Congenital Neck Masses

1. Congenital Neck Masses

2. Case review Patient was found to have a left sided neck mass on fetal ultrasound at the age of 16 weeks gestation. EXIT (Ex-utero Intrapartum Treatment) procedure was planned due to high level of concern for CHAOS (Congenital High-Airway Obstruction Syndrome) based on high resolution US studies. Three days prior to planned procedure, the mother presented in preterm labor, EXIT procedure was performed with competent airway observed.

5. Case review Patient subsequently admitted to CHOC due to increased size of the cystic lesion with development of mild inspiratory stridor. Surgical excision performed on day of life 10 through left neck incision only. The remaining hospital course was uneventful.

6. Neck Masses - Considerations Age Location Lateral � branchial cleft cysts and laryngoceles Midline � thyroglossal duct cyst, dermoid cyst, thymic cyst, and teratoma Exceptions � hemangiomas and vascular malformation lesions

7. Mulliken and Glowacki: simple biologic classification Hemangiomas and vascular malformations Hemangioma: not evident at birth, rapid endothelial proliferation followed by slow involution. Vascular malformation: present at birth, normal rate of endothelial turnover, lesion grows with the child, progressive dilation of vessels Vascular Lesions - Classification

8. Hemangiomas Most common pediatric tumor. Rapid proliferation of endothelium, slow progressive involution. Less than 33% present at birth 90% of lesion involute CT w/ contrast or MRI w/ Gadolinium. If associated w/ stridor, must rule out subglottic hemangioma. Kasabach-Merritt syndrome relation?

9. Vascular malformations Nevus flammeus vs. port wine stain Sturge-Weber Syndrome Venous Malformations Lips and cheeks Expand with jugular venous congestion Intraosseous �soap bubble� appearance AVM High flow � CHF Thrill/bruit Pain, ulceration, bleeding and pulsatile tinnitus

10. Lymphangioma Microcystic and macrocystic Large, soft, compressible masses 60% presenting in 1st year, 90% by three years of age Type I and Type II Anterior/OC/FOM vs. Posterior triangle 40% presenting with airway compromise Centrifugal vs. Centripetal theory MRI Spontaneous regression is rare (8-15%) and surgical excision is the treatment of choice. Recurrence is 10-52%

11. Branchial System Six pairs of mesodermal arches separated externally by ectodermally-lined clefts and internally endodermally-lined pouches Each arch consists of a nerve, artery, muscle rudiment and cartilaginous skeleton Neck musculature gains contributions from cervical somites.

12. 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

13. Branchial System First Branchial Cleft persists as the external auditory canal, and tympanic membrane First Branchial Arch Nerve- 5th cranial nerve Artery- maxillary artery First Branchial Pouch persists as the Eustachian tube, middle ear, portions of the mastoid bone.

14. Branchial System Second Branchial Cleft: Cervical sinus of His 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

15. Branchial System Third Branchial Cleft: Cervical sinus of His 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. Third Branchial Pouch Inferior parathyroids Thymus gland and thymic duct

16. Branchial System Fourth Cleft: Cervical sinus of His 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 Fourth and Sixth Branchial arches fuse to form the laryngeal cartilages.

17. Branchial System Sixth Branchial Arch Muscles- remaining laryngeal musculature Nerve- Recurrent Laryngeal Nerve Artery- Pulmonary Artery and ductus arteriosus

18. First Branchial Cleft Cysts Type I Ectodermal duplication anomaly of the EAC with squamous epithelium only Fistulous tracts near the lower portion of the parotid gland Parallel to the EAC Pretragal/ postauricular sulcus Surgical Excision

19. First Branchial Cleft Cysts Type II Represents anomalous EAC and rudimentary pinna (epithelium, mesoderm) Cyst/ tract below angle of mandible and through the parotid in variable position to CN VII Tract runs from the neck to the EAC or middle ear Surgical excision- superficial parotidectomy

20. Second Branchial Cleft Cysts Most Common (90%) branchial anomaly � failure of obliteration of cervical sinus of His Painless, fluctuant mass in anterior triangle Can occur at carotid bifurcation or parapharyngeal space 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

21. Third Branchial Cleft Cysts Patients present with recurrent infections of the lower lateral neck Masses low in the anterior neck, more often on the left side Sinus tract starting at the piriform fossa, through the thyrohyoid membrane, tracking under CN XII and carotid, but anterior to CN X Often track through the upper pole of the thyroid

22. Fourth Branchial Cleft Cysts May have opening located near the apex of the pyriform sinus, fistula or sinus tract that travels between the superior and inferior laryngeal nerves, or an external opening along the anterior border of the sternocleidomastoid muscle in the lower neck. Very rare, first reported by Sanborn in 1972

23. Thyroglossal Duct Cyst Most common congenital midline mass Elevates with tongue protrusion Commonly at the level of the hyoid Ectopic thyroid tissue vs. thyroglossal duct cyst Ultrasound Radioisotope scan

24. Cervical Thymic Cysts Commonly in the lower neck, but anywhere from the pyriform sinus to the chest Failure of involution of the cervical thymopharyngeal ducts. Firm, mobile masses found in the lower aspects of the neck. CXR, CT scan

25. Dermoid Cysts Tissue from all three germinal layers Sweat and sebaceous glands Midline mass that does not elevate with tongue protrusion Misdiagnosed as thyroglossal duct cysts Total surgical excision to prevent recurrence

26. Teratoma All three germ cell layers, but foreign to the site of presentation Mature vs. immature Rarely present after the first year of life 20% associated maternal polyhydramnios Unlike adult teratomas, they rarely demonstrate malignant degeneration. Surgical excision.

27. Laryngoceles Enlarged laryngeal saccule Classified as internal, external, or both Internal Confined to larynx, involves FC and AE fold Hoarseness/ respiratory distress vs. neck mass External and Combined Laryngoceles 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

28. Plunging Ranula Simple - unilateral OC cystic lesion Plunging - though mylohyoid Cyst aspirate- high protein, amylase levels CT scan/MRI Treatment is intra-oral excision to include the sublingual gland of origin

29. Fibromatosis colli Torticollis with firm mass on the SCM Noted at birth or within 1st few weeks Inflammatory lesion of unknown etiology with muscle replacement by fibrosis Range of motion exercises Myoplasty of the SCM only if refractory to PT

30. Case Revisited What was the diagnosis?