HYDROCEPHALUS OF THE INFANT (ABOUT 86 CASES)

Transcription

1 HYDROCEPHALUS OF THE INFANT (ABOUT 86 CASES) K.EL KHOU;R.ANDALOUSSI;L.OUZIDANE Pediatric radiology department-chu Ibn Rochd Casablanca-Morroco Morroco.

2 Introduction Hydrocephalus of infant is a progressive distension of the ventricles,caused by an anomaly of production or circulation of the cerebrospinal fluid. It has a great impact on the neurologic and psychomotor development. It benefited a lot from the development of the neuroradiology and from the surgery.

3 Patients and methods It s s a retrospective study of 86 cases of infant hydrocephalus hospitalized in de neurosurgery department of the Ibn Rochd hospital of Casablanca on a period of 5 years.

4 Epidemiology RESULTS Female (52.3%)>male (47.7%). Age:0-24 months (most patients are under 3 months old). Maternal antecedents: Infections(14%) Drug administration(9%). Consanguinity (12.8%). Personal antecedents: Meningitis (20%) Fetal distress (7%). Infections (6%). No antecedents (61%).

5 Clinical RESULTS Symptoms: Increasing head circumference (48%). Spinal dysraphism(38%). Myelomeningocele ( Encephalocele. Intracranial hypertension and neurological troubles (50%): Psychomotor retardation (17%). Paraplegia (6%),paraparesis (13%). Convulsions (6%). Optic disorders: Setting sun sign (32.5%). Strabism (5%). Papilledema (5%). Optical atrophy (5%).

6 Diagnostic tests RESULTS Skull radiographs: Realised at 45 patients Normal (47%). Intracranial Intracranial hypertension signs (40%): Increasing head circumference. Disjunction of the sutures. Central markings. Calvaria thinning. Osseous gap of the base of the occiput (13%).

7 Diagnostic tests RESULTS Spine radiograph: Realized at 20 patients(23%). Spina bifida: Lumbo-sacral sacral(11%). Lumbar (7%). Dorso-lumbar (2.3%). Cranial sonography: Realized at 49 patients (57%). Confirmed the diagnosis of hydrocephalus: Triventricular hydrocephalus (36%) Biventricular hydrocephalus (13%). Tetraventricular hydrocephalus (7%). Etiology: Determined in 17%. Arnold-chiari deformity (12%). Meningo-encephalocele encephalocele (3.5%). Aqueduct of sylvius stenosis (1.2%).

8 Transfontanellar ultrasonography: Hydrocephalus with signs of ventriculitis.

9 Diagnostic tests RESULTS CT-scan scan: Performed at 55 patients(64%) Identified the etiology in 42% of cases: MRI: Aqueduct of sylvius stenosis (12%). Dandy walker syndrome (10%). Tumor (7%) Arnold-chiari deformity (5%). Encephalocele (4%) Undetermined etiology (24%). Performed at 3 patients (3.5%) Showed: Cerebellar tumor (1 case) Dandy walker syndrome(1 case) Meningo-encephalocele encephalocele(1 case)

10 Triventricular hydrocephalus with reduced cerebral mantle on an Arnold Chiari malformation.

11 Triventricular hydrocephalus on an Arnold Chiari malformation associated to a dysgenesis of corpus callosum

12 CT-scan: hydrocephalus with septums and signs of ventriculitis.

13 Ct-scan: Hydrocephalus on a Dandy-Walker malformation

14 CONTRAST ENHANCEND MRI: Tumor of the posterior fossa with upstream hydrocephalus.

15 Etiology RESULTS Spinal dysraphism (24.4%) Arnold chiari deformity (16.3%) Meningitis (16.3%) Aqueduct of sylvius stenosis (12.8%). Dandy walker malformation (10.5%) Tumors (7%). Proencephalic cavity (2.3%) Unknown (10.5%)

16 Treatment RESULTS Ventriculoperitoneal shunt 94%. External derivation 1.2%. Therapeutic abstention 3.6%. Evolution: complications(22%). Valve disfunction (3.6%). Meningitis (14%). Respiratory infection,parietal, suppuration. Death (2 cases)

17 Pathophysiology DISCUSSION Hydrocephalus can be subdivided into the following 3 forms: Disorders of CSF production: This is the rarest form of hydrocephalus. Choroid plexus papillomas and choroid plexus carcinomas can secrete CSF in excess of its absorption. Disorders of CSF circulation: This form of hydrocephalus results from obstruction of the pathways of CSF circulation. This can occur at the ventricles or arachnoid villi. Tumors, hemorrhages, congenital malformations (such( as aqueductal stenosis), and infections can cause obstruction at either point in the pathways. Disorders of CSF absorption: Conditions, such as the superior vena cava syndrome and sinus thrombosis, can interfere with CSF absorption.

18 Epidemiology DISCUSSION Incidence: 4 to 10 per 1000 births in USA. Sex-ratio male/female :1.5 Age of discovery :0-6 months. Maternal age :There: is a higher risk of congenital malformations when the maternal age is over 35 years. Maternal antecedents: Infections. Drug administration. Maternal diabetes. Pregnancy evolution: Mechanical dystocia++. Consanguinity++.

19 Clinical DISCUSSION Symptoms: Increasing head circumference. Intracranial hypertension signs and neurological disorders: headache, gait disturbance, vomiting, and visual changes Psychomotor retardation. Optic disorders: Setting sun sign+++. Strabism,nystagmus,,nystagmus,upgazeupgaze palsy. Optic atrophy,papilledema papilledema (hydrocephalus is considered as the third cause of optic atrophy of children after ventricular hemorraege and brain tumors). Associated malformations: Spinal dysraphism : First cause of congenital hydrocephalus. myelomeningocele.+++ Polymalformative syndrome.

20 Diagnostic tests DISCUSSION The introduction of modern imaging techniques, as brain CT- scan,transfontanellar ultrasonography,mri and brain doppler, allowed a less invasive diagnosis in comparison with ventriculography and pneumoencephalography.

21 Skull radiographs DISCUSSION Increasing head circumference. Disjunction of the sutures. Calvaria thinning. Etiology research :ex calcifications in toxoplasmosis. Evaluation of ventriculoperitoneal shunt disconnection or displacement of the shunt catheter.

22 Transfontanellar echography DISCUSSION Original reports discuss the limited use of US in patients with hydrocephalus and congenital cerebral anomalies, but current applications greatly expand the role of US in the evaluation of the developing central nervous system. In newborns,, 5-5 to 7.5-MHz transducers are used, and 3- to 5-MHz 5 transducers are used in older infants. US is suited especially for evaluating the ventricular system and the cerebral mantle.

23 Brain CT-scan DISCUSSION CT scan of the head delineates the degree of ventriculomegaly and,, in many cases, the etiology. When performed with contrast, it can show infection and tumors that cause obstruction. It also helps with operative planning and for the evaluation of suspected shunt malfunction. It can show associated malformations: Aqueduct of sylvius stenosis. Arnold-chiari malformation,cystic malformations of the posterior fossa. Dysgenesis of corpus callosum. Calcifications.

24 MRI DISCUSSION Malformations of the CNS are best delineated using MRI. MRI usually is required for better anatomic resolution prior to surgical intervention. Nonenhanced routine cranial MRI can image the altered anatomy and provide excellent images in all projections (sagittal, axial, coronal), of which the sagittal view is one of the most useful. This delineates the extent of associated brain anomalies such as corpus callosum agenesis, Chiari malformations, disorders of neuronal migration, and vascular malformations. T2-weighted images can show transependymal flow of cerebrospinal fluid (CSF).

25 Brain doppler DISCUSSION Transcranial doppler helps to study the flow in big vessels and the brain hemodynamic of infant and children in physiologic and noninvasive conditions. It can show an impairment of the cerebral blood flow related to an increasing intracranial pressure. It s s a recent method that s not enough evaluated.

26 Antenatal diagnosis DISCUSSION US: Helps for the diagnosis of some cerebral malformations such as: anencephalia,encephalocele encephalocele,myelomeningocele,hydrocephalus and posterior fossa cyst. Hydrocephalus can be diagnosed from 17 weeks of gestation. Fetal brain MRI: It s s a simple non invasive examination that is used when a malformation is suspected on the antenatal US. Alphafoetoprotein dosage : The concentration of this protein in the amniotic fluid is increased when there are anomalies in the closure of the neural tube. Amniocentesis and caryotype studies: To search associated chromosomic anomalies.

27 Etiology DISCUSSION Congenital hydrocephalus: Spinal dysraphism++: Myelomeningocele++: Hydrocephalus is associated to myelomeningocele in 65% to 95% of cases. The lumbar location is the most frequent. Is almost always associated to cerebral malformations (Arnold Chiari deformity). MRI++, CT. Aqueductal stenosis: Second etiology of congenital hydrocephalus. Familial occurrence has been often reported. CT: triventricular dilatation with normal fourth ventricle. MRI:more specific to show the level of obstruction.

28 Etiology DISCUSSION Dandy walker syndrome: This syndrome is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. Approximately 70-90% of patients have hydrocephalus, which often develops postnatally. US,MRI++. Arnold-chiari syndrome: The Chiari II malformation is a complex congenital malformation of the brain, nearly always associated with myelomeningocele. This condition includes downward displacement of the medulla, fourth ventricle, and cerebellum into the cervical spinal canal. MRI++,CT,US. Great cerebral vein aneurysm: Rare congenital arteriovenous malformation. Hydrocephalus is found in 90% of cases associated to heart failure signs. CT :pineal: mass that strongly enhances after contrast administration. Angio-MRI MRI++.

29 Etiology DISCUSSION Congenital infections: Cytomegalovirus,toxoplasmosis toxoplasmosis. Antenatal diagnosis++:us or MRI. Dysgenesis of corpus callosum. Arachnoid cysts of the midline. Acquired hydrocephalus: Infection: Secondary hydrocephalus by obstruction of the pathways of CSF circulation:aqueduct aqueduct,fourth ventricle issues,peripheral resorption. Meningitis:staphylococcus staphylococcus pneumoniae,tuberculosis ++ Tumors: MRI++. Intraventricular haemorhage: Premature newborn++.

30 Treatment DISCUSSION Medical therapy Medical therapy is usually a temporizing measure.. In transient conditions, such as sinus occlusion, meningitis,, or neonatal intraventricular hemorrhage, medical therapy can be effective. Surgical therapy Ventriculoperitoneal shunting :This procedure is by far the most common procedure for CSF diversion. Ventriculoatrial shunting: : This procedure is usually the first choice for patients who are unable to have distal abdominal catheters Lumboperitoneal shunts are used in communicating hydrocephalus. External shunt. Ventriculostomy.

31 Evolution DISCUSSION Complications: The most common complications differ depending on the type of shunt and the underlying pathophysiology Infection Subdural hematomas occur almost exclusively in children with completed head growth. Shunt failure. Overdrainage. Outcome and prognosis: In general, outcome is good, The neurologic function of children is optimized with shunting.. Infection, especially if repeated, may affect cognitive status.

32 Conclusion Hydrocephalusof the infant is a frequent pathology in our context. Development of antenatal diagnosis,of modern imaging techniques, specifically,, MRI, has radically changed the prognosis of this pathology. The treatment is essentially symptomatic by ventriculoperitoneal shunting. Shunted hydrocephalus should be carefully followed up by clinical and radiologic surveillance.

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