Clinical characteristics of oral Propranolol in the treatment of 4 patients with PHACES syndrome and literature review / 中华实用儿科临床杂志

Objective:To summarize the clinical data and imaging characteristics of patients with PHACES syndrome treated with oral Propranolol.Methods:The clinical data of 4 cases of PHACES syndrome treated with oral Propranolol in Children′s Hospital, Capital Institute of Pediatrics from October 2018 to October 2022 were retrospectively analyzed.Relevant studies reporting the treatment of PHACES with Propranolol were retrieved in PubMed, China National Knowledge Infrastructure (CNKI) and Wanfang Data.Results:Three cases of the 4 patients with PHACES syndrome treated with Propranolol obtained relieved symptoms of facial hemangioma, and 1 case died due to late treatment, complication of severe cardiovascular malformation, and treatment abandonment by parents.A total of 7 clinical studies on the use of Propranolol in the treatment of PHACES were retrieved, including 6 retrospective studies and 1 observational study.Most studies have shown that Propranolol is well tolerated in the treatment of PHACES syndrome, and most of cases have relieved facial hemangioma.The main factors affecting the prognosis are the degree of damage to middle and small arteries such at brain, aorta, chest and neck.Propranolol treatment can improve the prognosis.Conclusions:Oral Propranolol is currently the first-line treatment for PHACES syndrome, and most patients tolerate oral Propranolol well.

Compliance and withdraw reason of sublingual immunotherapy in 245 patients with allergic rhinitis / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the compliance of patients with allergic rhinitis（AR） receiving sublingual immunotherapy and its influencing factors. Methods:The clinical data of 291 AR patients who received sublingual immunotherapy for dust mites at the First Hospital of Peking University from January 2016 to January 2018 were retrospectively analyzed, and their outpatient or telephone follow-up was conducted. For patients whose treatment time was less than 2 years, the time and reason for the loss were recorded, and the factors affecting their compliance were discussed from the aspects of gender, age, and education. Results:Among the 291 patients, 245 cases（84.2%） were successfully followed up, and 193 cases（78.8%） fell off midway（treatment time<2 years）. The overall compliance rate was 21.22%（52/245）. The compliance rate of children is higher than that of adults（χ²=21.306, P<0.05）, and gender and education level have no significant effect on the compliance rate. The time period for the largest number of shedding was 6-<12 months after treatment（68 cases, 27.8%）. The main cause of shedding was symptom relief, which was considered cured（16.7%）. Secondly, within 3 months after treatment, a total of 61 patients（24.9%） fell off, of which 34 cases（13.9%） fell off because of troublesome medication, often missed medication, and simply stopped taking the drug. Statistics on the overall reasons for shedding in 193 patients, the top three shedding reasons were: cured after symptom relief（59 cases, 30.6%）, troublesome medication, discontinuation after missed dose（44 cases, 22.8%）, slow onset or ineffectiveness（26 cases, 13.5%）. Conclusion:The overall compliance of sublingual immunotherapy in patients with allergic rhinitis is poor, and the compliance of children is better than that of adults. Clinicians should focus on the reasons for patients to fall off at various times, strengthen patient education, enhance patient confidence in treatment, and improve the compliance of patients.

Tumors originated from the inferior nasal turbinates: clinical features in 34 patients / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#The purpose of this study was to analyzed the histopathologic spectrum and clinical features of the tumors originated from the inferior nasal turbinates.@*METHOD@#Clinical data of 34 patients with tumours of the inferior nasal turbinates, treated from Jan. 1998 to Dec. 2012, were retrospectively reviewed. Of the 34 patients, 18 male and 16 female, aged from 25 years to 76 years (mean: 45.6yr).@*RESULT@#(1) Pathology : Of the 34 patients, 23 (67.6%) were benign and 11 (32.4%) were malignant. Hemangiomas were the most frequent benign tumour accounting for 18/23 (78.3%) in benign tumour and for 52.9% in all the tumors originated from inferior nasal turbinates. Other benign tumours included inverted papilloma, squamous papillomas and extrapleural solitary neurofibroma tumor. The most common malignant tumour was non-Hodgkin's lymphoma accounting for 6/11 (54.5%) in malignant tumour and for 17.6% in all the tumors. Other malignant tumour included squamous cell carcinoma, melanoma and undifferentiated carcinoma. (2) Distribution: Most tumors (31/34) arose from unilateral turbinates. Hemangiomas is the commonest tumor originated from anterior part of the turbinate in 14 cases of 18 (77.8%). NHL and melanoma involved through turbinates. 3. Symtoms: Nasal obstruction was the most common symtom (25/34, 73.5%). Epistaxis and bloody nasal discharge were the chief complaint in hemangiomas.@*CONCLUSION@#1. The most common benign and malignant tumors of the inferior turbinate were hemangioma and NHL. The majority of the hemangiomas (14/18) arose from the anterior part of the inferior turbinate. NHL or melanoma often presented progressive, unilateral or bilateral diffuse enlargement of the inferior turbinate with poor vascular contractile reactivity to the ephedrine.

Surgical treatment for advanced carcinoma of hypopharynx and laryngeal function preservation / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To evaluate the treatment outcome advanced hypopharyngeal carcinoma by surgery with laryngeal function preservation.</p><p><b>METHODS</b>Twenty cases with squamous cell carcinoma of the hypopharynx who were treated by the function preserved surgery between January 2000 and March 2012 were reviewed. Of the patients 19 were males and 1 female, and their median age was 62.0 years. Eight cases were only applied with total or subtotal hypopharyngectomy, and others received total or subtotal hypopharyngectomy with partial-laryngectomy. Postoperative functional training was performed. Radiotherapy was used in all cases from 2 to 4 weeks after surgery.</p><p><b>RESULTS</b>Speech intelligibility remained in all 20 patients and 80.0% of the patients were extracted the trachea tube in the average 60 days.</p><p><b>CONCLUSIONS</b>Laryngeal functions of larynx can be preserved in advanced hypopharyngeal carcinoma by total or subtotal hypopharyngectomy alone or plus partial laryngectomy with functional reconstruction, and the patients can maintain their rational speech and swallowing functions.</p>

Extrapleural solitary fibrous tumor in nose: 2 cases report and review of literature / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To analyze the clinical characteristics, imaging features, pathological findings, treatment and prognosis of the extrapleural solitary fibrous tumor (EPSFT) in nose, and then to improve the quality of diagnosis and treatment of EPSFT.@*METHOD@#Clinical data of two patients with EPSFT were collected in recent 2 years. All the information, including clinical symptoms, imaging characteristics, blood routine, serum biochemical, treatment options, pathological immunohistochemical findings, and following-up results were reported and analyzed, and the clinical features of EPSFT in the nose were summarized with review of the literature.@*RESULT@#(1) The main clinical characteristics were nasal obstructive and epistaxis. (2)There were no imaging differences essentially between EPSFT and other tumors in nasal cavity. In terms of enhancement CT scanning, the image manifestations of EPSET were similar to that of hemangioma to a certain degree in nasal cavity. (3) Routine blood and serum biochemical test were normal in the patients with EPSFT in the nose. (4)Confirmed diagnosis mainly depended on pathological and immunohistochemical findings. (5) These two patients underwent surgical treatment, and the tumors were en bloc resected, and were in full recovery. Follow-up time is 2 years in one case and the other one is 1 year. No recurrence or metastasis were found in these two patients.@*CONCLUSION@#DSA and vascular embolism is recommended before surgical treatment in some patients. En bloc resection is the preferred treatment option with definite curative effect for the patients with EPSFT in nose, and the prognosis is favorable.

Management and classification of first branchial cleft anomalies / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#We aimed to identify the different courses of first branchial cleft anomalies and to discuss the management and classification of these anomalies.@*METHOD@#Twenty-four patients with first branchial cleft anomalies were reviewed. The courses of first branchial cleft anomalies and their corresponding managements were analyzed. Each case was classified according to Olsen's criteria and Works criteria.@*RESULT@#According to Olsen's criteria, 3 types of first branchial cleft anomalies are identified: cysts (n = 4), sinuses (n = 13), and fistulas (n = 7). The internal opening was in the external auditory meatus in 16 cases. Two fistulas were parallel to the external auditory canal and the Eustachian tube, with the internal openings on the Eustachian tube. Fourteen cases had close relations to the parotid gland and dissection of the facial nerve had to be done in the operation. Temporary weakness of the mandibular branch of facial nerve occurred in 2 cases. Salivary fistula of the parotid gland occurred in one patient, which was managed by pressure dressing for two weeks. Canal stenosis occurred in one patient, who underwent canalplasty after three months. The presence of squamous epithelium was reported in all cases, adnexal skin structures in 6 cases, and cartilage in 14 cases. The specimens of the fistula which extended to the nasopharynx were reported as tracts lined with squamous epithelium (the external part) and ciliated columnar epithelium (the internal part). According to Work's criteria, 9 cases were classified as Type I lesions, 13 cases were classified as Type II lesions, and two special cases could not be classified. The average follow-up was 83 months (ranging from 12 to 152 months). No recurrence was found.@*CONCLUSION@#First branchial cleft anomalies have high variability in the courses. If a patient is suspected to have first branchial anomalies, the external auditory canal must be examined for the internal opening. CT should be done to understand the extension of the lesion. For cases without internal openings in the external auditory canal, CT fistulography should be done to demonstrate the courses, followed by corresponding treatment. Two special cases might be classified as a new type of lesions.

Cervical necrotizing fasciitis caused by Klebsiella pneumoniae: three cases report and review of literature / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To analyze the clinical manifestations of three cases of cervical necrotizing fasciitis caused by klebsiella pneumoniae and to analyze the published articles concerning the relationship between invasive klebsiella syndrome and necrotizing fasciitis in Chinese Mainland.@*METHOD@#We have retrospectively analyzed three cases of cervical necrotizing fasciitis caused by klebsiella pneumoniae treated in our department between 2003 and 2012. We also reviewed the Chinese-language scientific literature included in the WanFang data by searching with the following key words: necrotizing fasciitis, klebsiella pneumoniae and liver abscess.@*RESULT@#These patients recovered uneventfully without obvious complications or disseminated infection foci. Sporadic cases of invasive klebsiella syndrome were reported without necrotizing fasciitis involvement in Chinese Mainland.@*CONCLUSION@#Cervical necrotizing fasciitis caused by klebsiella pneumoniae may give rise to disseminated infection but there has been no such case report in Chinese Mainland.

Selective neck dissection and the management of the hypopharyngeal cancer / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To determine the most appropriate form of selective neck dissection(SND) in the hypopharyngeal cancer with cervical lymph node metastasis.@*METHOD@#We have retrospectively analyzed the distribution and prevalence of cervical metastasis in 26 patients with hypopharyngeal squamous cell carcinoma from January 1998 to December 2008. All the patients underwent SND as part of the primary treatment. There were 34 elective SNDs and 17 therapeutic SNDs from 11 node-negative hypopharyngeal cancers and others node-positive.@*RESULT@#Occult metastasis was found in 6 patients (55%) with cervical metastasis confined to level II and III. Clinical node-positive necks were all pathologically identified with 6.7%, 66.7%, 86.7%, 46.7%, and 20.0% of the prevalence of metastasis to level I, II, III, IV and V respectively. The regional recurrences were found in 4 patients during the follow-up, which were all from cN+ patients. No patient experienced level I recurrence.@*CONCLUSION@#The results of this study suggest that SND (I-III) may be feasible for the treatment of cN0 hypopharyngeal cancer, which needs a larger sample to verify. Meanwhile, from our data, it has a satisfactory result to perform SND (II-V) with adjuvant radiotherapy for the cN+ patients.

Bacteriological analysis of persistent rhinosinusitis after endoscopic sinus surgery / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the bacterial characteristics of persistent rhinosinusitis after functional endoscopic sinus surgery (FESS).@*METHOD@#Twenty patients with nasal septum deviation, 30 patients with chronic rhinosinusitis (CRS) and 20 patients with persistent rhinosinusitis, were selected to take discharges from middle meatus during the operation. Bacteria culture and drug susceptibility of the discharges were compared between three groups.@*RESULT@#There were 13, 15 and 15 isolates detected in nasal septum deviation group, CRS group and persistent rhinosinusitis group. There was no significant difference among the three groups at the detection rate of Gram-positive bacteria. But there was significant difference between the persistent rhinosinusitis group and the other two groups at the detection rate of Gram-negative bacteria. The detection rate of antibiotic-resistant bacteria were significantly higher in persistent rhinosinusitis group than in CRS group.@*CONCLUSION@#Aerobic bacteria can live in nasal cavity. Bacteria infection is one of the etiological factors of persistent rhinosinusitis after FESS. Gram-negative bacteria and antibiotic resistant bacteria are increased in patients with persistent rhinosinusitis. To treat the persistent rhinosinusitis after surgery, the antibiotics should be reasonably used according to the bacteria culture and the drug susceptibility.

Presentation and management of allergic fungal rhinosinusitis / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To assess the presentation of allergic fungal rhinosinusitis (AFRS) and describe the line of management in our setup.@*METHOD@#Twenty-six cases of AFRS from October 2002 to June 2006 were retrospectively analyzed for the study in Department of Otorhinolaryngology Head and Neck Surgery, Peking University First Hospital. Laboratory findings (especially total serum IgE level and special serum IgE level) and computed tomography were noted preoperation. Surgical specimens were sent for mycology and histopathologic analysis. The management included endoscopic sinus debridement, adequate sinus aeration, pre- and post-operative use of steroids and saline irrigations with antifungal drugs.@*RESULT@#Nasal obstruction and hyposmia were the commonest presentations. In 21 (80.8%) of 26 patients, AFRS was fund to be associated with allergic diseases. CT scan showed serpiginous or patchy increased attenuation within the completely opacified sinuses on reconstructed soft tissue window. Some of the involved sinuses had bone erosion and expansion. 84.6% (22/26) patients had elevated total IgE levels, 76.9% (20/26) had positive for sIgE levels to fungal allergen. Fungal smear were positive for all 26 patients. But only 14 surgical specimens were positive for fungal cultures. The most common causative agent was Aspergillus. The involved mucosa and allergic mucin with H & E staining contained clusters or sheets of degenerating eosinophils. Charcot-Leyden crystals was found in 8 surgical specimens. The follow-up after a year, the mucosa recovered epithelization in 19 patients.@*CONCLUSION@#The diagnosis of AFRS required to depend on history, CT scanning, histopathology, mycologic and immunologic monitoring. Comprehensive treatment with endoscopic sinus surgery, steroids and saline irrigations with antifungal drugs is effective method.

Cervical necrotising fasciitis--report of two cases and review of the literature / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To explore the causes, clinical features, diagnosis and treatment of cervical extensive necrotizing fasciitis, a rare clinical occurrence, and to improve the clinical recognition and appreciation of it.@*METHOD@#Two cases of cervical extensive necrotizing fasciitis were studied and relevant literatures were reviewed. The causes, clinical manifestation, experience of diagnosis and treatment were summarized.@*RESULT@#One of two cases was secondary to foreign body of hypopharynx, and the other with unknown cause. Apathy, crepitation and diffuse swelling and rubor following with abscess formation on the neck are main characteristics. Mixed synergistic infection was confirmed by drainage culturing. All two cases were treated actively by large dosage and effective broad spectrum antibiotics, and sustaining therapy and surgical treatment, including local incision and drainage, aggressive surgical debridement and tracheotomy.@*CONCLUSION@#Cervical extensive necrotizing fasciitis is a potentially life-threatening soft tissue infection. The keys of successful treatment were early diagnosis and surgical intervention. Rational antibiotics application and systemic supporting therapeutics were also recommended.

The Second China National Sample Survey on Disability:Standard of Speech Disability / 中国康复理论与实践

@#The standard of speech disability used in Chinese Second Disability Sampling Survey is introduced in the paper,including classification,screening,survey instrument,diagnose,cause analysis and rehabilitation advice.

Comparison of Two Kinds of Internal Fixation after High Tibia Osteotomy for the Treatment of Osteoarthritis of Knee with Genu Varus Deformity / 中国康复理论与实践

@#ObjectiveTo compare the effect and prognosis of two kinds of internal fixation (improved Giebel blade plate and traditional straddle nail) after high tibial osteotomy (HTO) on osteoarthritis of knee with genu varus deformity. Methods37 knees of 32 cases were treated with straddle nail (25 knees) or Giebel blade plate (12 knees). All the cases were followed up for 6～28 months. ResultsThe clinical bone healing time of osteotomy was 8～12 weeks. There was no significant differences between 2 groups in the increased score in HSS Standard and in the clinical bone healing time. ConclusionBoth internal fixation with improved Giebel blade plate and traditional straddle nail get similarly satisfactory prognosis, while the former shows more advantages to allow early functional exercises.

Mucosal malignant melanoma of the nasal cavity and paranasal sinuses: two case reports of nonspecific clinical features / 北京大学学报(医学版)

SUMMARY Mucosal malignant melanoma of the nasal cavity and paranasal sinuses are rare. Because symptoms and radiological examinations of this disease are generally nonspecific, the diagnosis is often difficult. Diagnosis can be confirmed by the immunohistochemical profile of staining with HMB-45 and S-100. This study deals with two cases of nonspecific clinical features. The factors of misdiagnosis were discussed and the lesson was summarized.