Current classification of vascular anomalies of the head and neck.

Vascular anomalies of the head and neck comprise a wide spectrum of phenotypically diverse lesions. Optimal diagnosis and management of these lesions are critically dependent upon establishment of uniform and well-defined histopathologic, clinical, and radiological criteria, but these remain subject of debate. In this paper, we describe the International Society for the Study of Vascular Anomalies classification scheme, which was first published in 1996 and updated in 2014. The strength of this proposal rests on its distinction between vascular malformations and tumors, and is responsible for its wide adoption. This paradigm serves as a developing platform for diagnosis, inter-collegial communication, and treatment, and adhering to it will help clinicians to improve the management of vascular anomalies.

Angiosarcomas of the head and neck: Impact of large data analysis on clinical management.

Angiosarcoma is a rare but often fatal malignancy from blood and lymphatic vessels that can arise anywhere in the body and often affects the head and neck region. Although its dismal prognosis is predominantly explained by its aggressive biology, several secondary factors contribute to poor outcomes. These include a phenotypic resemblance to innocuous blood vessel lesions, which contributes to a significant degree of late diagnosis. Another important factor is the rarity of angiosarcoma, which has impaired scientific determination of its optimal treatment significantly. As a result, treatment of angiosarcomas has largely been guided by information derived from the study of sarcomas at large, themselves a highly heterogeneous group of mesenchymal cancers both from a diagnostic as well as therapeutical perspective. The Digital Revolution and resultant Information Age promise to transform the clinical management of rare cancers from a generic to a more customized approach. In this paper, we review the current understanding of head and neck angiosarcomas within the context of this process.

Hearing loss and speech perception in noise difficulties in Fanconi anemia.

OBJECTIVES/HYPOTHESIS: Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing loss and speech perception in noise difficulties in Dutch Fanconi anemia patients. STUDY DESIGN: Retrospective chart review. METHODS: A retrospective chart review was conducted at a Dutch tertiary care center. All patients with Fanconi anemia at clinical follow-up in our hospital were included. Medical files were reviewed to collect data on hearing loss and speech perception in noise difficulties. RESULTS: In total, 49 Fanconi anemia patients were included. Audiograms were available in 29 patients and showed hearing loss in 16 patients (55%). Conductive hearing loss was present in 24.1%, sensorineural in 20.7%, and mixed in 10.3%. A speech in noise test was performed in 17 patients; speech perception in noise was subnormal in nine patients (52.9%) and abnormal in two patients (11.7%). CONCLUSIONS: Hearing loss and speech perception in noise abnormalities are common in Fanconi anemia. Therefore, pure tone audiograms and speech in noise tests should be performed, preferably already at a young age, because hearing aids or assistive listening devices could be very valuable in developing language and communication skills. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2358-2361, 2017.

The role of the tensor veli palatini muscle in the development of cleft palate-associated middle ear problems.

OBJECTIVE: Otitis media with effusion is common in infants with an unrepaired cleft palate. Although its prevalence is reduced after cleft surgery, many children continue to suffer from middle ear problems during childhood. While the tensor veli palatini muscle is thought to be involved in middle ear ventilation, evidence about its exact anatomy, function, and role in cleft palate surgery is limited. This study aimed to perform a thorough review of the literature on (1) the role of the tensor veli palatini muscle in the Eustachian tube opening and middle ear ventilation, (2) anatomical anomalies in cleft palate infants related to middle ear disease, and (3) their implications for surgical techniques used in cleft palate repair. MATERIALS AND METHODS: A literature search on the MEDLINE database was performed using a combination of the keywords "tensor veli palatini muscle," "Eustachian tube," "otitis media with effusion," and "cleft palate." RESULTS: Several studies confirm the important role of the tensor veli palatini muscle in the Eustachian tube opening mechanism. Maintaining the integrity of the tensor veli palatini muscle during cleft palate surgery seems to improve long-term otological outcome. However, anatomical variations in cleft palate children may alter the effect of the tensor veli palatini muscle on the Eustachian tube's dilatation mechanism. CONCLUSION: More research is warranted to clarify the role of the tensor veli palatini muscle in cleft palate-associated Eustachian tube dysfunction and development of middle ear problems. CLINICAL RELEVANCE: Optimized surgical management of cleft palate could potentially reduce associated middle ear problems.

Surgery versus endoscopic cauterization in patients with third or fourth branchial pouch sinuses: A systematic review.

OBJECTIVES: To systematically review the current literature on treatment of third and fourth branchial pouch sinuses with endoscopic cauterization, including chemocauterization and electrocauterization, in comparison to surgical treatment. DATA SOURCES: PubMed, Embase, and the Cochrane Library. REVIEW METHODS: We conducted a systematic search. Studies reporting original study data were included. After assessing the directness of evidence and risk of bias, studies with a low directness of evidence or a high risk of bias were excluded from analysis. Cumulative success rates after initial and recurrent treatments were calculated for both methods. A meta-analysis was conducted comparing the success rate of electrocauterization and surgery. RESULTS: A total of 2,263 articles were retrieved, of which seven retrospective and one prospective article were eligible for analysis. The cumulative success rate after primary treatment with cauterization ranged from 66.7% to 100%, and ranged from 77.8% to 100% after a second cauterization. The cumulative success rate after the first surgical treatment ranged from 50% to 100% and was 100% after the second surgical attempt. Meta-analysis on electrocauterization showed a nonsignificant risk ratio of 1.35 (95% confidence interval: 0.78-2.33). CONCLUSIONS: The effectiveness of cauterization in preventing recurrence seems to be comparable to surgical treatment. However, we suggest endoscopic cauterization as the treatment of choice for third and fourth branchial pouch sinuses because of the lower morbidity rate.

Endoscopic transmucosal direct puncture sclerotherapy for management of airway vascular malformations.

OBJECTIVES/HYPOTHESIS: To describe a multidisciplinary approach to the treatment of airway vascular malformations (venous or lymphatic) with direct suspension rigid laryngoscopy and direct puncture transmucosal bleomycin sclerotherapy injected under road-mapping fluoroscopic monitoring, supplemented by Dyna-computed tomography utilization. STUDY DESIGN: Case series. METHODS: We performed a retrospective medical record and imaging review of four patients with venous malformations or lymphatic malformations located in the airway. Patients were treated with a combination of direct suspension laryngoscopy or rigid nasopharyngoscopy and image-guided direct puncture bleomycin sclerotherapy. RESULTS: Two patients presented to our institution with extensive lymphatic malformation of the neck, parapharyngeal, and retropharyngeal spaces, and two presented with venous malformation of the nasopharynx and oropharynx. All patients were treated with multiple sclerotherapy and debulking procedures before undergoing combined direct transmucosal puncture bleomycin sclerotherapy guided by direct laryngoscopy or nasopharyngoscopy. All patients had complete resolution of disease while maintaining a safe airway. CONCLUSIONS: A multidisciplinary approach to airway vascular malformations with a combination of endoscopy and direct puncture bleomycin sclerotherapy was demonstrated to be a safe and effective treatment in our patient cohort. Direct laryngoscopy and nasopharyngoscopy provide easy access to the nasopharynx, oropharynx, retro- and/or parapharyngeal spaces and larynx. Unlike traditional agents, bleomycin induces minimal edema and therefore is an ideal substance to treat airway lesions.

Tracheal cartilaginous sleeve association with syndromic midface hypoplasia.

IMPORTANCE: We report 2 new cases of tracheal cartilaginous sleeve and perform a systematic literature review of all documented cases of this condition. We aim to increase awareness of this anomaly and provide recommendations for both intraoperative and postoperative management of patients with tracheal cartilaginous sleeve undergoing tracheostomy. OBSERVATIONS: We studied the clinical records of 2 children with tracheal cartilaginous sleeves and short tracheas treated at our institution. One of these patients had Beare-Stevenson syndrome, and the other had Crouzon syndrome. Both patients required tracheostomy for persistent upper airway obstruction, and both required custom-length tracheostomy tubes. Bronchoscopy and needle localization were beneficial in performing tracheostomy on these patients. All documented cases of tracheal cartilaginous sleeve in the literature were found to be associated with midface hypoplasia secondary to syndromic craniosynostosis. Seventy-five percent of cases required tracheostomy. CONCLUSIONS AND RELEVANCE: Tracheal cartilaginous sleeve can be associated with Beare-Stevenson syndrome. In infants with midface hypoplasia associated with a craniosynostosis syndrome undergoing tracheostomy, the surgeon must be prepared to encounter and manage tracheal cartilaginous sleeve intraoperatively. Bronchoscopy and needle localization can be beneficial when performing tracheostomy in these patients. Customized, shorter-length tracheostomy tubes should be considered for these patients.

Thyroglossal duct cyst and ectopic thyroid: surgical management.

The embryology, presentation, imaging, and treatment of the thyroglossal duct cyst will be reviewed. Anatomic features and surgical technique to prevent complications and recurrence will be discussed. Included in the discussion will be the management of thyroglossal duct cyst malignancy and ectopic thyroid.

Occult laryngomalacia resulting in obstructive sleep apnea in an infant.

Classic laryngomalacia presents in the awake infant with progressive stridor when agitated. Occult laryngomalacia usually presents with stridor in children older than 2 years and is limited to sleep or exercise. There have been no documented cases of occult laryngomalacia causing obstructive sleep apnea in infants. We report the youngest documented case of an infant with state-dependent laryngomalacia resulting in severe obstructive sleep apnea. This patient was successfully treated with supraglottoplasty, with resolution of symptoms. In conclusion, state-dependent laryngomalacia resulting in obstructive sleep apnea may present in children younger than 12 months of age. In these individuals, supraglottoplasty should be considered.

Floor of mouth masses in children: proposal of a new algorithm.

OBJECTIVE: Many surgical techniques have been described to manage floor of mouth masses, but few studies have described the approach to these masses in children. This case series summarizes a single institution's experience with pediatric floor of mouth masses. METHODS: We performed a retrospective chart review of all children who presented at our tertiary care facility with FOM masses between 2007 and 2012. Charts were reviewed for clinical presentation, preoperative, intraoperative and postoperative management. RESULTS: Thirteen cases were retrieved: 6 dermoid cysts, 4 ranulas, 1 lymphatic malformation, 1 imperforate submandibular duct, and 1 enlarged salivary gland. In 10 of 13 patients, clinical diagnosis was consistent with postoperative diagnosis. Imaging was consistent with postoperative diagnosis in 8 of 9 cases. Ten of 13 masses were managed transorally; 7 were excised, 2 were marsupialized and 1 was managed with submandibular duct dilation. Three masses with a larger submental component, 2 dermoids and 1 ranula, were removed transcervically. Most patients undergoing transoral excision underwent nasotracheal intubation; patients who underwent marsupialization underwent orotracheal intubation. There were no recurrences, complications or postoperative infections. An additional surgical procedure was necessary in one patient. CONCLUSION: Our cohort displays a common distribution of lesion types when compared to the literature. Low recurrence and infection rates are observed when oral masses are removed transorally, and masses with a larger cervical component are removed transcervically. More complex masses may warrant additional surgical procedures.

Evidence-based practice: pediatric tonsillectomy.

Tonsillectomy is one of the most common surgical procedures performed in children in the United States. Indications and recommendations for perioperative management are multiple and may vary among clinicians. Although tonsillectomy is a safe procedure, it can be associated with morbidity. Several techniques have been developed to reduce perioperative complications, but evidence of this reduction is lacking. This article provides clinicians with evidence-based guidance on perioperative clinical decision making and surgical technique for tonsillectomy.

Sinonasal manifestations in cystic fibrosis.

Cystic fibrosis is a genetic disease, characterized by accumulation of thickened mucous secretions in exocrine glands. Although the major clinical manifestations of the disease are pancreatic and pulmonary disease, the majority of cystic fibrosis patients will develop sinonasal manifestations as well. This paper outlines the etiology, evaluation, and management of the nasal and sinus manifestations in patients with cystic fibrosis.

Neurochemical characterization of pterygopalatine ganglion branches in humans.

BACKGROUND: Pterygopalatine ganglion (PPG) branches, seem to be involved in the pathophysiology of facial pain. The functions of these branches, including a recently discovered orbital branch, are not completely known but could be of clinical significance. This study was designed to characterize PPG branches through immunohistochemical stain and study their anatomy, specifically the orbital branches. METHODS: In a cadaver study of four specimens, the pterygopalatine fossa (PPF) was dissected out of its bony surroundings as a tissue block. Subsequently, cryostat sectioning of these blocks was performed. In one specimen the PPF was microscopically dissected. Recently discovered neural structures were identified, dissected out of the tissue block, and cryosectioned. All cryostat sectionings were immunohistochemically stained for protein gene product (PGP) 9.5, nitric oxide synthase (NOS), and tyrosine hydroxylase (TH). RESULTS: A recently discovered neural connection between the PPG and the ophthalmic nerve could be confirmed and classified as an orbital PPG branch. The connection stained throughout for PGP 9.5 and partially stained for NOS. In other orbital branches, both NOS and TH(+) nerve fibers were found. The PPG contained NOS(+) cells. TH labeling was also found in nerve fibers running through the PPG and the vidian nerve. CONCLUSION: The recently discovered orbital PPG branch is of a mixed parasympathetic and sensory nature. In the other orbital branches, sympathetic fibers were shown as well. This knowledge may add to understanding the symptomatology and therapies of headache syndromes such as nerve block.

Effects of radiofrequency thermocoagulation of the sphenopalatine ganglion on headache and facial pain: correlation with diagnosis.

AIMS: To study the effect of radiofrequency thermocoagulation (RFT) of the sphenopalatine ganglion (SPG) on headache and facial pain conditions following critical reevaluation of the original diagnosis. METHODS: This was a retrospective study of clinical records gathered over 4 consecutive years of all 15 facial pain or headache patients who underwent RFT of the SPG at a tertiary pain clinic; diagnoses were reevaluated, after which the effect of RFT on facial pain was assessed. RESULTS: After application of new criteria for Sluder's neuralgia (SN) and strict criteria for cluster headache (CH), seven patients out of the 15 turned out to have been diagnosed correctly. Nine of the 15 patients showed considerable pain relief after RFT of the SPG. Positive results were most frequent among patients with Sluder's neuropathy, atypical facial pain, and CH. However, repeated RFT procedures were needed in most patients. CONCLUSION: Correct headache and facial pain diagnosis is vital to assess the outcome of different treatment strategies. Even in a tertiary center, headache and facial pain can be misdiagnosed. RFT of the SPG may be effective in patients with facial pain, but repeated procedures are often needed.

A previously undescribed branch of the pterygopalatine ganglion.

BACKGROUND: Endonasal and infrazygomatic pterygopalatine ganglion (PPG) block for facial pain provides pain relief in a broader area than expected on anatomic grounds. The aim of this study was to search for neural structures in the pterygopalatine fossa (PPF) that could explain unexpected pain relief after PPG blockage. METHODS: The neural PPF content was explored through human cadaver study and nerve-specific staining. Five human PPF specimens were dissected as whole-mount preparations with the aid of an operation microscope and stained for acetylcholinesterase. One of these specimens was partially sectioned and analyzed through nitric oxide synthase (NOS) immunohistochemistry. RESULTS: A previously unknown nerve was identified. The nerve runs between the PPG and the ophthalmic nerve and was identified in all five specimens. NOS-containing nerve fibers were present but did not occupy the complete nerve area. CONCLUSION: Because it is likely that the nerve contains sensory fibers, our findings may provide an anatomic basis for unexplained pain relief in the ophthalmic area after PPG blockage.

Effect of adenotonsillectomy on middle ear status in children.

OBJECTIVE: To determine the effects of adenotonsillectomy as compared with watchful waiting on the middle ear status of children. STUDY DESIGN: Randomized controlled trial. METHODS: We recruited 300 children between 2 and 8 years of age who were selected for adenotonsillectomy according to current medical practice. Excluded from the trial were children with very frequent throat infections (more than 6 per year) or obstructive sleep apnea. Participants were randomly assigned to either adenotonsillectomy or watchful waiting. Main outcome measure was the percentage of children with unilateral or bilateral otitis media diagnosed at the scheduled follow-up visits according to an algorithm combining tympanometry and otoscopy. RESULTS: The percentages of children in the adenotonsillectomy and watchful waiting group diagnosed with otitis media at baseline and at 3, 6, 12, 18, and 24 months were 27.7 versus 30.5, 16.8 versus 25.2, 18.3 versus 21.2, 12.3 versus 15.2, 17.6 versus 15.5, and 14.7 versus 10.3%, respectively (P < .10). In the subgroup of children selected for adenotonsillectomy predominantly because of recurrent or persistent otitis media, hearing loss, or recurrent upper respiratory tract infections (n = 111) and in the subgroup of children diagnosed with otitis media at inclusion (n = 82), the occurrence of otitis media did not differ significantly between the adenotonsillectomy and watchful waiting group during the entire follow-up period. CONCLUSION: We conclude that in a large proportion of children selected for adenotonsillectomy according to current medical practice, including those with otitis media or related complaints, no beneficial effect of adenotonsillectomy on middle ear status is to be expected.