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Clinical case: Horner's syndrome
This is a clinical case of a Horner's Syndrome.
After reviewing this case a student should be able to describe:
- The arrangement of the lymph nodes and the drainage points of the lymphatic vessels of the neck. What might cause cervical lymphadenopathy?
- The condition that the child likely would have had if the tumor had invaded the sternocleidomastoid muscle and caused the muscle to become fibrotic.
- What is Horner syndrome and why it developed in this patient? Also, in addition to the sympathetic trunk, what nerves might be at risk in the surgical extirpation done in this case and the consequences of injury to those nerves?
- The origin of the internal carotid artery, including the vertebral level of the cervical segment. The relationship of this artery to the sympathetic innervation of the head.
This article is based on a case report published in the Journal "Case Reports in Otolaryngology" in 2016, by Oz. Erol, Al. Koycu, and Er. Aydin.
It has been modified and reviewed by Joel A. Vilensky PhD, Carlos A. Suárez-Quian PhD, Aykut Üren, MD.
Case description
History
The patient was a five-year old boy complaining of a non-painful swelling in the left side of the neck that had been present for the past four months. The boy had not had any other complaints such as loss of weight, fever, night sweating, respiratory distress, or difficulty swallowing.
Physical examination
The physical examination revealed a hard, mobile mass with an approximate size of 2 X 3 cm on the anterior surface of the sternocleidomastoid muscle in the patient’s neck (Figure 1).
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Imaging
A prior neck ultrasonography had suggested a diagnosis of lymphadenopathy. MRI revealed a limited 2.8 X 2.3 cm lesion on the sternocleidomastoid muscle and internal carotid artery. The lesion had a mass effect in that the parotid gland was displaced anteriorly (Figure 2B).
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Diagnosis
A fine-needle aspiration biopsy was refused by the family. Examination of the patient one month later revealed additional growth of the tumor; the tumor was now approximately 4 X 4 cm. Surgery was recommended to the patient’s family with the caveat that:
- the tumor was deep to the large arteries in the neck,
- that it involves nerves in addition to the presumed enlarged lymph node, and accordingly that there were surgical risks of a droopy left eyelid, respiratory changes and some loss of vocal control.
The family gave written consent for the surgery. The patient underwent the surgery with provisional diagnosis of lymphadenopathy with neural involvement.
Management
The encapsulated tumor on the sternocleidomastoid was found to be displacing the internal carotid artery and internal jugular vein laterally (Figure 2A); it was separated from the surrounding tissue by blunt dissection. The tumor was found to have continuity superiorly and inferiorly with nerve elements, and that it was entirely of neural origin (not an enlarged lymph node). The total tumor was extracted (Figure 3B).
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Histopathology
Subsequent, pathological evaluation revealed the nerve to be an intermixed type of ganglioneuroblastoma (Figure 4), consisting of:
- Schwannian stroma
- Neuropil-like islands
- Differentiating neuroblasts
- Immature and dysplastic ganglion cells
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Evolution
After the surgery the patient developed an ipsilateral Horner syndrome (miosis, ptosis, and anhidrosis). However, these signs were not apparent at follow-up three months post surgery.
After a I-131 Metaiodobenzylguanidine (I-131 MIBG) scan in the 6th- month postoperative examination, an involvement was observed in the contralateral cervical region; the patient was directed to undergo adjuvant chemotherapy to treat this contralateral tumor.
Metaiodobenzylguanidine [MIBG] is a catecholamine analog similar to noradrenaline, which can be up taken by hyperactive endocrine tissues and certain types of tumors including medullary carcinoma of thyroid, pheochromocytoma, neuroblastoma, paraganglioma and other neuroendocrine tumors. Combining a radioactive tracer [131I-MIBG], provides both a diagnostic tool and a therapeutic agent. 131I-MIBG is administered intravenously, and the whole body imaging of the patient is acquired 24 and 48 hour later. The patient in this case thus had a diagnostic 131I-MIBG examination.
At 12 months postoperative there was no indication of any recurrence presumably as a result of the adjuvant chemotherapy.
Anatomical and medical considerations
Neuroblastomas
Neuroblastomas are malignant solid tumors that develop from abnormal replication of immature sympathetic neurons. Neuroblastomas can develop anywhere sympathetic nervous tissues are present. They are most commonly seen in the suprarenal gland and within the sympathetic trunks.
Ganglioneuroma
Different levels of microscopic differentiations are observed in the majority of neuroblastomas. These tumors may transform into ganglioneuroma on the basis of fibrous stroma. Various levels of differentiation may be observed between pure neuroblastoma and pure ganglioneuroma, and these are called ganglioneuroblastoma. Cervical tumors are common pathologies in children. Whereas most are benign, nevertheless the suspicion of malignancy must come into play in cases of unusual tumors.
Ganglioneuroblastomas
Ganglioneuroblastomas represent a histological subgroup of rare neuroblastic tumors with moderate malignancy potential. They are very difficult to diagnose based solely on only physical examination and imaging modalities. Typically, final diagnosis is possible only after cytological and immuno-histochemical analysis following surgical excision.
In cases in which surgical excision is recommended, the parents consent should be obtained and they should be told of possible complications, such as:
- Horner syndrome due to sympathetic nerve chain incision
- respiratory, vocal and nutrition problems due to vagal nerve incision, etc.
Establishing Prognosis
Most neurogenic tumors secrete catecholamines. High levels of catecholamines may indicate the likelihood of recurrence. Genetic characteristics also may be used to predict tumor behavior. N-myc is a protooncogen, in which its chromosome arm resides at the far end of 2p. Detection of multiple N-myc copies indicates a fast tumor growth and a negative prognosis in patients who show these histological characteristics and are over 1 year of age. Also, a scintigraphic scan with MIBG should be conducted and invasion should be determined. In our patient, vanilmandelic acid (VMA) and N-myc protooncogene were found to be negative during the postoperative period.
Explanations to objectives
Objectives
- The arrangement of the lymph nodes and the drainage points of the lymphatic vessels of the neck. What might cause cervical lymphadenopathy?
- The condition that the child likely would have had if the tumor had invaded the sternocleidomastoid muscle and caused the muscle to become fibrotic.
- What is Horner syndrome and why it developed in this patient? Also, in addition to the sympathetic trunk, what nerves might be at risk in the surgical extirpation done in this case and the consequences of injury to those nerves?
- The origin of the internal carotid artery, including the vertebral level of the cervical segment. The relationship of this artery to the sympathetic innervation of the head.
Cervical lymph nodes and lymphadenopathy
Horizontal rings
Three hundred of the estimated 800 lymph nodes in the body are found in the neck. Lymph nodes in the neck are organized in two horizontal rings and two vertical chains on either side of the neck. The outer (superficial) ring is comprised of the:
- occipital
- preauricular (parotid)
- submandibular,
- submental nodes
The inner (deep) ring is formed by clusters of mucosa associated lymphoid tissue (MALT) located primarily in the nasopharynx and oropharynx (Waldeyer's ring).
Vertical chains
The vertical chain of lymph nodes consists of superior and inferior groups of deep cervical nodes associated with the carotid sheath [jugular groups (a node from such a group was initially thought to be enlarged in the patient described here)]. These nodes lie in the connective tissue of the carotid sheath; they are particularly closely related to the internal jugular vein. All lymph vessels of the head and neck drain to these nodes, either directly from the tissues or indirectly via outlying nodes. Lymph from the deep cervical nodes is returned to the systemic venous circulation via either the right lymphatic duct or the thoracic duct.
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Cervical Lymphadenopathy
Enlargement of the deep cervical lymph nodes (cervical lymphadenopathy) is a sign of a condition; it is not in itself a diagnosis. The causes of cervical lymphadenopathy may be:
- inflammatory
- degenerative
- neoplastic
In children, most palpable cervical lymphadenopathy is reactive or infective especially related to an upper respiratory infection. In individuals over the age of 50, metastatic enlargement from cancers of the respiratory or upper digestive tract is the most likely cause of cervical lymphadenopathy. The very wide drainage patterns of the deep cervical nodes render them susceptible to many diseases or abnormal processes within the head and neck, and also the thorax (e.g., lung and breast cancer).
Toricollis
Torticollis is also known as wry neck and is caused by a mechanical shortening of the sternocleidomastoid or spastic contraction of the muscle. It is sometimes seen in newborns. No one understands why some babies get torticollis and others don't. Developing the condition may be related to the cramping of a fetus inside the uterus or abnormal positioning (such as being in the breech position). The use of forceps or vacuum devices during childbirth is also associated with torticollis. Fortunately, the condition typically resolves with stretching exercises.
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Horner syndrome and endangered nerves
Horner syndrome is a group of ipsilateral signs and symptoms that result from interruption of the cervical sympathetic trunk to the head. The condition is characterized by:
- miosis (constricted pupil)
- partial ptosis (a weak, droopy eyelid)
- anhidrosis of face (decreased sweating)
- apparent enopthalmos (sunken eye, which is “apparent” because of the ptosis; the eye does not actually sink)
Preganglionic sympathetic neurons destined to innervate structures within the head arise from the upper part of the thoracic-lumbar sympathetic outflow (mainly T2) and ascend in the cervical sympathetic trunk to synapse in the cervical sympathetic ganglia (mainly superior cervical ganglion). They then ascend into the head using primarily arteries as pathways (internal carotid artery). The primary additional nerves that were at risk in this surgery were:
- the left superior laryngeal
- the left vagus
- the left glossopharyngeal
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The left vagus gives off the the left recurrent laryngeal nerve so if severed again there would be likely be vocal disturbances. Additionally, the vagus supplies parasympathetic innervation to the thoracic and abdominal viscera so the visceral effects associated with parasympathetic fibers would be affected (e.g., reduced peristalsis, dyspnea).
The superior laryngeal nerve supplies the mucosa to the upper larynx and is responsible for part of the cough reflex (via the internal laryngeal nerve), and also innervates the cricothyroid muscle via the external laryngeal nerve.
The glossopharyngeal provides much of the swallowing reflex but unilateral paralysis does not result in a significant disability.
Origin and relationships of the internal carotid artery
The cervical or C1 segment of the internal carotid (ICA), extends superiorly from the artery’s origin from the common carotid artery at the upper border of the thyroid cartilage, or at about the level of the fourth cervical vertebra. This segment terminates at the entrance to the carotid canal in the petrous part of the temporal bone.
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During this part of its course, the ICA lies anterior to the transverse processes of the upper three cervical vertebrae. The ICA is relatively superficial at its origin, where it is contained within the carotid triangle of the neck, and lies posterior and medial to the external carotid artery. The sternocleidomastoid muscle partially overlaps the vessel, which is also covered by deep fascia and the platysma.
As the ICA enters the carotid canal in the skull it provides a pathway for the internal carotid nerve, which contains mainly postganglionic sympathetic neurons destined to innervate all of the internal viscera and blood vessels of the head that receive sympathetic innervation. Most notably, the sympathetic fibers that dilate the pupil travel with the artery to the cavernous sinus where the fibers then join the ophthalmic division of the trigeminal nerve to reach the eye.
Clinical case: Horner's syndrome: want to learn more about it?
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