eMedicine Specialties > Pediatrics: Surgery > General Surgery

Surgical Aspects of Cystic Fibrosis and Meconium Ileus: Follow-up

Author: Michael S Irish, MD, Assistant Professor, Department of Surgery, The University of Iowa; Consulting Pediatric Surgeon, Department of Pediatric Surgery, Blank Children's Hospital and Children's Hospital Physicians Group
Coauthor(s): Philip M Bovet, DO, MPH, Resident, Department of Pediatric Surgery, Blank Children's Hospital
Contributor Information and Disclosures

Updated: Jul 31, 2006

Outcome and Prognosis

Hiatt and Wilson reported the first survivors with MI in 1948, and early series reported 50-67% mortality rates. The advent of improved nonoperative and operative treatments, nutritional support, and treatment of bacterial infection have combined to improve reported survival rates for infants with both complicated and simple MI to 85-100%. Of the MI mortalities, Escobar et al showed 8 (9%) deaths over 32 years, with 5 of these patients having complicated MI and 3 having simple MI.

However, patients with CF who have a history of MI have significantly worse pulmonary outcomes after age 8-10 years, as measured by forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC), forced expiratory flow at 25-75% (FEF25-75), and total lung capacity (TLC). Whether the MI was treated surgically or medically did not change future pulmonary status. Overall, patients with CF and MI have worse lung function and a higher rate of obstructive disease than patients with CF who do not have a history of MI. These data support MI as being a different phenotype in CF.

An epidemiological study of 27,703 patients formalized a baseline risk, using age of CF diagnosis and the initial disease presentation in order to build survival models and demonstrate disease severity in isolated patients with CF. This study revealed that patients diagnosed by MI had statistically significant higher risks of a shorter lifespan (1.80; 95% CI: 1.27-2.56, p = 0.001), acquiring Pseudomonas aeruginosa (1.23; 95% CI: 1.08-1.39, p = 0.001), and a trend toward FEV1 below 70% (OR = 1.39, p = 0.07) in comparison to the SCREEN group diagnosed by a newborn screening program. Additionally, each year increase in age at diagnosis resulted in a 3% decrease in risk of shortened lifespan (95% CI: 0.96-0.98, p < 0.001), a 5% decrease in acquiring P aeruginosa (95% CI: 0.93-0.97, p < 0.001), and 3% decreased risk in FEV1 less than 70% (95% CI: 0.95-0.99, p < 0.001).

Prenatal diagnosis of MI with obstetrical ultrasonography, coupled with improved biochemical and molecular techniques for diagnosis of CF, now enable a perinatal team to counsel a family about the likelihood of CF in the fetus. Prenatal diagnosis also allows physicians to monitor and manage an affected fetus to ensure an optimal outcome. Advances in neonatal care and surgical procedures for infants with CF complicated by MI have greatly improved survival rates.

Future and Controversies

Advances in perinatal diagnosis and management of MI and CF, combined with greater understanding of the CFTR protein, have vastly improved the outlook for affected infants. Continued successful care for these patients depends upon prenatal diagnosis, multidisciplinary care, and innovative antenatal therapy strategies.

Future goals should include discoveries of new ways to reduce the perinatal complications that increase morbidity, mortality, and medical care costs.

The ability to detect both MI and CF prenatally means physicians should begin to consider strategies that would prevent simple MI from progressing to complex MI. The creation of mouse models for CF provides a unique opportunity to study the basic pathophysiology of MI. This knowledge should enable the development and prospective evaluation of new treatments for this disorder. Future prenatal interventions such as gene therapy may even prevent MI.

 


More on Surgical Aspects of Cystic Fibrosis and Meconium Ileus

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Workup: Surgical Aspects of Cystic Fibrosis and Meconium Ileus
Treatment: Surgical Aspects of Cystic Fibrosis and Meconium Ileus
Follow-up: Surgical Aspects of Cystic Fibrosis and Meconium Ileus
References
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References

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Further Reading

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Keywords

cystic fibrosis, CF, meconium ileus, MI, simple meconium ileus, complicated meconium ileus

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Contributor Information and Disclosures

Author

Michael S Irish, MD, Assistant Professor, Department of Surgery, The University of Iowa; Consulting Pediatric Surgeon, Department of Pediatric Surgery, Blank Children's Hospital and Children's Hospital Physicians Group
Michael S Irish, MD is a member of the following medical societies: International Pediatric Endosurgery Group and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Philip M Bovet, DO, MPH, Resident, Department of Pediatric Surgery, Blank Children's Hospital
Philip M Bovet, DO, MPH is a member of the following medical societies: American Academy of Osteopathy and American College of Osteopathic Pediatricians
Disclosure: Nothing to disclose.

Medical Editor

Denis Bensard, MD, Director, Pediatric Trauma, Division of Pediatric Surgery, Children's Hospital of Denver; Associate Professor, University of Colorado Health Sciences Center
Denis Bensard, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, and Society of University Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Gail E Besner, MD, Professor of Surgery and Pediatrics, Department of Surgery, Ohio State University College of Medicine and Public Health; Director, Pediatric Surgical Research, Department of Surgery, Children's Hospital
Gail E Besner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Burn Association, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Medical Women's Association, American Pediatric Surgical Association, Association for Academic Surgery, Federation of American Societies for Experimental Biology, Society of Critical Care Medicine, Society of Surgical Oncology, and Society of University Surgeons
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Pediatric Oncology Group, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
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