Simultaneous giant hydatid cysts of brain and liver.

While hepatic hydatid cysts are most common in occurrence, intracranial hydatid cyst is less common. Simultaneous presence of both varieties is quite rare and poses a challenge for management in terms of involvement of multidisciplinary team and multiple interventions. An 8-year boy presented with neurological symptoms of a space occupying lesion. There was also hepatomegaly. Radiological investigations revealed giant hydatid cysts involving left cerebral hemisphere and left lobe of liver. Cerebral hydatid cyst was operated first by pediatric neurosurgeons. After 10 days of stabilization period, hepatic lesion was removed by pediatric surgeons. Patient showed uneventful recovery and discharged on oral albendazole for 6 months. There was no recurrence at follow-up.

Transorbital penetrating brain injury to frontal lobe by a wheel spoke.

Penetrating brain injury (PBI) is rare and the severest form of head injury with a high morbidity and mortality. A 3.5-year-old girl presented with PBI with a wheel spoke. Computerized tomography scan with three-dimensional skull reconstruction depicted its extent from the medial side of the roof of the right orbit to the right frontal lobe with a cavitation around the spoke. The spoke was removed by manipulation under general anesthesia from the entry site without a formal craniotomy. Postoperative outcome was uneventful.

Childhood primary angiitis of the central nervous system.

OBJECTIVE: To analyze the clinical course and magnetic resonance angiographic (MRA) abnormalities in children with primary angiitis of the central nervous system (cPACNS). STUDY DESIGN: Cohort study. PLACE AND DURATION OF STUDY: Neurosciences and Neuroradiology Department of the Children's Hospital, Lahore, from January 2009 to December 2010. METHODOLOGY: The cohort comprised consecutive patients diagnosed as having cPACNS based on clinical findings and identification of arterial stenosis on magnetic resonance angiography (MRA) in the absence of an underlying condition that could cause these findings. The treatment protocol for ischaemic infarcts consisted of induction therapy with intravenous steroids pulses and intravenous immunoglobulin followed by maintenance therapy with azathioprine and low dose aspirin. When indicated, they were treated with anticoagulants at least for 4 weeks along with induction therapy. Patients were followed at a single centre and systemically assessed for clinical presentation, classification of disease as progressive or non-progressive, adverse effects of anticoagulants, aspirin, azathioprine and their hospital course. RESULTS: Sixty-eight children with medium-large vessel cPACNS (62% boys, 38% girls) with mean age of 8.5 ± 3.5 years were enrolled in this study. Motor deficit (70%); headache (64%) and fever (20%) were the commonest symptoms; whereas hemiparesis (60%); seizures 55% (focal 35%, generalized 20%) and decreased conscious level (30%), were the commonest neurological findings. Neuroradiological findings were ischaemic strokes in 50 (73.5%), haemorrhagic strokes in 10 (14.7%) and ischaemic haemorrhagic lesions in 8 cases (11.8%). Angiographically 51 (51/68, 75%) of the cohort had non-progressive (obliterative) and 17 (17/68, 25%) had evidence of progressive arteriopathy at the time of admission. No secondary haemorrhagic lesions were documented among infarcts strokes, which were treated with heparin and oral anticoagulants. Outcome was survival in 56 cases (81.5%) and death in 12 cases (18.5%). All survivors were discharged on long-term oral aspirin; 15 of them were also commenced on azathioprine. Neurological findings among the 56 survivors were; normal 20%, minor disabilities in 25%, moderate disabilities in 20% and severe disabilities in 35%. CONCLUSION: The spectrum of cPACNS includes both progressive and non-progressive forms with significant morbidity and mortality. This treatment protocol of immunosuppressive therapy may improve long-term neurological outcome in children with medium-large vessel childhood primary angiitis of the CNS.

Aplasia cutis congenita scalp presenting with life threatening hemorrhage: a case report.

Aplasia cutis congenita is a rare congenital anomaly characterized by the absence of a patch of skin since birth. It may lead to life threatening complications at times. A 5-day-old neonate with Aplasia cutis congenita was received in a state of shock due to tremendous blood loss from the superior sagittal sinus. The neonate was resuscitated immediately followed by closure of the superior sagittal sinus and flap coverage to the defect as a life saving procedure.

Neurofibromatosis type II: a rare neurocutaneous syndrome.

Neurocutaneous syndromes are heterogeneous group of disorders with abnormalities of central as well as peripheral nervous system. Neurofibromatosis type II (NF-II) is an autosomal dominant neurocutaneous syndrome rarely diagnosed in pediatric population. Diagnosis is based on clinical history and radioimaging. We present a 14 years old boy with headache and decreased hearing, who turned to be a case of neurofibromatosis type II.