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Pediatric Neck Masses

Pediatric Neck Masses. Ankur Patel DO MPH Wright State University Boonshoft School of Medicine Department of Surgery. Pre-test. What is the most common congenital neck mass in the pediatric population? A. First Branchial Cleft Anomaly B. Thyroglossal Duct Cyst C. Dermoid Cyst

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Pediatric Neck Masses

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  1. Pediatric Neck Masses Ankur Patel DO MPH Wright State University Boonshoft School of Medicine Department of Surgery

  2. Pre-test • What is the most common congenital neck mass in the pediatric population? • A. First Branchial Cleft Anomaly • B. Thyroglossal Duct Cyst • C. Dermoid Cyst • D. Second Branchial Cleft Anomaly

  3. Pre-test • Which test is the best way to evaluate a thyroglossal duct cyst? • A. Nuclear Medicine Scan • B Thyroid Function Test • C CT scan of the Neck • D Ultrasound of the thyroid/neck

  4. Pharyngeal Clefts • The 1st cleft forms the external auditory canal • The 2nd, 3rd and 4th Clefts are obliterated by expansion of the 2nd Arch.

  5. Embryology of the head and neck Congenital head and neck masses History (as it pertains to the head and neck) Physical exam (emphasis on the neck) Radiologic studies Differential diagnosis

  6. First Arch(Manidular Arch) • Skeletal contributions • Maxillary process • Mandibular projection (Meckel’s cartilage) • Muscles • Muscles of mastication, anterior belly of digastric, mylohyoid, tensor tympani, tensor veli palatini • Nerve • Trigeminal Nerve (V3) • Artery • Maxillary Artery

  7. Second Arch(Hyoid Arch) • Skeletal Contributions(Reicherts cartilage) • Stapes, Styloid process, lesser horn of hyoid • Muscles • Muscles of facial expression, stapedius, posterior belly of digastric, stylohyoid • Nerve • Facial Nerve • Artery • Stapedial Artery

  8. Third Arch • Skeletal contributions • Greater horn of the Hyoid and lower body • Muscles • Stylopharyngeus • Nerve • Glossopharyngeal Nerve • Artery • Common Carotid and proximal internal carotid

  9. Fourth Arch • Skeletal contributions • Thyroid and epiglottic cartilages • Muscles • Cricothyroid, muscles of the soft palate, pharyngeal muscles • Nerve • CN X, Superior laryngeal nerve • Artery • Right: subclavian • Left:Aortic Arch

  10. Sixth Arch • Skeletal Contributions • Cricoid, arytenoid, and corniculate cartilages • Muscles • Intrinsic muscles of the larynx • Nerve • CN X, recurrent laryngeal nerve • Artery • Right: Pulmonary Artery • Left: Pulmonary Artery and Ductuc Arteriosus

  11. Pharyngeal Pouches • 1st Pouch • Middle ear cavity and eustachian tube • 2nd Pouch • Crypt of the palatine tonsils • 3rd Pouch • Inferior parathyroid glands and thymus • 4th Pouch • Superior parathyroid gland and ultimobranchial body

  12. Branchial Cleft Anomalies • These anomalies compose approximately 30% of congenital neck masses[1]. • During embryologic development, the tissues of the neck are derived from branchial arches that are separated externally by grooves and internally by pharyngeal pouches. Incomplete or aberrant fusion of 2 adjacent arches can result in the formation of branchial cleft anomalies that include cysts, internal sinuses, external sinuses, and fistulas[2,3,4] • They are equally common in males and females and usually present in childhood or early adulthood.

  13. Diagnosis • Complete history and physical exam • Ultrasound, CT, and MRI can be used to help define the lesion and its course. • CT is able to demonstrate the fistula in up to 64% of cases[5] • Barium esophagram can also be helpful with a 50%-80% sensitivity for 3rd and 4th branchial fistulae[10].

  14. Treatment • Definitive treatment is complete surgical excision. • Unresected cysts and sinuses have a high risk for infection and incomplete resection results in high rates of recurrence[7]. • Timing for resection is controversial with some advocating for early resection to prevent infection whereas others support waiting until age 2 to 3 yrs of age[7,9] • Approx 20% of lesions have been infected at least once before the time of surgery[9] • Acute infections should be treated first.

  15. First branchial cleft sinus or fistula type II type I

  16. First Cleft Anomalies • Account for only 1% of branchial cleft malformations[7]. • These can present as cysts, sinuses, or fistula located between the external auditory canal and the submandibular area. • More common in females, and often misdiagnosed leading to a delay in exicision[9,10]

  17. First Cleft Anomalies • Type 1 • These are duplications of the membranous EAC, and are composed of ectoderm only. • They course lateral to the CN 7. • Usually present as swelling near the ear. • Type 2 • These contain ectoderm and mesoderm. • They course medial to CN 7. • Can present as preauricular, infra-auricular, or postauricular swellings inferior to the angle of the mandible or anterior to the SCM[1,7]

  18. Presentation • Can present with cervical, parotid, or auricular signs • Cervical signs consist of drainage from a pit-like depression at the angel of the mandible. • Can have purulent discharge if infected • Parotid involvement is likely if there is rapid enlargement due to inflammation. • Auricular signs can include swelling or otorrhea,

  19. Surgical Resection • Resection usually often requires at least partial facial nerve dissection and superficial parotidectomy. • It is also necessary to excise any involved skin or cartilage of the EAC. • Some tracts may extend medially toward the tympanic membrane, these tend to lie deep to the facial nerve[5]. • Some tracts can split around the facial nerve, making recurrence common in these cases[9]. • The average number of procedures required to achieve complete resection is 2.4 per pt[7].

  20. Second branchial cleft sinus or fistula

  21. Second Cleft Anomalies • These are the most common branchial cleft anomaly representing 95% of all cases[3]. • Second cleft fistulas course from the skin in the lower anterolateral neck and course superiorly between the internal and external carotid and pass over CN9 and CN 12 to enter the supratonsillar fossa.

  22. Second Cleft Anomalies • There are 4 types • Type A lesions lie anterior to the SCM and do not contact the carotid sheath • Type B(most common) • Lie deep to SCM and either ant or post to carotid sheath • Type C pass between ICA and ECA and adjacent to pharynx • Type D lie medial to carotid sheath and adjacent to tonsillar fossa

  23. Presentation • These usually present as a fistula or cyst in the lower anterolateral neck • Most cysts are diagnosed in adults during the 3rd to 5th decades of life as a nontender mass that can acutely increase in size after an URI[1]. • The enlargement can lead to respiratory compromise, torticollis, or dysphagia. • Fistula are usually diagnosed in infancy or childhood as chronic drainage from an opening along the anterior border of the SCM in the lower 3rd of the neck[1,7]

  24. Surgical Resection • Resection can be approached via a transverse incision placed within a natural skin fold. • Careful exploration for an associated fistula tract must be performed for complete excision. • A fistula can be cannulated with a lacrimal probe to help facilitate excision. • As the track is followed, the skin incision may have to be extended or step-ladder incision may be used for better visualization.

  25. Third & Fourth branchial cleft sinuses or fistulae

  26. 3rd & 4th Cleft Anomalies • These anomalies are rare • These anomalies usually contain thymic tissue • Both 3rd and 4th branchial cleft sinuses and fistula will open into the pyriform sinus.

  27. Presentation of 3rd Cleft Anomalies • They usually present as a cystic mass located at the lower anterior border of SCM, at the level of the superior pole of the thyroid gland[1,7] • They pass deep to internal carotid artery and CN 9, entering the thyrohyoid membrane above the internal branch of the superior laryngeal nerve, then entering the pyriform sinus. • Third arch cysts can cause hypoglossal nerve palsy if infected.

  28. Presentation of 4th Cleft Anomalies • These lesions present as lateral cysts in the lower third of the neck. • The tract depends on the side. • Right: the lesion loops around the subclavian, passes deep to the ICA, ascending to the level of CN XII, then it descends along the anterior border of the SCM to enter the pharynx at the pyriform apex. • Left: the tract descends into the mediastinum, looping around the aortic arch, medial to the ligamentum arteriosus, then ascends on a similar course to the right

  29. Presentation • Either can also present with tracheal compression and airway compromise in neonate due to rapid enlargement in size. • They can also present as cold nodules in the thyroid gland. • They can also present as recurrent URI’s, neck or thyroid pain, or thyroid abscess.

  30. Surgical Resection • Resection is similar to 2nd arch anomalies. • Endoscopy should be performed to identify the pyriform sinus entry point. • There are case reports of cauterization of these tracts[13] • Fourth anomaly arch resections require ipsilateral hemithryoidectomy to completely excise the tract and partial resection of the thyroid cartilage to provide adequate exposure of the pyriform sinus[14].

  31. Branchiotorenal syndrome (BOR) • Autosomal Dominant disorder • Prevalence is 1:40,000 to 1:700,000 • Typical phenotype • Cup-shaped pinnae, preauricular pits, branchial fistula, CHL, SNHL, or mixed hearing loss, and renal anomalies ranging from mild hypoplasia to complete absence. • Hearing loss and pre-auricular pits are the most common findings. Branchial cleft Fistula occur in approximately 50% of individuals[12].

  32. Thyroglossal duct cyst

  33. TGDC • Thyroglossal duct anomalies are the second most common pediatric neck mass, behind adenopathy[1]. • Thyroglossal duct remnants occur in approximately 7% of the population, although only a minority of these become symptomatic[1].

  34. Embrylology • The thyroid gland forms from a diverticulum located between the anterior and posterior muscle complexes of the tongue at week 3 of gestation • This descends caudally and anteriorly to or through the hyoid bone. • By weeks 5-8 of gestation the thyroglossal duct obliterates, leaving a proximal remnant, the foramen cecum, and a distal remnant, the pyramidal lobe of the thyroid. • If the duct fails to obliterate before the formation of the mesodermal anlage of the hyoid bone, it persists as a cyst.

  35. Presentation • 2/3 of TGDC anomalies are diagnosed within the first 3 decades of life, with more than half being identified before age 10[1]. • Most common presentation is that of a painless mass near the hyoid bone in the midline[14]. • Although they are most common immediately adjacent to the hyoid (66%), they can also be located between the tongue and hyoid, between the hyoid and pyramidal lobe, within the tongue, or within the thyroid.[15,16].

  36. Presentation • The mass usually moves with swallowing or protrusion of the tongue. • Approximately 1/3 present with concurrent or prior infection, which is the more common presentation in adults[15]. • ¼ of patients present with a draining sinus that results from spontaneous drainage or surgical drainage of an abscess[15].

  37. Pre-operative Evaluation • Complete history and physical examination. • Imaging? • Ultrasound vs CT scan vs MRI vs Radionuclide scanning • Role of imaging is to look at lesion and presence of normal thyroid. • Median ectopic thyroid occurs in 1%-2% of TGDC[17]. • Role of pre-op TSH level? • Go by history, if any concerns for hypothyroidism, can obtain.

  38. Treatment • Elective surgical excision is the treatment of choice for uncomplicated TGDC to prevent infection of the cyst. • Sistrunk procedure is performed rather than simple excision to reduce the recurrence risk[15] • Infected cysts or sinuses are first managed by relieving the infection. • Most commonly infected with H. flu, Staph aureus, and Staph epidermidis.[15] • Formal I&D should be avoided to prevent seeding of the ductal cell outside the cyst, if possible. • Needle aspiration may be helpful for decompression and identification of organisms.

  39. Treatment • If a solid mass is encountered during exicision, a small piece can be sent for frozen section to rule out median ectopic thyroid. • If there is normal thyroid in place continue with the Sistrunk procedure. • If this is the patients only thyroid tissue than management is controversial. • Option 1 is to leave the tissue either in situ or repositioning it elsewhere in the body. • Option 2 is for removal due to the possibility of malignant degeneration.

  40. Treatment • Less than 1% of TGDC have malignant tissue, usually well-differentiated thyroid carcinoma. • This occurs more commonly in adults, but has been reported in children as young as 6[18]. • Papillary carcinoma is the most common[15]. • Sistrunk is associated with a 95% cure rate. • Some investigators recommend completion total thyroidectomy with nodal dissection, and radioactive iodine ablation is capsular invasion is present[15].

  41. Treatment • Recurrence rates for TGDC after complete excision using Sistrunk procedure is between 2.6%-5%[1] • Several factors have been identified predisposing patients to increased risk for recurrence. • Simple excision can result in recurrence rates as high as 38%-70%. • Children less than 2 yrs of age. • Intraoperative cyst rupture • Presence of a cutaneous component. • Historically pre-op or concurrent infection of the cyst was thought to increase recurrence, but a review showed that post op infections rather pre-op infections were associated with increased recurrence[15, 19].

  42. Recurrence • Recurrent TGDC excision has a higher risk for recurrence, 20%-35%.[15] • With recurrent disease a wider en bloc resection is required, ie central neck dissection. • Do you need to take tongue base mucosa? • Usually not in the majority of cases • Taking 5-10mm core of tongue base up to mucosa is sufficient[20].

  43. Dermoid Cyst • Dermoid cysts result from entrapment of epithelial elements along embryonic lines of fusion ( median and paramedian) and contain ectodermal and endodermal elements[1]. • They are lined by epithelium but contain epithelial appendages, such as hair follicles and sebaceous glands[15]. • Cervical dermoid cyst represent only 20% of head and neck dermoids[1].

  44. Presentation • Present as painless superficial subcutaneous masses in the anterior neck. • They can be close to the hyoid and move with swallowing or tongue protrusion confusing them with TGDC. • They do gradually increase in size due to the accumulation of sebum. • Infection is rare, but they can rupture and present with granulomatous inflammation. • They are often diagnosed before 3 yrs of age[1].

  45. Treatment • Ultrasound can be used to help delineate the depth of the lesion, and it’s relationship to the hyoid. • Complete surgical excision is recommended. • If it is found to be attached to the hyoid a Sistrunck should be performed to prevent inadequate excision of an atypical TGDC[21].

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