Fetal Surgery Experts Review Status of a Medical Frontier

February 28, 2013 — Fetal surgery — including surgical interventions to repair birth defects in the womb — is progressing into medical practice in highly specialized centers. Advanced imaging and diagnostic tools, new instruments and medications, and greater depth of experience are driving this rapidly evolving frontier of medicine.

“Birth defects remain the leading cause of infant mortality in the United States, but we have been privileged to help advance the treatment of these defects in ways that were only dreamed of a generation ago,” said fetal and pediatric general surgeon N. Scott Adzick, MD, medical director of the Center for Fetal Diagnosis and Treatment (CFDT) at The Children’s Hospital of Philadelphia (CHOP), where he is surgeon-in-chief.

Internationally prominent as a pioneer in fetal surgery, Adzick is the guest editor of the February 2013 issue of the journal Seminars in Pediatric Surgery, an issue entirely devoted to advances in fetal surgery. This is the third time in 20 years that Adzick has taken on this role, updating the state of this field since his previous contributions to the same journal in 1993 and 2003.

Focus on diagnostic and treatment innovations, basic science insights, and therapy improvements

Adzick and the article authors, all current or former members of the CFDT at CHOP, describe innovations in imaging modalities, surgical techniques and instrumentation, insights from basic science, and improvements to therapy grounded in experience and practice, including a landmark clinical trial of fetal surgery for spina bifida. “The presentations reflect the philosophy of one center, gleaned from two decades of clinical and experimental work,” Adzick writes in his preface to the special issue.

The CFDT is a premier program, one of a handful worldwide to offer a full range of fetal procedures. Since the center opened in 1995, more than 13,000 parents have used its services, from all 50 U.S. states and from over 50 other countries.

The 10 review articles in the journal cover a range of topics including: 

• Fetal Surgery for Spina Bifida: Past, Present, Future by N. Scott Adzick, MD, summarizes the history and current status of fetal surgery for spina bifida, highlighting the results of the federally sponsored, prospective randomized trial, the Management of Myelomeningocele (MOMS) study, reported in 2011. Adzick, as a co-leader of the study, reviews the improvements in clinical outcomes for children who underwent this procedure before 26 weeks’ gestation. Future research, currently underway, will evaluate long-term impact of this in utero intervention and will refine timing and techniques.
• Fetoscopic Laser Surgery for Twin-Twin Transfusion Syndrome by Nahla Khalek, MD, MPH; Mark P. Johnson, MD; and Michael W. Bebbington, MD, reviews the current status of fetoscopic laser ablation of abnormal shared placental vessels to treat TTTS. TTTS is a life-threatening condition in which blood passes unequally between identical twins sharing a placenta. The authors describe recent progress in treating this condition prenatally, by inserting a tiny laser into the mother’s uterus to seal off the abnormal blood circulation.
• Management of Prenatally Diagnosed Congenital Diaphragmatic Hernia, by Holly L. Hedrick, MD, describes prenatal and postnatal management of the fetus with congenital diaphragmatic hernia, an opening in the diaphragm that may cause devastating physiologic consequences. The experience of European centers in using minimally invasive tracheal balloons to improve prenatal lung growth shows promise, and the author endorses plans for a prospective randomized trial to evaluate efficacy. In the meantime, adds Hedrick, increased survival rates for children with this condition justify the creation of coordinated long-term follow-up programs.
• Stem Cell and Genetic Therapies for the Fetus by Erik G. Pearson, MD, and Alan W. Flake, MD, demonstrates that prenatal stem cell therapy and gene therapy hold the potential to change the treatment paradigm for genetic disorders by preempting postnatal clinical manifestations of disease. For many genetic diseases, the fetal period may be the most favorable time to prevent those manifestations. In utero stem cell transplantation may be a promising approach to treating fetuses with hematologic and immunologic disorders; the near future may see prenatal treatment of sickle cell anemia.
• The Special Delivery Unit by Lori J. Howell, MS, RN, describes the Special Delivery Unit at CHOP, opened in 2008 as the world’s first birthing unit dedicated to mothers carrying fetuses with known birth defects. Howell presents the rationale for this specialized obstetric delivery facility and outlines its operations.

Additional articles examine these topics:

• Management of Prenatally Diagnosed Lung Lesions
• Use of Ultrasound and MRI to Measure Lung Volumes in Fetuses with Isolated Left Congenital Diaphragmatic Hernia
• Ex Utero Intrapartum Therapy (EXIT)
• Anesthesia for Fetal Surgery
• Clinical Implications of Maternal-Fetal Cell Trafficking

Note: Dr. Bebbington, co-author of the article on twin-twin transfusion syndrome, was formerly at the CFDT at CHOP and is now at the Texas Fetal Center, University of Texas Medical School at Houston.

More information

Access the article

Seminars in Pediatric Surgery - February 2013: http://www.sempedsurg.org

About the Center for Fetal Diagnosis and Treatment at CHOP

The Center for Fetal Diagnosis and Treatment at The Children’s Hospital of Philadelphia is an internationally recognized leader in fetal surgery and fetal care. One of the only programs of its kind in the world, it offers a comprehensive breadth of services, including fetal therapy, to support patients from prenatal evaluation through delivery, postnatal care, and long-term follow-up. Established in 1995, the Center has welcomed more than 13,000 expectant parents and received referrals from all 50 states and more than 50 countries. Its multidisciplinary team brings decades of experience to the care and treatment of the fetus and the expectant mother. The Center has performed nearly 1000 fetal surgeries, including complex open procedures for birth defects such as spina bifida; less invasive fetoscopic or ultrasound-guided surgeries for conditions such as twin-twin transfusion syndrome; and specialized coordinated delivery approaches for babies that require surgical intervention while still on maternal-placental life support (EXIT delivery). 

Contact

To arrange an interview with Dr. N. Scott Adzick or other fetal medicine experts at The Children’s Hospital of Philadelphia, please contact Ashley Moore at 267-426-6071 or moorea1@email.chop.edu. She also can provide family stories about now-healthy children who underwent fetal surgery at Children’s Hospital.