6. CONGENITAL NECK MASSES.pptx23333455678

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1. CONGENITAL NECK MASSES By: Dr. J. K. Sesay Senior Registrar @ MOHS Specialist: General Surgeon Dip. Int. Health, M.D., M.Med . Surgery USLTHC, Connaught

2. OUTLINE Summary Thyroglossal duct cyst Branchial cleft cyst Cystic hygroma Differential Diagnosis Reference

3. SUMMARY Congenital neck masses are developmental anomalies that can manifest either at  birth  or later in life, usually following a respiratory infection. The most common congenital neck masses are  thyroglossal duct cysts ,  branchial cleft cysts , and  cystic hygromas . These  malformations  manifest as painless neck masses that, as they grow, can cause  dysphagia , respiratory distress, and neck  pain  by compressing surrounding structures. The location of the mass depends on the embryological structure the cyst arises from. Diagnosis is based on clinical findings and imaging results ( ultrasound , CT,  MRI ), which also help in surgical planning. Treatment consists of complete surgical resection to prevent recurrence and complications such as infection or  abscess  formation.

4. THYROGLOSSAL DUCT CYST (TDC) Definition : a remnant of the  thyroglossal duct , which forms during the  embryonic development  of the  thyroid gland  and normally regresses before  birth. Epidemiology Incidence : ∼ 7% of the population Second most common neck abnormality after  lymphadenopathy Pathophysiology   The  thyroid gland  originates from the  foramen cecum  at the base of the  tongue  and descends  caudally  into the neck, forming the  thyroglossal duct . If the  thyroglossal duct   fails to obliterate , midline neck cysts or ectopic  thyroid tissue  can develop anywhere along its path.

5. TDC contd. Clinical features : The cyst is present from  birth  and is usually detected during early childhood. Painless, firm, midline neck mass  that elevates with  swallowing  and  tongue  protrusion Usually located  near the  hyoid bone   May cause  dysphagia  or neck/throat  pain  if the cyst enlarges Diagnostics Neck and  thyroid examination Ultrasound  of the neck to evaluate the cyst and confirm the location of the  thyroid   Patients with  thyroglossal duct cysts  can have an ectopic  thyroid gland .  In the absence of ectopy, it is important to assess the anatomical relation of the cyst to the  thyroid  for preoperative planning. TSH  levels  If an infection is suspected,  fine needle aspiration  should be performed for  Gram stain  and culture (including AFB and  mycobacterial  culture).

6. TDC contd. Treatment   Elective surgical excision (Sistrunk procedure) to prevent infection: includes removal of the cyst, a portion of the  hyoid bone , and excision of tissue comprising the path of descent from the  foramen cecum Treatment of any active infection with  antibiotics  before surgery  Complications   Infection of the cyst with possible  abscess  formation Sinus tract formation with persistent drainage Ectopic  thyroid tissue Malignancy  arising from ectopic  thyroid tissue

7. Thyroglossal Duct Cyst There is a mass located in the patient's midline anterior cervical area near the thyroid cartilage.

8. Thyroglossal Duct Cyst Neck ultrasound (transverse section) A circular, sharply demarcated, hypoechoic lesion (green overlay) is visible dorsal to the thyroid gland.

9. Thyroglossal Duct Cyst CT head (without contrast; a: axial plane; b: sagittal plane) A hypodense mass (green overlay) with a fluid density value (mean 20 HU) is present anterior to the hyoid bone (arrows).

10. BRANCHIAL CLEFT CYST (BCC) Definition : remnants of the embryological second  branchial cleft or  cervical sinus , which normally regresses before  birth Epidemiology Accounts for ∼ 20% of pediatric neck masses ∼ 95% of all  branchial cleft   malformations  are anomalies of the second  branchial cleft . Pathophysiology : formed due to incomplete obliteration of  branchial clefts  and pouches Clinical features : usually diagnosed in late childhood or in adulthood after a previously undiagnosed cyst becomes infected  [3] History of upper respiratory infection Painless, firm mass Located  lateral  to the midline , usually  anterior to the  sternocleidomastoid muscle Does not move with  swallowing There may be a small draining opening if a fistula is present.

11. BCC contd. Diagnostics Neck examination Ultrasound CT or  MRI  to further assess anatomical structures for surgical planning Treatment : complete surgical excision of both the cyst and any associated tracts  Complications : infection of the cyst, tract, or sinus, with possible  abscess  formation

12. CYSTIC HYGROMA (CH) Definition :  a congenital lymphatic cyst (macrocystic lymphangioma) in the  posterior triangle of the neck  caused by  malformation  and obstruction of the fetal  lymphatic system   Epidemiology ∼ 1:6,000  live births Strongly associated with fetal  aneuploidy  (e.g.,  Turner syndrome ,  trisomy 21 ) and congenital  malformations  (e.g., congenital  heart  defects) Clinical features Present at  birth  as a soft, compressible,  painless,   posterior  triangle  neck mass  Can cause  dysphagia  or  airway  compromise Positive transillumination test 

13. CH contd. Diagnostics Prenatal  ultrasound   : fluid-filled neck mass with or without septations  [5] Ultrasound  to identify mass in  infancy CT or  MRI  may be used to further assess anatomical structures for surgical planning. Treatment : Small masses may regress spontaneously, but surgical excision is usually indicated to prevent infection or  airway  compromise, as well as for cosmesis.  Prognosis : Recurrence is common following surgical excision of extensive hygromas.

14. Cystic Hygroma A mass is visible anterior to the sternocleidomastoid muscle in the superior carotid triangle.

15. Cystic Hygroma Right lateral region of the neck (1) A massive, lobulated swelling (green overlay) is visible in the posterior triangle. This is the most common location of a cystic hygroma. (2) Patient after surgical excision of the hygroma (posterior triangle: blue overlay)

16. Surgical excision of cystic hygroma A cystic hygroma is visible in the middle of the incision. The internal jugular vein can be seen right above the cystic hygroma. If the cystic duct reaches up to the ipsilateral tonsil, the tonsil is also excised in surgery.

17. DIFFERENTIAL DIAGNOSIS (DDx) UNILATERAL CERVICAL LYMPHADENOPATHY (UCL): Unilateral cervical  lymphadenopathy  (UCL) refers to the localized swollen  lymph node (s) on one side of the neck and is usually associated with bacterial infections. Acute UCL is most commonly caused by  S. aureus  and  Streptococcus  species, while chronic UCL is the result of tuberculous or  nontuberculous mycobacterial infections . UCL is most often seen in children under 5 years of age and typically affects the submandibular or  deep cervical lymph nodes . FISTULA : An abnormal connection between two epithelium-lined surfaces. Examples include gastrocolic fistula, pancreaticoduodenal fistula, and arteriovenous fistula. HEMANGIOMA : A vascular tumor that is typically benign. There are several types of hemangiomas, including congenital hemangiomas, infantile hemangiomas, and hepatic hemangiomas.

18. DDx contd. CERVICAL  TERATOMA : Cervical  teratoma  manifests as a firm  lateral  neck mass that may cause respiratory distress or  dysphagia  through compression of the  trachea  and  esophagus . Cervical  teratomas  can be diagnosed on prenatal  ultrasound . A  cesarean delivery  may be required to expedite delivery and enable immediate treatment (surgical excision). DERMOID CYSTS : A mature cystic teratoma that can occur on the head, the neck, spine, cranium, and in the abdomen (most common ovarian tumor in females). Cysts typically become symptomatic as they grow in size. They are treated with complete surgical excision.

19. DDx contd. MIDLINE CERVICAL CLEFT CYST : Congenital midline cervical cleft (CMCC) is a very uncommon congenital anomaly of the anterior neck that has very characteristic features: (1) a nipple-like protuberance (skin tag), (2) a skin defect in the middle with palpable subcutaneous fibrous cord, and (3) a blind sinus at the end.

20. MIDLINE CERVICAL CLEFT CYST

21. REFERENCES Foley DS, Fallat ME. Thyroglossal duct and other congenital midline cervical anomalies. Semin Pediatric Surg .2006; 15(2): p.70–75. doi:  10.1053/j.sempedsurg.2006.02.003 . | Open in Read by QxMD 2.Jarrell BE. NMS Surgery. Lippincott Williams & Wilkins; 2015 3.Acierno SP, Waldhausen JH. Congenital Cervical Cysts, Sinuses and Fistulae. Otolaryngology Clin N Am .2007; 40(1): p.161–176. doi:  10.1016/j.otc.2006.10.009 . | Open in Read by QxMD 4.Sannoh S, Quezada E, Merer DM, Moscatello A, Golombek SG. Cystic hygroma and potential airway obstruction in a newborn: a case report and review of the literature. Cases Journal .2009; 2(1). doi:  10.1186/1757-1626-2-48 . | Open in Read by QxMD 5.Chen Y-N, Chen C-P, Lin C-J, Chen S-W. Prenatal Ultrasound Evaluation and Outcome of Pregnancy with Fetal Cystic Hygromas and Lymphangiomas. J Med Ultrasound .2017; 25(1): p.12-15. doi:  10.1016/j.jmu.2017.02.001 . | Open in Read by QxMD