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Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective evaluation was carried out to define the incidence of hydrocephalus and associated factors in 44 patients with Apert syndrome treated at The Hospital for Sick Children in Toronto over a 22-year period. Forty-three of these patients underwent cranioorbital decompressive procedures within 1 year of birth. Fifteen of 25 (60%) patients who had either a computed tomography scan or pneumoencephalogram had ventriculomegaly, and 3 of the 25 (12%) had associated brain anomalies. Ten of the 44 (23%) patients had cerebrospinal fluid (CSF) shunts placed, 7 lumboperitoneal and 3 ventriculoperitoneal. Six of the shunts were placed early after cranioorbital procedures (CSF leaks in 5 cases and a subgaleal fluid collection in 1 case). The average IQ of 15 patients evaluated by the Wechsler Intelligence Scale was 72.5, indicative of significant intellectual impairment. There was no correlation between IQ and ventricular size. Although hydrocephalus characterized by progressive ventricular dilatation is uncommon in Apert syndrome, postoperative problems related to impaired CSF circulation are common and may indicate an underlying CSF absorptive deficit.
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PMID:Hydrocephalus in Apert syndrome: a retrospective review. 768 1

This paper discusses 33 cases of Apert's syndrome which were treated in the Australian Craniofacial unit at the adelaide Children's Hospital. The main features were discussed. We found that mild ventricular dilatation is common in Apert's syndrome but without associated raised intracranial pressure. Severe ventricular dilatation was seen in only one case. No shunt procedures were performed. We also studied the changes in the ventricular size after transcranial corrective procedures. There was no significant change in the ventricular size, the increase in the skull volume was compensated by expansion of the brain tissue and to some extent by increase in the subarachnoid space. Two cases with unusual features are also described.
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PMID:Apert's syndrome. 825 21

Patients with syndromic craniosynostosis are at risk for elevated intracranial pressure because of various physiologic and anatomic abnormalities. The aims of this study were to determine the prevalence of papilledema in syndromic craniosynostosis, to evaluate the results of the treatment, and to examine the risk factors. This is a retrospective study on 84 patients with Apert, Crouzon, or Pfeiffer syndrome. Papilledema was defined as blurring of the margins of the optic disk. The association between clinical symptoms, beaten-copper pattern on skull radiograph, ventricular dilatation on computed tomography scan, and papilledema was assessed. Papilledema was present in 51% of the patients. No relation between specific clinical symptoms and papilledema was found. The significant associations were complex craniosynostosis, exorbitism, and ventricular dilatation. The prevalence of papilledema in patients with Apert, Crouzon, or Pfeiffer syndrome is high, not only before cranial decompression but also after vault expansion. Annual fundoscopy is recommended to screen for papilledema. We consider that early decompressive surgery (within the first year of age) prevents the development of papilledema and, most likely, elevated intracranial pressure.
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PMID:Papilledema in patients with Apert, Crouzon, and Pfeiffer syndrome: prevalence, efficacy of treatment, and risk factors. 1821 76

We report a case of a brain abscess caused by Haemophilus influenzae type e in a 12 year-old patient suffering from Apert syndrome. Apert syndrome is characterized by the premature closure of cranial sutures. In 2010 the patient suffered head trauma in the frontal area with cranial fracture and a cerebrospinal fluid fistula. In February 2013 he was admitted to hospital with fever, vomiting and generalized tonic-clonic seizure with deteriorating mental status/progressive sensory impairment. The computerized axial tomographic scan showed a right frontal lesion, perilesional edema, mild ventricular dilatation and pansinusitis. A brain abscess was diagnosed and drained. The clinical sample was then cultured. A gram negative coccobacillus was isolated and identified as Haemophilus influenzae serotype e. Empirical treatment was started with meropenem (120 mg/kg/day) and vancomycin (60 mg/kg/day), which was later switched to ceftriaxone (100 mg/kg/day) and metronidazole (500 mg/8 h) after culture results arrived. The patient was discharged in good clinical condition.
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PMID:[Brain abscess caused by Haemophilus influenzae type E in a pediatric patient suffering from Apert syndrome]. 2557 11