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Weekly Chest CasesImaging Conference Cases

Case No : 6

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  • Age/Sex 10 / M
  • Case Title A 10-month-old boy presented with fever, dehydration and malnutrition. His oral intake was decreased and activity was dropped for 2 days. He have been suffering from repeated vomiting. The breathing sounds were coarse in both lung fields and leuk
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Diagnosis With Brief Discussion

Courtesy
C
Imaging Findings
Chest radiograph shows soft tissue opacity in the right lower hemithorax posterolateral to the heart.

This opacity obscures the right cardiac margin & right hemidiaphragm.

CT shows 5 x 3 x 3 cm-sized cystic mass in the right posterior mediastinum.

Follow up chest radiograph and CT reveals an opacity with air-fluid level in the right thorax and the distal part of nasogastric tube is located within the mass.

A barium meal examination shows a partial right intrathoracic stomach and non-obstructive 1800 organoaxial torsion with the the greater curvature lying against the right chest wall. The esophagogastric junction is above the diaphragm.

At surgery, the gastric fundus was found to lie in the right chest cavity above the diaphragm and the esophageal hiatus was wide. The stomach was moved into the abdominal cavity and Nissen fundoplication was done.
Discussion
Diaphragmatic hernias are classified as Bochdalek's hernia (90%), hiatal hernia, and Morgagni's hernia. The parahiatal hernias are characterized by herniation through a small defect adjacent to the esophageal hiatus. They are rarely seen and may be associated with previous trauma.


The hiatal hernias are classified according to their anatomic characterirstics: as sliding (type I), charaterized by a upward migration of the gastroesophageal junction into the posterior mediastinum, paraesophageal (type II), characterized by an upward dislocation of the gastric fundus alongside a normally positioned gastroesophageal junction.

A mixed hernia (type III), charaterized by upward dislocation of both cardia and gastric fundus. The paraesophageal hiatal hernia accounts for only five percent of al diaphragmatic defects. In fact, more than 90% of paraesophageal hernias are actually of the mixed type.

The end stage of a type I or type II hernia occurs when the whole stomach migrates up into the chest by rotating 180 degree around its longitudinal axis with the cardia and pylorus as fixed points (intrathoracic stomach), and the last one is multiorgan hiatal hernia (type IV; herniation of viscera other than stomach).


A right intrathoracic stomach is a rare form of congenital hiatal hernia seen in childhood. It appears to have a male predominance and might be influenced by family history and genetics. Unlike other congenital diaphragmatic defects, a right intrathoracic stomach are discovered later in childhood because of the absence of a mass effect, pulmonary hypoplasia, and signs of incarceration or strangulation.

The symptoms are usually chronic because of gastroesophageal reflux or compromising the gastric outlet. The esophageal hiatus is anatomically situated at the level of the T10 vertebra. In this case the lower esophagus is short and the gastroesophageal junction is located high above the esophageal hiatus. The intrathoracic, extrapleural stomach is not dilated or obstructed.


It must be differentiated from a Morgagni's hernia and the rare right posterolateral Bochdalek's hernia (Lt : Rt = 5 : 1 or 83-94% in Lt), which usually contains the liver, small and large intestine and is associated with a mass effect and a shift of the mediastinal structures into the left hemithorax.


It also be differentiated from mediastinal cysts. Most mediastinal cysts are of congenital origin and include foregut-duplication cyst (bronchogenic, duplication, and neurenteric cysts), pleuropericardial cyst and thymic cyst.


Mediastinal bronchogenic cysts result from abnormal budding of the ventral diverticulum of the foregut. Usually there is no direct communication with the tracheobronchial tree, althouth connection by a stalk is common. Thus infection is less likely. Most mediastinal bronchogenic cyst are located in paratracheal (19%) and near the carina (52%), Less commonly, they are located in paraesophageal (14%), hilar, and miscellaneous (thymus, pericardium, and anterlateral surface of a thoracic vertebral bodies) areas. They rarely occur in the anterior mediastinum or the inferior aspect of the posterior mediastinum.


Esophageal duplication cysts have been hypothesized to result from either abnormal budding of the forgut ot a failure of complete vacuoation of the solid esophagus. They are asymptomatic or present symptoms relating to respiratory distress or swallowing difficulty. Approximately 10-15 % of all gastroenteric duplicationas are located in the thorax adjacent to the esophagus. The cysts usually located within or adjacent to the wall of the esophagus. The lesions rarely communicate with the esophageal lumen. The mass tend to have smooth outlines and usually impinges on barium-filled esophagus.


Neurenteric cysts contain both neural and gastrointestinal elements. The cyst is connected by a stalk to the meninges and associated with a vertebral anomaly. Vertebral anomalies include hemivertebrae, fusion anomalies and spina bifida.


Lymphangioma can occur in the posterior mediastinum, althouth they are more commonly found anteriorly.
Pancreatic pseudocyst is rare in this age.


Elective repair is offered as the treatment of choice in the paraesophageal hernia. Surgical techniques include hernia reduction, crural closure, and fundoplication.
Reference
1. Haddad MC, Youssef BA, Sammak BM, Duff A. Right intrathoracic stomach secondary
to congenital hiatal hernia and organoaxila torsion. AJR 1996;167:66-68
2. Hashemi M, Sillin LF, Peters JH. Current concepts in the management of paraesophageal hiatal hernia. J Clin Gastroenterol 1999;29(1):8-13
Keywords
Diaphragm, Congenital,
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