Clinical Quiz

A baby girl was born by normal vaginal delivery at 39 weeks gestation, with birth weight of 3.12 kg. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Limited resuscitation was required with free flow oxygen. The baby started to vomit on day 1 of life. The first three episodes were minor and consisted of gastric contents. The next two episodes of vomiting consisted of small amounts of fresh blood. Physical examination was normal at this time. She had a third episode of hematemesis at 27 hours of life, associated with pallor and a poor capillary refill time of 3 seconds. She required fluid resuscitation with intravenous normal saline (10 cc/kg) to obtain hemodynamic stability. Blood transfusion was not required. Apt test on vomited blood suggested the blood was of fetal origin. Chest X-ray revealed a normal mediastinum, and abdominal sonography was unremarkable. Coagulation studies could not be obtained due to clots in the specimen. Hemoglobin concentration and platelet count were within normal limits. Upper GI endoscopy performed the following day revealed a prominent, nonpulsatile purplish black mucosal lesion in the distal esophagus approximately 2 × 2 cm in size, 11 cm from the gum margins (Fig. 1). There was an associated small circular mucosal break overlying the lesion. A second prominent purplish black mucosal lesion (approximately 5 × 5 mm) was seen 1 cm below the first lesion. Endoscopy performed 2 months later revealed resolution of the lesions.FIG. 1: Lesion seen in distal esophagus.What is the likely diagnosis? Mallory-Weiss tear Arteriovenous malformation Intramural esophageal hematoma Boerhaave's syndrome ANSWER/DISCUSSION ANSWER C: Intramural esophageal hematoma (IEH) Intramural esophageal hematoma (IEH) is an uncommon cause of hematemesis (1). This rare condition is a form of esophageal injury and distinct from complete transmural rupture of the esophagus (Boerhaave's syndrome) and mucosal tear (Mallory-Weiss syndrome) (1). IEH has not been described as a cause of upper GI bleed in neonates to the best of our knowledge. Pathogenesis of IEH is unknown. It is thought to result from dissection of the mucosa from the muscular layers with formation of a hematoma (1). Multiple etiologies are known to cause this acute esophageal injury, which include esophageal instrumentation (2), impaired hemostasis, food impaction (3) and foreign bodies. Vomiting is a known precipitating cause of IEH (4). Upper GI endoscopy reveals a bluish/purplish swelling of the esophageal mucosa (4). Upper GI contrast radiography and CT scan of the chest may aid in the diagnosis (1). In our case, we believe that vomiting caused the intramural esophageal hematoma. Hematemesis, odynophagia or dysphagia, and chest pain are the cardinal symptoms in patients with IEH. Evaluating odynophagia in newborn babies is difficult. This baby was started on intravenous fluids after the first episode of hematemesis. Endoscopy revealed the typical endoscopic features of IEH. IEH did not completely occlude the lumen. This patient also had a small circular mucosal break which was not suggestive of a Mallory-Weiss tear. Typical Mallory-Weiss tears are linear breaks in the mucosa at the esophagogastric junction. However, these two forms of esophageal injury could be seen concomitantly (4). Boerhaave's syndrome usually presents with pneumomediastinum, pleural effusion or pneumothorax, along with chest pain and respiratory distress. This case was managed conservatively as suggested in the literature (4). IEH resolves spontaneously without any therapeutic intervention; however, endoscopic intervention has been reported. In one case, attempted biopsy of undiagnosed IEH resulted in rapid resolution of the lesion (5). The mainstays of treatment are elimination of oral feeds, IV fluid and parenteral nutrition (5). This baby resumed oral feeds the day after endoscopy and did not require parenteral nutrition. Healing was evident on the second endoscopy with ridging and adjacent depression of the esophagus. This endoscopic feature is suggestive of a healed intramural esophageal hematoma (5). This case illustrates the importance of endoscopic evaluation of newborn babies with significant upper GI bleed.

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