Multiple intracranial abscesses: Heralding asymptomatic venosus ASD
A 13-year-old Saudi girl presented with 1 week history of headache, vomiting, and fever. She was treated by oral antibiotics with no relief. Two days prior to admission, she started talking incomprehensively, developed photophobia, and ataxia. Patient had chicken pox 4-weeks prior to admission that lasted 2 weeks. She also complained of chronic recurrent headache and fainting attacks with some abnormal movements, 4-5 times in the last 5 years. Each attack lasted a few minutes. She had exertional chest pain, dyspnoea, and palpitation on walking short distance. No treatment was sought for these conditions. There was no history of cyanotic or rheumatic heart disease or chronic infection. She had no exposure to chronic contagious diseases or animals.\nOn admission, HR was 105/min. Temperature and BP were normal and RR 32/min. No cyanosis, clubbing or lymphadenopathy. Paranasal sinuses were normal. No cardiac murmur. Other systemic examinations were normal. Neurologically, she was fully conscious and oriented with gross ataxia. Fundus showed early papilloedema. Muscle tone and power were normal in all the four limbs. There was no nystagmus or tremor. She could not walk without support.\nHematological tests revealed normal hemoglobin and polymorphonuclear pleocytosis with 80% neurtrophils. RBC count was normal. ESR was 62 mm 1st h and CRP 27 mg/L. Serum biochemistry showed raised GGT with reversed albumin globulin ratio. Blood culture was sterile. Tests for other infective and parasitic conditions were normal. Stool did not show any ova or cyst. PPD and HIV, both were negative. X-ray chest, ECG, and transthoracic echocardiogram (TTE) were normal. Transesophageal echocardiography (TEE) was refused by the patient. CT scan of the brain revealed almost 12 identical contrast enhancing ring lesions on both sides of the brain with uniform wall thickness and perilesional edema suggestive of multiple intracranial abscesses []. All the lesions were almost of same size indicating same age. Abdominal and pelvic ultrasounds were normal.\nShe was treated with Ceftriaxone, Vancomycin, Metronidazole, Mannitol, Dexamethasone, and Diphenyl hydantoin. She made significant improvement. Dexamethasone and Mannitol were withdrawn gradually once condition got stabilized. Two weeks after admission, the patient suddenly deteriorated, became bradycardic, lethargic, and had screaming attacks with labile mood and visual hallucinations. No cranial nerve or motor deficits were present. Mannitol and Dexamethasone were restarted. EEG showed abnormal slow waves with underlying multifocal epileptic discharges predominantly on right temporo-occipital region. Repeat CT scan of brain showed increase in the size of right thalamic lesion compressing the posterior III ventricle/aqueduct with obstructive triventricular dilatation and periventricular lucency. There were no changes in other lesions. She underwent stereotactic aspiration of the right thalamic lesion and 5 cc of brownish pus was aspirated. Gram stain showed gram positive cocci in chains. However, the culture was sterile. Repeatedly, blood cultures and TTE were normal. Empirically, Gentamycin was added to the treatment regimen and the patient gradually started improving. Repeat CT scan of brain showed gradual reduction in the size of abscesses. She was given IV antibiotics for 6 weeks and another 8 weeks of oral antibiotics.\nResolution of lesions was very gradual and total clearance of all the lesion took 30 months on MRI. Antiepileptic drugs were continued. Repeat TTE, 36 months later showed enlarged right heart chambers with a suggestion of venosus atrial septal defect (ASD) []. Pulmonary artery pressure (PAP) was 25-30 mmHg. TEE confirmed 1.8 cms sinus venosus ASD from the SVC side [] with anomalous drainage of right upper pulmonary vein. Right heart chambers were dilated. The shunt was left to right. She underwent correction of the defect and did well.