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Chapter 11 - Pentalogy of Cantrell

from Section 2 - Right-Sided Obstructive Lesions

Published online by Cambridge University Press:  09 September 2021

By
Jennifer E. Lam
Edited by
Laura K. Berenstain  and
James P. Spaeth
Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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Summary

Pentalogy of Cantrell is a syndrome consisting of (1) a midline, supraumbilical abdominal wall defect; (2) a congenital heart defect; (3) a lower sternal defect; (4) an anterior diaphragmatic defect; and (5) a diaphragmatic pericardial defect. Patients present a challenge to both the surgeon and anesthesiologist in the operating room due to the wide spectrum of anomalies with various complexities they bring. Planning dictates a multidisciplinary approach involving neonatology, cardiology, cardiac surgery, general surgery, anesthesiology, and pertinent subspecialties to determine the best course of action. Generally, corrective strategies include separation of the peritoneal and pericardial cavities with coverage of midline defects, omphalocele correction, repair of intracardiac lesions, and restoration of the heart into the thoracic cavity, all while preserving/establishing musculoskeletal structural integrity and hemodynamic stability. The potential for significant cardiopulmonary compromise is high and must be understood and anticipated when caring for these patients.

Keywords

abdominal wall defectdiaphragmatic defectectopic cordisomphalocelepentalogy of Cantrellpericardial defectsternal malformationventricular diverticulum
Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
 , pp. 64 - 69
Publisher: Cambridge University Press
Print publication year: 2021

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References

References

Cantrell, J. R., Haller, J. A., and Ravitch, M. M.. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 1958; 107(5): 602–14.Google ScholarPubMed
Toyama, W.. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: a case report and review of the syndrome. Pediatrics 1972; 50(5): 778–92.Google Scholar
Vazquez-Jimenez, J. F., Meuhler, E. G., Daebritz, S., et al. Cantrell’s syndrome: a challenge to the surgeon. Ann Thorac Surg 1998; 65: 1178–85.CrossRefGoogle ScholarPubMed
Engum, S. A.. Embryology, sternal clefts, ectopia cordis, and Cantrell’s pentalogy. Semin Pediatr Surg 2008; 17: 154–60.CrossRefGoogle ScholarPubMed
Kawata, H., Kishimoto, H., Ueno, T., et al. Repair of left ventricular diverticulum with ventricular bigeminy in an infant. Ann Thorac Surg 1998; 66: 1421–3.CrossRefGoogle ScholarPubMed
Shamberger, R. C. and Welch, K. J.. Sternal defects. Pediatr Surg Int 1990; 5: 156–64.CrossRefGoogle Scholar
Duggan, E. and Puligandla, P. S.. Respiratory disorders in patients with omphalocele. Semin Pediatr Surg 2019; 28: 115–17.CrossRefGoogle ScholarPubMed
O’Gorman, C. S., Tortoriello, T. A., and McMahon, C. J.. Outcome of children with pentalogy of Cantrell following cardiac surgery. Pediatr Cardiol 2009; 30: 426–30.CrossRefGoogle ScholarPubMed

Suggested Reading

Balderrabano-Saucedo, N., Vizcaino-Alarcon, A., Sandoval-Serrano, E., et al. Pentalogy of Cantrell: forty-two years of experience in the hospital infantile de Mexico Federico Gomez. World J Pediatr Congenit Heart Surg 2011; 2: 211–18.CrossRefGoogle Scholar
Cantrell, J. R., Haller, J. A., and Ravitch, M. M. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 1958; 107: 602–14.Google ScholarPubMed
Duggan, E. and Puligandla, P. S. Respiratory disorders in patients with omphalocele. Semin Pediatr Surg 2019; 28: 115–17.CrossRefGoogle ScholarPubMed
Laloyaux, P., Veyckemans, F., and Van Dyck, M. Anaesthetic management of a prematurely born infant with Cantrell’s pentalogy. Pediatr Anesth 1998; 8: 163–66.CrossRefGoogle ScholarPubMed
Nicols, J. H. and Nasr, V. G. Sternal malformations and anesthetic management. Pediatr Anesth 2017; 27: 1084–90.Google Scholar
Williams, A. P., Marayati, R., and Beierle, E. A. Pentalogy of Cantrell. Semin Pediatr Surg 2019; 28: 106–10.Google Scholar

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