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Unusual variant of Cantrell's pentalogy?

Kumar B, Sharma SB, Kandpal DK, Agrawal LD - Ann Thorac Med (2008)

Bottom Line: A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress.A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly.Is congenital defect of chest wall associated with diaphragmatic hernia without ectopia cordis and omphalocele, an unusual variant of Cantrell's pentalogy?

Affiliation: Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute (JayKayLon Hospital), SMS Medical College, Jaipur, India. drbkpg75@rediff.com

ABSTRACT

A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress. A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly. The child expired after 6 hours of admission because of respiratory distress and electrolyte imbalance. Is congenital defect of chest wall associated with diaphragmatic hernia without ectopia cordis and omphalocele, an unusual variant of Cantrell's pentalogy?

Chest wall defect with prolapsed abdominal contents
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Figure 0001: Chest wall defect with prolapsed abdominal contents

Mentions: On examination, the baby had severe respiratory distress with peripheral cyanosis. Chest movement on left side was absent, and apex beat was seen on right side near midclavicular line at fourth intercostal space. The protruded swelling present on left side of chest wall appeared as having bowel loops with it [Figure 1]. A defect of about 3 × 4 cm in 4th/5th intercostal space was felt on left side of chest wall, through which sac was protruding containing stomach, small and large bowel, spleen, and part of liver. Overlying sac was ruptured. On auscultation, heart sound was heard on right side of chest without any appreciable murmur or added sound with decreased air entry at inframammary region. No air entry was found on left side of chest. On x-ray chest, mediastinum was found to be shifted on the right side without any bony deformity [Figure 2]. ECG was normal. Laboratory findings suggested that the baby was in severe respiratory acidosis with hypercarbia and electrolyte imbalance. The baby died before any surgical intervention in spite of all our medical efforts.

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Unusual variant of Cantrell's pentalogy?

Kumar B, Sharma SB, Kandpal DK, Agrawal LD - Ann Thorac Med (2008)

Chest wall defect with prolapsed abdominal contents
© Copyright Policy - open-access
Figure 0001: Chest wall defect with prolapsed abdominal contents
Mentions: On examination, the baby had severe respiratory distress with peripheral cyanosis. Chest movement on left side was absent, and apex beat was seen on right side near midclavicular line at fourth intercostal space. The protruded swelling present on left side of chest wall appeared as having bowel loops with it [Figure 1]. A defect of about 3 × 4 cm in 4th/5th intercostal space was felt on left side of chest wall, through which sac was protruding containing stomach, small and large bowel, spleen, and part of liver. Overlying sac was ruptured. On auscultation, heart sound was heard on right side of chest without any appreciable murmur or added sound with decreased air entry at inframammary region. No air entry was found on left side of chest. On x-ray chest, mediastinum was found to be shifted on the right side without any bony deformity [Figure 2]. ECG was normal. Laboratory findings suggested that the baby was in severe respiratory acidosis with hypercarbia and electrolyte imbalance. The baby died before any surgical intervention in spite of all our medical efforts.

Bottom Line: A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress.A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly.Is congenital defect of chest wall associated with diaphragmatic hernia without ectopia cordis and omphalocele, an unusual variant of Cantrell's pentalogy?

Affiliation: Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute (JayKayLon Hospital), SMS Medical College, Jaipur, India. drbkpg75@rediff.com

ABSTRACT

A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress. A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly. The child expired after 6 hours of admission because of respiratory distress and electrolyte imbalance. Is congenital defect of chest wall associated with diaphragmatic hernia without ectopia cordis and omphalocele, an unusual variant of Cantrell's pentalogy?

View Similar Images In: Results  - Collection
View Article: PubMed Central -  PubMed
Show All Figures - Show MeSH
getmorefigures.php?pmc=2700442&rFormat=json&query=null&req=5