Kinderchirurgie: Fötale Chirurgie bei Spina bifida

Martin Meuli a c, Roland Zimmermann b c, Nicole Ochsenbein b c, Ueli Möhrlen a c

Neues Verständnis der Pathogenese als Grundstein für die intrauterine Chirurgie

Journal: Swiss Medical Forum, (14):976-978

Date: 01/04/2014

a Universitäts-Kinderspital Zürich, Chirurgische Klinik, Steinwiesstrasse 75, 8032 Zürich, Schweiz;
b Klinik für Geburtshilfe, UniversitätsSpital Zürich, Frauenklinikstrasse 10, 8091 Zürich, Schweiz;
c Zentrum für Fetale Diagnostik und Therapie, Frauenklinikstrstrasse 10, 8091 Zürich, Schweiz

Premiere use of Integra™ artificial skin to close an extensive fetal skin defect during open in utero repair of myelomeningocele

Martin Meuli  1 , Claudia Meuli-Simmen, Alan W Flake, Roland Zimmermann, Nicole Ochsenbein, Ianina Scheer, Luca Mazzone, Ueli Moehrlen

There are fetuses demonstrating very large myelomeningocele lesion which can not be covered with autochothonous skin.

Journal: Springer Link Pediatric Surgery International 29, 1321-1326 (2013)

Date: 22/09/2013

1 The Zurich Center for Fetal Diagnosis and Therapy, Zurich, Switzerland,

Fetal Surgery for Myelomeningocele: A Critical Appraisal

Martin Meuli  1 , Ueli Moehrlen

This article narrates the thrilling story of how the pathogenetic understanding of myelomeningocele was fundamentally revised during the last decades and how these new insights, in particular the "two-hit hypothesis," have prepared the terrain for human fetal surgery. Formerly, the devastating cluster of neurologic and neurogenic problems was mainly attributed to the primary malformation, that is, failure of neurulation. At present, there is solid evidence that in early gestation the nonneurulated spinal cord functions well, but suffers from progressive traumatic and degenerative damage in later gestation because it is openly exposed to the amniotic cavity. There is no doubt that the secondary, in utero acquired spinal cord destruction is mainly responsible for the disastrous and irreversible peripheral neurologic deficit present at birth, and there is no doubt either that timely prenatal protective coverage can potentially arrest these deleterious dynamics and preserve neurologic function. Also, tethering of the cord within and constant outflow of cerebrospinal fluid from the lesion are seen as the driving forces behind the Chiari II malformation and consequent ventriculomegaly. Untethering and watertight sealing of the lesion reverses hindbrain herniation and lowers the risk for a relevant hydrocephalus. This article then details how human fetal surgery started in the late 1990s and follows the evolution from the pioneer case studies via the first case series providing encouraging results to the ground breaking Management of Myelomeningocele Study Trial, published in The New England Journal of Medicine in February 2011 by Adzick et al, that has, for the first time, generated unequivocal evidence that patients with prenatal repair do significantly better than those with postnatal care only. Finally, this review looks at several other critical issues, including the hitherto immature endoscopic approach to fetal repair, some future directions of research and clinical practice, and also utters a plea for concentration of these equally rare and complex cases to a few truly qualified centers worldwide. The conclusion derived from all data existing today is that maternal-fetal surgery, although not a cure and not free of risks, represents a novel standard of care for select mothers and their fetuses suffering from one of the most ruinous nonlethal congenital malformations.

Journal: Georg Thieme Verlag KG European Journal of Pediatric Surgery

Date: 09/04/2013

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

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.

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

Date: 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.

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

Date: 08/04/2008

1 Department of Pediatric Surgery, University Children's Hospital Zürich, 8032 Zürich, Switzerland.