Treatment for delayed presentation of sagittal synostosis: challenges pertaining to occult intracranial hypertension

Clinical article

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Object

Patients with delayed presentation of isolated sagittal synostosis (ISS) pose unique surgical challenges. Intracranial hypertension can be missed in the absence of overt findings. Here, an algorithm is presented for the elucidation of intracranial hypertension and approaches to surgical treatment during calvarial reconstruction.

Methods

Patients with delayed presentation (age > 15 months) of ISS between 1997 and 2009 were identified. Symptoms, signs, and radiological evidence of intracranial hypertension were noted. Intraoperative management included calvarial reconstruction in conjunction with ventriculostomy for intracranial pressure (ICP) monitoring and CSF drainage in the setting of suspected intracranial hypertension.

Results

Seventeen patients underwent calvarial reconstruction for delayed presentation of ISS. The mean surgical age was 40.5 months (16.2–82.9 months), and the average follow-up was 34.2 months (0.6–92.2 months). Eleven patients with subtle findings of intracranial hypertension underwent ICP monitoring during calvarial reconstruction. The mean opening ICP was 23.5 cm H2O (16.5–29.5 cm H2O), and the mean closing ICP was 7.0 cm H2O (3.5–17.0 cm H2O). Nine (81.8%) of 11 monitored patients demonstrated intracranial hypertension (ICP ≥ 20 cm H2O); the other 2 had borderline increased ICP. Perioperative morbidity was 5.9%, with 1 patient medically treated for transient, new-onset intracranial hypertension. The postoperative Whitaker category was I in 94.1% of patients, II in 5.9%, and III/IV in 0%. There were no reoperations or deaths.

Conclusions

In this consecutive series, 11 of 17 patients with delayed presentation of ISS underwent ICP monitoring during calvarial reconstruction as a result of subtle clinical findings of intracranial hypertension. Nine (81.8%) of 11 monitored patients demonstrated intracranial hypertension. Calvarial reshaping along with ICP monitoring and CSF drainage facilitated reconstruction and resulted in good outcomes and the resolution of intracranial hypertension.

Abbreviations used in this paper: CN = cranial nerve; ICP = intracranial pressure; ISS = isolated sagittal synostosis.
Article Information

Contributor Notes

Address correspondence to: Robert F. Keating, M.D., Department of Neurosurgery, Children's National Medical Center, 111 Michigan Avenue NW, 4th Floor Main Building, Washington, DC 20010. email: rkeating@cnmc.org.
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References
  • 1

    Anderson FMGeiger L: Craniosynostosis: a survey of 204 cases. J Neurosurg 22:2292401965

  • 2

    Arnaud ERenier DMarchac D: Prognosis for mental function in scaphocephaly. J Neurosurg 83:4764791995

  • 3

    Boltshauser ELudwig SDietrich FLandolt MA: Sagittal craniosynostosis: cognitive development, behaviour, and quality of life in unoperated children. Neuropediatrics 34:2933002003

    • Search Google Scholar
    • Export Citation
  • 4

    Da Costa ACWalters ISavarirayan RAnderson VAWrennall JAMeara JG: Intellectual outcomes in children and adolescents with syndromic and nonsyndromic craniosynostosis. Plast Reconstr Surg 118:1751832006

    • Search Google Scholar
    • Export Citation
  • 5

    David DJPoswillo DSimpson D: The Craniosynostoses: Causes Natural History and Management New YorkSpringer1982

  • 6

    Gault DTRenier DMarchac DJones BM: Intracranial pressure and intracranial volume in children with craniosynostosis. Plast Reconstr Surg 90:3773811992

    • Search Google Scholar
    • Export Citation
  • 7

    Horínek DHoza DTomek AHort JHäckel MTichý M: Lumbar pressure and transcranial Doppler sonography in children with scaphocephaly. Br J Neurosurg 22:2192232008

    • Search Google Scholar
    • Export Citation
  • 8

    Hudgins RJBurstein FDBoydston WR: Total calvarial reconstruction for sagittal synostosis in older infants and children. J Neurosurg 78:1992041993

    • Search Google Scholar
    • Export Citation
  • 9

    Hunter AGRudd NL: Craniosynostosis. I. Sagittal synostosis: its genetics and associated clinical findings in 214 patients who lacked involvement of the coronal suture(s). Teratology 14:1851931976

    • Search Google Scholar
    • Export Citation
  • 10

    Inagaki TKyutoku SSeno TKawaguchi TYamahara TOshige H: The intracranial pressure of the patients with mild form of craniosynostosis. Childs Nerv Syst 23:145514592007

    • Search Google Scholar
    • Export Citation
  • 11

    Kapp-Simon KA: Mental development and learning disorders in children with single suture craniosynostosis. Cleft Palate Craniofac J 35:1972031998

    • Search Google Scholar
    • Export Citation
  • 12

    Kapp-Simon KASpeltz MLCunningham MLPatel PKTomita T: Neurodevelopment of children with single suture craniosynostosis: a review. Childs Nerv Syst 23:2692812007

    • Search Google Scholar
    • Export Citation
  • 13

    Magge SNWesterveld MPruzinsky TPersing JA: Long-term neuropsychological effects of sagittal craniosynostosis on child development. J Craniofac Surg 13:991042002

    • Search Google Scholar
    • Export Citation
  • 14

    Morritt DGYeh FJWall SARichards PGJayamohan JJohnson D: Management of isolated sagittal synostosis in the absence of scaphocephaly: a series of eight cases. Plast Reconstr Surg 126:5725802010

    • Search Google Scholar
    • Export Citation
  • 15

    Pensler JMCiletti SJTomita T: Late correction of sagittal synostosis in children. Plast Reconstr Surg 97:136213701996

  • 16

    Renier DSainte-Rose CMarchac DHirsch JF: Intracranial pressure in craniostenosis. J Neurosurg 57:3703771982

  • 17

    Scott JRIsom CNGruss JSSalemy SEllenbogen RGAvellino A: Symptom outcomes following cranial vault expansion for craniosynostosis in children older than 2 years. Plast Reconstr Surg 123:2892992009

    • Search Google Scholar
    • Export Citation
  • 18

    Speltz MLEndriga MCMouradian WE: Presurgical and postsurgical mental and psychomotor development of infants with sagittal synostosis. Cleft Palate Craniofac J 34:3743791997

    • Search Google Scholar
    • Export Citation
  • 19

    Speltz MLKapp-Simon KCollett BKeich YGaither RCradock MM: Neurodevelopment of infants with singlesuture craniosynostosis: presurgery comparisons with case-matched controls. Plast Reconstr Surg 119:187418812007

    • Search Google Scholar
    • Export Citation
  • 20

    Starr JRKapp-Simon KACloonan YKCollett BRCradock MMBuono L: Presurgical and postsurgical assessment of the neurodevelopment of infants with single-suture craniosynostosis: comparison with controls. J Neurosurg 107:2 Suppl1031102007

    • Search Google Scholar
    • Export Citation
  • 21

    Sutton LNBarlett SPDuhaime ACMarkakis D: Total cranial vault reconstruction for the older child with scaphocephaly. Pediatr Neurosurg 19:63721993

    • Search Google Scholar
    • Export Citation
  • 22

    Thompson DNHarkness WJones BGonsalez SAndar UHayward R: Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management. Childs Nerv Syst 11:2692751995

    • Search Google Scholar
    • Export Citation
  • 23

    Tuite GFChong WKEvanson JNarita ATaylor DHarkness WF: The effectiveness of papilledema as an indicator of raised intracranial pressure in children with craniosynostosis. Neurosurgery 38:2722781996

    • Search Google Scholar
    • Export Citation
  • 24

    Tuite GFEvanson JChong WKThompson DNHarkness WFJones BM: The beaten copper cranium: a correlation between intracranial pressure, cranial radiographs, and computed tomographic scans in children with craniosynostosis. Neurosurgery 39:6916991996

    • Search Google Scholar
    • Export Citation
  • 25

    Virtanen RKorhonen TFagerholm JViljanto J: Neurocognitive sequelae of scaphocephaly. Pediatrics 103:7917951999

  • 26

    Walters FJM: Neuropharmacology - intracranial pressure and cerebral blood flow. Update in Anaesthesia 8:29371998

  • 27

    Weinzweig JBaker SBWhitaker LASutton LNBartlett SP: Delayed cranial vault reconstruction for sagittal synostosis in older children: an algorithm for tailoring the reconstructive approach to the craniofacial deformity. Plast Reconstr Surg 110:3974082002

    • Search Google Scholar
    • Export Citation
  • 28

    Whitaker LABartlett SPSchut LBruce D: Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients. Plast Reconstr Surg 80:1952121987

    • Search Google Scholar
    • Export Citation
  • 29

    Wilkie AOWall SA: Craniosynostosis: novel insights into pathogenesis and treatment. Curr Opin Neurol 9:1461521996

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