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Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 450-451
Pentology of cantrell


Dept of Radiodiagnosis, Lady Hardinge Medical College, India

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How to cite this article:
Marwah V V, Narula M K, Anand R, Solanki R. Pentology of cantrell. Indian J Radiol Imaging 2002;12:450-1

How to cite this URL:
Marwah V V, Narula M K, Anand R, Solanki R. Pentology of cantrell. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Aug 4];12:450-1. Available from: http://www.ijri.org/text.asp?2002/12/3/450/28513
Sir,

We report a case of Pentalogy of Cantrell, detected antenatally on routine ultrasonography in a 23 yr. Old primigravida. There was no history of any illness or drug intake during pregnancy.

Sonography demonstrated a single live intrauterine pregnancy of about 22 weeks 2 weeks gestational age, with adequate liquor amnii. Placenta was in the posterior and superior segment. Foetal spine showed a kyphotic deformity in the thoracolumbar region along with widening of the posterior elements and a large cystic mass lying posteriorly, suggestive of a meningocoele. There was no hydrocephalus. The anterior abdominal wall was deficient in the supra umbilical region, with herniation of most of the abdominal contents (esp, liver and bowel) which were encapsulated within a membrane. No ascites was seen within the sac. Foetal kidneys were identified in the abdomen. Lower part of the sternum was not seen. Foetal heart along with the origin of great vessels was displaced inferiorly and lying just superior to the omphalocoele and inferior to the sternum. No intracardiac abnormality could be detected. The foetal heart rate was normal. Umbilical cord was attached to the tip of the omphalocoele and demonstrated the presence of 2 vessels. (One artery and one vein). Doppler study of the umbilical artery demonstrated high resistance flow (SD-4.5). Foetal skull, face and limbs were normal. Gender of the baby could not be made out due to the large omphalocoele. The patient opted for a mid-trimester abortion and the findings were confirmed. Fetal karyotyping and autopsy could not be performed as the parents were unwilling. In view of these findings the diagnosis of pentalogy of Cantrell was made.

Pentalogy of Cantrell is a rare sporadic abnormality and only about 50 cases have been reported in literature. It was first described by Cantrell et al in 1958. The components of pentalogy are: i) Supraumbilical anterior abdominal wall defect. ii) defect of the lower sternum, iii) deficiency of the anterior diaphragm, iv) defect of diaphragmatic pericardium, v) various intracardiac defects. Ventromedial folding of the mesoderm layers give rise to thoracic and abdominal walls, sternum, septum transversum of the diaphragm, pericardium and epimyocardium. Midline fusion and formation of thoracic and abdominal wall is complete by the 9th week. The defect is believed to result from defective lateral mesoderm folds around 28-32 days of menstrual age. The causes postulated are: i) Vascular dysplasias resulting in vascular steal phenomenon, ii) Mechanical teratogenesis by amnion rupture.

Associated systemic congenital anomalies reported in literature are: i) CVS - intracardiac defects especially ASD, VSD, PDA, Aortic stenosis, double outlet right ventricle, hypoplastic pulmonary trunk and total anomalous pulmonary venous drainage, ii) CNS - Encephalocele, syringomyelia, exencephaly, spina bifida, hydrocephalus, iii) GIT - Omphalocoele, imperforate anus, anterior diaphragmatic hernia, iv) GUT - Horse-shoe kidneys, cystic changes in both kidneys, v) Skeletal system - deformed anterior rib cage, kypho-scoliotic spine, spina bifida, vi) Single umbilical artery and polyhydramnios.

Pentalogy of Cantrell has to differentiated from:-

i) Isolated thoracic ectopia - which consists of a) sternal defect, b) pericardial defect, c) whole or part of the heart protruding through the defect.

ii) Amniotic band syndrome - consists of a) concentric band, b) limb amputation / edema, c) facial clefts in bizarre location, d) large abdominal and chest wall defects, e) encephaloceles that are not midline.

iii) Limb body wall complex - consists of a) omphalocoele with eccentric defect in anterior abdominal wall, b) placenta attached to the site of defect, c) cardiac abnormalities, d) scoliosis, e) cranial defects and f) limb defects.

The prognosis depends on the severity of the anomalies. Complications include rupture of viscera during delivery, sepsis, cyanosis, congestive heart failure, cardio-respiratory difficulty and cardiac compression. Many patients have undergone repair of what seemed to be a simple omphalocoele and were later found to have pentalogy of Cantrell. Most of the cases detected prenatally have a fatal outcome. For the severe forms, the prognosis is very poor, since the reduction of heart in the chest causes kinking of great vessels. Once the diagnosis is established a fetal karyotype should be obtained and a complete search for associated anomalies performed.[4]

 
   References Top

1.Denath FM et al . Ultrasonographic findings of exencephaly in pentology of Cantrell case report and review of literature. J Clin Ultrasound 1994 June; 22 (5): 351 - 4.  Back to cited text no. 1    
2.Tongsong T. et al . Prenatal sonographic diagnosis of ectopia cordis. J. Clin Ultrasound, 1999 Oct; 27 (8): 440-5.  Back to cited text no. 2    
3.Hsieh Y et al . Prenatal sonographic diagnosis of Cantrell's pentology with cystic hygroma in the first trimester. J. Clin Ultrasound, 1998 Oct.; 26 (8): 409 - 12.  Back to cited text no. 3    
4.Fernandez MS et al . Cantrell's pentology. Report of four cases and their management. Pediatr Surg Int; 1997 July: 12 (5-6): 428-31.  Back to cited text no. 4    

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Correspondence Address:
V V Marwah
Dept of Radiodiagnosis, Lady Hardinge Medical College
India
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Source of Support: None, Conflict of Interest: None


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