Chiari type I malformation

To highlight an unusual cause of chronic cough, we present two cases of chronic cough in older children referred to a pediatric otolaryngology outpatient clinic with persistent symptoms despite extensive previous workup and treatment. Cranial neuropathy was identified in each case. As a result, magnetic resonance imaging was performed revealing Chiari type I malformation. In each case, surgical decompression provided symptom improvement. Chronic cough is a rare presenting symptom in children with Chiari type I malformation. We emphasize the significance of awareness for unusual cases of cough to aid in the correct identification and treatment in children. Laryngoscope, 123:2888–2891, 2013     

Chiari Type I malformation and upper airway obstruction in adolescents

Chiari Type I malformation is characterized by cerebellar tonsil herniation through the foramen magnum. Manifestations may include new-onset upper airway obstruction. We describe two patients who had bilateral vocal cord paralysis and Chiari Type I malformation. These cases highlight the importance of suspecting a neurologic abnormality such as Chiari malformation in new-onset upper airway obstruction and/or bilateral vocal cord paralysis in adolescents in the absence of an obstructive lesion, though the development of cranial neuropathies or radiographic evidence may be delayed. Establishing a proper diagnosis is critical as Chiari I malformation can be treated with neurosurgical decompression, with possible improvement.     

Sensorineural hearing impairment in children with Chiari I malformation

OBJECTIVES: We describe the prevalance of Chiari I malformation in children presenting to a pediatric tertiary care hearing loss clinic, characterize the phenotype of hearing loss in children with Chiari I malformation, and discuss the potential pathophysiology of sensorineural hearing impairment (SNHI) related to Chiari I malformation. METHODS: This study was a retrospective case series of patients seen in a pediatric tertiary care hearing loss clinic affiliated with an academic medical center. We considered 481 patients with SNHI who had radiographic imaging as part of the evaluation for the cause of their hearing impairment. Hearing impairment was determined by behavioral audiogram and/or auditory brain stem response testing. Radiographic imaging was performed with computed tomography and/or magnetic resonance imaging. RESULTS: Six children with SNHI also had a diagnosis of Chiari I malformation. Four of these 6 children had unilateral hearing impairment. One child had asymmetric bilateral impairment; 1 child had symmetric bilateral impairment. The severity of hearing impairment varied from mild to profound. All children were referred to the neurosurgery department. None underwent surgical decompression. CONCLUSIONS: Chiari I malformation may be associated with SNHI. Central nervous system imaging for the evaluation of children with SNHI may be indicated, particularly in individuals with negative results on temporal bone computed tomography and genetic testing.     

Adult chiari malformation presenting as bilateral vocal cord paralysis

In the Chiari malformations, herniation of the cerebellar tonsils through the foramen magnum into the cervical spinal canal can often result in headaches and spinal cord deficits. Bilateral vocal cord paralysis has been reported as a result of Chiari malformation but is rare and the vast majority of reports have been in the paediatric population. We report a case in a 68-year-old man with adult Chiari malformation where aspiration pneumonia from bilateral vocal cord palsy was the only manifestation of this malformation.     

Abductor vocal fold palsy as a manifestation of type one Arnold Chiari malformation

We report a rare case of type 1 Chiari malformation which presented to the Otolaryngology department with abductor vocal fold palsy following an aspiration pneumonia secondary to absent laryngeal sensation. Chiari malformations and related cases are discussed.     

Type I Chiari malformation as a cause of inferior vertical nystagmus

Down beat nystagmus is a central nystagmus. In the literature reviewed the two most common causes that can produce it are cerebellar degenerations and Chiari malformation. The site of lesion causing this nystagmus appears to be the brainstem or the cerebellum, although the definitive etiopathogeny remains controversial. We report the case of a 73-year-old woman with sudden unsteadiness of gait and down beating nystagmus, without any other vestibular or neurologic signs. Simple radiology and MRI evidenciated a Chiari malformation type 1 associated to plastybasia.     

睡眠呼吸障碍为首发表现的Chiari畸形幼儿1例报道并文献复习

目的 探讨中枢性睡眠呼吸暂停的特点及其引起疾病的临床特征,提高Chiari畸形合并中枢性睡眠呼吸暂停的认识.方法 回顾性分析1例以睡眠呼吸障碍为首发表现的Chiari畸形患儿的临床资料,结合文献复习探讨Chiari畸形合并中枢性睡眠呼吸暂停(CSA)的特征.结果 患儿通过影像学及多导睡眠监测证实符合Chiari畸形Ⅰ型...     

Type I Chiari malformation presenting central sleep apnea

Sleep apnea is a rare but a well-known clinical feature of type I Chiari malformation. It may be obstructive or central in nature. Sleep apnea in patients with type I Chiari malformation rarely presents without accompanying neurological signs or symptoms. We here report a case of a 10-year-old girl who presented with central sleep apnea without any other neurological signs but was ultimately diagnosed with type I Chiari malformation. The patient initially showed mild improvement in symptoms after administration of an acetazolamide. Finally, posterior fossa decompression dramatically improved her respiratory status during sleep, both clinically and on polysomnography. This case suggests that type I Chiari malformation should be considered in the differential diagnoses of central apneas in children, even if there are no other neurological signs and symptoms. Furthermore, sagittal craniocervical magnetic resonance imaging may be necessary for a definitive diagnosis.     

Clinical characteristics of aero-digestive foreign bodies in neurologically impaired children

OBJECTIVE: To determine if children with neurological impairment are at additional risk for foreign body aspiration or impaction and if they present with a different clinical course than normal children. DESIGN: Retrospective cohort study. SETTING: Tertiary care children's hospital. PATIENTS AND OTHER PARTICIPANTS: A retrospective chart review of 328 consecutive procedures for aero-digestive foreign body removal over a 6 year period was performed. Of these patients 52 (15.9%) were identified with neurological impairment. RESULTS: Compared with the non-impaired children, those with neurological impairment were older, diagnosed later, hospitalized longer and had a higher incidence of complications. CONCLUSION: A greater index of suspicion is needed to timely treat aero-digestive foreign bodies in children with neurological impairment. These children may not be receiving the necessary attention when presenting with non-specific symptoms.     

前后路联合治疗颅底凹陷合并小脑扁桃体下疝患者的临床疗效及预后分析

目的分析前后路联合治疗颅底凹陷合并小脑扁桃体下疝（Chiari）畸形患者的临床疗效及预后。方法选取2009年2月~2016年2月诊治的12例颅底凹陷症合并Chiari畸形患者。分析患者术后的临床疗效、影像参数变化及预后。结果所有患者术后随访7 d至20个月,患者的临床症状、体征均有明显的改善;术后的JOA评分（14.14±2.04）分明显高于术前的JOA评分（6.65±1.23）分（P<0.05）;12例患者术后CL值、ML值、WL值、Klaus高度指数、延颈髓角及颅底角参数较术前明显改善（P<0.05）;术后并发症少。结论经前后路联合治疗颅底凹陷合并小脑扁桃体下疝患者的临床效果较好,对脊髓功能改善明显,并发症发生率少,短期预后良好。     

Management of a giant lymphatic malformation of the tongue

Lymphatic malformations can occur anywhere in the head and neck, and when they do so in the oral cavity, they can present a potential hazard to the airway. We describe a 4-year-old girl with a giant lymphatic malformation of the tongue and neck. This report illustrates the particular difficulties that may be encountered in cases involving giant lymphatic malformations of the cervicofacial region in the pediatric population. An overview of the etiology, diagnosis, histology, and options for the management of such lesions is outlined.     

An unusual respiratory tract foreign body: A case report of pediatric broncholithiasis

Tracheobronchial foreign body aspiration is one of the most life-threatening diseases in children. Its symptom is non-specific sometimes; therefore the distinction with some similar clinical presentation pulmonary disease should be performed. Here, we reported a 5-year-old girl who was misdiagnosed with foreign body aspiration twice based on the broncho-obstructive symptom and radiologic finding. Finally, the diagnosis of broncholithiasis was confirmed by finding broncholiths in the respiratory tract through bronchoscopy. It is replied that bronchoscopy should be performed in the children with unexplained airway obstruction symptom. Furthermore, it is the first report about the broncholiths located at the upper main trachea.     

Respiratory obstruction as a sign of brainstem dysfunction in infants with Chiari malformations

Laryngeal respiratory obstruction associated with Chiari malformations was first described in 1932. We studied this type of obstruction in six children with one or several disorders pointing to brainstem dysfunction (failure to thrive, velopharyngeal incompetence, gastroesophageal reflux, or vagal hypertonia). The nature of the laryngeal obstruction was highly variable (vocal cord paralysis, paradoxical vocal cord motion, laryngomalacia) as were the frequency and severity of associated disorders. Chiari malformations should be routinely sought in a child with laryngeal respiratory obstruction occurring at birth or later, whatever the endoscopic diagnosis, especially when signs of brainstem dysfunction are present. The best tool for diagnosing the Chiari malformation is T1- and T2-weighted MRI. Signs of brainstem dysfunction must be treated symptomatically, before treating Chiari malformations by decompressive surgery. This latter approach led to full functional recovery in all five children who underwent the procedure. Palliative surgical treatment should be reserved for patients in whom this procedure is unsuccessful.     

慢性严重误吸的手术治疗

目的：探讨不同病因所致慢性严重误吸患者的最佳手术治疗方式。方法：对8例慢性严重误吸患者分别采用3种手术方式治疗；①气管切开术(5例)、颈气管永久性造瘘术(1例)，气管套管佩带气囊，进食时气囊充气以阻止误吸；②会厌瓣缝合术(即喉口闭锁术，1例)；③胃永久性造瘘术(1例)。结果：8例患者均能经口进食，食物的性状种类不受限制，且未发现吸入性肺炎。其中5例带气囊者仅在进食后放气时，略有轻微刺激性咳嗽，1例喉癌行Arslan术式者，仍有较轻误吸现象，以上6例患者堵管时均能发声，可日常交谈；1例采用会厌瓣缝合术患者，术后经口进食已完全杜绝误吸现象；1例行胃永久性造瘘术者，能较好解决进食误吸，无胃反流现象。结论：会厌瓣缝合术(喉口闭锁术)、气管切开术(或颈气管永久性造瘘术)和胃永久性造瘘术3种术式，均是解决慢性误吸的外科治疗方法，可依不同的病因及患者的具体情况选择最佳术式。     

Neuro-otological manifestations as presentacion of type I Chiari malformation

The type I Chiari malformation consists of an caudal descent of the cerebellar tonsils through the foramen magno towards the spinal cervical channel. The usual clinical presentation are occipital headaches and cervical pain, as well as some otoneurological symptoms. Among them are common dizziness and crisis of central positional vertigo in which down beating nystagmus can be observed. We present our experience with three cases presenting with neurotological manifestations in which MRI showed a type I Chiari malformation. Case 1: a patient of 24 years that had frequent instability, and common crisis ofpositional vertigo. It was possible to see the presence of positional down-beating nystagmus, of central characteristics, that improved after neurosurgical treatment. Case 2: patient of 11 years who suffered from occasional headaches, dizziness and positional vertigo, without severe handicap, and did not need surgery. Case 3: lady of 63 years who had common episodes of vertigo with head tilt, and unsteadiness during walks. Due to the coexistence of hydrocephalus and syringomyelia, surgery was indicated. A revision is done on otoneurological presentation of type I Chiari malformation, especially as differential diagnosis on central positional vertigo.     

Sympathetic neuropathy and dysphagia following doxycycline sclerotherapy

This case report demonstrates neurologic sequela following treatment with doxycycline sclerotherapy. A six-week-old child presented with respiratory distress from a macrocystic lymphatic malformation, extending from the skull base to the anterior mediastinum. Following doxycycline sclerotherapy, the airway symptoms resolved; however, the child developed silent aspiration and Horner's syndrome. Two months following treatment the patient resumed oral diet and at one year post-intervention there has been no recurrence of symptoms, with only mild ptosis remaining.     

Extraluminal arytenoid reconstruction: laryngeal framework surgery applied to a pediatric problem

Laryngeal framework surgery has proved to be an important alternative in the treatment of vocal cord paralysis in adults. We have modified one of these operations in order to manage debilitating aspiration in a 14-year-old boy. His laryngeal incompetence resulted from a severe neurologic injury and laryngeal scarring from a unilateral arytenoidectomy and multiple Teflon injections. To help him, we studied a series of pediatric larynges and devised an operation that succeeded in rebuilding the bulk of his excised arytenoid cartilage and markedly reducing aspiration.     

Hiccups. A case presentation and etiologic review.

Hiccups (singultus) usually present as a common annoyance lasting for short periods. Rarely, they may be the harbinger of a serious disease. We present the case of a 19-year-old man in which intractable hiccups was the first and most prominent symptom of a serious underlying neurologic disorder. The patient had been examined by his pediatrician, and despite multiple medical regiments and physical maneuvers, his symptoms persisted. A thorough head and neck examination revealed a right-sided vocal cord paralysis. This finding prompted obtaining a magnetic resonance imaging scan, which demonstrated a type I Arnold-Chiari malformation associated with a large cervicothoracic syringomyelia. The patient was referred to the neurosurgical service and subsequently underwent a ventriculoperitoneal shunt placement. There was considerable initial improvement in his neurologic status and cessation of the hiccups. However, the symptoms recurred within 1 month. The case report as well as a brief review of the relevant pathophysiologic and etiologic considerations and several treatment modalities for hiccups is presented.     

Pediatric type I thyroplasty: an evolving procedure

The treatment of vocal fold paralysis by type I thyroplasty in the pediatric age group has not been reported. From 1990 to 1998, 12 type I thyroplasty procedures were performed on 8 patients between 2 and 17 years of age. The most common cause of vocal fold paralysis was neurologic, followed by vagal injury from a cardiac procedure. The most common indications for the procedure were aspiration and dysphonia. In our early thyroplasty experience, adult techniques and measurements adapted after Isshiki or Netterville were used. Postoperative laryngoscopy showed that in most cases, the placement of the implant was too high. There were variable outcomes in aspiration and dysphonia with this technique. These findings appear to be independent of thyroplasty approach or of implant design type. We conclude that the standard approach for vocal fold medialization in the adult cannot be applied accurately in the pediatric population. In performing pediatric thyroplasty, the anatomically lower position of the vocal fold must be taken into consideration. We have since modified our technique to adjust for accurate identification of the vocal fold line and medialization. The modified approach for vocal fold medialization in the pediatric population is discussed.     

颅底陷入症合并小脑扁桃体下疝畸形的联合手术治疗(附4例报告)

目的探讨颅底陷入症(BasilarinvaginationBI)合并小脑扁桃体下疝畸形(Chiarimalformation)的手术治疗方法。方法对4例颅底陷入症合并小脑扁桃体下疝畸形病人经CT、MRI检查并测量Klaus高度指数、齿状突尖到桥延交界处的距离、小脑扁桃体下疝深度,所有病人均首先行后路减压、枕颈钛板固定、植骨融合,然后再行经口咽齿突磨除术。结果所有病人均于术后短期内症状缓解,术后半年4名病人均能生活自理;术后1年2名病人已能参与一般的体育活动,另2名病人也能进行较轻的劳动。结论对颅底陷入症合并小脑扁桃体下疝畸形的病人先行后路减压加固定、植骨融合,再行前路经口咽入路磨除齿突的联合手术,是一种有效的治疗方法,值得进一步应用研究。