PENTALOGY OF CANTRELL: A CLINICAL CASE


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Abstract

Background. Pentalogy of Cantrell is a rare combined malformation presented with 5 signs: thoracoabdominal defect in the anterior abdominal wall, defects in the lower sternum, the anterior diaphragm and the diaphragmatic pericardium, and congenital heart anomalies. The prognosis is poor. The disease may be diagnosed just in the f irst trimester of pregnancy. Its outcomes and treatment policy are determined by the pattern of cardiac defect and the severity of extracardiac abnormalities. Description. We present a case of three-month survival in a baby from a twin with complete pentalogy of Cantrell. His examination revealed a large anterior abdominal wall defect covered with thin shells. Contractions of the ectopic heart were supraumbilically detected. There were maxillofacial deformities as cleft lip and cleft palate. Systemic muscular hypotonia was pronounced. The fact that the baby had a complex heart defect was a contraindication to surgical correction of omphalocele. In this connection, an attempt was made to partially plunge the eventrated organs into the abdomen by therapeutic methods - omphalocele was vertically recorded behind the umbilical cord at birth (Fig. 1, an insert). The infant with stable hemodynamic parameters maintained with an oxygen concentrator was discharged home when he was 3 months old. Conclusion. When a newborn infant has pentalogy of Cantrell, his management tactic and strategy should be based on the stabilization of hemodynamic parameters and on preparation for further surgical treatment using the current principles of treatment and nursing.

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About the authors

Daria Vladimirovna Asherova-Yushkova

Yaroslavl Regional Perinatal Center

Email: dasherova@yandex.ru
chief of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Larisa Alexandrovna Krasnova

Academician V. I. Kulakov Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia

Email: l_krasnova@oparina4.ru
doctor of the surgery, intensive care department of neonatology and pediatrics Moscow 117997, Ac. Oparina str. 4, Russia

Tatiana Zacharovna Yasneva

Yaroslavl Regional Perinatal Center

Email: priemnaya.optz@yandex.ru
МD, neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Sergey Nikolaevich Kocheshkov

Yaroslavl Regional Perinatal Center

Email: sergej-kocheshkov@yandex.ru
neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Anatoly Yurjevich Lyashenko

Yaroslavl Regional Perinatal Center

Email: lyashenko70@mail.ru
PhD, neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Tatyana Vladimirovna Chaparova

Yaroslavl Regional Perinatal Center

Email: tatyana.chaparova@gmail.com
neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Elena Viktorovna Gorodova

Yaroslavl Regional Perinatal Center

Email: e.gor.24@mail.ru
neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Anna Alexandrovna Shmeliova

Yaroslavl Regional Perinatal Center

Email: niura.schmeliova@yandex.ru
neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Yuliya Sergeevna Badanina

Yaroslavl Regional Perinatal Center

Email: yulya-badanina@mail.ru
neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

Marina Olegovna Protasova

Yaroslavl Regional Perinatal Center

Email: priemnaya.optz@yandex.ru
neonatologist of neonatal intensive care unit Yaroslavl, Tutaevskoe sh. 31B, Russia

References

  1. Cantrell J.R., Haller J.A., Ravitch M.M. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg. Gynecol. Obstet. 1958; 107: 602-14.
  2. Van Allen M.I., Curry C., Walden C.E., Gallagher L., Patten R.M. Limb-body wall complex: II. Limb and spine defects. Am. J. Med. Genet. 1987; 28(3): 549-65.
  3. Carmi R., Boughman J.A. Pentalogy of Cantrell and associated midline anomalies: a possible ventral midline developmental field. Am. J. Med. Genet. 1992; 42(1): 90-5.
  4. Toyama W.M. 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.
  5. Bittmann S., Ulus H., Springer A. Combined pentalogy of Cantrell with tetralogy of Fallot, gallbladder agenesis, and polyspleenia: a case report. J. Pediatr. Surg. 2004; 39(1): 107-9.
  6. Assaqqat M.A., Al-Fayyadh M.I., Bulbul Z.R. Exteriorisation of the heart in two siblings. Cardiol. Young. 2003; 13(1): 98-100.
  7. Vazquez-Jimenez J.F, Muehler E.G., Daebritz S., Keutel J., Nishigaki K., Huegel W et al. Cantrell’s syndrome: A challenge to the surgeon. Ann. Thorac. Surg. 1998; 65(4): 1178-85.
  8. Jafarian A.H., Omidi A.A., Fazel A., Sadeghian H., Joushan B. Pentalogy of Cantrell: a case report. J. Res. Med. Sci. 2011; 16(1): 105-9.
  9. Morales J.M., Patel S.G., Duff J.A., Villareal R.L., Simpson J.W. Ectopia cordis and other midline defects. Ann. Thorac. Surg. 2000; 70: 111-4.
  10. Chen C.P. Syndromes and disorders associated with omphalocele (II): OEIS complex and pentalogy of Cantrell. Taiwan J. Obstet. Gynecol. 2007; 46(2): 103-10.
  11. Rodgers E.B., Monteagudo A., Santos R., Greco A., Timor-Tritsch I.E. Diagnosis of pentalogy of Cantrell using 2- and 3-dimensional sonography. J. Ultrasound Med. 2010; 29: 1825-8.
  12. Fernandez M.S., Lopez A., Vila J.J., Lluna J., Miranda J. Cantrell’s pentalogy. Report of four cases and their management. Pediatr. Surg. Int. 1997; 12(5-6): 428-31.
  13. Norma B.S., Alfredo V.A., Erika S.S., Begona S.S., Luis A.A., Lorenzo R.C. et al. Pentalogy of Cantrell: Forty- two years of Experience in the Hospital Infantil de Mexico Federico Gomez. World J. Pediatr. Congenit. Heart Surg. 2011; 2(2): 211-8.
  14. Gruberg L., Goldstein S.A., Pfister A.J., Monsein L.H., Evans D.M., Leon M.B. Cantrell’s syndrome: left ventricular diverticulum in an adult patient. Circulation. 2000;101:109-10.
  15. Hornberger L.K., Colan S.D., Lock J.E., Wessel D.L., Mayer J.E. Jr. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996; 94: 32-7.
  16. Van Hoorn J.H., Moonen R.M., Huysentruyt C.J., van Heurn L.W., Offermans J.P., Mulder A.L. Pentalogy of Cantrell: Two patients and a review to determine prognostic factors for optimal approach. Eur. J. Pediatr. 2008; 167: 29-35.

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