Retropharyngeal Soft Tissue Research Articles

MEDB-34. A very rare case: medulloblastoma relapse with bone marrow infiltration in a toddler

We report about a female toddler congenitally deaf and diagnosed with a non-metastatic desmoplastic medulloblastoma (SHH activated, TP53-wt, variant in LDB1 gene). No tumor predisposition syndrome was found. After complete tumor resection the patient was treated according to I-HIT-MED-Guidance protocol. Five months later an asymptomatic localised relapse (same histology, PTEN frameshift deletion, TERT mutation, LDB1 mutation) detected by routine MRI was treated by complete resection, craniospinal irradiation and an antiangiogenic regimen adapted from the MEMMAT scheme including fenofibrate, thalidomide, celecoxib, topotecan, temozolomide, bevacizumab and intraventricular cytarabine. Before start of systemic treatment blood cell counts were normal. In the second cycle we had to interrupt chemotherapy due to a leukopenia while continuing the antiangiogenic treatment. In order to avoid relevant bone marrow toxicity chemotherapy doses were reduced. Nevertheless we had to stop the fourth cycle because of a severe pancytopenia. Same time the girl presented with fever, neck and leg pain. A full blood count showed: hemoglobin 6.92 g/dl, leukocytes 640/µl, platelets 8,000/µl. Suspecting an infection supported by the presence of a high CrP value of 230 mg/l the patient was treated with i.v. antibiotics. MRI showed an unspecific retropharyngeal soft tissue augmentation, a pleural effusion and high T2 signals in multiple vertebral bodies but no central tumor relapse. The bone marrow diagnostics revealed a diffuse medulloblastoma cell infiltration with the known PTEN frameshift deletion and LDB1 mutation. The liquor was tumor-cell free. We report on an extremely rare case of an early local relapse of desmoplastic medulloblastoma progressing to a diffuse bone marrow infiltration in a toddler. The girl died due to therapy resistance 9 weeks after bone marrow relapse. It remains unclear whether the fatal course was related to the hereditary deafness syndrome and the molecular alterations of the tumor.

Differential tumor biology between locoregional and distant metastasis in a patient with TENIS with TKI-resistant aggressive recurrent disease: a comparative evaluation with FDG, 68Ga-DOTATATE and 68Ga-PSMA-11 PET-CT.

The molecular PET-CT imaging profile of an interesting case of differentiated thyroid carcinoma, later transformed into with thyroglobulin elevation and negative iodine scintigraphy (TENIS) with tyrosine kinase inhibitor (TKI) resistant recurrent aggressive disease, is presented. The patient was evaluated to assess SSTR-2 or PSMA expression to explore the possibility of any effective targeted nuclear therapy. 18F-FDG, 68Ga-DOTATATE and 68Ga-PSMA-11 PET/CT was performed, which revealed tracer avidity in all 3 scans in the extensive loco-regional disease of large ill-defined retropharyngeal and retro-tracheal soft tissue eroding cricoid cartilage, extending into tracheal lumen and left sided strap muscles. On the contrary, there was no definite uptake in the multiple bilateral lung nodules, the scan findings indicating a differential tumor biology between loco-regional and distant metastasis.

1768 Esophagitis Dissecans Superficialis Secondary to Ingestion of Hair Dye

INTRODUCTION: Esophagitis dissecans superficialis (EDS) is a rare and underdiagnosed esophageal lesion characterized by sloughing of the esophageal mucosa that has been associated with various autoimmune disorders and exposure to some chemical irritants. Our case highlights a 65-year-old woman diagnosed with EDS after incidental ingestion of hair dye containing resorcinol and paraphenylenediamine (PPD). CASE DESCRIPTION/METHODS: The patient is a 65 year-old female with a medical history of diabetes, hypertension, major depressive disorder, asthma, alcohol use disorder who presented to our emergency department complaining of nausea and vomiting after ingesting a colored hair dye. The patient stated she accidentally ingested 2 oz of hair dye believing it was cough syrup. Notable laboratory findings on admission included a complete blood count within normal limits; a venous blood gas showing a pH of 7.274; pCO2 of 39.2 mm Hg; HCO3 of 17.2 mEq/L and a lactic acid of 5.1. Notable imaging included a chest x-ray showing focal patchy opacities at both lung bases; and an X-ray of the neck showed no retropharyngeal soft tissue swelling. Physical exam was unremarkable. After initial evaluation esophagogastroduodenoscopy (EGD) was done and showed mild superficial mucosal desquamation in entire esophagus suggestive of esophagitis dissecans superficialis (Figure 1). After the procedure the patient was started on pantoprazole and admitted to the general medical floor for further monitoring. The following night the patients symptoms resolved and she was discharge with follow up appointments. DISCUSSION: PPD is an aromatic amine that is widely used in hair dye due to its favorable cosmetic outcomes. Adverse effects of PPD include cervicofacial edema, upper airway tract edema, oliguria, and shock. Resorcinol is a phenolic chemical that is also used in hair dye. Readily absorbed by the gastrointestinal tract, resorcinol is a corrosive acid that causes severe burns on direct contact. Its consumption may also result in pulmonary edema, hemolysis, and metabolic acidosis. Clinical manifestations of EDS are non-specific and include vomiting and dysphagia. Endoscopically it is characterized by sheets of sloughed squamous tissue which typically appear as multiple columns of strips, often described as "gift wrap paper" like distribution as was present in our case. Its prognosis is very favorable and long term complications are rare. To our knowledge this makes the first reported case of EDS occurring secondary to ingestion of hair dye.

Ganglioneuroma of the retropharyngeal space in a patient with glottic cancer

We describe the case of a 71-year-old man with a ganglioneuroma of the retropharyngeal space. The patient presented with a submucosal bulge of the left oropharyngeal wall during follow-up examination...

Influence of prevertebral soft tissue swelling on dysphagia after anterior cervical discectomy and fusion using a rectangular titanium stand-alone cage.

Background:Postoperative oropharyngeal complications such as dysphagia after anterior cervical spine surgery are some of the least discussed surgery-related complications. The purpose of this retrospective study is to investigate the incidence and possible risk factors for 30-day postoperative dysphagia after anterior cervical discectomy and fusion (ACDF).Materials and Methods:This study included 152 consecutive patients who underwent 1- or 2-level ACDF using a rectangular titanium stand-alone cage in our institutes. Surgery-related dysphagia early after surgery was analyzed based on hospital charts. Radiological evaluation of prevertebral soft tissue swelling (PSTS) was performed by comparing plain lateral radiographs of the cervical spine before surgery with those after surgery. The percentage of PSTS (%PSTS) was defined by retropharyngeal soft tissue diameter divided by vertebral diameter. Positive %PSTS was determined when %PSTS exceeded its mean + 2 standard deviations.Results:Twelve patients (7.9%) demonstrated prolonged symptoms of dysphagia within 30-day postoperatively. All patients eventually demonstrated satisfactory or acceptable recovery late after surgery, except one case of hypoglossal nerve palsy. %PSTS was significantly highest early after surgery and returned to presurgical levels within 30 days after surgery. Statistical analysis suggested that the positive %PSTS at C3 or C4 level early after surgery was significantly associated with the occurrence of postoperative dysphagia.Conclusions:Although the possible reasons for postoperative dysphagia may not only be multifactorial but also be highly surgeon-dependent, such a complication is still underestimated and needs to be carefully resolved. %PSTS appeared to be easy and reliable index to judge the possible risk of postoperative dysphagia.

Diagnosing Epiglottitis with Radiographs: Can we be More Objective?

Objective The classical radiographic signs in acute epiglottitis are qualitative. The purpose of our study was to identify objective radiographic parameters to aid diagnosis of acute epiglottitis in Chinese adults. Methods A case-control study of Chinese adult patients attending the accident and emergency department (AED) who were subsequently diagnosed to have acute epiglottitis from 1st January 2009 to 31st December 2013 in a public hospital in Hong Kong. Controls were one-to-one age- and sex-matched patients who attended the AED during the study period for orthopaedic problems with cervical spine radiographs taken. Cases and controls were retrieved from the hospital computer system and their radiographs were interpreted by two specialist radiologists with various pharyngeal and laryngeal parameters, including the dimensions of the third cervical vertebral body, epiglottis, aryepiglottic fold, hypopharynx, retropharyngeal soft tissue and retrotracheal soft tissue, measured and analysed. Sensitivity, specificity, positive and negative likelihood ratios were calculated for each of the parameters. Results Twenty-six patients and 26 age- and sex-matched controls were included in the study. Epiglottis width of 5.5 mm or more was found to be 96.2% sensitive and 100% specific in diagnosing acute epiglottitis. Positive and negative likelihood ratios were infinity and 0.04 respectively. Aryepiglottic fold width of 5.9 mm or more was 92.3% sensitive and 80.8% specific. Positive and negative likelihood ratios were 4.80 and 0.10 respectively. Conclusion The identified objective radiographic parameters should aid in the diagnosis of acute epiglottitis in Chinese adults. (Hong Kong j. emerg.med. 2016;23:168-175)

Traditional Threshold for Retropharyngeal Soft-Tissue Swelling Is Poorly Sensitive for the Detection of Cervical Spine Injury on Computed Tomography in Adult Trauma Patients

Retrospective cohort study. To examine the diagnostic value of prevertebral soft-tissue swelling in the setting of cervical spine trauma. In adult patients with trauma, an increase in the thickness of the retropharyngeal soft tissues is commonly used as a potential indicator of occult injury, but no studies have examined this parameter using computed tomography (CT) as a screening modality. A total of 541 patients with trauma with injuries at any level of the spine underwent CT. Patients with cervical injury were divided into those requiring noninvasive (observation or cervical collar, n = 142) management, and those requiring invasive (surgery or halo, n = 61) treatment. A control group of patients with isolated thoracic or lumbar injuries was used for comparison (n = 542). Retropharyngeal soft tissues were measured at the cranial and caudal endplates of all cervical levels on sagittal and axial CT. Sensitivity and specificity were calculated for +1, +2, and +3 standard deviations from mean values. Sensitivity for detection of injury was found to be universally poor for all measurement groups. This ranged from 14.4% to 21.2% at +1 SD to 5.3% to 8.7% at +2 SD. Positive and negative predictive values for injury were also universally poor, ranging from 38% to 75%. Soft-tissue swelling as a sentinel sign of cervical spine injury demonstrates consistently high specificity and low sensitivity, precisely the opposite of what would be desired in a screening test. This study shows at best a sensitivity of 21.6% when using this parameter for the detection of these injuries in adult patients with trauma. On the basis of the results of this study, we recommend against the routine use of measurement of the prevertebral soft tissues on CT as a screening tool for cervical spine injury in adult patients with trauma. 3.

Variability of Postoperative Esophagrams after Endoscopic Cricopharyngeal Myotomy: Technique Dependence

We illustrate the dependence of postoperative day (POD) 1 esophagram findings on the closure technique used after endoscopic cricopharyngeal myotomy (ECPM). We performed a retrospective chart review of POD 1 fluoroscopic examinations of the cervical esophagus utilizing contrast dye after ECPM to assess radiologic findings associated with three different techniques of addressing the exposed buccopharyngeal fascia (BPF). Each technique resulted in specific and different findings on the POD 1 esophagram. When the BPF was untreated, the esophagram demonstrated a pseudodiverticulum with free flow of contrast dye. When a fibrin glue seal was used, the esophagram demonstrated a curvilinear focus of contrast dye projected over the retropharyngeal soft tissue persisting after the swallow, similar to a leak. When fibrin glue application was combined with single-suture reapproximation of the mucosal incision, the pattern was similar to esophagrams performed 6 weeks after myotomy. Different techniques used to address the exposed BPF following ECPM result in specific findings on the POD 1 esophagram. Recognition of these imaging differences and open communication with the fluoroscopist will avoid a misdiagnosis of a pharyngeal leak, which might cause an unnecessary delay of oral feeding and hospital discharge.

Grisel's Syndrome: A Case Report

Grisel’s syndrome is a non-traumatic atlantoaxial subluxation associated with inflammatory conditions of head and neck which occurs primarily in children. Increased flexibility of the ligaments during the inflammation is implicated in the pathogenesis of the subluxation between the axis and atlas. The diagnosis is made by clinical and radiological findings. In this article, we present a 19-year-old male patient who was admitted with a complaint of gradually increasing neck pain and stiffness. He had no history of trauma. Cervical spine radiography and computed tomography revealed atlantoaxial subluxation. Magnetic resonance imaging revealed subluxation and retropharyngeal soft tissue infection. Based on the clinical and radiological findings, we diagnosed the patient as having Grisel’s syndrome, and the patient was treated with intravenous antibiotics and internal immobilization. Although the potential sequelae may be severe, early diagnosis and treatment of Grisel’s syndrome can prevent tragic outcomes.

A 2-Year Follow-Up of Swallowing Function After Radiation Therapy in Patients With Nasopharyngeal Carcinoma

Chang Y-C, Chen S-Y, Ting L-L, Peng SS, Wang T-C, Wang T-G. A 2-year follow-up of swallowing function after radiation therapy in patients with nasopharyngeal carcinoma. Objective To evaluate over a 2-year period the serial swallowing function of patients with nasopharyngeal carcinoma (NPC) after completing radiotherapy (RT). Design Prospective longitudinal follow-up. Setting University hospital. Participants Patients with NPC (N=76) referred for RT: 53 of them at 1 year after RT, and 23 at 2 years after RT. Interventions Not applicable. Main Outcome Measures Participants completed a questionnaire and had a video-recorded fluoroscopic swallowing study before RT and 1 month, 1 year, and 2 years after RT. Results The highest incidence of dysphagia symptoms and retropharyngeal soft tissue swelling occurred in the first month after RT and decreased over time. Pharyngeal transit time was prolonged continuously up to 1 year after RT. Epiglottic vallecular stasis and pharyngeal mucosal coating were worst in the first month after RT and stable afterwards. Aspiration was uncommon during the first 2 years after RT. Conclusions At a 2-year follow-up after RT, patients with NPC had a progressively increasing pharyngeal transit time, although the subjectively identified symptoms of dysphagia decreased after the first month after RT.

Kawasaki Disease Presenting as Retropharyngeal Cellulitis

Objective: Kawasaki disease (KD), which occurs in infants and children, is an acute multisystemic vasculitis of unknown etiology. Several reports mentioned retropharyngeal cellulitis as a rare manifestation of KD. The purpose of this study is to investigate the population and characteristics of KD manifested by retropharyngeal soft tissue swelling. Method: A retrospective review of 213 patients (mean age of 2 years, age range of 7 months to 10 years) diagnosed as KD between 2005 and 2010. Results: Enhanced computed tomography showed an abnormal low density area without ring enhancement in the retropharyngeal space in 9 cases (4.2%). All of them had fever and cervical lymphadenopathy as the first symptom. Though cervical lymphadenopathy is one of the 6 diagnostic features of KD (fever, skin rash, cervical lymphadenopathy, conjunctivitis, fissuring of lips, and strawberry tongue), the occurrence is reported to be the lowest (about 50%). They were treated by a pediatrician as suppurative lymphadenitis in 6 cases and as retropharyngeal cellulitis in 3 cases, and they were finally diagnosed as KD owing to ineffectiveness of antibiotics or emergence of the other symptoms. Conclusion: A low density area on computed tomography in the retropharyngeal space was identified in 4.2% of KD. Early diagnosis is essential in KD because coronary artery lesions emerge in 50% of patients without treatment. If a young child has fever, cervical lymphadenopathy and swelling of the retropharyngeal space, KD should be within differential diagnoses.

Brucellar spondylitis presenting with neck stiffness and accompanying retropharyngeal inflammatory soft tissue mass

Brucellosis is a systemic inflammatory condition caused most commonly by Brucella Melitensis, a gram negative coccobacilli which is transmitted by infected animals and their products. Ondulent fever, night sweats and generalized weakness are the most common clinical symptoms but the wide spectrum of complaints can even include psychiatric disorders. We present a case who had neck stiffness and difficulty in neck movement as the initial complaints and radiologically, retropharyngeal soft tissue swelling at upper cervical level in MRI. This case report reveals the value of MRI in the further characterisation of pharyngeal involvement by brucellosis with an atypical presentation.

Poster 120: Crowned Dens Syndrome: A Case Report

Setting: Tertiary care hospital. Patients: 82-year-old man with acute onset neck pain. Assessment/Results: An 82-year-old man with history of myelodysplastic syndrome and diabetes presented with acute onset of neck pain 4 days prior to initial evaluation. He was seen in the emergency department and then by his primary care provider. Working diagnosis included neck strain or disk herniation and he was treated with a trigger point injection, muscle relaxant, and oral narcotics. He eventually required hospital admission for intractable pain. His workup revealed an elevated ESR and a magnetic resonance imaging which showed retropharyngeal soft tissue edema concerning for hemorrhage or infection. Computed tomography (CT)-guided aspiration was negative. He was referred to physical medicine and rehabilitation for further evaluation. Physical exam showed limited, painful cervical range of motion (ROM) and pain with palpation and ROM of the left shoulder, right wrist, and right knee. An inflammatory arthropathy was suspected and rheumatology department was consulted. ESR was elevated and aspiration of the right knee showed positively birefringent crystals under polarized light. Review of the cervical CT scan revealed calcification of the transverse ligament of the atlas consistent with Crowned Dens syndrome which is typically caused by calcium pyrophosphate dihydrate deposition disease (CPPD or pseudogout). The patient improved readily with oral steroid therapy. Conclusions: Crowned Dens syndrome is an uncommon, or probably under-reported, cause of acute neck pain. It may be more prevalent in those with concomitant CPPD affecting other joints. As in this case, the diagnosis may be elusive, mimicking other causes such as radiculopathy, neck strain, or infection. The onset of pain may be acute and severe, commonly affecting older and female patients. Evaluation should include a comprehensive physical exam with attention to cervical rotational ROM. Identification of inflammatory markers, cervical CT showing crescentic calcification posterior to the dens, plain films of other involved joints, and synovial fluid analysis showing positively birefringent crystals, helps confirm the diagnosis. Treatment with nonsteroidal anti-inflammatory drugs or corticosteroids is usually successful. Thorough evaluation is necessary as complications may be disabling.

Pediatric airway

To become familiar with common causes of acute airway obstruction in children. To develop an imaging approach to the infant with stridor or wheeze. To receive an introduction to chronic airway obstructions or masses unique to children. Shoulder bolster is very helpful for soft tissue neck films. Fluoro can be helpful to see if child needs CT. Best sign of retropharyngeal soft tissue swelling is loss of normal step-off. Delayed CT images through coalescent low density area can help distinguish abscess from focal cellulitis. Maintain a high index of suspicion for aspirated foreign body in craw-ling infants. Consider decubitus or expiratory views to show air-trapping. Nasopharyngeal or sinus neoplasm in young child is most likely rhabdomyosarcoma (not squamous cell carcinoma). Subglottic mass in infant most likely hemangioma. Clinical, pathologie, and imaging features of common conditions causing acute airway obstruction and more chronic conditions affecting the pediatrie airway will be presented.

PASTEURELLA MULTOCIDA MENINGITIS AND CERVICAL SPINE OSTEOMYELITIS IN A NEONATE

A 20-day-old male infant presented with fever, decreased alertness and quadriparesis as a result of Pasteurella multocida meningitis and C1-2 vertebral osteomyelitis. Although his household contained 2 pet cats, there was no history of bites, scratches or licks. We speculate that colonization of the nasopharynx was followed by contiguous spread to the retropharyngeal soft tissue, cervical vertebrae and meninges.

Ｂａｒｒ´ｅ‐Ｌｉ´ｅｏｕ徴候を伴った頚椎カリエスの１例

Tuberculosis of the cervical spine is uncommon to arise in the whole apine. We report a case of tuberculous cervical spondylitis with Barre-Lieou syndrome.The case was a 61 year old female, who complained of mild muscle weakness and strong dizziness at motion, headache, nausea, so called Barre-Lieou syndrome. MRI showed rim enhancement of the retropharyngeal soft tissue and peridural abscess. We therefore performed the operation of curretage and anterior spinal body fusion by use of autogenous fibula bone, adding the chemotherapy of INH, RFP and SM. She gradually recovered from Barre-Lieou syndrome.The origin of Barre-Lieou syndrome in this case was unsidered the source of the irritation of the vertebral nerve by tuberculous abscess.

Retropharyngeal cellulitis: A manifestation of kawasaki disease in children

Kawasaki disease (KD) or mucocutaneous lymph node syndrome is an acute febrile illness with prominent vasculitic features. The cause is unknown and the disease is seen most commonly in young children. After its initial description in Japan, the disease was thought to be rare in the United States, but recognition has dramatically increased over the past decade. Common symptoms of the disease for which the otolaryngologist may be initially consulted include inflammation of the lips and oral cavity and acute cervical lymphadenopathy. We present a case of Kawasaki disease initially complicated by retropharyngeal soft tissue swelling. Similar reports are rare in the pediatric literature; however, no such reports appear in the otolaryngology literature.

Dynamic radiographic imaging following total laryngectomy.

Cinepharyngoesophograms of the pharynx were obtained in 51 patients after total laryngectomy for squamous cell carcinoma. The radiological findings were correlated with operative reports and follow-up findings in 47 patients. Postoperative anatomic changes were well-demonstrated radiographically. A spectrum of radiographic findings were observed and included narrowing at the superior surgical closure site in 52% and pseudodiverticula in 47% of all patients. Cricopharyngeal prominence was observed in 15%, fistulae in 10%, and pharyngeal pouches in 6% of all cases. Increased retropharyngeal soft tissue thickness was not found to be indicative of tumor recurrence. We conclude that cineradiography is a useful method for demonstrating both functional and structural changes following laryngectomy for carcinoma.

The value of retropharyngeal soft tissue measurements in trauma of the adult cervical spine. Cervical spine soft tissue measurements.

Widening of the retropharyngeal soft tissue space (RSTS) has been interpreted as a sign of cervical spine trauma. Widely differing measurements are reported in the literature. However it is clear that, using the currently acceptable limits of normal, a large number of patients without spinal injury are subjected to further tests. The RSTS on lateral cervical spine radiographs of 318 patients with a history of cervical spine trauma were measured at the C2-C4 level. The values for patients found to be normal or with fractures/dislocations were evaluated. Statistical analysis of the results demonstrates a considerable overlap in the RSTS of normal and abnormal patients. Therefore we suggest that RSTS measurements are of limited diagnostic value. Using the statistical probability of abnormality, guidelines that indicate which patients may require additional study are suggested.

Radiographic parameters in adult epiglottitis

A retrospective study was undertaken to define precise radiographic criteria for the diagnosis of epiglottitis in the adult. We reviewed the standard lateral neck films of six patients over the age of 18 with epiglottitis and five with a normal epiglottis. Radiographic anatomy measured included the angle of the valleculae, retropharyngeal soft tissue width at C2, retrotracheal soft tissue width at C6, width of the epiglottis, width of the aryepiglottic folds, and the hypopharyngeal to tracheal air column ratio. The measurement differences were significant between the groups only for the width of the epiglottis and aryepiglottic folds (P less than .01). Width of the epiglottis greater than 8 mm and of the aryepiglottic folds greater than 7 mm seem highly suggestive of epiglottitis in the adult.