PEDIATRICS WK 3 HEAD AND NECK ALISON WALLACE MD, PHD

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Irene Gilmore
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1 PEDIATRICS WK 3 HEAD AND NECK ALISON WALLACE MD, PHD

2 Topics 1. Cervical lymphadenopathy 2. Lymphatic malformation 3. Thyroglossal duct cysts 4. Branchial cleft cysts 5. Thyroid masses

3 CASE 1

4 Case 1 A 2 yo boy is seen in your office with a midline neck mass that has been present for 2 months.

5 Differential diagnosis for a midline neck mass?

6 Differential diagnosis for a midline neck mass? Lymphadenopathy Ectopic thyroid Thyroglossal duct cyst Thymic cyst Dermoid cyst Ranula

7 Case 1 History? Physical? Work up?

8 Case 1 On examination, the mass is 2 cm in size, is not tender or pulsatile, and moves with protrusion of his tongue.

9 Case 1 Ultrasound of the neck demonstrates a midline cystic lesion sitting deep to the strap muscles with no surrounding lymphadenopathy or other pathology. The thyroid gland is noted in the normal location. Findings on thyroid function studies are normal.

10 U/S - Painless, fluctuant mass, spreads the strap muscles

11 CT - Thin walled, smooth, well defined homogeneously attenuating lesions

12 Case 1 - Summary A 2 yo boy is seen in your office with a midline neck mass that has been present for 2 months. On examination, the mass is 2 cm in size, is not tender or pulsatile, and moves with protrusion of his tongue. Ultrasound of the neck demonstrates a midline cystic lesion sitting deep to the strap muscles with no surrounding lymphadenopathy or other pathology. The thyroid gland is noted in the normal location. Findings on thyroid function studies are normal.

13 Case 1 Thyroglssal duct cyst

14 Which of the following statements is true regarding a thyroglossal duct cyst? 1. Simple excision of the mass is sufficient?

15 Which of the following statements is true regarding a thyroglossal duct cyst? 1. Simple excision of the mass is sufficient? 2. This mass most likely represents ectopic thyroid tissue?

16 Which of the following statements is true regarding a thyroglossal duct cyst? 1. Simple excision of the mass is sufficient? 2. This mass most likely represents ectopic thyroid tissue? 3. The rate of recurrence is very high after appropriate therapy?

17 Which of the following statements is true regarding a thyroglossal duct cyst? 1. Simple excision of the mass is sufficient? 2. This mass most likely represents ectopic thyroid tissue? 3. The rate of recurrence is very high after appropriate therapy? 4. These lesions can be found along the base of the tongue and hyoid bone?

18 Which of the following statements is true regarding a thyroglossal duct cyst? 1. Simple excision of the mass is sufficient? 2. This mass most likely represents ectopic thyroid tissue? 3. The rate of recurrence is very high after appropriate therapy? 4. These lesions can be found along the base of the tongue and hyoid bone? 5. Most lesions are associated with a draining cutaneous fistula?

19 TYROGLOSSAL DUCT CYSTS

20 Thyroglossal duct The thyroglossal duct is an epitheliumlined connection between the foramen caecum and the thyroid that develops during the descent of the thyroid. It usually involutes in the 8th-10th week of gestation.

21 Gross Anatomy The thyroglossal duct arises from foramen caecum located at the junction of anterior two thirds and posterior third of the tongue. From there it passes in front of the body of the hyoid bone, curving backwards and superiorly to reach behind the bone before once more turning inferiorly and continuing to the isthmus of the thyroid. The pyramidal lobe, if present, marks this point.

22 Thyroglossal duct cyst Failure of obliteration of the duct may lead to the formation of a thyroglossal duct cyst.

23 Epidemiology Typically present during childhood 90% present before the age of 10 If infected, they can present at any time Account for 70% of all congenital neck anomalies Second most common benign neck mass, after lymphadenopathy Most common midline neck mass in young patients

24 Clinical presentation Presentation is typically either as a painless rounded midline anterior neck swelling If infected, as a red warm painful lump It may move with swallowing and classically elevates on tongue protrusion

25 Location The cysts can occur anywhere along the course of the thyroglossal duct: Infrahyoid location is most common: ~45% (range 25-65%) At the level of hyoid bone: ~30% (range 15-50% Suprahyoid: 20-25% (less common in adults ~5%) Typically located in the midline (~70%) with those offmidline characteristically tucked next to the thyroid cartilage. Almost all are located within 2 cm of the midline, with more inferior lesions tending to be off midline.

26 Associations Ectopic thyroid: ~40%

27 Treatment and prognosis Complete resection of the cyst and duct up to the foramen caecum is curative. The Sistrunk procedure includes resection of the middle third of the hyoid bone. There is small risk of recurrence (~2.5%).

32 Complications Infection Malignancy Rare Seen in <1% of cysts Most frequently papillary thyroid carcinoma

33 Which of the following statements is true regarding a thyroglossal duct cyst? 1. Simple excision of the mass is sufficient? 2. This mass most likely represents ectopic thyroid tissue? 3. The rate of recurrence is very high after appropriate therapy? 4. These lesions can be found along the base of the tongue and hyoid bone? 5. Most lesions are associated with a draining cutaneous fistula?

34 Comments Most do not have a draining fistula tact, as with branchial cleft remnants. Ultrasound and thyroid function tests should be performed to be certain that there is thyroid tissue in the normal anatomic location so that the patient is not rendered hypothyroid in the postoperative period. Surgical treatment involves removal of the cyst tract along with the central hyoid bone the Sistrunk procedure. Simple cyst excision alone results in high rates of recurrence, where as a Sistrunk procedure has reported recurrence rates of less than 5%.

36 CASE 2

37 Case 2 A 2 yo girl is seen in your office with a lateral neck mass that has been present for 2 months.

38 Differential diagnosis for a lateral neck mass?

39 Differential diagnosis for a lateral neck mass? Paramedian thyroglossal duct cyst Cystic lymph nodes Necrotic nodal metastases, especially SCC and papillary thyroid cancer Tuberculous adenitis Vascular lesions jugular vein thrombosis mycotic aneurysm of the neck Neurogenic tumours Neuroblastoma Schwannoma Neurofibroma Ganglioneuroma Cervical dermoid cyst Atypical mycobacterial infection

40 Case 2 History? Physical? Work up?

41 Case 2 On examination, there is a rounded swelling just below angle of mandible. It is non-tender and fluctuant.

42 U/s - sharply demarcated, anechoic

43 CT - rounded, sharply circumscribed fluid density

44 Case 2 Branchial cleft cyst

45 Which of the following statements is false in regard to branchial cleft remnants? 1. Branchial fistulas are more common than external sinuses, which are more common than branchial cysts.

46 Which of the following statements is false in regard to branchial cleft remnants? 1. Branchial fistulas are more common than external sinuses, which are more common than branchial cysts. 2. First branchial cleft remnants are typically located along the anterior border of the SCM muscle.

47 Which of the following statements is false in regard to branchial cleft remnants? 1. Branchial fistulas are more common than external sinuses, which are more common than branchial cysts. 2. First branchial cleft remnants are typically located along the anterior border of the SCM muscle. 3. Second branchial cleft remnants are the most common branchial cleft remnants.

48 Which of the following statements is false in regard to branchial cleft remnants? 1. Branchial fistulas are more common than external sinuses, which are more common than branchial cysts. 2. First branchial cleft remnants are typically located along the anterior border of the SCM muscle. 3. Second branchial cleft remnants are the most common branchial cleft remnants. 4. Third branchial cleft remnants are typically located in the suprasternal notch or clavicular region.

49 Which of the following statements is false in regard to branchial cleft remnants? 1. Branchial fistulas are more common than external sinuses, which are more common than branchial cysts. 2. First branchial cleft remnants are typically located along the anterior border of the SCM muscle. 3. Second branchial cleft remnants are the most common branchial cleft remnants. 4. Third branchial cleft remnants are typically located in the suprasternal notch or clavicular region. 5. Second branchial cleft remnants are typicaly located along the anterior border of the sternocleidomastoid muscle.

50 BRANCHIAL CLEFT REMNANTS

51 Branchial Cleft Remnants Structures of the head and neck are derived from six pairs of branchial arches with intervening external clefts and internal pouches. Failure of these structures to regress leads to congenital branchial fistulas, sinuses and cysts. Fistula > sinus > cyst

52 Branchial Cleft Remnants

53 ANOMALIES The range of anomalies can include: Cyst: no internal or external communication Fistula: communicates both internally and externally Sinus: incomplete tract

54 First branchial remnants Typically located: In the front or back of the ear In the region of the mandible Fistulas typically course through the parotid gland deep or through branches of the facial nerve and end in the external auditory canal

55 Second branchial remnants Most common Typically located: Along the anterior border of the SCM muscle

56 Third branchial remnants Typically located: At the sternal notch or the clavicular region

57 Second branchial remnants Most common. Second branchial cleft cysts are a cystic dilatation of the remnant of the 2nd branchial apparatus, and along with 2nd branchial fistulae and sinuses accounts for 95% of all branchial cleft anomalies. Typically located along the anterior border of the sternocleidomastoid muscle.

58 Clinical Presentation Although a congenital abnormality, cysts tend to present in early adulthood (10-40 years of age) often after minor trauma or infection. Cysts present as a rounded swelling in the lateral neck Sinus or fistulas present earlier as continuous mucoid drainage.

59 Treatment Surgical excision

60 COMPLICATIONS Superimposed infection

61 Which of the following statements is false in regard to branchial cleft remnants? 1. Branchial fistulas are more common than external sinuses, which are more common than branchial cysts. 2. First branchial cleft remnants are typically located along the anterior border of the SCM muscle. 3. Second branchial cleft remnants are the most common branchial cleft remnants. 4. Third branchial cleft remnants are typically located in the suprasternal notch or clavicular region. 5. Second branchial cleft remnants are typicaly located along the anterior border of the sternocleidomastoid muscle.

63 CASE 3

64 Case 3 A 6 yo girl from Belarus is seen in your office with a neck mass that has been increasing in size for 2 months.

65 Case 3 History? Physical? Work up?

66 Case 3

67 Case 3

68 Case 3 Thyroid cancer

69 Thyroid nodules Uncommon in children Nodules diagnosed in children carry a greater risk of malignancy compared to those in adults (22%-26% vs 5%- 10%) Among 15- to 19-year- old adolescents, thyroid cancer is the eighth most frequently diagnosed cancer and the second most common cancer among girls.

70 Risk factors Radiation exposure Iodine deficiency A history of antecedent thyroid disease Several genetic syndromes High risk group - cancer survivors who were treated for their primary malignancy with radiation

72 Initial evaluation, treatment, and follow-up of the pediatric thyroid nodule Surgery implies lobectomy plus isthmusectomy in most cases

73 Initial postoperative staging

75 CERVICAL LYMPHADENOPATHY

76 Cervical lymphadenopathy Enlarged lymph nodes is one of the most common pediatric conditions. They occur usually along the sternocleidomastoid muscle border, often presenting in clusters.

77 Work up History Physical Imaging

78 Presentation Typical A small, mobile, rubbery, palpable mass in the anterior cervical triangle. Worrisome Fixed, nontender, progressively enlarging nodes in the supraclavicular region. Night sweats and a history of weight loss.

79 Workup Biopsy indications Worrisome features >2 cm Present for > 6 wks, not responsive to antibiotics Negative serology TB, EBV, Bartonella

80 Cervical lymphadenopathy

82 LYMPHATIC MALFORMATION

83 Lymphatic malformations Rare non-malignant masses consisting of fluid-filled channels or spaces thought to be caused by the abnormal development of the lymphatic system. Usually apparent at birth or by two years of age. Can affect any area of the body (except the brain), but most commonly affect the head and neck. Lymphatic malformations are not cancerous and there is no known risk of malignant transformation.

84 Presentation Soft cystic masses that distort the surrounding anatomy, including the airway Posterior neck region

85 Complications Airway compromise Infection Hemorrhage

86 Classification 1. Macrocystic 2. Microcystic 3. Mixed (a combination of the other two)

87 Macrocystic Characterized by a single or multiple fluid-filled pockets or cysts Generally form soft, large translucent masses Overlying skin may have a bluish tinge Macrocystic lymphatic malformations can potentially be extremely large, even large enough to obstruct the airway at delivery

88 Microcystic Appear as several small, raised sacs (vesicles) on the skin that contain clear or bloody (hemorrhagic) fluid. They generally grow slowly usually in proportion with a growing child. Microcystic lymphatic malformations can thicken or swell causing enlargement of surrounding soft tissue and bones.

89 Diagnosis?

90 Treatment options 1. Percutaneous drainage 2. Surgery 3. Sclerotherapy 4. Laser therapy 5. Radiofrequency ablation These different treatment options may be used in various combinations.

91 Treatment Complete surgical excision Difficult because of the intimate involvement with surrounding vital structures Radical resection with sacrifice of vital structures is not advocated Sclerosing agents include bleomycin or OK-432, derived from Streptococcus pyogenes

Congenital Neck Masses C. Stefan Kénel-Pierre, MD

Congenital Neck Masses C. Stefan Kénel-Pierre, MD SUNY-LICH Medical Center Department of Surgery Case Presentation xx year old male presents with sudden onset left lower neck swelling x 1 week Denies pain,