Erstellt von Sridhar Kaushik
vor mehr als 5 Jahre
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Case: Male infant; 39 5/7 wks GA; Mom: 21 y.o. G1P0 with no medical problems. Pregnancy uncomplicated. Normal prenatal labs. NSVD with apgars 8 and 9. B.Wt 3.34 kg, L 52 cm, H.C 32.3 cm all WNL. Initial NB exam WNL; AGA baby. Tolerated both breast and bottle and was voiding well and passing stool normally at discharge (2 days old). D/C wt 3.3 kg with 1.2% wt loss. On day 4 of birth: Infant brought to ER with feeding intolerance, non-bloody, non-bilious vomiting after each feeds. Noted to have 12% wt loss with self limiting brady episodes to 70 BPM. ECG- sinus bradycardia. 2 bolus of NS 10 ml/kg given with hR improvement to 100 BPM and intermittent HR dips to 90 BPM. Other vitals normal Sepsis w/u done, infant started on IV ampicillin/ gentamicin. Labs: Rapid RSV, Influenza A, B tests negative. CBC: WC: 8000/µL with normal diff. Hgb 17.6/ HCt 53.2; Plt: 409,000/µL Serum Na 151 mEq/L; rest of lytes, glucose normal. BUN 11 mg/dL Cr 0.5 mg/dL Ca 10.1 mg/dL CXR- normal Baby transferred to NICU. Abd X-ray there shows gasless abdomen and a cystic lucency projecting over lower mediastinum.
He is given nothing by mouth on admission and started on intravenous fluids. Pediatric surgery is consulted and computed tomography (CT) of the chest, abdomen, and pelvis is completed, which shows a complex gastric hernia with the gastroesophageal junction above the diaphragm (Fig 2).
A subsequent barium esophagography confirms a hiatal hernia or intrathoracic stomach with significant partial obstruction (Fig 3). Echocardiography is also performed, which shows a structurally normal heart with good function but an external impingement on the posterior aspect of the left atrium is noted.
A diagnosis of a hiatal hernia with compression of the heart is made, which is likely the factor contributing to the episodic bradycardia. Surgery done for hiatal hernia with gastrostomy tube. Post-op recovery and subsequent growth were good. Discussion Congenital hiatus hernia (CHH) is rare in NB period and is due to the herniation of the abdominal organs, most commonly the stomach, into the thorax from a physiologic opening caused by the laxity of the attachment of the stomach and gastro-esophageal junction. It is different from Congenital Diaphragmatic Hernia which is due to a pathological defect in the diaphragm. Hiatal hernias have been classified into 4 types: Type 1- sliding hernia, the most common type, accounting for more than 90% of cases. Type 2- rolling or paraesophageal hernia Type 3- a combination of these two types and Type 4- characterized by the presence of a structure other than the stomach, such as the omentum, colon, or small bowel within the hernia sac The most common of these is a sliding-type hernia accounting for more than 90% of all the cases of hiatal hernia. Although a paraesophageal hernia is a rare entity it is more prone to incarceration, strangulation, complete gastric herniation with organoaxial volvulus (upside down stomach), and a perforation of herniated viscera. Symptoms of CHH respiratory distress vomiting poor feeding failure to thrive, poor feeding, Other signs such as bradycardia Diagnosis of CHH is usually made with a barium swallow esophagography, upper endoscopy, or CT scan showing the herniation of intra-abdominal contents into the thorax. Chest radiographs may show opacity in the posterior mediastinum with or without an air-fluid level. Barium swallow will show three or more gastric folds above the diaphragm hiatus and a pouch of stomach more than two cm above the hiatus. On CT scan, the diaphragmatic crura are seen separated by more than 15mm and the protrusion of hernia above the diaphragm hiatus. Differential Diagnosis would include: Sepsis Gastroesophageal Chest radiographs may show opacity in the posterior mediasti-num with or without an air-fluid level. Barium swallow will show three or more gastric folds above the diaphragm hiatus and a pouch of stomach more than two cm above the hiatus. On CT scan, the diaphragmatic crura are seen separated by more than 15mm and the protrusion of her-nia above the diaphragm hiatusreflux disease Respiratory: pneumonia, pneumatocele, pneumothorax, pleural effusion, and Congenital diaphragmatic hernia References See Prenatal diagnosis of hiatus hernia in a fetus with asplenia syndrome Prenatal diagnosis of congenital hiatus hernia
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