular system and a subdural effusion in the right occipital region. TABLE. REPORTED INCIDENCE OF CHOROID PLEXUS PAPILLOMA IN CHILDREN Year %
A 5-month-old infant is reported who presented with fever, nonspecific symptoms, a rapidly enlarging head, and a regression in motor development. Investigations subsequently confirmed the diagnosis of a choroid plexus papilloma. (J NatI Med Assoc. 1992;84:888891.) Key words * choroid plexus tumors * choroid plexus papillomas * intracranial tumors Choroid plexus papillomas are rare lesions that are seen more frequently in children.I-I° They comprise less than 1 % of intracranial tumors, the first case having been documented in 1833 by Guerard et al. I In a recent series reviewed by Humphreys et al,2 52% of 23 children with choroid plexus tumors were under the age of 1 year. These lesions should be suspected in cases of hydrocephalus where there appears to be no obvious cause or neurological deficit.
CASE REPORT A 5-month-old male child was referred by a general practitioner and admitted to the Queen Elizabeth Hospital, St Michael, Barbados, West Indies on July 16, 1988 with a 2-day history of having a large head, drowsiness, fever, irritability, and poor feeding. He was an only child and had been born in the Queen Elizabeth Hospital to a 30-year-old gravida 5 para 4 mother, by a normal vaginal delivery with a birthweight of 2880 kg, head circumference 32 cm, and length 53 cm. The infant had a normal neonatal period. Developmental history From the Departments of Pediatrics and Surgery, Queen Elizabeth Hospital, Barbados, West Indies. Requests for reprints should be addressed to Dr M. Anne St John, Queen Elizabeth Hospital, Martindales Rd, St Michael, Barbados, West Indies. 888
revealed that he was not able to lift his head off the bed nor was he able to sit up. He had received three combined diphtheria, pertussis, and tetanus immunizations. A physical examination revealed a well-nourished, but irritable infant. He was febrile (37.2°C, axillary) with a pulse of 128/minute, weighed 8 kg (75th percentile), had a head circumference of 47 cm (>95th percentile), and his length was 74 cm (97th percentile). Both fontanelles were open and flat. He had a significant degree of head lag and was unable to lift his head off the bed or roll over. He did not follow or focus on objects with his eyes, the latter having a "setting sun" appearance. His fundi revealed Grade 1 papillodema with congested reddened discs. He was hyperreflexic. The rest of his physical examination was normal. Investigations revealed normal complete blood count, serum electrolytes, and blood glucose. A lumbar puncture revealed increased cerebrospinal fluid (CSF) pressure, increased CSF protein (600 mg/L), normal CSF glucose, and a cell count of 87 red cells and 0 white cells, India ink and Gram stain negative. A throat swab grew normal flora, and CSF and blood cultures were negative. A skull radiograph showed widened coronal and sagittal sutures, with thinning of the outer table. On July 22, an axial computerized tomography (CT) scan revealed marked ventricular dilatation, with a large enhancing lesion in the right occipital horn of the lateral ventricle and marked hydrocephalus (Figures 1 and 2). During the next few days, his head circumference increased to 48.5 cm. He was evaluated by the neurosurgeon and then transferred to The Hospital for Sick Children, Toronto, Canada on July 27 for surgery. A carotid angiography investigation did not show a tumor blush. Surgery was performed 48 hours later, and the mass in the right lateral ventricle was excised. A postoperative CT scan revealed that the entire tumor had been removed. Histology reports confirmed a JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 10
CHOROID PLEXUS TUMORS
Figure 1. Unenhanced axial CT scan of the brain showing marked ventricular dilatation. The 4th ventricle is communicating widely with a dilated cisterna magna.
Figure 2. Enhanced axial CT scan of the brain showing marked ventricular dilatation with a large enhancing lesion in the right occipital horn of the lateral ventricle.
benign choroid plexus papilloma. The initial postoperative clinical examination revealed a mild left-sided weakness with a major visual loss, without an ability to fixate or follow light. The wound healed well, and the patient was discharged from the hospital on August 20. At a follow-up visit on September 16, he was observed to have searching ocular movements and bilateral subdural effusions. He was readmitted for insertion of a subdural peritoneal shunt. He and his mother subsequently remained in Canada and returned to Barbados during the first week of December 1989. A few days later, he was admitted to the Queen Elizabeth Hospital pediatric medical ward because of shaking of his left hand for 1 minute. His physical examination was normal except for tonic-clonic movements of his left upper limb. He was unable to follow or fixate on a light. His head circumference was 53.5 cm. He was treated with intravenous phenobarbital, and the seizure movements disappeared. He was continued on phenytoin in maintenance doses. On December 13, an enhanced CT scan (Figure 3) showed dilatation of all ventricles and a subdural effusion in the right occipital region. The patient became febrile. A shunt infection was suspected, and he was treated with intravenous cloxacillin and gentamicin. A new shunt was inserted on the next day. Postoperatively, he again became febrile. Shunt fluid was taken from the reservoir and his
antibiotic therapy was changed to ceftazidime for 1 week. An area of redness was subsequently observed over the shunt site on his scalp, and parenteral cloxacillin treatment was restarted. Within a few days, he became afebrile and was discharged on oral phenytoin and a 10-day course of cloxacillin orally. His developmental follow-up examination in February 1990 revealed that he could say two-word phrases, see objects from his left eye, and was stepping with support and reaching for objects. He had an obvious strabismus and nystagmus. His head circumference was 53 cm (>95% percentile). Phenytoin was discontinued, and he was referred to the Children's Development Centre and an ophthalmology clinic. He was subsequently admitted to the the Queen Elizabeth Hospital on March 20 with a 2-hour history of continuous seizures involving the left upper and lower limbs, and the face. On examination, he was afebrile, drowsy, and responded poorly to pain. His weight was 14 kg, respiratory rate 24/minute, and pulse 144/minute. Significant abnormal findings included tense shunt reservoir pressures and bilateral nystagmus to the left. Investigations included a normal blood count, differential, and electrolytes. An unenhanced CT scan performed (Figure 4) revealed ventricular dilatation with the shunt catheter tip positioned in the right frontal region and a small subdural effusion in the right occipital region. He received a loading dose of phenytoin and was
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 84, NO. 10
889
CHOROID PLEXUS TUMORS
....
Figure 3. Enhanced CT scan of the bra'in showing dilatation of the ventricular system and a subdural effusion in the right occipital region. TABLE. REPORTED INCIDENCE OF CHOROID PLEXUS PAPILLOMA IN CHILDREN Year %
