Evaluation and Management of Pediatric Neck masses :Evaluation and Management of Pediatric Neck masses Steven T. Wright, M.D. Ronald Deskin, M.D. April 23, 2003


Pediatric Neck Masses :Pediatric Neck Masses Congenital masses Benign lesions Vascular and lymphatic malformations Infectious and inflammatory conditions Malignant lesions


Embryology and Anatomy :Embryology and Anatomy Branchial System- 6 pairs of pharyngeal arches separated by endodermally lined pouches and ectodermally lined clefts. Each arch consists of a nerve, artery, and cartilaginous structures. The remaining neck musculature gains contributions from cervical somites.


Branchial system :Branchial system First Branchial arch Maxillary and mandibular (Meckel’s) process regress to leave the malleus and incus. Ossification around Meckel’s cartilage gives rise to the mandible, sphenomandibular ligament, and anterior mallear ligaments. Muscles- temporalis, masseter, pterygoids, mylohyoid, ant belly of digastric, tensor tympani, tensor veli palatini


Branchial system :Branchial system First Branchial Arch Nerve- 5th cranial nerve Artery- maxillary artery


Branchial system :Branchial system First Branchial Pouch persists as the Eustachian tube, middle ear, portions of the mastoid bone. First Branchial Cleft persists as the external auditory canal, and tympanic membrane


Branchial system :Branchial system Second Branchial Arch Reichert’s cartilage contributes to the superstructure of the stapes, the upper body and lesser cornu of the hyoid, the styloid process and stylohyoid ligament. Muscles- platysma, muscles of facial expression, posterior belly of digastric, stylohyoid, and stapedius Nerve- 7th cranial nerve Artery- stapedial artery


Branchial system :Branchial system Third Branchial Arch Lower body of the hyoid and greater cornu. Muscles- stylopharyngeus, superior and middle pharyngeal constrictors. Nerve- 9th cranial nerve Artery- common carotid and proximal portions of the internal and external carotid.


Branchial system :Branchial system Third Branchial Pouch Inferior parathyroids Thymus gland and thymic duct


Branchial system :Branchial system Fourth and Sixth Branchial arches fuse to form the laryngeal cartilages. Fourth Arch Muscles- cricothyroid, inferior pharyngeal constrictors Nerve- Superior Laryngeal Nerve Artery- Right Subclavian, Aortic arch Fourth Pouch- superior parathyroid glands and parafollicular thyroid cells


Branchial system :Branchial system Sixth Branchial Arch Muscles- remaining laryngeal musculature Nerve- Recurrent Laryngeal Nerve Artery- Pulmonary Artery and ductus arteriosus


Branchial system :Branchial system Epipericardial ridge- mesodermal elements of the sternocleidomastoid, trapezius, and lingual and infrahyoid musculature. Nerve- hypoglossal and spinal accessory nerve Cervical Sinus of His


Thyroid Gland :Thyroid Gland Endoderm of the floor of mouth between the 1st and 2nd arches. Descends as a bilobed diverticulum from the foramen cecum


First Branchial Cleft Cysts :First Branchial Cleft Cysts Type I Ectodermal Duplication anomaly of the EAC with squamous epithelium only. Parallel to the EAC Pretragal, post auricular Surgical Excision


First Branchial Cleft Cysts :First Branchial Cleft Cysts Type II Squamous epithelium and other ectodermal components Anterior neck, superior to hyoid bone. Courses over the mandible and through the parotid in variable position to the Facial Nerve. Terminates near the EAC bony-cartilaginous junction. Surgical excision- superficial parotidectomy


Second Branchial Cleft Cysts :Second Branchial Cleft Cysts Most Common (90%) branchial anomaly Painless, fluctuant mass in anterior triangle Inferior-middle 2/3 junction of SCM, deep to platysma, lateral to IX, X, XII, between the internal and external carotid and terminate in the tonsillar fossa Surgical treatment may include tonsillectomy


Fourth Branchial Cleft Cysts :Fourth Branchial Cleft Cysts Courses from pyriform sinus caudal to superior laryngeal nerve, to emerge near the cricothyroid joint, and descend superficial to the recurrent laryngeal nerve.


Thyroglossal Duct Cyst :Thyroglossal Duct Cyst Most common congenital midline mass Ectopic thyroid tissue vs. thyroglossal duct cyst Asymptomatic mass at or below the hyoid bone that elevates with tongue protrusion. Ultrasound Thyroid Scan in patients that do not demonstrate a normal thyroid by US.


Thyroglossal Duct Cyst :Thyroglossal Duct Cyst Simple Excision leads to high recurrence rate Sistrunk Procedure Patients at high risk for recurrence- Modified Sistrunk Procedure


Cervical Thymic Cysts :Cervical Thymic Cysts Failure of involution of the cervical thymopharyngeal ducts. Firm, mobile masses found in the lower aspects of the neck. CXR, CT scan


Dermoid and Teratoid Cysts :Dermoid and Teratoid Cysts Developmental anomalies composed of different germ cell layers. Isolation of pluripotent stem cells or closure of germ cell layers within points of failed embryonic fusion lines. Classified according to composition.


Dermoid Cysts :Dermoid Cysts Mesoderm and Ectoderm Midline, paramedian, painless masses that usually do not elevate with tongue protrusion. Commonly misdiagnosed as Thyroglossal Duct Cysts. Treatment is simple surgical excision


Teratoid Cysts and Teratomas :Teratoid Cysts and Teratomas All three germ cell layers- Endoderm, mesoderm and ectoderm. Larger midline masses, present earlier in life. 20% associated maternal polyhydramnios Unlike adult teratomas, they rarely demonstrate malignant degeneration. Surgical excision.


Laryngoceles :Laryngoceles Congenitally from an enlarged laryngeal saccule. Classified as internal, external, or both Internal Confined to larynx, usually involves the false cord and aryepiglottic fold. Hoarseness and respiratory distress vs. neck mass.


Laryngoceles :Laryngoceles External and Combined Laryngoceles Soft, compressible, lateral neck mass that distends with increases in intralaryngeal pressures. Through the thyrohyoid membrane at the entrance of the Superior Laryngeal Nerve. CT scan Asymptomatic vs. Symptomatic laryngoceles.


Vascular Lesions :Vascular Lesions Hemangiomas are the most common pediatric tumor. Rapid Growth, quiescence, involution. Not present at birth 70% resolution by age 7. CT w/ contrast or MRI w/ Gadolinium. If associated w/ stridor, must rule out Subglottic hemangioma.


Lymphangiomas :Lymphangiomas Classified as capillary, cavernous, and cystic Large, soft, compressible masses Posterior vs. anterior triangle location CT scan Spontaneous regression is rare and surgical excision is the treatment of choice.


Plunging Ranula :Plunging Ranula Simple ranula- unilateral oral cavity cystic lesion. Plunging ranula- pierce the mylohyoid to present as a paramedian or lateral neck mass. Cyst aspirate- high protein, amylase levels CT scan/MRI Treatment is intra-oral excision to include the sublingual gland of origin.


Sternomastoid Tumor of Infancy(Pseudotumor) :Sternomastoid Tumor of Infancy(Pseudotumor) Firm mass of the SCM, chin turned away and head tilted toward the mass. Hematoma with subsequent fibrotic replacement. Ultrasound Physical therapy is very successful. Myoplasty of the SCM only if refractory to PT.


Infectious and Inflammatory Lesions :Infectious and Inflammatory Lesions 40% of infants have palpable LAD 55% of pediatric patients. Most commonly involving the submandibular and deep cervical nodes.


Bacterial Cervical Adenitis :Bacterial Cervical Adenitis Tender, enlarged nodes Organisms- Staphylococcus, Group A Streptococcus Treatment- Beta-lactamase resistant antibiotic Fine Needle Aspiration


Deep Space Neck Abscess :Deep Space Neck Abscess Most commonly involves the retropharyngeal and parapharyngeal spaces. Polymicrobial Organisms CT scan Intra-oral vs. External surgical drainage. Lemierre’s syndrome Fusobacterium necrophorum


Tuberculous Mycobacteria :Tuberculous Mycobacteria Classically present with a single enlarged node, fevers, malaise. PPD is usually strongly reactive. CXR to rule out pulmonary disease. Treatment is similar to pulmonary TB 3-6 months of isoniazid, ethambutol, streptomycin, rifampin combination therapy


Nontuberculous Mycobacteria :Nontuberculous Mycobacteria More common than tuberculous mycobacteria Atypical presentations- usually without fever or systemic symptoms. CXR rarely positive. PPD is usually normal to intermediate reactivity. Treatment is less definitive.


Cat Scratch Disease :Cat Scratch Disease Bartonella henselae Fever, malaise, cervical LAD Warthin-Starry Stain- pleomorphic gram negative rods 10% of patients may require I&D Antibiotic therapy is anecdotal.


Viral Adenitis :Viral Adenitis Most common infectious process in the neck. Rhinovirus, adenovirus, enterovirus.


Infectious Mononucleosis :Infectious Mononucleosis Ebstein Barr Virus Exudative, necrotic tonsillitis Heterophile Antibodies, EBV IgG & IgM CMV/HIV can present with similar cervical lymphadenopathy.


Kawasaki Syndrome :Kawasaki Syndrome Multisystem vasculitis of unknown etiology Diagnosis includes 5 of 6 criteria: Fever >5 days, conjunctival injection, reddening/desquamation of palms/soles, injected oral cavity, polymorphous rash, cervical LAD Permanent Cardiac Damage in 20% of untreated cases. Treatment in the acute phase is with high dose aspirins and immunoglobulins.


Bibliography :Bibliography Bailey BJ, ed. Head and Neck Surgery- Otolaryngology. J.B. Lippincott. Philadelphia. 1993. Bluestone CD and Stool SE. eds. Pediatric Otolaryngology. Second Edition. W.B. Saunders Company. 1990. Burton DM, Pransky SM. Practical Aspects of Managing non-malignant Lumps of the Neck. The Journal of Otolaryngology 21:6, 1992. Cunningham MJ. The Management of Congenital Neck Masses. American Journal of Otolaryngology. Vol 13 (2): 78-92. March-April 1992. Kobayashi, T. Blanket Removal of the Sublingual Gland for Treatment of Plunging Ranula. Laryngoscope. Vol 113 (2), February 2003. Todd NW. Common Congenital Anomalies of the Neck. Surgical Anatomy and Embryology. Vol 73 (4), August 1993. Torsiglieri, AJ. Pediatric Neck Masses: Guidelines for Evaluation. International Journal of Pediatric Otorhinolaryngology. Vol 16 (1988). Pgs 199-210. Triglia JM, Nicollas R. First Branchial Cleft Anomalies. Archives of Otolaryngology- Head and Neck Surgery. Vol 124, March 1998. Pgs 291-295. Tunkel DE, Domenach EE. Radioisotope Scanning of the Thyroid Gland Prior to Thyroglossal Duct Cyst Excision. Archives of Otolaryngology- Head and Neck Surgery. Vol 124. May 1998. Wetmore WF, Muntz HR. Pediatric Otolaryngology- Principles and Practice Pathways. Thieme. New York. 2000.