Anesthesiology

Congenital Cysts of Neck (Thyroglossal Duct Cysts including Lingual, Brachial Cleft Cysts

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What the Anesthesiologist Should Know before the Operative Procedure

Congenital cystic lesions of the neck include midline and lateral defects, in descending order of frequency, thyroglossal duct cyst, branchial cleft anomalies, dermoid cysts, hemangiomas, and lymphangiomas.

Malformations of the midline

Thyroglossal duct cysts (TDCs) are the most common congenital cyst encountered in the neck, representing 54.6% of all congenital cervical cysts and are three times as common as branchial cleft abnormalities. Etiology is persistent epithelial remnants of the thyroglossal duct present during descent of the thyroid gland from the foramen cecum to its final position in the anterior neck. Cystic change is initiated when lymphoid tissue of the neck close to these structures reacts to repeated upper respiratory infections. TDCs can be found anywhere along the thyroglossal duct, but the majority occur in the midline of the neck close to the hyoid bone. In a large reported meta-analysis, 60.9% of TDCs were located thyrohyoid, 24.1% were suprahyoid, 12.9% were suprasternal 12.9%, and 2.1% were lingual. Malignancy is rarely encountered in TDCs (1%), usually papillary or squamous carcinoma.

Lingual thyroglossal duct cysts (LTDCs) are rare, seated posterior and associated with dysphagia, inspiratory stridor, dyspnea, respiratory distress and failure to thrive in newborns and infants. Involvement of TDC of the larynx has been described. There is an equal distribution of TDC among males and females. Presentation is usually in the first 6 months of life. The most common signs and symptoms of LTDC relate to upper airway obstruction and include intermittent or continuous stridor, accessory muscle retraction and dyspnea. These may relate to the feedings or precipitated by placing the infant in the supine position.

Differential diagnosis: dermoid cyst, lymphadenopathy, cystic hygroma.

Congenital inclusion dermoid cysts account for up to 25% of midline cervical anomalies. They are derived along the defective closure of the midline (lines of embryologic fusion) as a result of inclusion of sequestered epithelial remnants or from ectodermal displacement by in growing membranous bone. Etiology is uncertain. Lingual dermoid cysts are very rare and only eight have been reported in the literature. Postulated theories include entrapment of epithelial rests during midline fusion of lateral processes of embryonic tongue during third and fourth intrauterine weeks, failure of disappearance of embryonic cleft, trauma or displacement of normal epithelial bands. Etiology is result of entrapment of epithelial elements during development. They present as painless, superficial subcutaneous masses in the anterior neck. Because they have no connection to the oropharynx, infection is rare. Complete excision is curative for nonruptured dermoid cysts.

Malformations of the lateral cervical region

Branchial cysts (BCs) are cystic swellings of embryonic origin and comprise approximately 30% of congenital neck masses. Etiology is persistence of remnants of branchial clefts during fetal development. There is no sex predominance, and presentation as soft nontender fluctuant lateral neck masses is usually evident in childhood or early adulthood. BCs are more laterally situated along the anterior border of the sternocleidomastoid muscle and are more likely to be infected than TDCs.The majority of branchial arch anomalies (85%) derive from the second arch, with the most common site of occurrence at the junction of the upper one-third and lower two-thirds of the anterior border of the sternocleidomastoid muscle. First branchial arch derivatives are much less common and usually appear on the face on related to the auricle. Third branchial defects are rare and fourth arch defects course inferior to superior laryngeal nerve. Rupture of these cysts can occur spontaneously, following acute infection or ill advised attempts at drainage. Complete excision is the only treatment

1. What is the urgency of the surgery?

What is the risk of delay in order to obtain additional preoperative information?

Incision and drainage of infected TDC may be necessary, but should be avoided when possible to decrease recurrence.

Treatment of choice for all patients with uncomplicated TDC and all branchial anomalies to prevent infection of the cyst. Abscesses forming in these lesions should be treated first by incision and drainage. Early excision for branchial cysts should be performed to prevent incidence of secondary infection and predisposition to chronic infection.

2. Preoperative evaluation

Careful airway evaluation, especially if lingual thyroid suspected. TDC typically presents as a mobile, painless mass in the anterior midline of the neck, usually in close proximity to the hyoid bone. Less often TDC present with signs and symptoms of secondary infection or fistula.

Patients present with an infected TDC may be managed initially with attempts at alleviation of the infection, whereby antibiotics directed toward the most common causative organism are initiated.

Despite historical association of preoperative or concurrent infection with increased recurrence rates and more difficult complete surgical excision, a recent review of 100 patients from a major pediatric hospital found no such association between preoperative infection and increased recurrence rates. However, infection in the immediate postoperative period was associated with a statistically increased rate of recurrence.

3. What are the implications of co-existing disease on perioperative care?

Perioperative evaluation

TDCs usually present as a painless cystic mass in the region of the hyoid bone at or near the midline. Most TDC present during the first five years of life. TDC are prone to infection. Up to one-third of patients present with a concurrent or previous history of infection in the cyst, and the risk of infection are the primary indications for surgical intervention. The most common responsible pathogens include Haemophilus influenzae, Staphylococcus aureus, and Staphylococcus epidermidis.

History and physical with particular attention to rule out inspiratory stridor, chest retractions, feeding difficulties that might portend upper airway obstruction secondary to possible lingual thyroglossal duct cyst. Questions directed to improvement /worsening of respiratory symptoms when the infant is placed in the prone versus supine positions. Dysphagia, dysarthria, and choking sensation in older infants and young children accentuated during upper respiratory infection.

Familial TDCs have been reported and may be autosomal recessive, dominant, or multifactorial.

b. Cardiovascular system:

N/A

c. Pulmonary:

N/A

d. Renal-GI:

N/A

e. Neurologic:

N/A

f. Endocrine:

N/A

g. Additional systems/conditions which may be of concern in a patient undergoing this procedure and are relevant for the anesthetic plan (e.g., musculoskeletal in orthopedic procedures, hematologic in a cancer patient)

4. What are the patient's medications and how should they be managed in the perioperative period?

N/A

h. Are there medications commonly seen in patients undergoing this procedure and for which should there be greater concern?

N/A

i. What should be recommended with regard to continuation of medications taken chronically?

N/A

j. How to modify care for patients with known allergies

N/A

k. Latex allergy - If the patient has a sensitivity to latex (e.g., rash from gloves, underwear, etc.) versus anaphylactic reaction, prepare the operating room with latex-free products.

N/A

l. Does the patient have any antibiotic allergies - Common antibiotic allergies and alternative antibiotics

If a patient is allergic to beta-lactams, fluoroquinolones or clindamycin is used in selected situations.

m. Does the patient have a history of allergy to anesthesia?

N/A

5. What laboratory tests should be obtained and has everything been reviewed?

Ultrasound of neck for TDCs and branchial arch anomalies; or DCs to evaluate depth of lesion. Lateral neck radiograph delineates tongue, epiglottis, glottis, and subglottic areas, and edema impinging on the airway.

Branchial cysts

CT Neck Imaging modality of choice for branchial cleft anomalies which appear as thin walled cystic lesions. MRI Neck May better demonstrate deep tissue extent of cyst.

Barium esophagram can be helpful for third and fourth branchial fistulae.

Dermoid cysts

MRI neck hypointense to muscle on T1-weighted images and hyperintense on T2-weighted images.

Other tests

  • Thyroid function tests including screening TSH level.

  • Iodine-131 thyroid scintis can of oropharynx and neck to rule out a median ectopic thyroid and identify the extent of functional thyroid tissue in the neck for patients in whom hypothyroidism is suggested by history, physical or elevated TSH levels and those in whom the mass is predominantly solid in nature or if US cannot identify the thyroid gland in its normal location. It may reveal ectopic thyroid tissue in a lingual mass and whether functional thyroid gland exists in normal position.

  • Flexible nasolaryngoscopy for infants for TDC with existing respiratory distress.

  • Upper airway endoscopy to locate the pharyngeal opening in BCs.

Intraoperative Management: What are the options for anesthetic management and how to determine the best technique?

General anesthesia is administered. The standard surgical approach to TDC is Sistrunk’s operation, involving removal of the mid-portion of the hyoid bone in continuity with the TDC and excision of a core of tissue between hyoid bone and foramen cecum. After intubation, patients are placed in a supine position with the neck extended.

General anesthesia

Acute respiratory symptoms caused by LTDC may be relieved by careful endotracheal intubation and by aspiration of the cyst. Definitive treatment of LTDC is excision along with removal of lingual thyroid tissue. Tracheostomy is often required prophylactically at the time of excision in anticipation of postoperative edema which can compromise the airway.

6. What is the author's preferred method of anesthesia technique and why?

Oral premedication midazolam 0.3 mg/kg; maximum 16 mg. Induction: Inhalation with oxygen, nitrous oxide, sevoflurane, followed by placement of an intravenous catheter and intubation with an oral endotracheal tube or nasal RAE tube. Maintenance: balanced technique with titrated fentanyl. Local anesthetic infiltration by surgeon at the end of the procedure.

What prophylactic antibiotics should be administered?

Prophylactic antibiotic received within one hour prior to surgical incision. First or second generation cephalosporins satisfy the criteria for most operations. If a patient is allergic to beta-lactams, fluoroquinolones and clindamycin in selected situations. Prophylactic antibiotics discontinued within 24 hours after surgery end time. (Downloaded from SCIP (August 13, 2011). "CMS Core Measure SCIP Surgical Care Improvement Project," at http://www.sjhlex.org/documents/Physicians/SCIP_Poster_Full_Size.pdf.

What do I need to know about the surgical technique to optimize my anesthetic care?

Neck will be extended for transverse midline incision for TDC or DC.

What can I do intraoperatively to assist the surgeon and optimize patient care?

Positioning with head up may reduce bleeding. Good communication with surgical team concerning possible entry into vascular or airway structures essential. May be asked to depress base of tongue to move cyst to facilitate surgical identification of proximal lingual attachment. Careful attention to sterile technique is important to facilitate a successful outcome.

a. Neurologic:

N/A

b. If the patient is intubated, are there any special criteria for extubation?

N/A

c. Postoperative management

What analgesic modalities can I implement?

Acetaminophen, local anesthetic infiltration by surgeon, short acting opioids.

What level bed acuity is appropriate?

PACU and discharge home for majority; LTDC PICU because of airway involvement.

What are common postoperative complications, and ways to prevent and treat them?

Incidence of complications has been reported at less than 10% and mostly recurrence and infection. Major complications of TDC surgery are rare and include recurrence, abscess or hematoma potentially compressing vascular and/or airway structures in neck requiring surgical drainage, entry in the airway, need for tracheostomy, nerve paralysis, hypothyroidism and death. Minor complications such as seroma, local wound infection and stitch abscesses were reported. Young age and cyst infection may be related to an increase in recurrence rate. Air emphysema in the neck has been reported after excision of a thyroglossal duct cyst caused by a pharyngeal breach from energetic coughing postoperatively.

Postoperative infection is associated with increased recurrence TDC.

What's the Evidence?

Acierno, SP, Waldhausen, JHT. "Congenital cervical cysts, sinuses and fistulae". Otolaryngol Clin N Am. vol. 40. 2007. pp. 161-76.

(Discuss embryology, clinical presentation, diagnosis, and treatment for congenital cystic lesions of neck.)

Agaton-Bonilla, FC, Gay-Escoda, C. "Diagnosis and treatment of branchial cleft cysts and fistulae: a retrospective study of 183 patients". Int J Oral Maxillofac Surg. vol. 25. 1996. pp. 449-52.

(Author discusses surgical treatment and complications of branchial cleft cysts.)

Al-Khateeb, TH, Al Zoubi, F. "Congenital neck masses: a descriptive retrospective study of 252 cases". J Oral Maxillofac Surg. vol. 65. 2007. pp. 2242-7.

(Authors describe embryology, epidemiology, imaging, and surgical procedures, including indications for elective surgical excision.)

Campbell, R, Walker, P. "Management of congenital lingual dermoid cysts". Int J Pediatr Otorhinolaryngol. vol. 74. 2010. pp. 567-71.

(Discusses anesthetic management for laser excision of lingual dermoid.)

Dabholkar, JP. "Congenital cystic lesions in head and neck". Ind J Otolaryngol Head Neck Surg. vol. 55. 2003. pp. 128-30.

(Includes embryology and clinical presentation of branchial and dermoid cysts of neck.)

Dearlove, OR, Bowen, J. "Air emphysema in the neck after excision of a thyroglossal cyst". Anaesthesia. vol. 59. 2004. pp. 193-4.

(Authors discuss anesthetic management, and complications including air emphysema in neck.)

Erikci, V, Hosgor, M. "Management of congenital neck lesions in children". J Plastic, Reconstructive & Aesthetic Surg. vol. 67. 2014. pp. e217-222.

(Describes clinical features, outcomes of treatment, and complications in children with congenital neck lesions.)

Foley, DS, Fallat, ME. "Thyroglossal duct and other congenital midline cervical anomalies". Semin Pediar Surg. vol. 15. 2006. pp. 70-5.

(Describe embryology, clinical presentation, diagnosis, surgical procedures, and complications.)

Kepertis, C, Anastasiadis, K, Lambropouls, V, Mouravas, V, Spyridakis, I. "Diagnostic and surgical approach of thyroglossal duct cyst in children: ten years data review". J Clin Diagn Res. vol. 9. 2015.

(Describes the clinical features, treatment, complications and outcomes in children with thyroglossal duct cyst.)

Kolomvos, N, Theologie-Lygidakis, N, Tzerbos, F, Pavli, M, Leventis, M, Iatrou, I. "Surgical treatment of oral and facial soft tissue cystic lesions in children. A retrospective analysis of 60 consecutive cases with literature review". J Cranio-Maxillo-Facial Surg.. vol. 42. 2014. pp. 392-396.

(Authors describe the characteristics and treatment of soft tissue cysts of the oral and maxillofacial region in children.)

McAvoy, JM, Zuckerbraun, L. "Dermoid cysts of the head and neck in children". Arch Otolaryngol. vol. 102. 1976. pp. 529-31.

(Summarizes various types and locations of dermoid cysts of the head and neck in children.)

Mondin, V. "Thyroglossal duct cyst: personal experience and literature review". Auris Nasus Larynx. vol. 35. 2008. pp. 35-11.

(Authors include embryology, epidemiology, etiology, clinical presentation, preoperative evaluation and imaging, differential diagnosis, surgical procedures, and complications.)

Paez, P. "Stridor as the presenting symptom of lingual thyroglossal duct cyst in an infant". Clin Pediatr. vol. 13. 1974. pp. 1077-8.

(Authors address lingual thyroglossal duct cyst clinical presentation, imaging, and surgical treatments.)

Samuel, M. "Lingual thyroglossal duct cyst presenting in infancy". J Pediatr Surg. vol. 28. 1993. pp. 891-3.

(Authors include embryology, clinical presentation, diagnosis, and surgical management.)

Schwartz, AJ, Litman, RS. "Thyroglossal duct cyst". Pocket Clinician Pediatric Anesthesia Practice. Cambridge University Press. 2006. pp. 186-7.

(Author discusses pre-, intra-, and postanesthetic management and complications.)

CMS Core Measure SCIP Surgical Care Improvement Project. August 13, 2011. http://www.sjhlex.org/documents/Physicians/SCIP_Poster_Full_Size.pdf.

(Provides latest SCIP guidelines)

Sistrunk, WE. "The surgical treatment of cysts of the thyroglossal tract". Ann Surg. vol. 21. 1920. pp. 121-3.

(Describes Sistrunk procedure.)

Weldon, BC, Krafcik, JM. "Breath-holding-like spells in an infant: an unusual presentation of lingual thyroglossal duct cyst". J Pediatr Surg. vol. 35. 2000. pp. 1381-4.

(Authors discuss clinical presentation of lingual thyroglossal duct cyst and airway management.)
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