The mass was slightly larger , brighter , and more myxoid than depicted on the MRI from two years prior . After discussion of the risks and benefits , the patient and her mother opted for surgical excision .
FIGURE 2 : T2-weighted coronal MRI of the head and neck showing a heterogeneous high signal oval mass measuring 4.7 cm by 2.1 cm x 1.2 cm in the left side of the neck deep to the sternocleidomastoid muscle and separate from the cervical spine . There is a subtle whorled appearance .
Surgical dissection involved lateral retraction of the sternocleidomastoid to identify the carotid sheath . The carotid sheath structures were dissected . Careful dissection was performed to protect the laryngeal and vagus nerves . The carotid sheath structures were then retracted medially to expose the sympathetic chain and mass deep and lateral to the sheath . Dissection proceeded circumferentially around the mass until it was isolated at its superior and inferior extent on the sympathetic chain . The mass could not be freed from the sympathetic chain with blunt or sharp dissection ; therefore , the mass was excised with sacrifice of the sympathetic chain superiorly and inferiorly with application of surgical clips . The vagus and hypoglossal nerves , internal jugular vein , and carotid arteries were preserved .
The patient was discharged on postoperative day one . Pathologic examination revealed a 4.8 cm x 2.5 cm x 2.1 cm ganglioneuroma ( GN ). At her one-week postoperative check , the patient reported a slight worsening of eyelid droop but improved swallowing . She had no other complaints . At her 11-month postoperative follow-up , her incision was healed very well with no recurrence of the mass and no significant difference in ptosis from preoperative status .
DISCUSSION
Horner ’ s syndrome commonly presents as a triad of ptosis , miosis , and anhidrosis . It is caused by a disruption in the oculosympathetic pathway . The disruption can occur on the hypothalamospinal , preganglionic sympathetic , or postganglionic sympathetic tract , which is caused by first , second , or third-order lesions , respectively . Disruptions in the oculosympathetic pathway can be congenital or acquired and can be caused by trauma , inflammation , or neoplasms . 1 We present a case of an 11- year-old female with a neck mass and a history of Horner ' s syndrome . The mass was a GN that was successfully excised with no complications . There have not been reported cases , to our knowledge , of GNs presenting as Horner ' s syndrome , specifically left-sided ptosis , miosis , and anhidrosis .
GNs are well-differentiated neoplasms that originate from neural crest cells and arise in the autonomic nervous system . They are most commonly found in the sympathetic chain ganglia in the posterior mediastinum , retroperitoneum , and adrenal gland , and account for approximately 1 % of all soft tissue neoplasms . 2 Other less common locations include the middle ear , skin , parapharynx , orbit , and gastrointestinal tract . 3 , 4 , 5 The neck is a rare location of GNs , appearing there in approximately 5 % of all cases . 6
Although GNs are usually asymptomatic , mild compressive symptoms can occur , as well as diarrhea due to increased vasoactive peptide and rarely hypertension from increased catecholamines . 7 , 8 Most GNs in the cervical region present as an enlarging neck mass and less commonly throat pain or compressive symptoms such as dyspnea , dysphagia , and snoring . 9 Because GNs are so rare and the presentation is vague , it is important to obtain the proper diagnostic studies to narrow down the differential diagnosis . Characteristic findings on ultrasonogram , CT , and MRI can help narrow the differential diagnosis . Classic ultrasonogram findings include a well-circumscribed mass with a homogenous and hypoechoic texture . 10 Our patient had a well-defined homogenous mass but with sparse hyperechoic areas . Common CT findings for GNs include low attenuation on non-contrasted images with slight to moderate enhancement . Up to 60 % of GNs have calcifications , and some have patchy fat density . 10 If the CT study is dynamically enhanced , the arterial phase reflects none to slight enhancement with a CT value of 0-12 hounsfield units ( HU ). In delayed phase , enhancement is strengthened progressively with a CT value of 10-20 HU seen after 120 seconds . 11 While our studies were not dynamically enhanced , our images did show low attenuation . Our patient ’ s CT images did not
West Virginia Medical Journal • June 2022 • 21