Management of difficult airway during induction of anesthesia in a patient with Hallermann-Streiff syndrome

An 18-year-old female with Hallermann-Streiff syndrome underwent the fixation of prolapsus recti. She had significant microgenia, mental retardation and pharyngeal airway stenosis. During induction of anesthesia with halothane and nitrous oxide, severe upper airway obstruction and respiratory depression occurred. The mask ventilation with jaw lift maneuver was impossible. Lowering anesthetic level restored her spontaneous breathing and airway patency. Although the direct laryngoscopic view under light anesthesia with halothane was limited to the epiglottic tip, blind orotracheal intubation using stylet was accomplish after several attempts. At the end of anesthesia, the tracheal tube was extubated after the patient had become fully awake and had recovered completely from neuromuscular blockade monitored by electromyography.     

Bronchiolitis obliterans in a patient with localized scleroderma treated with D-penicillamine

D-penicillamine-associated bronchiolitis obliterans (BO) is a rare but well-known pulmonary complication in patients with rheumatoid arthritis or progressive systemic sclerosis. It has been assumed that in most, if not all cases, BO is a complication of the underlying disease rather than a side-effect of treatment. We report the case of a 46 year old man with scleroderma localized to his lower legs (morphea), who received a daily dose of 750 mg D-penicillamine. During the treatment of 1 yr duration, he developed progressive shortness of breath due to a worsening obstructive ventilatory defect suggesting BO, which was confirmed by surgical lung biopsy (constrictive BO). Bronchial obstruction progressed over the next 5 yrs and did not respond to corticosteroids. The patient finally underwent a successful single left lung transplantation. The histological features of constrictive BO were confirmed in the explanted lung. This observation suggests that D-penicillamine may induce bronchiolitis obliterans in the absence of a systemic connective tissue disease.     

The unanticipated difficult airway with recommendations for management

PURPOSE: To review the current literature and generate recommendations on the role of newer technology in the management of the unanticipated difficult airway. METHODS: A literature search using key words and filters of English language and English abstracted publications from 1990-96 contained in the Medline, Current Contents and Biological Abstracts databases was carried out. The literature was reviewed and condensed and a series of evidence-based recommendations were evolved. CONCLUSIONS: The unanticipated difficult airway occurs with a low but consistent incidence in anaesthesia practice. Difficult direct laryngoscopy occurs in 1.5-8.5% of general anaesthetics and difficult intubation occurs with a similar incidence. Failed intubation occurs in 0.13-0.3% general anaesthetics. Current techniques for predicting difficulty with laryngoscopy and intubation are sensitive, non-specific and have a low positive predictive value. Assessment techniques which utilize multiple characteristics to derive a risk factor tend to be more accurate predictors. Devices such as the laryngeal mask, lighted stylet and rigid fibreoptic laryngoscopes, in the setting of unanticipated difficult airway, are effective in establishing a patient airway, may reduce morbidity and are occasionally lifesaving. Evidence supports their use in this setting as either alternatives to facemask and bag ventilation, when it is inadequate to support oxygenation, or to the direct laryngoscope, when tracheal intubation has failed. Specifically, the laryngeal mask and Combitube have proved to be effective in establishing and maintaining a patent airway in "cannot ventilate" situations. The lighted stylet and Bullard (rigid) fibreoptic scope are effective in many instances where the direct laryngoscope has failed to facilitate tracheal intubation. The data also support integration of these devices into strategies to manage difficult airway as the new standard of care. Training programmes should ensure graduate physicians are trained in the use of these alternatives. Continuing medical education courses should allow physicians in practice the opportunity to train with these alternative devices.     

Advances in palliative care for the patient with scleroderma

Few studies published within the past year have addressed palliative care for the patient with scleroderma or systemic sclerosis. However, progress continues to be made, and important contributions have been made with respect to different vasodilator preparations for Raynaud's phenomenon, a possible role for diltiazem in the treatment of calcinosis, and the treatment of gut dysmotility. A number of comprehensive review papers on different aspects of management, mainly organ based, were included in the recently published textbook Systemic Sclerosis. Until there is an effective disease-modifying treatment for systemic sclerosis, management will be largely palliative and is best delivered by a multidisciplinary team.     

A difficult case involving a bradycardic patient

A seven-week-old, male chow chow-keeshond cross puppy was presented with acute paraplegia and episcleral hemorrhage. An X-linked, hereditary coagulopathy was suspected and confirmed by clotting factor analysis and necropsy findings of intraspinal hemorrhage. In young, male puppies with acute spinal pain and long-tract signs, intraspinal hematoma secondary to inherited X-linked coagulation factor deficiencies should be considered in the differential diagnosis.     

Multiple stress fractures in a scleroderma patient on methotrexate therapy

A patient on methotrexate therapy for scleroderma developed four stress fractures within a period of 13 months. She was not on steroid therapy and had no risk factors for osteoporosis. A review of the literature found 13 cases of stress fractures under methotrexate therapy. Whether methotrexate can induce bone changes remains controversial.     

Failure to control the airway in a patient with Hunter''s syndrome

We report a 36-year-old man with proximal dominant muscle weakness, thymic tumor, diabetes mellitus, hypokalemia, and increased levels of plasma ACTH and cortisol. The diagnosis of carcinoid tumor was made on the basis of pathological findings in the biopsied specimen of the thymic tumor. The proximal muscle weakness was considered to be due to steroid myopathy resulting from the overproduction of cortisol from the adrenal glands induced by the ectopic ACTH secreted by the thymic carcinoid tumor. Although thymoma in frequently associated with myasthenia gravis, we should also consider carcinoid tumors in patients with a thymic tumor presenting with a proximal muscle weakness.     

Long-term propofol infusion and airway management in a patient with Goldenhar''s syndrome

SAPHO syndrome is characterized by osteoarticular involvement of ventral chest wall in the form of sternocostoclavicular osteoarthritis and hyperostosis and skin changes such as palmoplantar pustulosis and acne. Occasionally, psoriatic lesions and sacroiliitis are observed. However, despite the higher frequency of psoriasis in this syndrome, its inclusion in psoriatic arthropathy spectrum is not clearly established. The authors report three cases of SAPHO syndrome in psoriatic patients commenting on the difficulty in differentiating this entity from psoriatic arthritis as well as its relationship with seronegative spondyloarthropathies. This disease has been described mainly in Japan and only a few cases of this disease have been reported in the European or American literature.     

The use of the laryngeal mask in a female patient with an unpredictable difficult intubation

A laryngeal mask was used after repeated ineffective attempts at intubation. Preoperative examinations failed to defect signs of a possible difficult intubation. Laryngoscopy showed a true glottis, but it was impossible to insert tube No. 6 in the trachea. Failure of attempts at intubation made us use a laryngeal mask for maintaining the patency of the upper airways. Anesthesia coursed smoothly in the presence of stable hemodynamics and gas exchange. Use of laryngeal mask helped solve the problem of unpredictable difficult intubation and provide reliable patency of the upper respiratory airways in a female patient with latent stenosis of the subglottal space.     

The univent tube can facilitate difficult intubation in a patient undergoing thoracoscopy

A case of a subcutaneous haemangioma of the hand clinically mimicking a glomus tumour is described. Other discrete small hand tumours that may present with the triad of pain, tenderness and cold intolerance are discussed.     

Usefulness of the intubating laryngeal mask airway for cases with predicted difficult intubation

We evaluated the usefulness of the intubating laryngeal mask airway (ILMA) in patients who were predicted to have possible difficult airway. Patients with possible difficult airway were defined as those with limited head extension, Mallampati's classification of grade IV, thyro-mental distance < 4 cm, or Cormack grade III-IV on the laryngoscopy. The control group was consisted of the patients without these conditions or impaired mouth opening. Insertion of the ILMA was successfully performed in all patients of both groups. In the group of possible difficult airway, 83% of patients were intubated through the ILMA successfully, and in the control group, 86%. We conclude that the ILMA may become an additional tool in patients with difficult intubation.     

Continuous versus bilevel positive airway pressure in a patient with idiopathic central sleep apnea

A 57-yr-old man with idiopathic central apnea is reported. He presented at our hospital complaining of excessive daytime sleepiness. Polysomnography, including esophageal pressure monitoring, confirmed central sleep apnea with an apnea index of 27/h. He had mild non-insulin-dependent diabetes mellitus (NIDDM) but no signs of diabetic neuropathy or other background diseases. The ventilatory responses to hypoxia and hypercapnia tested while he was awake indicated increased respiratory chemosensitivity. We applied nasal continuous positive airway pressure (CPAP) and bilevel positive airway pressure (BPAP) in an attempt to compare the possible difference in therapeutic efficacy. Although nasal CPAP completely reversed central apnea, nasal BPAP adversely affected both apnea length and frequency in an applied pressure-dependent manner. Arterial blood gas analyses while he was being treated indicted alveolar hypoventilation with CPAP and hyperventilation with BPAP. Additionally, administration of a mixed gas containing 5% CO2 through a face mask had a significant effect on the disappearance of central apnea in this patient. These findings support the theory that the arterial PCO2 level is critical in generating idiopathic central apnea and that nasal CPAP therapy may be effective in eliminating central apnea by raising the PaCO2.     

The reinforced laryngeal mask airway (LMA) as an alternative airway device to manage the difficult airway

BACKGROUND: Infection with human parvovirus B19 (B19) has been reported in a few patients with various vasculitis syndromes. Mixed cryoglobulinaemia (MC), a model of small vessel size vasculitis, may result from numerous infectious diseases, particularly hepatitis C virus (HCV) infection. AIM: To assess the prevalence of seric B19 infection markers in a large series of patients with MC, with or without HCV infection. PATIENTS AND METHODS: Sixty-four patients were studied: essential MC (EMC, n = 19), MC associated with non-infectious diseases (non-essential MC, n = 9), and patients with HCV infection with (HCV-MC, n = 18) or without MC (HCV-no-MC, n = 18). Patients were considered to have MC if two successive determinations of their serum cryoglobulin concentration were above 0.05 g/l. Serum samples were analysed for specific IgG and IgM antibodies to B19 by enzyme immunoassay. B19 DNA detection was performed by polymerase chain reaction using a set of primers located in the VP1 gene, separately in serum and in cryoprecipitates to investigate a possible capture of B19 DNA in cryoprecipitate. The study also looked for a possible enrichment for of IgG antibodies to B19 in MC. RESULTS: The presence of specific IgG antibodies to B19 was found in 68% EMC, 56% non-essential MC, 78% HCV-MC, and 78% HCV-no-MC. No patient of either group had specific IgM antibodies to B19, or B19 DNA in serum or in cryoprecipitate. Overall, IgG antibodies to B19 were found in 46 of 64 (72%) serum samples, a prevalence quite similar to the prevalence in general adult population (> 60%). A specific enrichment of IgG antibodies to B19 in the MC was not found. CONCLUSION: These results suggest that B19 infection is neither an aetiological factor of EMC, nor a cofactor that may lead to MC production in patients with chronic HCV infection.     

Case report: severe fibrosis in a patient with scleroderma and previous radiotherapy--a case report and literature review

Patients with scleroderma may develop marked local cutaneous fibrosis following radiation therapy. We describe one patient treated with wide local excision and radiation therapy, who subsequently developed scleroderma with severe fibrosis in the irradiated field.