In The Name of God Congenital Neck Masses Dr. Babak Saedi otolaryngologist imam Khomeini hospital 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 Thyroglossal Cyst Painless, cystic midline swelling in the region of the hyoid bone Most common congenital midline mass Can occur any age most common up to 12 years F/M: 1 Commonly at the level of the hyoid Become symptomatic through inflammation – Pain and swelling Moves up on swallowing and protrusion of the tongue Thyroglossal Duct Cyst Cyst is a portion of the thyroglossal duct, found at the base of the tongue that remains patent = cystic structure Ectopic thyroid tissue vs. thyroglossal duct cyst Base of tongue mass Thyroid Scan Thyroglossal Duct Cyst B-mode Ultrasound Thyroid Scan in patients that do not demonstrate a normal thyroid by US. Thyroglossal Duct Cyst Simple Excision leads to high recurrence rate Sistrunk Procedure Patients at high risk for recurrence- Modified Sistrunk Procedure Dermoids and Teratomas 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 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 All three germ cell layers- Endoderm, mesoderm and ectoderm. Larger midline masses, present earlier in life. Epignathi :most differantiated Teratoma Surgical excision. 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 This region develop between 3 to 7 week in fetus. Ectoderm Pharyngeal Pouches and Aortic Arch Cranial region of developing embryo 1.Arch components and floor of primordial pharynx 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 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. Pharyngeal Arches. Second arch growing over the third and fourth arch 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 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 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. Branchial System Sixth Branchial Arch Muscles- remaining laryngeal musculature Nerve- Recurrent Laryngeal Nerve Artery- Pulmonary Artery and ductus arteriosus Arch Nerve Muscles Trigemina Muscles of mastication (mandibula l (CN V) Mylohyoid and r) First Skeletal structures ligaments Malleus Incus Anterior ligament of malleus Sphenomandibular ligament Stapes Styloid process Lesser cornu of hyoid Upper part of body of hyoid bone Stylohyoid ligament anterior belly of digastric Tensor tympani Tensor veli palatini Second (hyoid) Facial (CN VII) Muscles of facial expression Stapedius Stylohyoid Posterior belly of digastric Arch Nerve Muscles Third Glossopharyngea Stylopharyngeus Greater cornu of l hyoid (CN IX) Cranial laryngeal Fourth and Sixth branch of vagus (CN X) Recurrent laryngeal branch of vagus (CN X) Skeletal Structures Lower part of body of hyoid bone Cricothyroid Levator veli palatini Constrictors of pharynx Intrinsic muscles of larynx Striated muscles of esophagus Thyroid cartilage Cricoid cartilage Arytenoid cartilage Corniculate cartilage Cuneiform cartilage 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 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 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 Second Branchial Cleft Cysts and Fistula 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 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% Lymphangioma Vascular Lesions - Classification 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 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. 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 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