Rapp Jordan,  Mallon Mea

Final Pr. ID: Poster #: CR-016

A 15 year old female with no significant past medical history except for long standing dysphagia and intermittent chest pain presented for a frontal and lateral radiograph. The x-ray showed a long segment density along the right heart border concerning for mediastinal mass. The patient subsequently underwent a contrast enhanced CT. Diffuse circumferential thickening of the esophagus began just below the thoracic inlet and extending for approximately 17cm to the level of the esophageal hiatus.

Evaluation of the esophageal lumen was performed at our institution utilizing reduced pediatric dose pulsed fluoroscopy with a barium esophagram. While the cervical and upper 1/3 of the thoracic esophagus had a normal lumen diameter and contour, there was irregular contractility and motility throughout the upper esophagus. The lower 2/3 showed narrowing which did distend with barium passage.

At this juncture a biopsy of the lesion was performed with the resulting pathology consistent with a leiomyoma. Preoperative planning MRI was then undertaken. As seen with the CT, diffuse circumferential thickening of the esophagus began just below the thoracic inlet with progressive thickening continuing distally to a maximum thickness just above the GE junction.

The patient subsequently went on to have an Ivor-Lewis esophagectomy with gastric pull through. The diagnosis of diffuse esophageal leiomyomatosis was confirmed by pathology.

Diffuse esophageal leiomyomatosis (DEL) was probably first described by Hall in 1916 in a case report of a 17 year old female who died of starvation due to dysphagia, with the diagnosis subsequently made on autopsy. While some cases of DEL are sporadic, as in our case, there is a well-established association with the x-linked Alport Syndrome, especially in the pediatric population. Up to 5% of Alport patients are affected by DEL and as much as 2/3 of pediatric patients with DEL carry the diagnosis of Alport Syndrome. Esophageal-Vulvar syndrome, characterized by leiomyomata of both the vulva and esophagus, presents with findings of DEL on imaging in many cases, often in young adult females. While presentations may vary, the majority of patients present with long standing dysphagia. An Iver-Lewis esophagectomy with a gastric pull-through is the treatment of choice. Read More

Authors:  Rapp Jordan , Mallon Mea

Keywords:  Esophagus, Leiomyomatosis

Guirguis Albair,  Lampl Brooke,  Park Ellen,  Mamoun Ihsan

Final Pr. ID: Poster #: EDU-028

Background: Esophageal disorders are relatively common in pediatric age group, clinically presenting with dysphagia, odynophagia, chest pain, cough, and aspiration. Etiologies may include infections and motility disorders, congenital disorders, and some traumatic (post foreign body or caustic ingestion)??? causes. Overlap exists in the appearance of esophageal pathology by fluoroscopy and cross-sectional evaluation. Recognition of the imaging features and how to differentiate various pathologies assist in diagnosis and further management.

Purpose: To review the esophageal disorders that can manifest in children with specific attention to the imaging features of different pathologies.

Educational goal: To recognize and describe the different pathologies and to emphasize the key imaging features encountered in esophageal disorders.

Technique and modalities of Imaging: Plain radiographs may be helpful in the newborn in the diagnosis of esophageal atresia, however esophagram is considered the primary tool for evaluation as it demonstrates both the anatomy and function of the esophagus. Cross sectional imaging such as CT and MRI may follow esophagram for further assessment of the extent of the diseases in some cases such as the wall involvement, extra-luminal extension, and extrinsic lesions.

Examples of cases (please refer to images)

Lymphoma involving the esophagus.
Ulcerating mass and irregular wall thickening of the lower esophagus.

Esophageal leimyomatosis (Alport syndrome).
Diffuse esophageal wall thickening and dilatation.

Congenital Esophageal stenosis.
Long segment of irregular concentric narrowing with mild proximal dilatation.

Listed below are some of the esophageal disorders included in the poster:
Congenital stenosis.
Achalasia.
Esophageal web.
Traumatic stricture (battery ingestion).
Esophageal perforation (post TOF repair).
Diffuse Esophageal Spasm.
Eosinophilic Esophagitis.
Tertiary contractions.
Hiatal hernia.



Conclusion: Imaging is one of the main tools in the work up for esophageal disorders, allowing for appropriate management and treatment. Several cases will be presented, including clinical information, illustrated images and descriptions of the imaging findings. These cases will cover different pathologies, as a quick comprehensive review for esophageal disorders. Read More

Authors:  Guirguis Albair , Lampl Brooke , Park Ellen , Mamoun Ihsan

Keywords:  Esophagus, Esophagram, Dysphagia