Multiple Esophageal Duplication Cysts in a Toddler: A Rare Scenario.

The foregut duplication cysts are rare congenital malformation and are usually "solitary" midline noncommunicating mediastinal structures. We present a 2-year-old asymptomatic boy having multiple noncommunicating foregut cysts in the neck as well as the thorax. Copyright:

INTRODUCTION

Foregut duplication cysts are rare entities but fairly common in pediatric super-specialty center. They represent abnormal migration or buddings from the primitive foregut. Robison et al. were probably the first to report a child with multiple esophageal duplication cysts (two thoracic).[1] There are only a handful of case reports published since then. Cocker et al. reported congenital multiple foregut duplication cysts (2 thoracic and 1 gastric) excised at the age of 20 months.[2] Kang et al. reported multiple esophageal (2 thoracic) duplication cysts in a 53-year-old man.[3] The index case had three esophageal duplication cysts (1 cervical and 2 thoracic) that has not been reported hitherto.

CASE REPORT

A 2-year-old boy presented with a complaint of progressively increasing left cervical swelling for 3 months [Figure 1a]. There was no history of trauma, fever, or dysphagia. The swelling was mildly fluctuant without any local rise of temperature, nontender, nontransilluminant, noncompressible and was going deep behind the clavicle. The dark old hemorrhagic fluid was aspirated from the swelling elsewhere before admission with us that led us to suspect it to be a foregut cyst. The hematological and biochemical parameters were within the normal limits. Ultrasonography of the abdomen was done to rule out any co-existing multiple duplication cysts of the bowel. Computed tomography of the chest was done to look for exact dimensions of the cyst, but to our surprise, it revealed two more noncommunicating cysts in the thorax [Figure 1b]. The patient was planned for the excision of cysts. First, the cervical cyst was excised and then, the thoracic cysts were excised through left posterolateral thoracotomy through 4th intercostal space. Both the cysts were noncommunicating, though they shared a common muscular wall with the esophagus.

Figure 1

(a) Intra-operative left cervical swelling. (b) Computed tomography of the thorax image showing noncommunicating cysts

All the cysts in the index case satisfied all the three histopathological criteria laid by Arbona et al.;[4] the cyst walls were focally lined by gastric mucosa with large areas of ulceration. The patient is doing well in follow-up for the past 2 years.

DISCUSSION

Foregut duplication cysts include enteric duplications and cysts (lined by intestinal epithelium), bronchogenic cysts (lined by respiratory epithelium), and neurenteric cysts (associated with vertebral anomalies or having a connection with the nervous system). These are usually mediastinal in location and are more common on the right side.

The symptoms depend on the precise location of the lesion and its surrounding pressure effects. It may include respiratory distress, chest pain, dysphagia, cardiac arrhythmias, recurrent respiratory infections, etc., or the cyst(s) may be asymptomatic as in our case. Even incidentally discovered and asymptomatic ones should be surgically treated in view of 75%–90% infection rate and rare chance of malignant transformation.

The differential diagnoses for neck swelling such as in the present patient include parapharngeal abscess, duplication cyst, lymphatic malformation, branchial cyst, thymic cyst, cervical bronchogenic cyst, etc., The possibility of the abscess was ruled out in view of the absence of signs of inflammation and normal laboratory parameters. Differential diagnoses that were entertained in this case included cervical lymphatic malformation with secondary bleeding or cervical foregut duplication cyst with ectopic mucosa. The bronchogenic cysts are usually located in the thorax, with a few case reports of ectopic location in the neck.

Kawashima et al. have recently tabulated 20 cases of solitary infantile cervical esophageal duplication cysts collected from available literature; most of them were symptomatic.[5] The clinical findings and radiological evaluation helped in guiding the surgical approach. Computed tomography scan was the mainstay of presurgical diagnosis; it helps in providing dimensions, anatomic relations to the vicinity of structure, and planning surgery.

The cyst puncture or aspiration should be avoided in the cervical duplication cyst as it may lead to respiratory compromise secondary to bleed from adjacent vessels. Surgery is curative, and no recurrence has been reported.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.