1/128. Severe progressive osteoporotic spine deformity with cardiopulmonary impairment in a young patient. A case report. STUDY DESIGN: This report describes a young patient with a rapidly progressive kyphosis caused by collapse of a severely osteoporotic thoracolumbar spine, which led to impairment of cardiopulmonary function. OBJECTIVES: To highlight the treatment strategy, difficulty of diagnosis, operative stabilization, and outcome. SUMMARY OF BACKGROUND DATE: Little is known about natural history, treatment options, and results of this condition. methods: The magnitude of bone loss was measured by dual-energy x-ray absorptiometry, and the deformity was visualized by computed tomography and magnetic resonance imaging. Laboratory investigations also were performed before and during halotraction in an attempt to establish a diagnosis. These data constituted the preoperation information required to assess later results of medical and surgical intervention. RESULTS: An extensive evaluation of possible underlying etiologies failed to identify a specific etiology. Before and during halotraction, bone mineral substitutes were given, partially correcting the bone mineral content as measured on repeated dual-energy x-ray absorptiometry scans. In addition, the thoracic kyphosis was partially corrected, from 100 degrees to 70 degrees Cobb's angle. Subsequently, a combined anterior and posterior stabilization was performed from C7 to S1 using a vascularized fibula graft, a double Isola rod system (AcroMed, Cleveland, OH), and a carbonate apatite cancellous bone cement to reinforce the pedicle screws. At follow-up assessment 40 months surgery, the patient was asymptomatic and fully mobilized, with radiographs showing complete incorporation of the grafts and no loosening of the fixation device. CONCLUSIONS: The diagnostic and therapeutic difficulties of progressive spine deformity caused by severe osteoporosis in young patients emphasizes the importance of a thoroughly planned treatment strategy. Halotraction is recommended to stop progression of the deformity, or even partially correct it, and to allow time to search for the diagnosis and bone mineral substitution. Surgical treatment using vascularized fibular strut grafts and a strong fixation device was successful. Biocompatible carbonated apatite cancellous bone cement was successfully used to reinforce pedicle screw fixation. ( info) |
2/128. Cor pulmonale presenting in a patient with congenital kyphoscoliosis following intercontinental air travel. We present the case of a 59-year-old man with congenital kyphoscoliosis who developed cor pulmonale for the first time following intercontinental air travel. Prolonged exposure to the low partial pressure of oxygen in the cabin of the aircraft led to pulmonary hypertension and right heart failure. The case highlights the potential for long-haul air travel to cause decompensation in patients with thoracic deformity and apparently stable cardiorespiratory function. It also emphasises the need for patients and their medical attendants to carefully consider the potential health implications of the hypoxic atmosphere in pressurised aircraft. ( info) |
3/128. Halo femoral traction and sliding rods in the treatment of a neurologically compromised congenital scoliosis: technique. In severe congenital scoliosis, traction (whether with a halo or instrumental) is known to expose patients to neurologic complications. However, patients with restrictive lung disease may benefit from halo traction during the course of the surgical treatment. The goal of treatment of such deformities is, therefore, twofold: improvement of the respiratory function and avoidance of any neurologic complications. We report our technique to treat a 17-year-old girl with a multi-operated congenital scoliosis of 145 degrees and cor pulmonale. Pre-operative halo gravity traction improved her vital capacity from 560 c.c. to 700 c.c., but led to mild neurologic symptoms (clonus in the legs). To avoid further neurologic compromise, her first surgery consisted of posterior osteotomies and the implantation of two sliding rods connected to loose dominoes without any attempt at correction. Correction was then achieved over a 3-week period with a halofemoral traction. This allowed the two rods to slide while the neurologic status of the patient was monitored. Her definitive surgery consisted of locking the dominoes and the application of a contralateral rod. Satisfactory outcome was achieved for both correction of the deformity (without neurologic sequels) and improvement of her pulmonary function (1200 c.c. at 2 years). This technique using sliding rods in combination with halofemoral traction can be useful in high-risk, very severe congenital scoliosis. ( info) |
4/128. Right heart failure as the dominant clinical picture in a case of primary amyloidosis affecting the pulmonary vasculature. A 91-year-old female patient died of right heart failure and pulmonary hypertension. The autopsy revealed multi-organ vascular amyloidosis and pulmonary alveolar septal amyloidosis with no evidence of parenchymal myocardial amyloid deposition. This is a rare example of cor pulmonale secondary to pulmonary amyloidosis. ( info) |
5/128. Reversible cardio-pulmonary changes due to adeno-tonsilar hypertrophy. Adeno-tonsillar hypertrophy, with signs of upper airway obstruction is a common presentation in ENT clinics. Recently it is identified as a major cause of sleep apnea syndrome. Several isolated case reports of pulmonary hypertension and corpulmonale appeared in the literature. The authors report two such children aged less than 2 years with cardio-pulmonary changes occurring secondary to chronic adeno-tonsillar hypertrophy that were successfully treated with the surgical removal. ( info) |
6/128. Phlegmasia cerulea dolens of the upper extremity. Phlegmasia cerulea dolens (PCD) is the term describing the painful venous congestion that results from near-total venous occlusion of a limb. It is unusual in the lower extremity but is decidedly rare in the upper extremity with only a handful of cases reported in the literature. PCD of the upper extremity usually occurs in patients with significant comorbid conditions such as severe cardiac failure or advanced malignancy. PCD of the upper extremity is associated with substantial morbidity and mortality. We present a case of upper extremity PCD in an elderly man with a complex medical history, complicated clinical course, and poor outcome that is typical for this rare disease. ( info) |
7/128. Severe pectus excavatum associated with cor pulmonale and chronic respiratory acidosis in a young woman. Pectus excavatum has never been reported to cause hypercapnic respiratory failure. In this report, we describe the first such case in a young woman with severe pectus excavatum who presented with chronic respiratory acidosis, pulmonary hypertension, and chronic cor pulmonale. An extensive diagnostic workup failed to uncover any other cause of respiratory acidosis, which led us to conclude that the severe chest wall deformity and the resulting severe restrictive defect were responsible for the development of chronic respiratory acidosis and cor pulmonale. ( info) |
8/128. Paradoxical embolism. An old but, paradoxically, under-estimated problem. The theoretical model of paradoxical embolism requires the presence of four parameters, namely, arterial embolism, venous thrombus, abnormal intracardiac communication and right-to-left shunt. Many aspects, however, of this well known entity are under consideration; diagnosis is often difficult to be established and the long term efficacy of preventive measures is undefined. We comment on a case report of recurrent paradoxical embolism with popliteal vein thrombosis and patent foramen ovale, and we briefly review the literature. ( info) |
9/128. Transitional cell carcinoma manifesting as acute cor pulmonale: cause of microscopic tumor embolism. Acute cor pulmonale is an uncommon manifestation of microscopic pulmonary tumor embolism. We describe the case of an 84-year-old man with a history of transitional cell carcinoma (TCC) of the urinary bladder who had acute cor pulmonale and died within a few hours after the onset of dyspnea. autopsy showed that the right ventricle was dilated without hypertrophy. Microscopic examination of the lung showed that the small arteries, arterioles, and capillaries were filled with micrometastases of TCC. Microscopic pulmonary tumor embolism has rarely been reported with TCC, and to the best of our knowledge, such a fulminant course has not been previously described in the English language. ( info) |
10/128. Pickwickian syndrome, 20 years later. The Pickwickian syndrome stimulated new pathophysiological concepts in regard to control of ventilation. With the advent of sleep laboratories, the peculiar sleep apnea occurring in some of these patients has been explained on the basis of intermittent upper airway obstruction. Two patients with different manifestations of the Pickwickian syndrome are presented. The suggestion is made that these two subsyndromes should have unique designations. The Auchincloss syndrome is manifested by right heart failure and respiratory acidosis in obese patients who are alert and have no major abnormality of breathing pattern. The fundamental cause of this abnormality is the increased work of breathing caused by the obesity. The cost of breathing is so high that the ventilatory regulation is compromised and respiratory acidosis results. The Gastaut syndrome is characterized principally by hypersomnia and sleep apnea. The fundamental defect is upper airway obstruction during sleep, resulting in increased work of breathing, which together with the increased work caused by obesity leads to respiratory acidosis and right ventricular failure. Hypersomnia, rather than heart failure or respiratory acidosis, is the major manifestation of this syndrome, and is the result of sleep loss. ( info) |