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Acute epiglottitis in the adult: experience with a seasonal presentation.

Acute epiglottitis in adults may follow an unpredictable clinical course, complicated by acute airway obstruction. Our experience with this disease was evaluated by looking at yearly incidence, seasonal occurrence, and clinical variables of both intubated and conservatively managed patients. In our series of 17 cases from 1987 through 1990, 16 occurred in 1988 and 1989. Ten of 17 cases occurred during the summer months. The two patients who developed stridor went on to require intubation and tracheotomy. Statistical analysis of our data revealed that, in adult acute epiglottitis, stridor is a strong predictor of airway obstruction. A significant summer seasonal predominance was seen, as well as a suggestion of an epidemic in 1988 and 1989.

Acute Disease↗

Laser laryngoplasty for laryngomalacia.

Laryngomalacia is the most common cause of stridor in infants. In severe cases, it results in obstructive sleep apnea, cor pulmonale, gastroesophageal reflux, failure to thrive, and pectus excavatum. Our experience with 24 children who underwent laser excision of redundant supraglottic tissue from May 1987 to July 1991 is reviewed. The technique emphasizes preoperative planning with flexible fiberoptic laryngoscopy, apneic anesthetic for the procedure, conservative resection of tissue and the need for complete airway evaluation. In our series, 71% of patients experienced complete resolution of symptoms. Nine of 10 postoperative sleep studies indicated improvement. In 4 patients, collapse developed later in other areas of the supraglottis, resulting in recurrence of stridor several months after the initial procedure, and requiring revision laryngoplasty. In several patients with other significant sites of upper airway obstruction (e.g., tonsils and adenoids, and a tongue base cyst), laryngomalacia became more significant following alleviation of the primary source of airway obstruction. Twelve patients (50%) required additional airway procedures either before or following initial laryngoplasty. Patient selection, sleep study data, and complications are reviewed.

Child, Preschool↗

Functional laryngeal obstruction relieved by panting.

A 49-year-old man presented with a two-day history of severe recurrent dyspnea and inspiratory stridor. A chest roentgenogram, computed tomographic scan of the neck, direct laryngoscopy, and bronchoscopy excluded organic upper airway obstruction. Laryngospasm occurred during the bronchoscopy. Although flow volume loops revealed severe upper airway obstruction (inspiratory and expiratory), airway resistance measured plethysmographically (during panting) was normal. Because of this observation, panting was recommended for relief of the patient's recurrent attacks of functional laryngeal obstruction. The panting maneuver immediately and completely relieved all 25 to 30 subsequent attacks. After the patient recovered clinically, a flow volume loop was repeated and was found to be normal. The marked discrepancy between severe flow limitation (as detected by flow volume loops) and normal airway resistance (measured plethysmographically) may be a diagnostic test for functional laryngeal obstruction, and panting may be an effective emergency measure for its relief. Relief by panting may also suggest the diagnosis. A second patient with an almost identical symptom complex is described, in whom the panting maneuver was also dramatically successful in promptly aborting recurrent severe attacks of airway obstruction and stridor.

Airway Obstruction↗

Comparison of three different methods used to achieve local anesthesia for fiberoptic bronchoscopy.

A patient's tolerance of fiberoptic bronchoscopy depends on the effectiveness of local anesthesia. This study compares the three different methods of local anesthesia in common use After sedation, patients (n = 53) received either 4 ml of 2.5 percent cocaine by intratracheal injection (TI) (n = 18), by bronchoscopic injection (BI) (n = 19), or had 4 ml of 4 percent lidocaine delivered by nebulizer 20 min before the procedure (NEB) (n = 16). Patients and bronchoscopists scored the procedure using visual analog (VAS) and severity scales. Objective measurements of cough counts and episodes of stridor were recorded by phonopneumography. Patients' VAS scores showed a clear preference for the transtracheal method compared with either bronchoscopically injected cocaine (p less than 0.001) or nebulized lidocaine (p less than 0.001). Patients also reported that the TI method produced less cough during intubation of the larynx and inspection of the airways (BI and NEB, p less than 0.01). The TI method was also preferred by the bronchoscopists (BI and NEB, p less than 0.001); they reported less cough and easier tracheal intubation. The mean cough count was significantly lower for the TI group, 49 (43) compared with 95 (52) for BI (p less than 0.01), and 81 (43) for the NEB group (p less than 0.05). Patients' and bronchoscopists' VAS showed significant correlation with cough (r = 0.63-69, p less than 0.01). Stridor occurred in only two patients after TI, compared with 15 in the other two groups. Extra local anesthesia was required by 16 patients after BI, by all the NEB group, but by only one patient after TI. Subjective and objective measurement shows that 4 ml of 2.5 percent cocaine injected into the trachea produced excellent local anesthesia for fiberoptic bronchoscopy, there were no extra complications, and it was the method preferred by both patients and bronchoscopists.

Aerosols↗

Abnormal movement of the arytenoid region during exercise presenting as exercise-induced asthma in an adolescent athlete.

A 16-year-old female basketball player presented with a 2 1/2-year history of exercise-induced severe dyspnea, stridor, and mild wheezing that did not respond to prophylactic treatment with beta-agonists and cromolyn. Spirometric data at rest were normal, but flow-volume loops during exercise suggested a variable extrathoracic obstruction. Laryngoscopic evaluation while the patient was riding an exercise bicycle demonstrated an abnormal motion of the arytenoid region causing obstruction of the airway during inspiration. The vocal cords moved normally. This patient demonstrates the capacity of supraglottic tissue to obstruct the airway during exercise as a cause for exercise-induced dyspnea and stridor. Patients with this disorder may be misdiagnosed as having exercise-induced asthma.

Adolescent↗

Intratracheal ectopic thyroid tissue mass.

A diagnosis of severe stridor due to a subglottic tracheal mass was made in a 62-year-old woman. The tumor was removed by external transtracheal surgery, and the pathologic study disclosed that it was intratracheal ectopic thyroid tissue. In this particular case, 10 years prior to the onset of the stridor, normal thyroid tissue was seen in biopsy specimens of the same subglottic localization. Although the pathogenesis of a mass composed of intratracheal thyroid tissue is not known, this case shows that it can be a slowly progressive tumor that is asymptomatic for several years.

Choristoma↗

Evaluation of a new self-expandable silicone stent in an experimental tracheal stenosis.

STUDY OBJECTIVES: To assess the usefulness of an animal model for testing new tracheobronchial stents. SETTING: Animal laboratory of a university hospital. ANIMALS AND INTERVENTIONS: In a series with 12 mini-pigs, we induced a stable fibromalacic tracheal stenosis that was 50% to 70% of the normal tracheal diameter. After dilation we inserted a 16 x 40-mm self-expandable silicone stent into the stenotic part of the trachea in 10 of the mini-pigs. Five of the stents had a smooth outer surface, and five had additional silicone retaining spikes. Because of a long stenosis in two of the mini-pigs, two overlapping stents (one smooth and one with spikes) were inserted. MEASUREMENTS AND RESULTS: Stent deployment was successful and resulted in the disappearance of the slight to moderate stridor in all of the mini-pigs. Over a mean (+/- SD) observation period of 24 days (range, 10 to 41 days), all of the mini-pigs redeveloped stridor. Three of them died unexpectedly of suffocation: in all three a smooth stent had migrated, leading to total obstruction of the stenosis. In total, five of the six smooth stents migrated, and only one of the six spiked stents migrated. There was considerable granulation tissue formation at the ends of all of the stents. In the two control mini-pigs, a 12 x 35-mm Dumon stent was inserted. Both Dumon stents migrated, and one of them had considerable granuloma formation at its ends. At the end of the observation period, all stents were removed endoscopically and were found not to have deteriorated over time. CONCLUSIONS: Our model proved to be suitable for the evaluation of the technical aspects of the Polyflex stent. Spikes on the outer stent surface are more effective in preventing migration than smooth-surface stents. Long-term compatibility, however, seems to be difficult to test with our model because both the Polyflex and the Dumon stents had excessive granulation tissue formation at both ends, a factor which--in the case of the Dumon stent--does not occur to such a degree in benign human airway stenoses. Our results indicate a need for prospective long-term studies in benign human airway stenoses.

Animals↗

Extrathoracic obstruction and hypoxemia occurring during exercise in a competitive female cyclist.

A 22-year-old competitive female cyclist complained of cough, chest tightness, and wheeze during high-intensity exercise that had previously been diagnosed as exercise-induced bronchospasm (EIB). A loud stridor, a sensation of her "throat closing," and severe dyspnea developed during maximal cycling exercise with concomitant reductions in both inspiratory and expiratory flow rates. A decrease of 25 L/min (26%) in minute ventilation and arterial hypoxemia (PaO(2) decrease, 93 to 76.5 mm Hg) resulted from this obstruction. Spontaneous tidal flow-volume loops (FVLs) during exercise exhibited a sawtooth pattern during inspiration, and substantial drops in flow rates after the stridor developed. However, maximal FVLs were unchanged from baseline following exercise, indicating that the obstruction was not EIB. We suggest that the continuous measurement of spontaneous breath-by-breath tidal FVLs may be a useful diagnostic tool for the identification of exercise-induced extrathoracic obstruction. Additionally, extrathoracic obstruction should be considered as an uncommon but potential cause of inadequate ventilation and arterial hypoxemia during exercise.

Adult↗

Use of nasopharyngoscopy in the evaluation of children with noisy breathing.

STUDY OBJECTIVE: To evaluate the practice of using nasopharyngoscopy without routine fiberoptic bronchoscopy for children presenting to a pediatric pulmonary practice with nonspecific noisy breathing. DESIGN: Retrospective chart review. Records of patients who underwent nasopharyngoscopy between January 1, 1990, and December 31, 1999, were reviewed. Follow-up was obtained by office records and direct contact with the patient's family and/or primary care physician. SETTING: Academic, tertiary care facility. RESULTS: Eighty-one children who underwent upper airway endoscopy to evaluate noisy breathing consistent with extrathoracic lesions were identified. One child had two evaluations separated by years for differing complaints, making a total of 82 procedures. Stridor was the chief complaint in three fourths of the children. Half of the children with stridor were found to have laryngomalacia. Long-term follow-up was available for 75 of 81 children, with median follow-up of 6 years (range, 1 to 13 years). No medical problems related to missed airway lesions developed in any infants initially evaluated using nasopharyngoscopy. CONCLUSIONS: Nasopharyngoscopy without lower airway endoscopy can be used safely for the initial evaluation of noisy breathing in infants and children provided excellent follow-up is available.

Adolescent↗

Transalar sphenoidal encephalocele and respiratory distress in a neonate: a case report.

We present a full-term newborn infant who suffered from immediate postpartum severe respiratory distress. The infant had an inspiratory stridor as a result of a swelling of the soft palate, extending from the roof of the nasopharynx. Transoral endotracheal intubation resulted in normal saturation levels. Histologic examination after an open biopsy showed mature neuroglial tissue. Radiology demonstrated the presence of a right parapharyngeal process obstructing the nasopharynx and oropharynx and extending to the right middle and posterior fossa, via the foramen ovale. After transoral debulking, the infant was extubated successfully. After an uneventful period of 5 months, the patient was readmitted at our hospital for treatment of meningitis. Subsequently, the inspiratory stridor recurred, and staged surgery was performed. First, a transcranial approach was used to remove a large intradural part of the process and close the defect at Meckel's cave. Two weeks later the retro- and parapharyngeal part of the process were removed transorally. Given the site of the defect of the skull base and the intradural location of the process, the diagnosis is a transalar sphenoidal encephalocele. This is a rare type of basal encephalocele, and has never been reported in an infant nor known to present with respiratory distress. The pathogenesis, clinical presentation, pathology, and therapeutic implications of basal encephaloceles are discussed.

Encephalocele↗

Vocal cord paralysis secondary to impacted esophageal foreign bodies in young children.

Impacted foreign bodies in the esophagus can result in respiratory symptoms including stridor and aphonia. Several mechanisms have been proposed to explain these symptoms, but the possibility of vocal cord paralysis and its cause has not been adequately emphasized. Two cases of young children with esophageal foreign body are described; both presented with respiratory symptoms, 1 with aphonia and the other with stridor. In both cases, the symptoms were secondary to vocal cord paralysis. A possible mechanism of recurrent nerve injury is proposed.

Aphonia↗

Retropharyngeal aberrant thymus.

INTRODUCTION: Upper airway obstruction from a retropharyngeal mass requires urgent evaluation. In children, the differential diagnosis includes infection, trauma, neoplasm, and congenital abnormalities. Aberrant cervical thymic tissue, although occasionally observed on autopsy examination, is rarely clinically significant. We present the case of an infant with respiratory distress attributed to aberrant thymic tissue located in the retropharyngeal space. CASE: A 6-week-old infant was brought to the emergency department for evaluation of stridor associated with periodic episodes of cyanosis. Lateral neck radiograph revealed widening of the retropharyngeal soft tissues. The patient's symptoms did not improve with intravenous ampicillin-sulbactam. Magnetic resonance imaging (MRI) performed on the seventh day of hospitalization revealed a retropharyngeal mass that extended to the carotid space. The mass was easily resected using an intraoral approach. Microscopic examination demonstrated thymic tissue. A normal thymus was also observed in the anterior mediastinum on MRI. The patient recovered uneventfully and had no further episodes of stridor or cyanosis. DISCUSSION: Aberrant cervical thymic tissue may be cystic or solid. Cystic cervical thymus is more common, and 6% of these patients present with symptoms of dyspnea or dysphagia. Aberrant solid cervical thymus usually presents as an asymptomatic anterior neck mass. This case is unusual in that solid thymic tissue was located in the retropharynx, a finding not previously reported in the English literature. Additionally, the patient presented in acute respiratory distress, and the diagnosis was confounded by the presence of mild laryngomalacia. In retrospect, our patient likely had symptoms of intermittent upper airway obstruction since birth. The acute respiratory distress at presentation was likely the result of laryngomalacia exacerbated by the presence of aberrant thymic tissue and a superimposed viral infection. Aberrantly located thymic tissue arises as a consequence of migrational defects during thymic embryogenesis. The thymus is a paired organ derived from the third and, to a lesser extent, fourth pharyngeal pouches. After its appearance during the sixth week of fetal life, it descends to a final position in the anterior mediastinum, adjacent to the parietal pericardium. Aberrant thymic tissue results when this tissue breaks free from the thymus as it migrates caudally. Therefore, aberrant thymic tissue may be found in any position along a line from the angle of the mandible to the sternal notch, and in the anterior mediastinum to the level of the diaphragm. In an autopsy study of 3236 children, abnormally positioned thymic tissue was found in 34 cases (1%). The aberrant thymus was most often located near the thyroid gland (n = 19 cases) but was also detected lower in the anterior neck (n = 6 cases), higher in the anterior neck (n = 8 cases), and at the left base of the skull (n = 1 case). The presence of thymic tissue in the retropharyngeal space in our patient is more unusual given the typical embryologic origin and descent of the thymus in the anterior neck to the mediastinum. Children with aberrant thymus may have associated anomalies. Twenty-four of 34 children (71%) with aberrant thymus detected at autopsy had features consistent with DiGeorge syndrome, and only 5 of the remaining 10 patients had a normal mediastinal thymus present. Our patient had normal serum calcium levels after excision and a mediastinal thymus was visualized on MRI. Biospy is required for diagnosis of cervical thymus and should also be considered to exclude other causes. MRI is helpful in delineating the presence, position, and extent of thymic tissue. Immunologic sequelae or recurrence after resection of an aberrant cervical thymus has not been reported.

Airway Obstruction↗

Recurrent respiratory papillomatosis in children: masquerader of common respiratory diseases.

BACKGROUND: Recurrent respiratory papillomatosis in children is an uncommon but potentially life-threatening benign tumor of the respiratory tract with laryngeal predilection. The diagnosis of recurrent respiratory papillomatosis may be challenging unless there is a high index of suspicion and awareness of the variable presentations. METHODS: We reviewed the medical charts of children with recurrent respiratory papillomatosis treated at a tertiary children's hospital. The presentation of recurrent respiratory papillomatosis is illustrated by a series of case reports. We provide a paradigm to assist in the early diagnosis of children with recurrent respiratory papillomatosis. RESULTS: Five patients, aged 2 to 6 years, were erroneously diagnosed with recurrent croup, asthma, laryngeal hemangioma, and tracheomalacia after presenting with variable degrees of chronic dyspnea, cough, stridor, dysphonia, weak cry, and syncope. Once the diagnosis of recurrent respiratory papillomatosis was made, recurring surgical ablation of papillomata was initiated. CONCLUSIONS: Any child presenting with a voice disturbance with or without stridor is recommended to have diagnostic flexible fiber-optic laryngoscopy. Recurrent respiratory papillomatosis should be considered in children when other common pediatric airway diseases either do not follow the natural history or do not respond to treatment of the common disorder.

Child↗

Acoustical studies on respiratory sounds in asthmatic patients.

Changes in the breathing sounds such as stridor and dry and wet rales in asthmatic patients were studied. The breathing sounds were recorded with the small transistor warp type microphone inserted through the nasal orifice into the trachea, main bronchi and segmental bronchi, and were analyzed with sound analyzer. The results were as follows: The whistling rales were caused by the resonance of the bronchial wall, when the vibrating frequency was coincident with its specific frequency. Snoring rales were explained as the sound caused by the air flow passing promptly through contracted bronchi with little secretion. Resonance of either bronchial wall or highly viscous sputum was considered to be responsible for the sonorous rales. Sibilant rales were audible when bronchi promptly repeated dilation and contraction. Stridor was picked up at the upper part of the air tract, from the main bronchus to the trachea, and noise was the main component on the sonogram, presumably caused by the air turbulence due to the narrowing and deformity of bronchial tube or to the sputum adhering to the inner surface of the air tract.

Adolescent↗

Hydrogen peroxide poisoning.

Hydrogen peroxide is an oxidising agent that is used in a number of household products, including general-purpose disinfectants, chlorine-free bleaches, fabric stain removers, contact lens disinfectants and hair dyes, and it is a component of some tooth whitening products. In industry, the principal use of hydrogen peroxide is as a bleaching agent in the manufacture of paper and pulp. Hydrogen peroxide has been employed medicinally for wound irrigation and for the sterilisation of ophthalmic and endoscopic instruments. Hydrogen peroxide causes toxicity via three main mechanisms: corrosive damage, oxygen gas formation and lipid peroxidation. Concentrated hydrogen peroxide is caustic and exposure may result in local tissue damage. Ingestion of concentrated (>35%) hydrogen peroxide can also result in the generation of substantial volumes of oxygen. Where the amount of oxygen evolved exceeds its maximum solubility in blood, venous or arterial gas embolism may occur. The mechanism of CNS damage is thought to be arterial gas embolisation with subsequent brain infarction. Rapid generation of oxygen in closed body cavities can also cause mechanical distension and there is potential for the rupture of the hollow viscus secondary to oxygen liberation. In addition, intravascular foaming following absorption can seriously impede right ventricular output and produce complete loss of cardiac output. Hydrogen peroxide can also exert a direct cytotoxic effect via lipid peroxidation. Ingestion of hydrogen peroxide may cause irritation of the gastrointestinal tract with nausea, vomiting, haematemesis and foaming at the mouth; the foam may obstruct the respiratory tract or result in pulmonary aspiration. Painful gastric distension and belching may be caused by the liberation of large volumes of oxygen in the stomach. Blistering of the mucosae and oropharyngeal burns are common following ingestion of concentrated solutions, and laryngospasm and haemorrhagic gastritis have been reported. Sinus tachycardia, lethargy, confusion, coma, convulsions, stridor, sub-epiglottic narrowing, apnoea, cyanosis and cardiorespiratory arrest may ensue within minutes of ingestion. Oxygen gas embolism may produce multiple cerebral infarctions. Although most inhalational exposures cause little more than coughing and transient dyspnoea, inhalation of highly concentrated solutions of hydrogen peroxide can cause severe irritation and inflammation of mucous membranes, with coughing and dyspnoea. Shock, coma and convulsions may ensue and pulmonary oedema may occur up to 24-72 hours post exposure. Severe toxicity has resulted from the use of hydrogen peroxide solutions to irrigate wounds within closed body cavities or under pressure as oxygen gas embolism has resulted. Inflammation, blistering and severe skin damage may follow dermal contact. Ocular exposure to 3% solutions may cause immediate stinging, irritation, lacrimation and blurred vision, but severe injury is unlikely. Exposure to more concentrated hydrogen peroxide solutions (>10%) may result in ulceration or perforation of the cornea. Gut decontamination is not indicated following ingestion, due to the rapid decomposition of hydrogen peroxide by catalase to oxygen and water. If gastric distension is painful, a gastric tube should be passed to release gas. Early aggressive airway management is critical in patients who have ingested concentrated hydrogen peroxide, as respiratory failure and arrest appear to be the proximate cause of death. Endoscopy should be considered if there is persistent vomiting, haematemesis, significant oral burns, severe abdominal pain, dysphagia or stridor. Corticosteroids in high dosage have been recommended if laryngeal and pulmonary oedema supervene, but their value is unproven. Endotracheal intubation, or rarely, tracheostomy may be required for life-threatening laryngeal oedema. Contaminated skin should be washed with copious amounts of water. Skin lesions should be treated as thermal burns; surgery may be required for deep burns. In the case of eye exposure, the affected eye(s) shod eye(s) should be irrigated immediately and thoroughly with water or 0.9% saline for at least 10-15 minutes. Instillation of a local anaesthetic may reduce discomfort and assist more thorough decontamination.

Administration, Inhalation↗

Bilateral vocal cord paralysis in newborns with neuraxial malformations--two case reports--.

Two neonates presented with inspiratory stridor due to bilateral vocal cord paralysis associated with occipital encephalocele, Chiari malformation, and hydrocephalus in one patient, and cervical meningomyelocele and Chiari malformation in the other patient. The clinical symptoms dramatically regressed after repair of the encephalocele or meningomyelocele with no requirement for craniovertebral decompressive procedures or shunts in the acute phase. Careful evaluation of neonatal stridor and recognition of vocal cord paralysis are important, as treatment of associated congenital central nervous system anomalies is likely to achieve satisfactory surgical results.

Arnold-Chiari Malformation↗

[Clinical management of upper airway obstruction: epiglottitis and laryngotracheobronchitis]

OBJECTIVE: To present current concepts on diagnosis and treatment of upper airway obstruction, mainly related to differential diagnosis between acute viral laryngotracheobronchitis and epiglottitis.METHODS: Bibliographic review covering the last ten years, using both Medline system and direct research. The most relevant articles published about the subject were selected.RESULTS: Viral laryngotracheobronchitis is an acute self-limited disease of the upper airway in a child, clinically characterized by barking cough, stridor, hoarse voice, and upper respiratory symptoms. The disease is diagnosed by clinical signs and symptoms. Rarely, if no immediate airway management is needed, radiography of the neck may help to exclude other entities that cause laryngeal obstruction. In contrast to viral laryngotracheobronchitis, epiglottitis is characterized by inflammation of the supraglottic tissues and is caused mainly by Haemophilus influenzae type b. A previously healthy child suddenly develops a sore throat and fever. Within hours after the onset of symptoms the patient looks toxic, swallowing is painful and breathing is difficult. Drooling and cervical hyperextension are frequently present. Lateral neck radiograph is rarely required to the diagnosis and may delay appropriate management of the airway. Moderate viral laryngotracheobronchitis with stridor at rest and retractions should be treated with steroids (systemic or nebulized) and nebulized epinephrine. Severe viral laryngotracheobronchitis should be treated aggressively while arregements are made for endotracheal intubation. The diagnosis of epiglottitis requires immediate endotracheal intubation in the appropriate unit (emergency department, intensive care unit or surgical unit) and antimicrobial therapy. Alternatively at some medical centers children with severe upper airway obstruction have been treated with a mixture of helium and oxygen (70 to 80% concentration of helium) instead of room air or pure oxygen to avoid intubation.CONCLUSIONS: There are different levels of care for patients with upper airway obstruction, depending on their clinical presentation. The clinical manifestations of viral laryngotracheobronchitis may be confused with the presentation of epiglottitis. Despite this observation we believe that differential diagnosis between viral laryngotracheobronchitis and epiglottitis rests on clinical grounds.

Journal Article↗

Symptomatic Arnold-Chiari malformation: review of experience with 22 cases.

Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.

Adult↗