Severe Restrictive Lung Disease Research Articles

Lung volume histograms after computed tomography of the chest with three-dimensional imaging as a method to substantiate successful surgical expansion of the rib cage in achondroplasia

The authors describe a radiographic method to quantify a surgical procedure of thoracic expansion in a 2-year-old patient with achondroplasia, small chest cage, and severe restrictive lung disease. The patient had undergone three surgical procedures of thoracic expansion since birth. The authors utilized computer-generated lung volume histograms after spiral computed tomographic scanning and three-dimensional imaging of the lungs to calculate his lung volumes before and after the third surgical thoracic expansion. The lung volumes, calculated by the histograms, were 363 mL and 406 mL before and after surgery, respectively. This 40-mL difference in the patient's lung volumes (4 mL/kg) accounted for a significant clinical improvement. Lung volume histograms obtained by this radiographic method are very helpful in substantiating a successful surgical chest expansion or provide an explanation for an unsuccessful repair.

Operative management of asphyxiating thoracic dystrophy after pectus repair

Pectus excavatum repair usually results in unchanged or improved pulmonary function. However, a small subset of patients will experience severely impaired pulmonary function after pectus repair caused by restrictive lung disease, and no adequate surgical approach has been described for this condition. A procedure is described that is a variation of an operation for Jeune's thoracic dystrophy, that resulted in marked respiratory improvement in this setting. A 14-year-old boy had undergone standard pectus excavatum repair at age 4, from which he recovered uneventfully. Beginning at age 10 to 12 years progressive restrictive pulmonary disease, recurrent pneumonia, and cor pulmonale developed, which resulted in almost constant shortness of breath and the need for continuous nasal positive pressure support. Pulmonary function test results were markedly abnormal and worsening. He underwent an operative procedure consisting of sternal split that was wedged open permanently with rib struts, opening of pleura bilaterally, and six rib resections bilaterally. His postoperative recovery was satisfactory, and his pulmonary functions have shown steady improvement. He is now completely off oxygen and pressure support, has improved exercise tolerance, and has returned to school. Severe restrictive lung disease after pectus repair can be successfully managed with aggressive operative procedures. Patients should have close follow-up after pectus repair for the development of this potentially debilitating disorder to allow earlier repair.

Acute respiratory distress syndrome caused by respiratory syncytial virus.

Acute respiratory distress syndrome (ARDS) complicating severe respiratory syncytial virus (RSV) infection has been described in only a few infants. In contrast to the low mortality rates usually associated with RSV infections (< 5%), mortality rates in the range of 40-70% have been reported in pediatric patients with ARDS. However, studies on patients with ARDS are usually lumped with respect to causation, and the disease course of RSV-induced ARDS has not been previously studied. We examined the pulmonary function abnormalities of 37 infants with RSV-induced respiratory failure who were admitted to our pediatric intensive care unit for assisted ventilation. Measurements included respiratory mechanics, maximum expiratory flow-volume curves, and lung volumes. These allowed the calculation of a Murray lung injury score (modified for pediatric use) in which radiographic findings, ventilator settings, lung compliance, and blood gas results were considered. We identified ten infants with severe restrictive lung disease who fulfilled the clinical criteria for classification as ARDS. All had lung injury scores above 2.5, compatible with a diagnosis of ARDS. Twenty-seven infants had obstructive patterns of lung function consistent with a clinical diagnosis of RSV bronchiolitis. The patients with RSV-induced ARDS were significantly younger, and had a longer time on assisted ventilation (P < 0.05) and a higher proportion of predisposing illnesses (P < 0.05, odds ratio = 6.67, two-tailed Fisher's exact test) when compared with the patients who had obstructive disease. Only one patient (who had immunodeficiency) died, and all others were successfully managed on conventional mechanical ventilation. We conclude that RSV-induced respiratory failure represents a relatively benign cause of ARDS in pediatric patients. Our observations support the notion of differentiating ARDS with respect to causation, especially when novel and experimental therapy is considered and mortality rates are analyzed.

Reconstructive spine surgery in pediatric patients with major loss in vital capacity.

Thirty-two pediatric patients with severe restrictive lung disease identified with vital capacities < 40% of predicted, who had undergone major reconstructive spine surgery, were reviewed. There were 18 boys and 14 girls, the mean age was 13 years (range, 7-17), and the mean vital capacity was 31% of predicted (range, 16-39%). Fifty-four procedures were performed, 13 posterior only, one of which was staged, and 19 anterior and posterior procedures, of which 15 were staged and four were sequential. The incidence of pulmonary complications (pneumonia, reintubation, pneumothorax, respiratory arrest, or the need for tracheostomy) was 19% (six patients), and only three patients required tracheostomy. The surgical and perioperative mortality rate was zero. Patients who had a thoracotomy or a thoracoabdominal approach had a significantly higher number of pulmonary complications. The use of preoperative decreased vital capacity as a measure of inoperability excludes the young patient most in need of surgical intervention. With improved preoperative, intraoperative, and postoperative techniques, careful monitoring, and the cooperation of pediatric pulmonologists and intensivists, reconstructive spine surgery can be performed in the pediatric patient with severe decreased vital capacity with very acceptable morbidity and mortality.

Profiles of neuromuscular diseases. Spinal muscular atrophy.

Forty-five individuals with spinal muscular atrophy (SMA) types II and III were evaluated prospectively over a 10-yr period to develop an impairment and disability profile. SMA II subjects showed marked weakness and progressive decline of strength. Mean manual muscle test (MMT) score for all muscles combined was 2.3 +/- 0.6, with a decline in strength of -0.24 MMT units per decade. SMA III individuals had a relatively static or very slowly progressive course and were far stronger. Mean MMT score for all muscles combined was 3.8 +/- 0.7, and the decline in strength per decade was not significant. In both types proximal weakness was greater than distal, but there was greater involvement of the lower extremities and the extensor muscle groups only in SMA II. Contractures, progressive scoliosis, and restrictive lung disease (RLD) were present in most of the SMA II individuals, but these complications were rare in SMA III. Maximal expiratory pressures were affected earlier and to a greater degree than vital capacity. Seventy-eight percent of those with SMA II had scoliosis with a mean Cobb angle of the primary curve of 62 +/- 37 degrees. Forty-one percent had severe RLD, and 17% had moderate RLD. In both types, 63% had abnormal electrocardiograms although most had minor findings. Timed motor performance and functional evaluations indicated that muscle weakness translated to substantial disability in both SMA II and III, with more severe impairment noted in SMA II. Neither type was associated with abnormal means scores on intellectual and neuropsychologic test batteries.

Profiles of neuromuscular diseases. Limb-girdle syndromes.

Sixty-six individuals with Limb-Girdle Syndrome (LGS) were evaluated over a 10-yr period and classified into three types: 19 severe autosomal recessive muscular dystrophy of childhood (ARMDC), alternatively referred to by some as SCARMD, 18 autosomal dominant late onset (ADLO), and 29 pelvifemoral (PF) individuals. ARMDC subjects showed the greatest weakness, 2.5 +/- 1.0, mean Manual Muscle Test (MMT) grade for all muscles combined, and the only significant progression of loss of strength, -0.59 MMT unit decline per decade. Strength loss in ADLO and PF types was about the same, 3.7 +/- 0.7 and 4.0 +/- 0.7 grades, respectively. Quantitative strength measurements in ADLO and PF types were more sensitive than MMTs, showing losses of 30-40% strength in muscle groups with MMT grades of 4 or higher. All three types showed greater proximal and lower extremity weakness but usually no difference between flexor and extensor strength. There was a high percentage (44%) of mild very slowly progressive scoliosis in ARMDC, but spine deformity was unusual in ADLO and PF (11%) LGS. Contractures were few, slowly progressive, and usually mild in severity in all types, although more frequent in ARMDC. There also was a low frequency of severe restrictive lung disease in all types (10%) but a high percentage of electrocardiogram abnormalities (62-73%). The most common electrocardiogram abnormalities were increased R/S ratio in V1 and infranodal conduction defects. Intellectual and cognitive functions were within normal limits. Mobility and extremity function reflected the strength differences between the ARMDC and other types of LGS. Eight-five percent of ARMDC individuals relied on a wheelchair for all or part of their mobility, and all were unable to complete timed motor performance tests within the 99th percentile range for controls.

Severe restrictive lung disease in systemic sclerosis.

We sought to identify risk factors for developing severe restrictive lung disease and to determine the time of onset and rate of progression in patients with systemic sclerosis (SSc). Using the University of Pittsburgh Scleroderma Databank, we grouped patients according to their lowest forced vital capacity (FVC) value: > 75% predicted, 50-75% predicted, and < 50% predicted. In patients with severe restrictive disease, we examined serial pulmonary function test (PFT) results to determine the rate of loss of lung volume over time. Of 890 SSc patients, 60% (n = 531) never had an FVC < or = 75% predicted; 27% (n = 243) had moderate restrictive disease, with an FVC value of 50-75% predicted; and only 13% (n = 116) of the patients had severe restrictive disease, with FVC < or = 50% predicted. Black race, male sex, early disease, and primary cardiac involvement due to SSc were the features most frequently associated with severe restrictive lung disease (by multiple logistic regression). Fifty-five patients with severe restrictive lung disease had their first of at least 2 PFTs during the first 5 years after onset of any SSc (not pulmonary) symptoms. In 30 patients, the FVC declined by 32% per year in the first 2 years of illness, in 16 patients the annual loss was 12% in years 2-4 after disease onset, and in 9 patients annual loss was 3% during years 4-6 of disease (P < 0.005 by 1-way analysis of variance). In SSc patients, black men with early disease who have cardiac involvement are the most likely to have factors associated with the development of severe restrictive lung disease (which is increasingly becoming a major cause of death). Disease subtype (diffuse versus limited cutaneous) and serum anti-topoisomerase I antibody do not differentiate between moderate and severe restrictive disease. Careful monitoring of pulmonary function early in the disease, when the greatest loss of lung function occurs, may help identify patients likely to respond to new therapy.

Pectus excavatum repair in a patient with prune belly syndrome

SummaryWe describe the anaesthetic management of a child with ‘Prune Belly’ syndrome who was presented for repair of pectus excavatum. In addition to having severe restrictive lung disease, the patient was also asthmatic. Prior surgical procedures had resulted in long term debilitation because of pulmonary complications. Principles of anaesthetic management first described more than twenty years ago were utilized and combined with an effective perioperative pain management plan to provide for a smooth and satisfactory postoperative outcome. Repair of the patient's pectus excavatum deformity improved his respiratory mechanics, while the use of thoracic epidural anaesthesia in the immediate perioperative period allowed rapid improvement in pulmonary function.

RESTRICTIVE LUNG DISEASE IN PREGNANCY

Restrictive ventilatory defects characterized by a reduction in lung volumes and an increase in the ratio of forced expiratory volume in 1 second to forced vital capacity occur when lung expansion is limited because of alterations in the lung parenchyma or because of abnormalities in the pleura, chest wall, or neuromuscular apparatus. Few studies have examined pregnant women with carefully defined restrictive lung disorders. The majority of pulmonary diseases have their onset after the childbearing years. When present, most do not alter fertility. Further, these disorders are only a relative contraindication to pregnancy because both the fetus and mother are able to survive without a high risk of increased morbidity or mortality. The clinical course of sarcoidosis is generally not altered by pregnancy. Factors indicative of a poor prognosis in sarcoidosis and pregnancy include parenchymal lesions on chest radiography, advanced roentgenologic staging, advanced maternal age, low inflammatory activity, requirement for drugs other than corticosteroids, and the presence of extrapulmonary sarcoidosis. Pregnancy seldom has a significant effect on the course of the connective tissue diseases. In PSS with significant renal involvement, pregnancy has the potential for poor fetal prognosis and the risk of maternal death due to a lethal progression of renal failure. Worsening of SLE is uncommon in pregnancy, and prophylactic therapy is generally not necessary. Most women with LAM are advised to avoid pregnancy or the use of estrogens because of the concern that it will lead to worsening of their disease. The incidence of kyphoscoliosis in pregnancy is relatively high. Premature birth rates are higher than that in the normal population. The risk of progression of the abnormal curve in a scoliotic patient appears low. However, women with unstable scolioses at the time of pregnancy can demonstrate progression of the curve with the pregnancy. Respiratory complications during pregnancy in patients with kyphoscoliosis have been reported but in general are not serious if appropriately managed. As a rule, patients with severe restrictive lung disease (i.e., vital capacity < 1 L) should be advised to avoid pregnancy or consider therapeutic abortion. If such a patient decides to continue the pregnancy she should be provided with optimal medical management of her underlying disease and should consider delivery by cesarean section.

Lung Transplantation in a Patient with a Prior Bone Marrow Transplant

A 25-year-old woman with acute lymphoblastic leukemia underwent two bone marrow transplants. She subsequently developed severe restrictive lung disease which was successfully treated with a single lung transplant.

The Use of a Short-Length Transtracheal Oxygen Catheter in Patients of Small Stature with Restrictive Lung Disease

Transtracheal oxygen therapy is being used with increasing frequency because it is an effective mode of oxygen delivery and is well tolerated by patients. An increase in cough and mild intermittent hemoptysis are not uncommon in the early postinsertion period but usually resolve spontaneously. Herein we present two individuals of short stature with restrictive lung disease who had persistence of excessive cough and mild hemoptysis after insertion of a standard catheter (SCOOP). Bronchoscopic evaluation in one revealed erosions of the mucosa over the carina and take-off of the right main-stem bronchus. Symptoms resolved in both individuals following placement of a shorter catheter. We suggest that greater consideration be given to matching transtracheal catheter length to patient lung size, particularly in the face of severe restrictive lung disease.

Pulmonary Functions in Children With Progressive Systemic Sclerosis

The patterns of pulmonary involvement in 13 children with progressive systemic sclerosis were investigated. Eight patients (61%) had respiratory symptoms or signs and 7 patients (55%) had abnormalities on chest roentgenogram. Twelve patients (92%) had abnormal pulmonary function tests: 7 had restrictive disease, 2 had obstructive disease, 2 had small airway disease, and 1 had an isolated reduction in the diffusing capacity of carbon monoxide. Nine patients had the test performed during the first year of illness, 3 during the second year, and 1 at 5 years. All patients had abnormal pulmonary function tests when first studied. Subsequent pulmonary function tests over a period of 3 to 10 years (mean 6.2 years) showed substantial changes in only 2 patients (1 patient had initial worsening of diffusing capacity of carbon monoxide followed by normalization and another patient showed improvement of obstructive disease). Two patients died during follow-up, 1 of pulmonary hypertension, the other of severe restrictive lung disease and myocardial fibrosis. The major findings of this study were (1) high frequency of pulmonary disease in children with progressive systemic sclerosis, (2) early involvement of the lungs, (3) relatively indolent progression of lung disease, and (4) the prognostic importance of the severity of pulmonary disease. Pulmonary manifestations of progressive systemic sclerosis in children appear to be similar to those of affected adults.

Results of Single-Lung Transplantation for Bilateral Pulmonary Fibrosis

Between November 1983 and August 1989, we performed single-lung transplantation for end-stage pulmonary fibrosis in 20 patients. Nine patients (45 percent) who survived for more than one year form the basis of this report. Before surgery, the nine survivors had severe restrictive lung disease, with a mean (+/- SD) vital capacity (VC) of 43 +/- 9 percent, a forced expiratory volume in one second (FEV1) of 50 +/- 9 percent, and a single-breath diffusing capacity (DLCO) of 36 +/- 9 percent of predicted values. One year after transplantation, the patients' VC had reached 69 +/- 10 percent, FEV1 79 +/- 15 percent, and DLCO 62 +/- 16 percent of predicted values. Relative perfusion to the transplanted lung rose from 63 +/- 14 percent (three days after surgery) to 77 +/- 7 percent within three months and stayed constant or increased slightly thereafter. Before surgery, despite supplemental oxygen at flow rates varying from 1 to 9 liters per minute, none of the patients could exercise beyond stage 1/2 (2.7 km [1.7 miles] per hour, 5 percent grade) on a modified Bruce treadmill-exercise protocol. All eight patients tested one year or more after transplantation achieved at least stage 1 (2.7 km [1.7 miles] per hour, 10 percent grade), and usually a higher stage, without supplemental oxygen. Arterial oxygen tension returned to normal values in most patients (87 +/- 13 mm Hg), and supplemental oxygen, which all patients required before surgery, was no longer needed by any patient after transplantation. We conclude that in carefully selected patients with end-stage pulmonary fibrosis, single-lung transplantation is an effective treatment.

Breathing patterns and HbSaO2 changes during nocturnal sleep in patients with Duchenne muscular dystrophy.

A night-time polygraphic sleep recording with continuous HbSaO2 monitoring was performed in 11 chair-bound Duchenne muscular dystrophy patients with severe restrictive lung disease but with blood gas values within normal limits when awake. No abnormalities of sleep pattern were detected. Nocturnal sleep did not have significant adverse effects on respiration. However, in 6 patients, infrequent central apnoeas or hypopnoeas occurred which were associated with falls in HbSaO2 greater than those that have been reported to be in normal subjects. The magnitude of HbSaO2 falls appeared to be significantly correlated with functional residual capacity values. Overall, the findings revealed a relatively preserved, although unstable, blood O2 balance during nocturnal NREM and REM sleep in patients with Duchenne muscular dystrophy, even in an advanced stage of their illness.

Acquired immunodeficiency syndrome may present as severe restrictive lung disease

A majority of patients with the acquired immunodeficiency syndrome (AIDS) will develop pulmonary disease at some point during their clinical course. Most patients with AIDS-related pulmonary disease present with the subacute onset of fever, cough, and dyspnea often in the context of other clinical findings that suggest infection with human immunodeficiency virus (HIV) [l]. Clinicians must be alert to uncommon presentations of AIDS that may occur, particularly in patients who do not have apparent risk factors for infection with HIV. In the current report, we describe an atypical pulmonary presentation of AIDS that was associated with unusual histopathologic findings of Pneumocystis carinii pneumonia.

SARCOIDOSIS IN CHILDREN

Eight patients with sarcoidosis seen at the Royal Children's Hospital, Melbourne, during the past 10 years were reviewed. Five of the eight patients came form non-metropolitan areas. The major presenting symptoms were cough, fatigue and weight loss; peripheral lymphadenopathy and hepatomegaly were common. None of the patients had eye or central nervous system involvement. Seven patients had bilateral hilar adenopathy on chest radiography and six had parenchymal lung changes. Angiotensin converting enzyme was measured in six patients and was elevated in all, while hypercalcaemia was present in three patients. Five patients had a tissue biopsy showing the characteristic non-caseating granulomas. Corticosteroid therapy was used for four patients and was given for hypercalcaemia in three patients and for severe restrictive lung disease in one patient.

Sarcoidosis in children.

Eight patients with sarcoidosis seen at the Royal Children's Hospital, Melbourne, during the past 10 years were reviewed. Five of the eight patients came from non-metropolitan areas. The major presenting symptoms were cough, fatigue and weight loss; peripheral lymphadenopathy and hepatomegaly were common. None of the patients had eye or central nervous system involvement. Seven patients had bilateral hilar adenopathy on chest radiograph and six had parenchymal lung changes. Angiotensin converting enzyme was measured in six patients and was elevated in all, while hypercalcaemia was present in three patients. Five patients had a tissue biopsy showing the characteristic non-caseating granulomas. Corticosteroid therapy was used for four patients and was given for hypercalcaemia in three patients and for severe restrictive lung disease in one patient.

Sleep patterns in nonambulatory boys with Duchenne muscular dystrophy.

Sleep patterns and respiratory function during sleep were studied in five nonambulatory boys with Duchenne muscular dystrophy to clarify why patients with this disease awaken frequently at night. It was hypothesized that hypoxemia during sleep due to severe restrictive lung disease might cause nighttime arousals. Each boy underwent electroencephalography, electro-oculography and electromyography. Also determined were arterial oxyhemoglobin saturation, airflow from the nose and mouth, chest and abdominal excursions, and carbon dioxide tension of exhaled breaths. All five subjects had pulmonary function abnormalities consistent with severe restrictive lung disease and respiratory muscle weakness but none had evidence of respiratory failure or cor pulmonale. The boys awakened three times more frequently than age-matched published norms and experienced sleep stage shifts twice as often as normal children. Rapid-eye movement (REM) sleep as a proportion of total sleep was significantly reduced; sleep stage I was increased compared to normal values. No subject developed oxyhemoglobin desaturation during sleep. End-tidal CO2 tensions rose during sleep stages I, II and V (REM) in association with reduced chest wall excursion, suggesting transient episodes of mild hypoventilation which were not associated with arousals. Sleep fragmentation, frequent arousals and REM sleep deprivation occur in some boys with Duchenne muscular dystrophy but are not associated with significant disorders in breathing during sleep.

Surgical Palliation of Respiratory Insufficiency Secondary to Massive Exuberant Polyostotic Fibrous Dysplasia of the Ribs

A middle-aged man with long-standing polyostotic fibrous dysplasia had severe progressive restrictive lung disease with hypoxemia and pulmonary hypertension with heart failure because of exuberant intrathoracic, extraosseous proliferation of dysplastic tissue. Subtotal resection of this benign tissue mass ameliorated the respiratory insufficiency and led to sustained improvement in exercise tolerance, increase in pulmonary reserve, and decrease in signs of heart failure and pulmonary hypertension.

Successful treatment of varicella pneumonia with prolonged extracorporeal membrane oxygenation in a child with leukemia

A 5-year-old boy with acute lymphoblastic leukemia in complete continuous remission developed life-threatening varicella pneumonia and acute respiratory insufficiency (ARI). The child recovered after 92 hours of partial venoarterial perfusion with a membrane oxygenator. Functional asplenia developed. Serial pulmonary function tests after perfusion indicate moderately severe restrictive lung disease which has slightly improved during an 18 month period.