Doctor123.org

Branchial Cleft Fistula

Doctor123.org

Branchial Cleft Fistula

EPIDEMIOLOGY

para>Most occur in the pediatric age group á

Positive family history á

Microtia and aural atresia occur with failure of development of the 1st branchial cleft. á

Culture if signs of infection á

11 Nicoucar áK, Giger áR, Jaecklin áT, et al. Management of congenital third branchial arch anomalies: a systematic review. Otolaryngol Head Neck Surg. 2010;142(1):21.e2-28.e2.22 Liberman áM, Kay áS, Emil áS, et al. Ten years of experience with third and fourth branchial remnants. J Pediatr Surg. 2002;37(5):685-690.33 Waldhausen áJH. Branchial cleft and arch anomalies in children. Semin Pediatr Surg. 2006;15(2):64-69.44 Roback áSA, Telander áRL. Thyroglossal duct cysts and branchial cleft anomalies. Semin Pediatr Surg. 1994;3(3):142-146.

  • Predominant age: By definition, all branchial cleft fistulae are present at birth; however, they may remain unnoticed for some time.
  • Branchial cleft cysts may not present until adulthood and are commonly diagnosed in the 3rd and 5th decades of life.
  • Predominant sex: male = female

ETIOLOGY AND PATHOPHYSIOLOGY

  • Branchial anomalies result from the incomplete obliteration of pharyngeal clefts and pouches during embryogenesis.
  • Both respiratory and squamous epithelium (alone or together) may line branchial anomalies.
  • Squamous epithelium is found more commonly in cysts.
  • Ciliated, columnar epithelium is found more commonly in sinuses and fistulae.
  • The 1st branchial cleft contributes to the external auditory canal, middle ear cavity, mastoid air cells, and eustachian tube. Related fistulae are very rare and tend to be infra- or retroauricular. Preauricular cysts and sinuses are not thought to be of branchial cleft origin:1st branchial cleft anomalies enter the external auditory canal and/or occasionally the middle ear.They represent 1-4% of all branchial cleft malformations.Type I anomalies contain ectodermal elements only and course lateral to the facial nerve.Type II anomalies contain ectoderm and mesoderm, coursing medial to the facial nerve.
  • The 2nd branchial cleft forms the hyoid bone and tonsillar fossa. Related fistulas (most common variant) course between the internal and external carotid arteries:2nd branchial anomalies represent 90-95% of all branchial cleft lesions.They course close to the glossopharyngeal and hypoglossal nerves, entering the pharynx at the level of supratonsillar fossa.The external opening runs along anterior border of sternocleidomastoid muscle.2nd branchial cleft anomalies are subdivided into four subtypes:Type I lesions are anterior to the SCM and do not involve the carotid sheath.Type II lesions are the most common second arch anomalies, deep to the SCM, and anterior or posterior to the carotid artery.Type III lesions pass between the internal and external carotid arteries and are adjacent to the pharynx.Type IV lesions are medial to the sheath, adjacent to the tonsillar fossa.
  • The 3rd and 4th branchial clefts form the parathyroid glands, thymus, and portions of thyroid gland (parafollicular cells):3rd branchial cleft anomalies represent 5% of all branchial anomalies.Sinus tracts (also called pyriform sinuses) originate in the pyriform sinus and course adjacent to the thyroid cartilage.Fistulas are rare, usually resulting from recurrent infections and/or repeated surgery.Both 3rd and 4th fistulas should have external ostia on the lower anterior neck. Left-sided lesions are more common than right-sided ones.They are often called pyriform sinus "fistulae,"Ł despite the frequent lack of an external opening to the skin.Those from the 3rd branchial cleft course posterior to carotid artery.Differentiated from 2nd branchial cleft anomalies by the location of their internal opening (external openings should be the same)Presence of thymic tissue does not differentiate between 3rd and 4th branchial cleft anomalies, as accessory thymic tissue has been described in the latter (1).
  • 1st branchial cleft anomalies enter the external auditory canal and/or occasionally the middle ear.
  • They represent 1-4% of all branchial cleft malformations.
  • Type I anomalies contain ectodermal elements only and course lateral to the facial nerve.
  • Type II anomalies contain ectoderm and mesoderm, coursing medial to the facial nerve.
  • 2nd branchial anomalies represent 90-95% of all branchial cleft lesions.
  • They course close to the glossopharyngeal and hypoglossal nerves, entering the pharynx at the level of supratonsillar fossa.
  • The external opening runs along anterior border of sternocleidomastoid muscle.
  • 2nd branchial cleft anomalies are subdivided into four subtypes:Type I lesions are anterior to the SCM and do not involve the carotid sheath.Type II lesions are the most common second arch anomalies, deep to the SCM, and anterior or posterior to the carotid artery.Type III lesions pass between the internal and external carotid arteries and are adjacent to the pharynx.Type IV lesions are medial to the sheath, adjacent to the tonsillar fossa.
  • Type I lesions are anterior to the SCM and do not involve the carotid sheath.
  • Type II lesions are the most common second arch anomalies, deep to the SCM, and anterior or posterior to the carotid artery.
  • Type III lesions pass between the internal and external carotid arteries and are adjacent to the pharynx.
  • Type IV lesions are medial to the sheath, adjacent to the tonsillar fossa.
  • 3rd branchial cleft anomalies represent 5% of all branchial anomalies.
  • Sinus tracts (also called pyriform sinuses) originate in the pyriform sinus and course adjacent to the thyroid cartilage.
  • Fistulas are rare, usually resulting from recurrent infections and/or repeated surgery.
  • Both 3rd and 4th fistulas should have external ostia on the lower anterior neck. Left-sided lesions are more common than right-sided ones.
  • They are often called pyriform sinus "fistulae,"Ł despite the frequent lack of an external opening to the skin.
  • Those from the 3rd branchial cleft course posterior to carotid artery.
  • Differentiated from 2nd branchial cleft anomalies by the location of their internal opening (external openings should be the same)
  • Presence of thymic tissue does not differentiate between 3rd and 4th branchial cleft anomalies, as accessory thymic tissue has been described in the latter (1).

RISK FACTORS

COMMONLY ASSOCIATED CONDITIONS

DIAGNOSIS

HISTORY

  • History of drainage from the cervical area
  • Neck abscess or suppurative thyroiditis

PHYSICAL EXAM

  • Presence of a tiny external opening usually on middle to lower neck along anterior border of sternocleidomastoid muscle
  • Spontaneous mucoid drainage
  • External openings may be marked by a skin tag or cartilage.
  • Infection may be the presenting sign: Erythema, swelling, pain, or fever may also be present.
  • Thyroid abscesses in children often represent an underlying branchial remnant, especially with (+) mixed-flora cultures (2).
  • 10% are bilateral.
  • Small orifices located in the mid-neck, most commonly along the anterior border of the sternocleidomastoid muscle (less commonly in the lower neck or postauricular)
  • 3rd and 4th branchial cleft anomalies are predominantly left-sided (89%) (1).

DIFFERENTIAL DIAGNOSIS

  • External sinuses
  • Cystic hygroma
  • Dermoid cysts
  • Lymphadenopathy

DIAGNOSTIC TESTS & INTERPRETATION

  • CT of neck with IV contrast is occasionally beneficial in 3rd and 4th branchial cleft fistulas/sinus (2)[C].
  • CT may demonstrate the fistula tract in up to 64% of cases (3).
  • MRI and US may also be useful to define the tract.
  • A barium esophagogram may demonstrate the fistula tract and location; sensitivity rate of 50-80% when used to evaluate 3rd and 4th branchial anomalies (3)[C].
  • An incisional biopsy should not be performed in children because this may make subsequent resection more difficult.
  • Contrast diagnostic modalities are more accurate in the noninfected state due to reduced edema and improved contrast penetration into the fistula tract.
  • Sinogram or fistulogram may be done but are usually of little value.
  • Upper airway endoscopy may be useful to visualize the opening into the pharynx.
  • Cysts are more commonly lined by stratified squamous epithelium and may contain hair follicles, sweat glands, sebaceous glands, or cartilage.
  • Sinuses and fistulae are more commonly lined by ciliated, columnar epithelium.

TREATMENT

  • Complete surgical excision is required to prevent recurrence (4).
  • Outpatient status is usually appropriate.
  • Acute infections should be treated with antibiotics first; if this fails to resolve, incision and drainage may be required (3). Typical offending organisms include Streptococcus and Klebsiella, although infections may be polymicrobial.
  • If possible, an elective resection should be scheduled weeks after the acute infection.

SURGERY/OTHER PROCEDURES

  • Stepladder incisions may be needed to visualize the upper portion of the tract.
  • 1st branchial cleft lesions may require larger incisions.
  • Methylene blue injection into the fistula may be useful, although spillage complicates the dissection.
  • Cannulation of the tract or fistula with a 2-0 or 3-0 monofilament suture may aid in establishing the course of the tract.
  • Drains are not routinely used.
  • For 3rd and 4th arch anomalies, fiberoptic endoscopy may assist in locating the opening into the pyriform sinus.
  • 4th arch fistulas require an ipsilateral hemithyroidectomy to remove the tract; partial resection of the thyroid cartilage may be required to gain exposure to the tract as it enters the pyriform sinus (3).
  • Antibiotics are used only for infection.
  • Patients with abscesses related to 3rd branchial cleft anomalies frequently require initial incision and drainage (1)[B]:Incision and drainage failure rates is ~94% on the first attempt.Virtually all will require resection.Those with acute suppurative thyroiditis may require partial thyroidectomy and removal of the fistula tract. Partial thyroidectomy is rarely curative (1)[B].
  • Endoscopic cauterization of the internal orifice has been successfully used in some cases, especially in children <8 years (1)[B].
  • Incision and drainage failure rates is ~94% on the first attempt.
  • Virtually all will require resection.
  • Those with acute suppurative thyroiditis may require partial thyroidectomy and removal of the fistula tract. Partial thyroidectomy is rarely curative (1)[B].

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • If infected, follow at weekly intervals until resolution of abscess and inflammatory reaction; then excise.
  • Postoperative visit at 2 to 3 weeks
  • Lack of epithelium in the fistula tract pathology specimen may indicate incomplete resection; have a high index of suspicion for future recurrence.

PROGNOSIS

COMPLICATIONS

  • Facial nerve injury (1st arch anomalies)
  • Hypoglossal nerve injury
  • Spinal accessory nerve injury
  • Vagus nerve injury
  • Infection
  • Carotid artery injury
  • Recurrence (full excision of tract epithelium required)-common with 1st arch anomalies due to incomplete excision.
  • Neoplastic degeneration of branchial remnants (~250 reported cases) if not resected

REFERENCES

CODES

ICD10

ICD9

SNOMED

CLINICAL PEARLS

  • Most common: 2nd branchial cleft anomalies: an opening in the mid-neck at the anterior border of the sternocleidomastoid muscle with a history of an occasional discharge is diagnostic.
  • Radiographic confirmation is not needed; complete surgical excision as an outpatient is both diagnostic and therapeutic.
  • Branchial cleft remnants, sinuses, and cysts result from the failure of branchial arches to close and/or complete their normal development.