Publikationen

    Fetal surgery in Zurich: key features of our first open in utero repair of myelomeningocele

    Martin Meuli  1 , Ueli Moehrlen  1 , Alan Flake  2 , Nicole Ochsenbein  3 , Margaret Huesler  3 , Peter Biro  4 , Ianina Scheer  5 , Sasha Tharakan  1 , Peter Dürig  6 , Roland Zimmermann  3               

    The first description of apparently secondary, that is, in utero acquired, damage to the pathologically exposed spinal cord within the myelomeningocele (MMC) lesion dates back to 1956. For obvious reasons, the significance of this observation regarding therapeutic consequences was not recognized until fetal surgery became a reality in the 1980s. Only then was the hypothesis born that early in utero intervention might stop the ongoing neural tissue destruction and so reduce the neurologic deficit otherwise seen at birth.

    PMID: 23165510 DOI: 10.1055/s-0032-1329700

    Journal: Thieme European Journal of Pediatric Surgery 2013; 23(06): 494-498

    DateEnglish: 01/01/2013

    1 Department of Pediatric Surgery, University Children's Hospital Zurich, Zurich, Switzerland.

    2 The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States.

    3 Department of Obstetrics, University Hospital Zurich, Zurich, Switzerland.

    4 Institute of Anesthesiology, University Hospital Zurich, Zurich, Switzerland.

    5 Department of Radiology, University Children's Hospital Zurich, Zurich, Switzerland.

    6 Effinger Center for Obstetrics and Gynecology, Zurich, Switzerland.

    The dysraphic levels of skin and vertebrae are different in mouse fetuses and neonates with myelomeningocele

    Dorothea Stiefel  1 , Martin Meuli

    Mouse fetuses with spontaneous myelomeningocele (MMC) were investigated, determining the various levels of dysraphism in soft tissue, spinal cord, and vertebrae. Morphology was correlated with hind limb function.

    PMID: 18405716 DOI: 10.1016/j.jpedsurg.2007.12.008

    Journal: Journal of Pediatric Surgery (Volume 43, Issue 4, April 2008, Pages 683-690)

    DateEnglish: 08/04/2008

    1 Department of Pediatric Surgery, University Children's Hospital Zürich, 8032 Zürich, Switzerland. stiefel_d@yahoo.co.uk

    Scanning electron microscopy of fetal murine myelomeningocele reveals growth and development of the spinal cord in early gestation and neural tissue destruction around birth

    Dorothea Stiefel  1 , Martin Meuli

    Previous studies demonstrated that the spinal cord within a fetal myelomeningocele (MMC) lesion suffers progressive destruction during gestation. This study aims at elucidating this pathophysiologic feature on a cellular and ultrastructural level in a model of genetically determined MMC.

    PMID: 17848249 DOI: 10.1016/j.jpedsurg.2007.04.019

    Journal: Journal of Pediatric Surgery (Volume 42, Issue 9, September 2007, Pages 1561-1565)

    DateEnglish: 01/09/2007

    1 Neural Development Unit, Institute of Child Health, University College London, London, WC1N 1EH United Kingdom.

    Fetal spina bifida in a mouse model: loss of neural function in utero

    Dorothea Stiefel  1 , Andrew J Copp, Martin Meuli

    The devastating neurological deficit associated with myelomeningocele has previously been assumed to be a direct and inevitable consequence of the primary malformation-failure of neural tube closure. An alternative view is that secondary damage to the pathologically exposed spinal cord tissue in utero is responsible for the neurological deficiency. If the latter mechanism were shown to be correct, it would provide an objective rationale for the performance of in utero surgery for myelomeningocele, because coverage of the exposed spinal cord could be expected to alleviate or perhaps prevent neurodegeneration. To examine this question, the authors studied the development of neuronal connections and neurological function of mice during fetal and neonatal stages in a genetic model of exposed lumbosacral spina bifida.

    PMID: 17465388 PMCID: PMC3651953 DOI: 10.3171/ped.2007.106.3.213

    Journal: JNS Journal of Neurosurgery (Volume 106: Issue 3, March 2007)

    DateEnglish: 01/03/2007

    1 Department of Pediatric Surgery, University Children's Hospital Zurich, Switzerland. stiefel_d@yahoo.co.uk

    Tethering of the spinal cord in mouse fetuses and neonates with spina bifida

    Dorothea Stiefel  1 , Takashi Shibata, Martin Meuli, Patrick G Duffy, Andrew J Copp

    Tethering of the spinal cord is a well-known complication in humans with spina bifida aperta or occulta. Its pathogenesis consists of a pathological fixation of the spinal cord resulting in traction on the neural tissue which, in turn, leads to ischemia and progressive neurological deterioration. Although well established in humans, this phenomenon has not been described in animal models of spina bifida.

    PMID: 12956464 PMCID: PMC4432388 DOI: 10.3171/spi.2003.99.2.0206

    Journal: JNS Journal of Neurosurgery: Spine (Volume 99: Issue 2, Sep. 2003)

    DateEnglish: 01/09/2003

    1 Neural Development Unit, Institute of Child Health, University College London, United Kingdom.