Hydrocephalus in achondroplasia: the possible role of intracranial venous hypertension

Paul Steinbok M.B.,B.S., F.R.C.S.(C) 1 , Judith Hall M.D., F.R.C.P.(C) 1 and Olof Flodmark M.D., Ph.D. 1
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  • 1 Departments of Pediatric Neurosurgery, Medical Genetics, and Radiology, British Columbia Children's Hospital, and Division of Neurosurgery (Department of Surgery), and Departments of Medical Genetics and Radiology, University of British Columbia, Vancouver, British Columbia, Canada
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✓ The significance and cause of ventriculomegaly in achondroplasia was investigated in five achondroplastic children. The intraventricular pressure (IVP) was monitored over 24 hours, followed by intraventricular injection of radionuclide alone or in combination with water-soluble contrast material. The IVP was elevated and the reabsorption of cerebrospinal fluid (CSF) into the sagittal sinus was slow in all cases, but there was no obstruction to CSF flow. The spinal subarachnoid space was well seen in all patients. Jugular venograms with pressure monitoring were obtained in four patients (bilaterally in one). These studies confirmed a narrow jugular foramen in all patients with a significant venous pressure gradient (3 to 10 mm Hg) obtained while the catheter was being pulled back from the sigmoid sinus through the foramen. A second gradient was found in the jugular vein in two patients at the level of the upper thoracic aperture. This gradient was 6 and 14 mm Hg, respectively. Identical venograms and monitoring of the venous pressure in a control group showed no pressure gradients across the jugular foramen and smaller gradients (2 to 5 mm Hg) across the thoracic inlet.

It is concluded from these studies that ventriculomegaly in achondroplastic children represents hydrocephalus, which is likely secondary to raised intracranial venous pressure due to hemodynamically significant stenosis of the jugular foramen and, in some cases, the jugular vein in the thoracic aperture.

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Contributor Notes

Address reprint requests to: Paul Steinbok, M.B.,B.S., F.R.C.S.(C), Department of Neurosurgery, British Columbia Children's Hospital, 4480 Oak Street, Vancouver, British Columbia V6H 3V4, Canada.
  • 1.

    Beck DJK, & Russell DS: Experiments on thrombosis of the superior longitudinal sinus. J Neurosurg 3:337347, 1946 Beck DJK, Russell DS: Experiments on thrombosis of the superior longitudinal sinus. J Neurosurg 3:337–347, 1946

    • Search Google Scholar
    • Export Citation
  • 2.

    Bedford THB: The effect of prolonged occlusion of the external jugular veins on the cerebrospinal fluid and torcular venous pressures of the dog. Brain 59:324336, 1936 Bedford THB: The effect of prolonged occlusion of the external jugular veins on the cerebrospinal fluid and torcular venous pressures of the dog. Brain 59:324–336, 1936

    • Search Google Scholar
    • Export Citation
  • 3.

    Bedford THB: The effect of variations in the subarachnoid pressure on the venous pressure in the superior longitudinal sinus and in the torcular of the dog. J Physiol (Lond) 101:362368, 1942 Bedford THB: The effect of variations in the subarachnoid pressure on the venous pressure in the superior longitudinal sinus and in the torcular of the dog. J Physiol (Lond) 101:362–368, 1942

    • Search Google Scholar
    • Export Citation
  • 4.

    Bering EA, & Salibi B: Production of hydrocephalus by increased cephalic-venous pressure. Arch Neurol Psychiatry 81:693698, 1959 Bering EA, Salibi B: Production of hydrocephalus by increased cephalic-venous pressure. Arch Neurol Psychiatry 81:693–698, 1959

    • Search Google Scholar
    • Export Citation
  • 5.

    Cohen ME, , Rosenthal AD, & Matson DD: Neurological abnormalities in achondroplastic children. J Pediatr 71:367376, 1967 Cohen ME, Rosenthal AD, Matson DD: Neurological abnormalities in achondroplastic children. J Pediatr 71:367–376, 1967

    • Search Google Scholar
    • Export Citation
  • 6.

    Dandy WE: Hydrocephalus in chondrodystrophy. Johns Hopkins Hosp Bull 32:510, 1921 Dandy WE: Hydrocephalus in chondrodystrophy. Johns Hopkins Hosp Bull 32:5–10, 1921

    • Search Google Scholar
    • Export Citation
  • 7.

    DeLange SA, & De Vlieger M: Hydrocephalus associated with raised venous pressure. Dev Med Child Neurol 12 (Suppl 22):2832, 1970 DeLange SA, De Vlieger M: Hydrocephalus associated with raised venous pressure. Dev Med Child Neurol 12 (Suppl 22):28–32, 1970

    • Search Google Scholar
    • Export Citation
  • 8.

    Dennis JP, , Rosenberg HS, & Alvord EC Jr: Megalencephaly, internal hydrocephalus and other neurological aspects of achondroplasia. Brain 84:427444, 1961 Dennis JP, Rosenberg HS, Alvord EC Jr: Megalencephaly, internal hydrocephalus and other neurological aspects of achondroplasia. Brain 84:427–444, 1961

    • Search Google Scholar
    • Export Citation
  • 9.

    Depresseux JC, , Carlier G, & Stevenaert A: CSF scanning in achondroplastic children with cranial enlargement. Dev Med Child Neurol 17:224228, 1975 Depresseux JC, Carlier G, Stevenaert A: CSF scanning in achondroplastic children with cranial enlargement. Dev Med Child Neurol 17:224–228, 1975

    • Search Google Scholar
    • Export Citation
  • 10.

    Dorst JP: Functional craniology: an aid in interpreting roentgenograms of the skull. Radiol Clin North Am 2:347366, 1964 Dorst JP: Functional craniology: an aid in interpreting roentgenograms of the skull. Radiol Clin North Am 2:347–366, 1964

    • Search Google Scholar
    • Export Citation
  • 11.

    Emery JL, & Zachary RB: Hydrocephalus associated with obliteration of the longitudinal sinus. Arch Dis Child 31:288292, 1956 Emery JL, Zachary RB: Hydrocephalus associated with obliteration of the longitudinal sinus. Arch Dis Child 31:288–292, 1956

    • Search Google Scholar
    • Export Citation
  • 12.

    Friedman WA, & Mickle JP: Hydrocephalus in achondroplasia: a possible mechanism. Neurosurgery 7:150153, 1980 Friedman WA, Mickle JP: Hydrocephalus in achondroplasia: a possible mechanism. Neurosurgery 7:150–153, 1980

    • Search Google Scholar
    • Export Citation
  • 13.

    Haar FL, & Miller CA: Hydrocephalus resulting from superior vena cava thrombosis in an infant. Case report. J Neurosurg 42:597601, 1975 Haar FL, Miller CA: Hydrocephalus resulting from superior vena cava thrombosis in an infant. Case report. J Neurosurg 42:597–601, 1975

    • Search Google Scholar
    • Export Citation
  • 14.

    Hakim S, , Venegas JG, & Burton JD: The physics of the cranial cavity, hydrocephalus and normal pressure hydrocephalus. Mechanical interpretation and mathematical model. Surg Neurol 5:187210, 1976 Hakim S, Venegas JG, Burton JD: The physics of the cranial cavity, hydrocephalus and normal pressure hydrocephalus. Mechanical interpretation and mathematical model. Surg Neurol 5:187–210, 1976

    • Search Google Scholar
    • Export Citation
  • 15.

    Hecht JT, , Nelson FW, & Butler IJ, et al: Computerized tomography of the foramen magnum: achondroplastic values compared to normal standards. Am J Med Genet 20:355360, 1985 Hecht JT, Nelson FW, Butler IJ, et al: Computerized tomography of the foramen magnum: achondroplastic values compared to normal standards. Am J Med Genet 20:355–360, 1985

    • Search Google Scholar
    • Export Citation
  • 16.

    Heinz ER, , Ward A, & Drayer BP, et al: Distinction between obstructive and atrophic dilatation of ventricles in children. J Comput Assist Tomogr 4:320325, 1980 Heinz ER, Ward A, Drayer BP, et al: Distinction between obstructive and atrophic dilatation of ventricles in children. J Comput Assist Tomogr 4:320–325, 1980

    • Search Google Scholar
    • Export Citation
  • 17.

    Hooper R: Hydrocephalus and obstruction of the superior vena cava in infancy. Clinical study of the relationship between cerebrospinal fluid pressure and venous pressure. Pediatrics 28:792799, 1961 Hooper R: Hydrocephalus and obstruction of the superior vena cava in infancy. Clinical study of the relationship between cerebrospinal fluid pressure and venous pressure. Pediatrics 28:792–799, 1961

    • Search Google Scholar
    • Export Citation
  • 18.

    Horton WA, , Rotter JI, & Rimoin DL, et al: Standard growth curves for achondroplasia. J Pediatr 93:435438, 1978 Horton WA, Rotter JI, Rimoin DL, et al: Standard growth curves for achondroplasia. J Pediatr 93:435–438, 1978

    • Search Google Scholar
    • Export Citation
  • 19.

    James AE, , Dorst JP, & Mathews ES, et al: Hydrocephalus in achondroplasia studied by cisternography. Pediatrics 49:4649, 1972 James AE, Dorst JP, Mathews ES, et al: Hydrocephalus in achondroplasia studied by cisternography. Pediatrics 49:46–49, 1972

    • Search Google Scholar
    • Export Citation
  • 20.

    Kinal ME: Hydrocephalus and the dural venous sinuses. J Neurosurg 19:195201, 1962 Kinal ME: Hydrocephalus and the dural venous sinuses. J Neurosurg 19:195–201, 1962

    • Search Google Scholar
    • Export Citation
  • 21.

    Langer LO, , Baumann PA, & Gorlin RJ: Achondroplasia. AJR 100:1226, 1967 Langer LO, Baumann PA, Gorlin RJ: Achondroplasia. AJR 100:12–26, 1967

    • Search Google Scholar
    • Export Citation
  • 22.

    Maytal J, , Alvarez LA, & Elkin CM, et al: External hydrocephalus: radiologic spectrum and differentiation from cerebral atrophy. AJNR 8:217278, 1987 Maytal J, Alvarez LA, Elkin CM, et al: External hydrocephalus: radiologic spectrum and differentiation from cerebral atrophy. AJNR 8:217–278, 1987

    • Search Google Scholar
    • Export Citation
  • 23.

    Mueller SM: Enlarged cerebral ventricular system in infant achondroplastic dwarf. Neurology 30:767769, 1980 Mueller SM: Enlarged cerebral ventricular system in infant achondroplastic dwarf. Neurology 30:767–769, 1980

    • Search Google Scholar
    • Export Citation
  • 24.

    Mueller SM, , Bell W, & Cornell S, et al: Achondroplasia and hydrocephalus. A computerized tomographic, roentgenographic, and psychometric study. Neurology 27:430434, 1977 Mueller SM, Bell W, Cornell S, et al: Achondroplasia and hydrocephalus. A computerized tomographic, roentgenographic, and psychometric study. Neurology 27:430–434, 1977

    • Search Google Scholar
    • Export Citation
  • 25.

    Mueller SM, & Reinertson JE: Reversal of emissary vein blood flow in achondroplastic dwarfs. Neurology 30:769772, 1980 Mueller SM, Reinertson JE: Reversal of emissary vein blood flow in achondroplastic dwarfs. Neurology 30:769–772, 1980

    • Search Google Scholar
    • Export Citation
  • 26.

    Nellhaus G: Head circumference from birth to eighteen years. Practical composite international and interracial graphs. Pediatrics 41:106114, 1968 Nellhaus G: Head circumference from birth to eighteen years. Practical composite international and interracial graphs. Pediatrics 41:106–114, 1968

    • Search Google Scholar
    • Export Citation
  • 27.

    Norrell H, , Wilson C, & Howieson J, et al: Venous factors in infantile hydrocephalus. J Neurosurg 31:561569, 1969 Norrell H, Wilson C, Howieson J, et al: Venous factors in infantile hydrocephalus. J Neurosurg 31:561–569, 1969

    • Search Google Scholar
    • Export Citation
  • 28.

    Pierre-Kahn A, , Hirsch JF, & Renier D, et al: Hydrocephalus and achondroplasia. A study of 25 observations. Childs Brain 7:205219, 1980 Pierre-Kahn A, Hirsch JF, Renier D, et al: Hydrocephalus and achondroplasia. A study of 25 observations. Childs Brain 7:205–219, 1980

    • Search Google Scholar
    • Export Citation
  • 29.

    Rosman NP, & Shands KN: Hydrocephalus caused by increased intracranial venous pressure: a clinicopathological study. Ann Neurol 3:445450, 1978 Rosman NP, Shands KN: Hydrocephalus caused by increased intracranial venous pressure: a clinicopathological study. Ann Neurol 3:445–450, 1978

    • Search Google Scholar
    • Export Citation
  • 30.

    Sainte-Rose C, , LaCombe J, & Pierre-Kahn A, et al: Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60:727736, 1984 Sainte-Rose C, LaCombe J, Pierre-Kahn A, et al: Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60:727–736, 1984

    • Search Google Scholar
    • Export Citation
  • 31.

    Shulman K, & Ransohoff J: Sagittal sinus venous pressure in hydrocephalus. J Neurosurg 23:169173, 1965 Shulman K, Ransohoff J: Sagittal sinus venous pressure in hydrocephalus. J Neurosurg 23:169–173, 1965

    • Search Google Scholar
    • Export Citation
  • 32.

    Shulman K, , Yarnell P, & Ransohoff J: Dural sinus pressure in normal and hydrocephalic dogs. Arch Neurol 10:575580, 1964 Shulman K, Yarnell P, Ransohoff J: Dural sinus pressure in normal and hydrocephalic dogs. Arch Neurol 10:575–580, 1964

    • Search Google Scholar
    • Export Citation
  • 33.

    Stewart DR, , Johnson DG, & Myers GG: Hydrocephalus as a complication of jugular catheterization during total parenteral nutrition. J Pediatr Surg 10:771776, 1976 Stewart DR, Johnson DG, Myers GG: Hydrocephalus as a complication of jugular catheterization during total parenteral nutrition. J Pediatr Surg 10:771–776, 1976

    • Search Google Scholar
    • Export Citation
  • 34.

    Weed LH, & Flexner LB: The relations of the intracranial pressures. Am J Physiol 105:266272, 1933 Weed LH, Flexner LB: The relations of the intracranial pressures. Am J Physiol 105:266–272, 1933

    • Search Google Scholar
    • Export Citation
  • 35.

    Welter H: Zur frage des Hydrocephalus chondiodystrophicus co ngenitus. Beitr Pathol Anat 97:18, 1936 Welter H: Zur frage des Hydrocephalus chondiodystrophicus co ngenitus. Beitr Pathol Anat 97:1–8, 1936

    • Search Google Scholar
    • Export Citation
  • 36.

    Wise BL, , Sondheimer F, & Kaufman S: Achondroplasia and hydrocephalus. Neuropaediatrie 3:106113, 1970 Wise BL, Sondheimer F, Kaufman S: Achondroplasia and hydrocephalus. Neuropaediatrie 3:106–113, 1970

    • Search Google Scholar
    • Export Citation
  • 37.

    Wright RD: Experimental observations on increased intracranial pressure. Aust NZ J Surg 7:215235, 1938 Wright RD: Experimental observations on increased intracranial pressure. Aust NZ J Surg 7:215–235, 1938

    • Search Google Scholar
    • Export Citation
  • 38.

    Wu XR, & Swaiman KF: Reversible hydrocephalus caused by bilateral jugular vein catheterization. Brain Dev 4:397400, 1982 Wu XR, Swaiman KF: Reversible hydrocephalus caused by bilateral jugular vein catheterization. Brain Dev 4:397–400, 1982

    • Search Google Scholar
    • Export Citation
  • 39.

    Yamada H, , Nakamura S, & Tajima M, et al: Neurological manifestations of pediatric achondroplasia. J Neurosurg 54:4957, 1981 Yamada H, Nakamura S, Tajima M, et al: Neurological manifestations of pediatric achondroplasia. J Neurosurg 54:49–57, 1981

    • Search Google Scholar
    • Export Citation

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