Surgical intervention for malignant tumors of the skull base through collaboration among surgeons from the fields of neurosurgery, head and neck surgery, and plastic surgery has yielded good results over the last two decades. This is considered to be because craniofacial resection (CFR) can be performed without touching the tumor, while preserving the intracranial tissue, and the internal and external cranium can be safely shielded by reconstruction. On the other hand, the surgical risk, postoperative disorders and facial aesthetic problems still pose major problems.
We have developed some methods to make CFR more reliable and safe, e.g., 1) some adequate approaches to the infratemporal fossa; 2) combined use of an endoscope; 3) combined maxillary swing approach and infratemporal fossa approach for nasopharyngeal cancer; 4) skull reconstruction using a custom-made titanium mesh. We also introduced 1) a 3D virtual simulation and navigation system, and 2) psychiatric liaison activity, as examples of efforts to improve the safety.
Team approach is important for surgical treatment of malignant tumors of the skull base. In addition to the surgical team, the safety of skull base malignant tumor treatment will be improved by building a comprehensive team of oncologists, radiation therapists, psychiatrists, clinical psychologists, and nurses.
Infectious mononucleosis is a viral syndrome that is most often caused by Epstein-Barr virus (EBV). A few patients, at the time of the initial infection with EBV, reportedly manifest neurological symptoms, such as meningitis, Guillain-Barré syndrome, and encephalitis. We report a case of infectious myelitis in association with infectious mononucleosis caused by EBV. The patient was a 23-year-old woman who presented with persistent sore throat, fever, and cervical lymphadenopathy. Examination revealed bilateral tonsillar hypertrophy with redness and pus discharge. After making a diagnosis of infectious mononucleosis based on the laboratory data and findings of physical examination, we treated the patient with antibiotics. On the fourth day after the initiation of treatment, the patient showed unilateral motor and sensory paralysis of acute onset. Cervical MRI revealed high signal intensities in the C5 section of the spinal cord. Neurologists at our institution began the patient on pulse therapy with methylprednisolone. After two courses of steroid pulse therapy, the motor and sensory paralysis improved. Cerebrospinal fluid examination revealed a negative test result for EBV-DNA, but the patient was diagnosed as having infectious myelitis caused by EBV from the clinical course.
Although infectious mononucleosis is encountered frequently by otolaryngologists, care should be taken to watch out for the development of hidden complications in patients with acute tonsillitis presenting with atypical findings, such as neurologic symptoms.
Congenital middle ear anomalies could involve the ossicles, middle ear cleft, and middle ear muscles and tendons. Among these, the absence of a stapedius tendon is a rare middle ear malformation. Herein, we present a case of absent stapedius tendon not associated with hearing impairment.
A 24-year-old woman was referred to Kyoto University Hospital with left facial palsy after being prescribed systemic corticosteroid therapy. The hearing level in both ears was below the standard threshold, but the left stapedius muscle reflex was absent. Trans-mastoid decompression of the facial nerve canal was performed because of the low ENoG value. During the surgery, we found a defect in the left pyramidal eminence and an absent stapedius tendon. A review of the preoperative computed tomographic images also revealed a deficiency of the stapedius muscle.
Although in most previously reported clinical cases, the absence of a stapedius tendon was accompanied by hearing loss, our patient had a normal hearing level. This case suggests that a defect of the stapedius tendon might not affect the hearing ability. Because subjects with normal hearing would have limited opportunities to have their stapedius tendon examined, the incidence of an absent stapedius tendon in the clinical setting is lower than that in cadaver studies.
Glomangiopericytoma (GPC) is a rare sinonasal tumor. GPC is categorized as a low-grade neoplasm with borderline malignancy and shows a tendency for local recurrence. GPC is histologically characterized by perivascular proliferation of tumor cells and needs to be distinguished from other perivascular neoplasms, such as solitary fibrous tumor and glomus tumor. In addition to general pathological examinations, we performed genetic testing in this case.
We report a case of GPC in a 73-year-old female patient, that was accidentally diagnosed by MRI of the temple region. A reddish smooth tumor was observed in the right nasal cavity. Biopsy revealed a possible perivascular tumor, such as GPC. Thus, we performed complete resection by endoscopic surgery after embolization. The tumor cells showed positive immunohistochemical staining for smooth muscle actin and vimentin, but negative staining for STAT6, CD34 and bcl-2. The tumor cells also showed nuclear accumulation of beta-catenin. In addition, genetic testing revealed a CTNNB1 mutation (p.S37C).
There was no cytologic atypia or mitoses, and no necrosis was observed. A good prognosis can be expected in this case, because the patient could be successfully treated by endoscopic resection and no markers of aggressive behavior were observed. We propose to conduct genetic testing in cases of GPC in the future.
Teratocarcinosarcoma is a rare, highly malignant tumor, that occurs mainly in the nasal cavity and paranasal sinuses. The prognosis is poor because the tumor invades the surrounding tissues even from an early stage. We report a case of sinonasal teratocarcinosarcoma that was treated by transnasal endoscopic skull base surgery and radiotherapy.
The patient was a 65-year-old man who presented with a 2-month history of recurrent left epistaxis. Examination revealed a mass in the left nasal cavity. Computed tomography (CT) revealed a tumor extending from the left ethmoid sinus to the sphenoid sinus, and bone destruction of the anterior skull base. Magnetic resonance imaging (MRI) revealed intracranial extension of the tumor, but no apparent invasion of the brain parenchyma. Biopsy findings led to the suspicion of olfactory neuroblastoma. Since an olfactory neuroblastoma was suspected by biopsy and the possibility of invasion of the brain parenchyma was low, we performed endoscopic endonasal skull base surgery. The tumor was resected with the bone of the skull base and the dura. The skull base was reconstructed with the skull periosteum, fascia lata, and a nasal septum flap. Postoperative histopathological examination revealed the diagnosis of teratocarcinosarcoma. Therefore, after the surgery, the patient received radiotherapy. One year and ten months after the radiotherapy, metastasis to the left cervical lymph nodes was found, and neck dissection was performed. A follow-up examination at 4 years after the radiotherapy showed no evidence of local recurrence or distant metastases to other organs.
It was difficult to confirm the histological diagnosis before surgery, and the diagnosis could be confirmed only by histopathology of the resected tumor specimen. The main treatment modality for teratocarcinosarcoma is surgical excision. However, teratocarcinoma is a highly malignant tumor, and radiotherapy and chemotherapy may be useful in addition to surgery, to obtain a better prognosis.
Background: Endoscopic modified medial maxillectomy (EMMM) is a surgical procedure that allows good visualization of the maxillary sinus and good operability, without requiring alteration of the morphology of the nasal cavity. Endoscopic medial maxillectomy involves resection of the lateral nasal wall, including the inferior turbinate and nasolacrimal duct, whereas EMMM enables preservation of the lateral nasal wall and the structures along it. Therefore, most patients undergoing EMMM recover without sequelae, such as epiphora and empty nose syndrome. Recently, EMMM has been reported as effective surgery for many maxillary sinus diseases.
Methods: We examined the data of 14 patients who underwent EMMM at Ehime Prefectural Niihama Hospital between November 2016 and December 2019. Their medical records were reviewed retrospectively.
Results: The indication for surgery was postoperative maxillary cyst in 7 patients, inverted papilloma in 4 patients and maxillary sinus fungus ball in 3 patients. There were no recurrences during a mean follow-up period of 11 months. Although two of the 14 patients exhibited temporary numbness of the upper teeth due to anterior superior alveolar nerve injury, their symptoms improved over several months. There were no further postoperative sequelae.
Conclusion: EMMM is a safe and effective surgical technique for the treatment of maxillary sinus diseases. EMMM allows expansion of the indications for endoscopic surgery.
There are a few cases of foreign body in the tongue among patients presenting with the chief complaint of tongue pain. Lingual abscess caused by foreign body impaction is extremely rare. Herein, we report a case of lingual abscess in a 69-year-old man, which necessitated emergency surgery. He visited the hospital about a month after sustaining an injury of the tongue. He had an abscess at the base of the tongue at the time of consultation, and he was judged as being at risk of developing an airway emergency. Since visual inspection did not reveal the location of the foreign body, we confirmed the location by ultrasound and CT. Emergency incision and drainage was performed under intraoperative ultrasound guidance, and the foreign body (fish bone) was removed. Our experience suggests that use of intraoperative ultrasound for a lingual abscess may be effective for identifying the location of the foreign body.
Retropharyngeal hematoma can cause dyspnea due to upper airway obstruction. The major cause of retropharyngeal hematoma is trauma, and in a few cases, even mild head and neck injury could be the cause. In the era of a super aged society, because of the potential increase in the frequency of falls from the bed with the increase in the number of elderly people, the incidence of traumatic retropharyngeal hematoma is also expected to increase.
We report the cases of three patients who were referred to our hospital with dyspnea that developed tens of minutes to a few hours later after they sustained mild head and neck injury. Flexible nasopharyngoscopy revealed bulging of the posterior pharyngeal wall, and X-ray/computed tomography (CT) showed widening of the prevertebral soft tissue. We diagnosed the patients as having airway obstruction caused by a traumatic retropharyngeal hematoma. In two cases, we performed tracheal intubation immediately, because the airway was already so narrowed and we expected the retropharyngeal hematoma to increase in size with time. This disease is an emergency medical condition with a high risk of airway obstruction. Otorhinolaryngologists should be aware that even mild head and neck injury can cause severe airway obstruction. Appropriate diagnosis and prompt airway management are important.
Lyme disease, a tick-borne disease, is a systemic infection caused by Borrelia burgdorferi. In Japan, this disease has mainly been reported in Hokkaido prefecture. Although eczema is the most common symptom, some patients suffer from neurological complications. Herein, we report the case of a patient with Lyme disease who developed peripheral facial nerve palsy. The patient, a 72-year-old woman, was bitten by a tick in the right auricle. Two weeks later, she developed erythema and swelling of the right auricle, which were treated with ampicillin. A month later, she developed peripheral facial nerve palsy without other cranial nerve involvements, and was referred to our department. Based on the positive test result for serum anti-Borrelia burgdorferi antibody, the patient was diagnosed as having Lyme disease. She was treated with ceftriaxone and prednisolone, and the facial nerve palsy improved. Among Lyme disease-related neurological symptoms, facial nerve palsy is among the most frequent. It is necessary to be aware of the possible occurrence of facial nerve palsy as a manifestation of neuroborreliosis.
Retropharyngeal hematoma can induce dyspnea by causing upper airway obstruction. Therefore, early diagnosis and prompt securing of the airway, either by tracheal intubation or tracheostomy, are of importance. Tracheostomy is usually carried out between the 2nd and 4th tracheal ring. However, it is difficult to perform standard tracheostomy in patients with abnormalities of the cervical region, such as obesity, low-set larynx, neck stiffness, thyroid tumor, brachiocephalic meandering, or an unstable cervical spine. In 2007, Kano et al. reported “a newly developed surgical airway-opening technique (cricoid fenestration) using partial resection of the cricoid cartilage to form a stoma.”
The patient reported herein was a 70-year-old man with ankylosing spondylitis who presented with an unstable cervical spine fracture and a retropharyngeal hematoma. The patient sustained a fall after binge drinking and presented with breathlessness; he was diagnosed as having a cervical spine fracture and a retropharyngeal hematoma. An anesthesiologist performed tracheal intubation, however, laryngopharyngeal edema presented until 2 weeks later. Therefore, we performed cricoid fenestration as we could not perform a standard tracheostomy because of the difficulty in neck extension. The patient showed a good clinical course following this procedure.
Cricoid fenestration is a useful and safe technique for casess where standard tracheostomy is expected to be difficult. However, we have observed this patient for only less than one year after closure of the tracheal stoma, and must continue to observe the patient for any late complications.
Angioedema is characterized by localized, rapid and severe swelling of the subcutaneous and submucosal tissues. Angioedema of the upper airway can lead to life-threatening airway obstruction. Acquired angioedema (AAE) is known to be caused by a deficiency or dysfunction of C1-esterase inhibitor (C1-INH) related to underlying diseases. We report a case of acute angioedema that was strongly suspected, but could not be confirmed, as a case of AAE. A 70-year-old man presented to us with sudden non-pitting edema of the floor of his mouth, tongue and neck. The swelling appeared within a few hours after the patient had bitten his own tongue. He was treated with tranexamic acid and the symptom improved promptly. He had experienced two episodes of acute angioedema in the past, and the first episode was when he was 68 years of age. There was no family history of angioedema. Based on the findings, we considered that this was not a case of hereditary angioedema (HAE). Blood test revealed reduced serum levels of C4, C1-INH, and C1q, and we strongly suspected that the patient had AAE. However, we could not detect any underlying diseases that could predispose to the development of AAE. Five months later, the patient developed the fourth episode of AE. Treatment with tranexamic acid failed to improve the symptom, and we had to perform tracheostomy for upper airway obstruction. The treatment of first choice for patients with AAE is control of the underlying disease. However, when the cause of the AAE remains unclear, AAE should be treated according to the treatment principles for HAE.