- Email: [email protected]
Ectopic Intrathoracic Kidney with Right-Sided Congenital Diaphragmatic Hernia
Ectopic Intrathoracic Kidney with Right-Sided Congenital Diaphragmatic Hernia
A
male term infant (birth weight 3700 g) born via cesarean delivery after an uneventful pregnancy showed persistent cyanosis at 15 minutes. Radiograph revealed right-sided congenital diaphragmatic hernia (CDH) (Figure 1; available at www.jpeds.com). The infant was intubated. The echocardiograph showed pulmonary hypertension and bidirectional ductus arteriosus shunt. During primary hernia repair, the right kidney and part of
the right lobe of the liver were found to be herniated into the thorax. The right kidney was left at the thoracic level. Excretory phase computed tomography scan of the ectopic kidney at 1 month of age showed a normal-sized kidney with no dilatation in calices (Figure 2; available at www. jpeds.com). The infant was discharged home on room air on day 37. Contrast-enhanced coronal-plane magnetic resonance imaging at 3 months of age revealed normal parenchymal enhancement of the ectopic kidney (Figure 3). 99mTc-dimercaptosuccinic acid revealed renal functions of 52% (left kidney) and 48% (right kidney). CDH is a rare condition (0.01%-0.05% of births).1 The reported incidence of intrathoracic renal ectopia due to CDH is 0.25%.2 A right-sided thoracic kidney is much less common due to the location of the liver. Although intrathoracic kidney with CDH can be mobile,3 nephropexy was not performed in this case. Isolated intrathoracic kidney is usually asymptomatic and diagnosed incidentally on chest imaging. n Petek Genc Kayıran, MD Department of Pediatrics
Terman Gumus, MD Department of Radiology
Sinan Mahir Kayıran, MD Ilmay Bilge, MD Berkan Gurakan, MD Department of Pediatrics American Hospital Istanbul, Turkey
References
Figure 3. T1-weighted postcontrast coronal-plane magnetic resonance image showing normal parenchyma enhancement of the intrathoracic right kidney (arrow) compared with the left kidney in nephrographic phase (arrowhead). Left kidney (arrowhead) is normal in size and location.
J Pediatr 2013;163:1226. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.04.016
1226
1. Tovar JA. Congenital diaphragmatic hernia. Orphanet J Rare Dis 2012;7:1. 2. Donat SM, Donat PE. Intrathoracic kidney: a case report with a review of the world literature. J Urol 1988;140:131-3. 3. Murphy JJ, Altit G, Zerhouni S. The intrathoracic kidney: should we fix it? J Pediatr Surg 2012;47:970-3.
Vol. 163, No. 4 October 2013
Figure 1. Anteroposterior chest radiograph showing gasdistended bowel loops (arrows) inside the right hemithorax, consistent with right-sided diaphragmatic hernia.
Figure 2. Excretory phase computed tomography scan, reconstructed in the coronal plane, showing physiologic contrast filling of the right ectopic kidney calices (arrow). No dilatation is seen in pelvicalyceal system.
1226.e1
A
male term infant (birth weight 3700 g) born via cesarean delivery after an uneventful pregnancy showed persistent cyanosis at 15 minutes. Radiograph revealed right-sided congenital diaphragmatic hernia (CDH) (Figure 1; available at www.jpeds.com). The infant was intubated. The echocardiograph showed pulmonary hypertension and bidirectional ductus arteriosus shunt. During primary hernia repair, the right kidney and part of
the right lobe of the liver were found to be herniated into the thorax. The right kidney was left at the thoracic level. Excretory phase computed tomography scan of the ectopic kidney at 1 month of age showed a normal-sized kidney with no dilatation in calices (Figure 2; available at www. jpeds.com). The infant was discharged home on room air on day 37. Contrast-enhanced coronal-plane magnetic resonance imaging at 3 months of age revealed normal parenchymal enhancement of the ectopic kidney (Figure 3). 99mTc-dimercaptosuccinic acid revealed renal functions of 52% (left kidney) and 48% (right kidney). CDH is a rare condition (0.01%-0.05% of births).1 The reported incidence of intrathoracic renal ectopia due to CDH is 0.25%.2 A right-sided thoracic kidney is much less common due to the location of the liver. Although intrathoracic kidney with CDH can be mobile,3 nephropexy was not performed in this case. Isolated intrathoracic kidney is usually asymptomatic and diagnosed incidentally on chest imaging. n Petek Genc Kayıran, MD Department of Pediatrics
Terman Gumus, MD Department of Radiology
Sinan Mahir Kayıran, MD Ilmay Bilge, MD Berkan Gurakan, MD Department of Pediatrics American Hospital Istanbul, Turkey
References
Figure 3. T1-weighted postcontrast coronal-plane magnetic resonance image showing normal parenchyma enhancement of the intrathoracic right kidney (arrow) compared with the left kidney in nephrographic phase (arrowhead). Left kidney (arrowhead) is normal in size and location.
J Pediatr 2013;163:1226. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.04.016
1226
1. Tovar JA. Congenital diaphragmatic hernia. Orphanet J Rare Dis 2012;7:1. 2. Donat SM, Donat PE. Intrathoracic kidney: a case report with a review of the world literature. J Urol 1988;140:131-3. 3. Murphy JJ, Altit G, Zerhouni S. The intrathoracic kidney: should we fix it? J Pediatr Surg 2012;47:970-3.
Vol. 163, No. 4 October 2013
Figure 1. Anteroposterior chest radiograph showing gasdistended bowel loops (arrows) inside the right hemithorax, consistent with right-sided diaphragmatic hernia.
Figure 2. Excretory phase computed tomography scan, reconstructed in the coronal plane, showing physiologic contrast filling of the right ectopic kidney calices (arrow). No dilatation is seen in pelvicalyceal system.
1226.e1