Aqueduct stenosis

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Classification according to ICD-10
Q03.0 Malformations of the cerebral aqueduct
ICD-10 online (WHO version 2019)

The aqueduct is a constriction ( stenosis ) in the aqueduct of midbrain , the passageway 3 to 4 cerebral ventricles .

It is the most common cause of congenital obstructive hydrocephalus , but it can also be acquired.

The aqueductus cochleae and the aqueductus vestibulae should not be confused .

distribution

The frequency is estimated at 1 in 5,000, and varies considerably depending on the cause.

root cause

Possible causes are:

Clinical manifestations

The clinical picture depends primarily on the cause and extent of the obstruction to drainage.

If the stenosis is compensated, symptoms do not appear until there is an increased flow of CSF .

The main symptoms are headache , further signs of intracranial pressure , and increase in head circumference due to hydrocephalus if the cranial sutures are still open .

Eye symptoms such as vertical, conjugated eye paralysis can also be in the foreground as Parinaud's syndrome , English periaqueductal dysfunction; Koerber-Salus-Elschnig syndrome; Sylvian aqueduct syndrome .

Is next to a relocation of the aqueduct and obstruction of the interventricular foramen (foramen of Monro) before, a can of isolated third ventricle develop English Trapped Third Ventricle

diagnosis

An often symmetrical enlargement of the lateral ventricles and the 3rd ventricle , but not the 4th ventricle, is pathognomonic .

The diagnosis results from medical imaging and can already be recorded in the prenatal diagnosis .

A measurement of the CSF flow through the aqueduct can be carried out by means of magnetic resonance imaging .

therapy

Treatment depends on the cause, discharge can be provided as described in Hydrocephalus Treatment .

Measures are possible even in the womb ( therapy in utero ).

literature

  • BM Kline-Fath, MS Arroyo, MA Calvo-Garcia, PS Horn, C. Thomas: Congenital aqueduct stenosis: Progressive brain findings in utero to birth in the presence of severe hydrocephalus. In: Prenatal diagnosis. Volume 38, Number 9, August 2018, pp. 706-712, doi: 10.1002 / pd.5317 , PMID 29927492 .
  • KJ Heaphy-Henault, CV Guimaraes et al. a .: Congenital Aqueductal Stenosis: Findings at Fetal MRI That Accurately Predict a Postnatal Diagnosis. In: American Journal of Neuroradiology. 39, 2018, p. 942, doi: 10.3174 / ajnr.A5590 .

Individual evidence

  1. ^ Willibald Pschyrembel : Clinical Dictionary , 266th, updated edition, de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0
  2. ↑ a b Radiopaedia
  3. ↑ a b Entry on aqueduct stenosis in the Flexikon , a Wiki of the DocCheck company
  4. ↑ H. Vogel, EC Gessaga, DS Horoupian, H. Urich: Aqueductal atresia as a feature of arhinencephalic syndromes. In: Clinical neuropathology. Volume 9, Number 4, 1990 Jul-Aug, pp. 191-195, PMID 2225595 .
  5. ↑ MT Whitehead, AF Choudhri, J. Grimm, MD Nelson: Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children. In: Pediatric radiology. Volume 44, Number 7, July 2014, pp. 849-856, doi: 10.1007 / s00247-014-2877-4 , PMID 24633306 .
  6. ↑ N. Flora, N. Kulasekaran, SK Mudali, TS Swaminathan: Compensated aqueduct of sylvius obstruction by web-a case report. In: Neuroradiology Vol. 13, No. 1, 2005, pp. 19-20 IRJI
  7. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  8. ^ Hydrocephalus with stenosis of the aqueductus Sylvii. In: Orphanet (Rare Disease Database).
  9. ^ Rare Diseases
  10. ↑ S. Abbati: Idiopathic Aqueductal Stenosis. In: New England Journal of Medicine. 367, 2012, p. E30, doi: 10.1056 / NEJMicm1200835 .
  11. ↑ SR Tripathy, SS Mishra, RC Deo, I. Mohanta, K. Tripathy: Aqueductal Stenosis Has a New Dimension-Aspergillosis: A Rare Case Report and Review of the Literature. In: World Neurosurgery . Volume 84, number 5, November 2015, pp. 1494.e13–1494.e16, doi: 10.1016 / j.wneu.2015.05.005 , PMID 25982686 .
  12. ^ T. Pui Poon, EJ Arida, WP Tyschenko: Cerebral cysticercosis with aqueductal obstruction. In: Journal of Neurosurgery. 1980, p. 252, doi: 10.3171 / jns.1980.53.2.0252 .
  13. ^ R. Ford, EL Spatz: Post-Traumatic Obstruction of the Aqueduct of Sylvius and Postdecompression Cerebral Edema Treated by Ventriculostomy. In: New England Journal of Medicine. 263, 1960, p. 263, doi: 10.1056 / NEJM196008112630601 .
  14. ↑ a b W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Vol. 1, pp. 459ff, Springer 1996, ISBN 3-540-60224-0 .
  15. ↑ H. Maroulis, GM Halmagyi, R. Heard, RJ Cook: Sylvian aqueduct syndrome with slit ventricles in shunted hydrocephalus due to adult aqueduct stenosis. In: Journal of Neurosurgery. Volume 109, Number 5, November 2008, pp. 939-943, doi: 10.3171 / JNS / 2008/109/11/0939 , PMID 18976088 .
  16. ↑ EJ Kuiper, WP Vandertop: Trapped Third Ventricle. In: Acta Neurochirurgica. 143, 2001, p. 1169, doi: 10.1007 / s007010100009 .
  17. ↑ BM Kline-Fath, MS Arroyo, MA Calvo-Garcia, PS Horn, C. Thomas: Prenatal aqueduct stenosis: Association with rhombencephalosynapsis and neonatal outcome. In: Prenatal diagnosis. Volume 38, Number 13, December 2018, pp. 1028-1034, doi: 10.1002 / pd.5361 , PMID 30229955 .
  18. ^ E. Rault, A. Lacalm, M. Massoud et al .: The many faces of prenatal imaging diagnosis of primitive aqueduct obstruction. In: European Journal of Pediatric Neurology. 22, 2018, p. 910, doi: 10.1016 / j.ejpn.2018.07.015 .
  19. ↑ S. Ernst, RI Ernestus, H. Kugel, K. Lackner: MRI with CSF flow measurement before and after endoscopic ventriculostomy for aqueduct stenosis. In: RöFo: Advances in the field of X-rays and nuclear medicine. Volume 173, Number 6, June 2001, pp. 502-508, doi: 10.1055 / s-2001-14985 , PMID 11471290 .
  20. ↑ Radiopaedia
  21. ↑ CR Goodwin, EW Sankey, I. Jusué-Torres, BD Elder, TA Kosztowski, A. Liu, J. Hoffberger, J. Lu, AM Blitz, D. Rigamonti: The Use of an Aspirating / Resecting Device to Reduce Stoma Closure Following Endoscopic Third Ventriculostomy for Aqueductal Stenosis. In: Operative neurosurgery. Volume 11, number 4, December 2015, pp. 512-517, doi: 10.1227 / NEW.0000000000000920 , PMID 29506164 .
  22. ^ SP Emery, S. Greene, WA Hogge: Fetal Therapy for Isolated Aqueductal Stenosis. In: Fetal Diagnosis and Therapy . Volume 38, number 2, 2015, pp. 81-85, doi: 10.1159 / 000382015 , PMID 25997519 (review).