Most Frequently Missed Diagnosis: Retropharyngeal Suppurative Lymphadenitis

J Shah, MD; L Lanier, MD; C L Sistrom, MD; D Rajderkar, MD; A Ghaffari, MD; A Mancuso, MD;I M Schmalfuss, MD

University of Florida, Department of Radiology Contact: [email protected]

The authors have no financial disclosure in regard to this educational exhibit. Background:

 Computer aided online simulation (SIM) of emergency imaging studies was developed

 Designed to test residents for readiness for call

 Providing proficient & objective assessment of resident competence in the emergency/critical care imaging & affirmation of milestone achievements Background:

 8 hour simulation of 65 emergent & critical care cases of varying degrees of difficulty, including normal studies

 Presentation via full DICOM image sets

 Dictation of free responses into text boxes labelled:

o Critical findings

o Incidental findings

o Acuity ranking Results:

 SIM was taken by 103 first (R1) & second (R2) year residents from 9 USA radiology training programs in 2014

 Suppurative retropharyngeal space (RPS) lymphadenitis was presented

o 100% of residents failed to make the correct diagnosis o Incorrect answers included: • RPS abscess (48%) • Peritonsillar abscess (24%) • Tonsillar abscess (15%) • Abscess without localization (1%) Results:

 SIM was taken by 103 first (R1) & second (R2) year residents from 9 USA radiology training programs in 2014

 Suppurative retropharyngeal space (RPS) lymphadenitis was presented

o 100% of residents failed to make the correct diagnosis

Conclusion: Significant cognitive gap exists in differentiating suppurative RPS lymphadenitis from abscess placing patients at risk for unnecessary surgical procedure Teaching Points:

 Familiarize the radiologist with clinical and imaging findings of RPS suppurative lymphadenitis

 Discuss clinical and imaging features of mimics of RPS suppurative lymphadenitis to improve diagnostic competency Outline:

 SIM case presentation

 Discussion of normal RPS anatomy

 Presentation of clinical & imaging findings of RPS suppurative lymphadenitis and potential complications

 Review of mimics of RPS suppurative lymphadenitis SIM Case: 9 month old male with fever & decreased movement

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Axial contrast enhanced CT (CECT) shows mild narrowing of the upper airway (*) caused by a focal, rim enhancing hypodensity in the right RPS with marked surrounding edema. The lateral nature of the disease is not consistent with a RPS abscess but rather with suppurated RPS lymphadenitis. RPS Anatomy

 Boundaries: o Anterior: Middle layer of deep cervical o Posterior: Deep layer of deep o Lateral: Alar fascia (arises from ) o Superior: Skull base o Inferior: Fusion of middle & deep cervical fascia from T2 to T6

 Contents: o Loose connective tissue o Lateral and medial RPS lymph nodes RPS Imaging Anatomy: Axial plane - CT

Axial CECT shows thin layer of fat in the RPS between the pharyngeal constrictor & longus colli muscles. RPS lymph nodes are divided into lateral & medial groups (2) with the lateral being located medial to the internal carotid (ICA) while the medial ones are in midline. RPS Imaging Anatomy: Axial plane - MRI

Axial T1+Gd image reveals a normal lateral RPS lymph node located immediately medial to the ICA and lateral to the longus colli muscle. Axial T2 image in an infant shows a normal medial RPS lymph node located in the midline between the longus colli muscles and a normal left lateral RPS lymph node medial to the ICA. RPS Imaging Anatomy: Sagittal plane

Danger space is a potential space while RPS is an actual space. In a normal patient, the two cannot be distinguished with a small fat plane seen in the RPS on T1 image. RPS Imaging Anatomy: Sagittal plane

Sagittal CT in bone algorithm in a different patient shows air tracking inferiorly into the posterior to the esophagus consistent with the danger space as the RPS stops at the thoracic inlet level. RPS Suppurative Lymphadenitis

 Demographics: o Most common in children between ages of 2 to 6 years o Teens and young adults occasionally affected o No gender predilection

 Presentation: o Young children - high fevers, feeding problems and airway compromise o Older children - fevers, sore throat, odynophagia, and neck pain

 Pathophysiology: o Staphylococcus or Streptococcus head & neck infection -> RPS reactive lymphadenopathy -> RPS suppurative lymphadenitis -> RPS abscess RPS Diagnostic Confusion

 In RPS space: o RPS suppurative lymphadenitis o RPS abscess o RPS edema o Necrotic RPS nodal metastasis

 In adjacent anatomical structures: o Tonsillar abscess o Peritonsillar abscess o Longus colli calcific tendinitis o Soft tissue tumors / cysts Increasing Diagnostic Accuracy

RPS Suppurative Characteristic RPS Abscess RPS Edema Lymphadenitis Fills the RPS from Fills the RPS from Fluid Distribution Unilateral side to side side to side

“Bow-Tie” configuration on axial images, Rounded or ovoid, Rounded or ovoid; Configuration sagittal images mass effect mass effect and mass effect demonstrates depends on size depends on size tapered margins. Mild to no mass effect

Enhancing Wall Sometimes Always No • Necrotic metastatic RPS lymph nodes mimic RPS suppurative lymphadenitis but affect elderly patients. RPS Suppurative Lymphadenitis

Axial CECT images in a 8 year old boy with dysphagia, fever & neck pain reveals a hypodense, rim enhancing lesion in the right RPS as the lesion is medial to ICA & posterior to the pharyngeal constrictor muscle. Notice the reactive lymphadenopathy in level II with subtle edema in the RPS. The unilateral nature of the lesion is consistent with RPS suppurative lymphadenitis with clear fluid & negative cultures on aspiration, confirming the diagnosis and ruling out RPS abscess. RPS Abscess

CECT reveals an irregular, rim enhancing hypodensity in the RPS. The small air pockets & bilateral nature of the disease indicates RPS abscess rather than suppurated RPS lymph node. This requires search for underlying etiology, e.g. discitis/osteomyelitis as seen on sagittal T1+Gd image in this patient. RPS Abscess

CECT reveals a rim enhancing hypodensity in the RPS bilaterally consistent with RPS abscess which was related to recent anterior fusion surgery. The diagnosis of RPS abscess requires CT imaging to the carina to exclude thoracic involvement as seen in this patient. Notice also the small abscess in the sternocleidomastoid muscle. RPS Edema

CECT of a 60 year old male following anterior fusion surgery reveals hypodensity in the RPS bilaterally. The lack of enhancement is consistent with RPS edema rather than RPS abscess as seen in the patient on the prior slide. The RPS edema spontaneously resolved with the lateral cervical spine plain film performed 2 months later showing normal thickness of the prevertebral stripe. RPS Edema

Non-contrasted CT in a 65 year old shows hypodensity in the RPS bilaterally. The lack of mass effect, the tapered margins and additional subcutaneous stranding are consistent with RPS edema rather than RPS abscess. Search for venous thrombosis / obstruction is required in every patient with unexplained RPS edema. Superior vena cava syndrome due to mediastinal adenopathy was confirmed in this patient. RPS Edema

Bilateral RPS edema in two different patients related to venous thrombophlebitis with involvement of the internal jugular vein only in the patient on the left and extension into smaller neck veins in the patient on the right. Notice that the degree of the edema does not indicate the extent of thrombophlebitis. Necrotic RPS Nodal Metastasis

CECT in a 58 year old male reveals a focal rim enhancing hypodensity in the right RPS. The age of the patient & the associated large abnormal group IIB nodal conglomerate without adjacent inflammatory changes are consistent with metastatic disease rather than suppurative RPS adenitis. Fullness & fat plane obscuration was seen in the right nasopharynx and confirmed as cancer on subsequent biopsy. Necrotic RPS Nodal Metastasis

CECT in a 49 year old male reveals a right, oval hypodensity with rim enhancement & inflammatory thickening of the adjacent soft tissues mimicking suppurative RPS adenitis. However, in an adult patient this is a necrotic RPS metastasis until proven otherwise and requires search for an underlying malignancy in particular of the oropharynx & thyroid gland as in this patient. RPS Pathology - Management

 Varies based on pathology

RPS Suppurative Management RPS Abscess RPS Edema Lymphadenitis Intravenous Non-surgical & antibiotics; most Treatment Surgical drainage depending on patients improve in cause of edema 24 to 48 hours

Airway protection; Airway protection; Airway protection; Chest CT to exclude Incision & drainage if Search for Additional extension into progression to RPS underlying cause Recommen- mediastinum; Search abscess or if the e.g. venous dations for underlying cause lymph node exceeds thrombosis / e.g. discitis / 3 cm in short thrombophlebitis osteomyelitis RPS Pathology – Reporting Guidelines

 Is the process centered in the RPS lymph node or truly in the RPS?  If in RPS lymph node, is it inflammatory indicating suppurative adenitis or neoplastic?  If truly in the RPS, is it infectious indicating RPS abscess or just edema?  Associated findings / complications, such as: o Airway compromise? o Venous obstruction or thrombophlebitis? o Osteomyelitis/discitis? o Epidural abscess with spinal canal or cord compromise? o Oropharyngeal or thyroid gland malignancy?

 Extent of disease & relationship to carotid artery / jugular vein  Are additional studies or intervention such as aspiration/biopsy needed to establish the correct diagnosis? Additional RPS Lymphadenitis Mimics:

 Tonsillar abscess

 Peritonsillar abscess

 Longus colli calcific tendinitis

 Soft tissue tumors / cysts Tonsillar versus Peritonsillar Abscess

CECT in two different patients reveals focal fluid collections in the right tonsillar region. The differentiating feature between these two entities is the location of the tonsils. In the patient with tonsillar abscess, the fluid collection is centered in the tonsil while the tonsil is normal but medially displaced in peritonsillar abscess. Both collections are markedly anterior to the RPS. Longus Colli Calcific Tendinitis

 Clinical presentation mimics RPS infection: o Fevers, dysphagia, odynophagia & neck pain o May have elevated inflammatory markers including ESR & WBC

 Imaging findings on CT/MRI include: o Edema within the longus colli musculature & RPS o Calcification of the longus colli tendons = differentiating feature; may be subtle

 Treatment with NSAIDS Longus Colli Calcific Tendinitis

54 year old male with dysphagia & fevers is referred to the hospital for suspected discitis/osteomyelitis. CT scout image reveals marked prevertebral soft tissue thickening that is caused by widened and hypodense RPS without associated enhancement on the CECT consistent with RPS edema. Notice the small calcifications at C2 leading to the correct diagnosis of calcific longus colli tendinitis. Soft Tissue Tumors / Cysts

Axial T1 and T2 images in a 26 year female demonstrates a well-defined T1 hypointense & T2 hyperintense mass medial to the ICA that compresses the longus colli muscle. This could be mistaken for a suppurative RPS adenitis. The marked enhancement on the T1+Gd image is however not consistent with such a diagnosis. Pathology revealed a schwanomma likely arising from the sympathetic chain as the patient did not have vocal cord paralysis to indicate vagus origin. Soft Tissue Tumors / Cysts

T2 images in a 60 year old female reveal a well-defined, oval, hyperintense lesion that might be mistaken for suppurative RPS adenitis. Patient’s age, lack of inflammatory changes, location anterior to the ICA and the heterogeneous internal enhancement on the T1+Gd image contradict such a diagnosis. The location is characteristic of a parapharyngeal space mass with the pathology revealing benign minor salivary gland tumor. Soft Tissue Tumors / Cysts

CECT in a 57 year old male shows a well-defined, oval, hypodense lesion medial to ICA concerning for suppurative RPS adenitis. Patient’s age & the lack of enhancement are contradicting such a diagnosis. Axial T1 images reveal that the lesion is spontaneously hyperintense due to proteinaceous fluid & located anterior to the pharyngeal constrictor muscle consistent with a nasopharyngeal retention cyst. Conclusion:

 Identification of key clinical and imaging findings of suppurative RPS lymphadenitis requires attention in resident education

 This exhibit focuses on improving competence in diagnosing suppurative RPS lymphadenitis through

o Presentation of imaging features of common pathologies of the RPS that are confused with suppurative RPS adenitis (e.g. RPS abscess, RPS edema, & necrotic RPS nodal metastasis)

o Review of other mimics of RPS pathologies that affect adjacent anatomical structures (e.g. nasopharynx, tonsillar & paravertebral structures) References

• Mancuso AA, Hanafee WN. Head and Neck Radiology. Head and Neck Radiology. Volumes I and II. Lippincott & Williams and Wilkins, 2011.

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• Hoang JK, Branstetter BF, Eastwood JD, et al. Multiplanar CT and MRI of Collections in the Retropharyngeal Space: Is It an Abscess? AJR. 2011;196: W426-W432.

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• widionline.com