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Hepatic pulmonary fusion: a rare association of right-sided congenital diaphragmatic hernia.

Saurabh K, Kumar S, Chellani H, Aarya S - Ann Gastroenterol (2013)

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

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Hepatic pulmonary fusion is a rare association of right-sided congenital diaphragmatic hernia... The repair and reduction in this case depends on the extent of fusion to the lungs and the associated mediastinal structures... On examination, trachea was central in position with decreased air entry on right side of chest and right thumb and index finger syndactyly... Chest x-ray showed homogenous opacity involving right hemithorax mainly in mid and lower zone, with well-defined superior margin with no mediastinal shift and clefts in vertebrae (Fig. 1 A)... Contrast-enhanced computed tomography of thorax revealed migration of liver into the thoracic cavity in the posterior aspect with normal appearing anterior diaphragm with right lung hypoplasia or collapse and multiple cleft vertebrae (Fig. 1 B)... Peroperatively, the entire liver was found to be displaced into the right thoracic cavity with pneumatization of right lobe of liver and the diaphragm was stuck to remnant pulmonary tissue and adjacent mediastinal tissue... Most of the case reports favor that non-shifting of mediastinal structures away from the affected side in a right-sided congenital diaphragmatic hernia is an indicator of hepatic pulmonary fusion... This is also true in our case as there was no evidence of shifting of mediastinum in x-ray film... In summary, in a suspected case of right-sided congenital diaphragmatic hernia, hepatic pulmonary fusion should be suspected whenever imaging findings show intrathoracic liver and lack of mediastinal shift... Proper radiological imaging especially MRI should be considered for a better surgical outcome.

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(A) X-ray film of chest and abdomen showing homogenous opacity involving right hemithorax without mediastinal shift and presence of clefts in vertebrae. (B) Contrast-enhanced computed tomography of chest and abdomen showing migration of liver into the thoracic cavity in the posterior aspect with right lung hypoplasia/collapse
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Figure 1: (A) X-ray film of chest and abdomen showing homogenous opacity involving right hemithorax without mediastinal shift and presence of clefts in vertebrae. (B) Contrast-enhanced computed tomography of chest and abdomen showing migration of liver into the thoracic cavity in the posterior aspect with right lung hypoplasia/collapse

Mentions: This is a case of a full-term neonate who presented with signs of respiratory distress at 13 h of life. On examination, trachea was central in position with decreased air entry on right side of chest and right thumb and index finger syndactyly. Chest x-ray showed homogenous opacity involving right hemithorax mainly in mid and lower zone, with well-defined superior margin with no mediastinal shift and clefts in vertebrae (Fig. 1 A). Contrast-enhanced computed tomography of thorax revealed migration of liver into the thoracic cavity in the posterior aspect with normal appearing anterior diaphragm with right lung hypoplasia or collapse and multiple cleft vertebrae (Fig. 1 B). Peroperatively, the entire liver was found to be displaced into the right thoracic cavity with pneumatization of right lobe of liver and the diaphragm was stuck to remnant pulmonary tissue and adjacent mediastinal tissue. It was difficult to achieve a clear-cut line of cleavage. The diaphragmatic defect was partially approximated and sutured. Postoperatively, the patient died at day 11 of life due to respiratory insufficiency.


Hepatic pulmonary fusion: a rare association of right-sided congenital diaphragmatic hernia.

Saurabh K, Kumar S, Chellani H, Aarya S - Ann Gastroenterol (2013)

(A) X-ray film of chest and abdomen showing homogenous opacity involving right hemithorax without mediastinal shift and presence of clefts in vertebrae. (B) Contrast-enhanced computed tomography of chest and abdomen showing migration of liver into the thoracic cavity in the posterior aspect with right lung hypoplasia/collapse
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3959522&req=5

Figure 1: (A) X-ray film of chest and abdomen showing homogenous opacity involving right hemithorax without mediastinal shift and presence of clefts in vertebrae. (B) Contrast-enhanced computed tomography of chest and abdomen showing migration of liver into the thoracic cavity in the posterior aspect with right lung hypoplasia/collapse
Mentions: This is a case of a full-term neonate who presented with signs of respiratory distress at 13 h of life. On examination, trachea was central in position with decreased air entry on right side of chest and right thumb and index finger syndactyly. Chest x-ray showed homogenous opacity involving right hemithorax mainly in mid and lower zone, with well-defined superior margin with no mediastinal shift and clefts in vertebrae (Fig. 1 A). Contrast-enhanced computed tomography of thorax revealed migration of liver into the thoracic cavity in the posterior aspect with normal appearing anterior diaphragm with right lung hypoplasia or collapse and multiple cleft vertebrae (Fig. 1 B). Peroperatively, the entire liver was found to be displaced into the right thoracic cavity with pneumatization of right lobe of liver and the diaphragm was stuck to remnant pulmonary tissue and adjacent mediastinal tissue. It was difficult to achieve a clear-cut line of cleavage. The diaphragmatic defect was partially approximated and sutured. Postoperatively, the patient died at day 11 of life due to respiratory insufficiency.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Hepatic pulmonary fusion is a rare association of right-sided congenital diaphragmatic hernia... The repair and reduction in this case depends on the extent of fusion to the lungs and the associated mediastinal structures... On examination, trachea was central in position with decreased air entry on right side of chest and right thumb and index finger syndactyly... Chest x-ray showed homogenous opacity involving right hemithorax mainly in mid and lower zone, with well-defined superior margin with no mediastinal shift and clefts in vertebrae (Fig. 1 A)... Contrast-enhanced computed tomography of thorax revealed migration of liver into the thoracic cavity in the posterior aspect with normal appearing anterior diaphragm with right lung hypoplasia or collapse and multiple cleft vertebrae (Fig. 1 B)... Peroperatively, the entire liver was found to be displaced into the right thoracic cavity with pneumatization of right lobe of liver and the diaphragm was stuck to remnant pulmonary tissue and adjacent mediastinal tissue... Most of the case reports favor that non-shifting of mediastinal structures away from the affected side in a right-sided congenital diaphragmatic hernia is an indicator of hepatic pulmonary fusion... This is also true in our case as there was no evidence of shifting of mediastinum in x-ray film... In summary, in a suspected case of right-sided congenital diaphragmatic hernia, hepatic pulmonary fusion should be suspected whenever imaging findings show intrathoracic liver and lack of mediastinal shift... Proper radiological imaging especially MRI should be considered for a better surgical outcome.

No MeSH data available.


Related in: MedlinePlus