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Long-term follow-up of children with diaphragmatic hernia.

From 1962 to 1988, 147 neonates were admitted for operative repair of CDH to the Division of Pediatric Surgery in the Children's Hospital of Cologne. Follow-up studies were performed on 45 patients ages 1 to 25 years representing 54.2% of the 83 survivors. 18 patients (40%) were entirely without any complaints, 11 patients (24.4%) had increased rates of respiratory infections. Especially in early childhood they suffered from obstructive alterations in the respiratory tract. No restrictive pulmonary changes were observed. Scintigraphic ventilation tests were performed on 44 cases. 34 (77%) tests indicated absolutely normal results. Some rare local deficits of lung ventilation were based on adhesive anatomic alterations of the thoracic skeleton and the diaphragm. The lung function tests conducted in 29 persons ages 6 to 25 years revealed that the vital capacity and the forced expiratory volume were all normal. We found an increase of the intrathoracic gas volume in 11 infants (disposition to pulmonary inflation). In 9 cases we observed a decrease in the mid expiratory flow curves and in 6 patients we measured an increase in the specific resistance of airways (tendency towards obstructive airway alterations). Nevertheless even former hypoplastic lungs revealed a good extensibility.

Adolescent↗

Respiratory inductance plethysmography used to diagnose bilateral diaphragmatic paralysis: a case report.

OBJECTIVE: To report the use of respiratory inductance plethysmography in the diagnosis and management for a case of bilateral diaphragmatic paralysis after repeated sternotomies in a 23-month-old child. DESIGN: Case report. SETTING: A 15-bed pediatric cardiothoracic intensive care unit in an academic children's hospital. INTERVENTIONS: The patient could not be weaned from the ventilator after a repeat sternotomy for pulmonary artery reconstruction. Pulmonary function test results were within normal limits, and plain film radiography, ultrasonography, and fluoroscopy were unable to establish a definitive diagnosis. Evaluation of thoracoabdominal synchrony was undertaken using respiratory inductance plethysmography (RespiTrace). The work of breathing was assessed using esophageal manometry to obtain the pressure-rate product. RESULTS: During spontaneous breathing, complete thoracoabdominal asynchrony was noted, with clockwise Konno-Mead loops and associated phase angles of nearly 180 degrees. The pressure-rate product was 120 cm H(2)O/min, indicating elevated work of breathing. The pressure-rate product decreased dramatically, as indicated by measurement and observation, in response to increased levels of continuous positive airway pressure. CONCLUSIONS: The diagnosis of bilateral diaphragmatic paralysis can be confirmed by measurement of thoracoabdominal synchrony. Therapeutic and diagnostic application of continuous positive airway pressure may predict response to diaphragmatic plication. Controlled trials comparing measurement of thoracoabdominal synchrony with standard methods for the early diagnosis of diaphragmatic paralysis are needed.

Diaphragm↗

Cardiopulmonary function in fibrodysplasia ossificans progressiva.

Cardiopulmonary function was evaluated in 21 patients with fibrodysplasia ossificans progressiva. Neither cardiac enlargement nor failure was observed, but six patients had abnormal electrocardiograms. All had marked restrictive spirometry because of chest wall fixation and depended upon diaphragmatic respiration. The severity of chest restriction was independent of sex, age, duration of disease, and extnet of other physical disability. Progression to chronic respiratory failure was not observed. Chest infection in the presence of diminished pulmonary reserve is the major hazard of life in this rare disease and prophylactic measures should be considered.

Adolescent↗

Diaphragmatic pressures: transvenous vs. direct phrenic nerve stimulation.

Diaphragmatic force, determined by stimulating the phrenic nerve while simultaneously measuring the pressures in a closed respiratory system, was assessed in five anesthetized dogs over a 5-h period to evaluate the inherent variability of this technique. Transdiaphragmatic pressure (Pdi) was measured at functional residual capacity during stimulation (120 Hz, 0.2-ms duration) of one phrenic nerve by either direct phrenic nerve stimulation (DPNS) or transvenous phrenic nerve stimulation (TPNS). An analysis of variance showed no significant (P greater than 0.50) change during the 5-h period. There was a significant correlation (r = 0.94, P less than 0.001) between Pdi obtained by TPNS and that obtained by DPNS. It is concluded that either DPNS or TPNS can be used to evaluate diaphragmatic strength over a 5-h period and that TPNS can be used in lieu of DPNS.

Animals↗

Case report: Morgagni hernia.

The case reported here is a 32-year-old man with a sudden onset of chest pain and an acute deterioration of lung function. An incarcerated Morgagni hernia was diagnosed with a computer tomographic CT scan, and repaired electively via a midline laparotomy. Morgagni hernia is a rare type of congenital diaphragmatic hernia, which may not be symptomatic until adulthood when the patient presents with acute symptoms or incarceration.

Adult↗

Initial experience with non-thoracic, extraperitoneal, off-pump insertion of the Jarvik 2000 Heart in patients with previous median sternotomy.

BACKGROUND: Successful implantation of left ventricular assist devices (LVADs) in patients with previous median sternotomy remains challenging. METHODS: Seven patients underwent implantation of a Jarvik 2000 Heart by an extrathoracic, extraperitoneal, sub-costal surgical approach, which allows easy exposure of the diaphragmatic surface of the heart and the supraceliac aorta. All patients were at very high risk and were receiving high doses of inotropic agents to maintain their cardiac function. All had undergone prior median sternotomy. We compared data for blood loss and intensive care unit (ICU) stay with those of 15 patients in whom the HeartMate I vented electric LVAD was placed through a re-do sternotomy incision. RESULTS: All 7 patients survived the surgical procedure for implantation of the Jarvik 2000. All 7 patients were rapidly rehabilitated and had a short stay in the ICU (mean 3.3 days, range 1 to 8 days), as compared with the re-do HeartMate patients (mean 10.3 days, range 3 to 33 days) (p = 0.005). The average 12-hour blood loss was 635 ml in the Jarvik patients compared with 2,405 ml in the re-do HeartMate patients (p = 0.028). The cardiac index improved significantly in all Jarvik patients. CONCLUSIONS: The extrathoracic, extraperitoneal, sub-costal surgical approach is less invasive than a median sternotomy and allows the Jarvik 2000 to be implanted quickly and without cardiopulmonary bypass (CPB). By avoiding CPB and an extensive mediastinal dissection, bleeding is decreased in these hypocoagulable patients with compromised end-organ function. The decreased operative morbidity and mortality associated with this technique may allow consideration of the Jarvik 2000 Heart for safe and effective implantation in home-bound New York Heart Association (NYHA) Class III and IV patients.

Blood Loss, Surgical↗

[Disseminated erythematous lupus with bilateral diaphragmatic involvement].

The diaphragm is an unusual localization for disseminated erythematous lupus and is generally not recognized. We present the case of a 34-year-old woman who developed bilateral pain in the base of the thorax 15 days before hospitalization without any bronchial signs and NYHA stage II dyspnea. The patient's general health status remained satisfactory and the physical examination was normal except for diffuse inflammatory joint pain and cutaneous photosensitivization. The chest x-ray disclosed ascension of both hemidiaphragms with retracted lungs associated with bilateral basal atalectasia. The diagnosis of lupus with diaphragmatic involvement was retained due to the clinical presentation with diffuse joint pain, photosensitization with facial erythema, pericardial effusion and elevated antinuclear antibody and lymphopenia (1 100/mm3). Lung function tests revealed a restrictive syndrome. Oral corticosteroids 1 mg/kg/d enabled clinical and functional improvement. In light of this observation we discuss the pathogenic mechanisms of this uncommon localization of lupus and the difficulty of establishing a sure diagnosis.

Adrenal Cortex Hormones↗

Cardiac anatomy revisited.

In tomorrow's world of clinical medicine, students will increasingly be confronted by anatomic displays reconstructed from tomographically derived images. These images all display the structure of the various organs in anatomical orientation, this being determined in time-honoured fashion by describing the individual in the 'anatomical position', standing upright and facing the observer. It follows from this approach that all adjectives used to describe the organs should be related to the three orthogonal planes of the body. Unfortunately, at present this convention is not followed for the heart, even though most students are taught that the so-called 'right chambers' are, in reality, in front of their 'left' counterparts. Rigorous analysis of the tomographic images already available, along with comparison with dissected hearts displayed in attitudinally correct orientation, calls into question this continuing tendency to describe the heart in terms of its own orthogonal axes, but with the organ positioned on its apex, so that the chambers can artefactually be visualized with the right atrium and right ventricle in right-sided position. Although adequate for describing functional aspects, such as 'right-to-left' shunting across intracardiac communications, this convention falls short when used to describe the position of the artery that supplies the diaphragmatic surface of the heart. Currently known as the 'posterior descending artery', in reality it is positioned inferiorly, and its blockage produces inferior myocardial infarction. In this review, we extend the concept of describing cardiac structure in attitudinally correct orientation, showing also how access to tomographic images clarifies many aspects of cardiac structure previously considered mysterious and arcane. We use images prepared using new techniques such as magnetic resonance imaging and computerized tomography, and compare them with dissection of the heart made in time-honoured fashion, along with cartoons to illustrate contentious topics. We argue that there is much to gain by describing the components of the heart as seen in the anatomical position, along with all other organs and structures in the body. We recognize, nonetheless, that such changes will take many years to be put into practice, if at all.

Aorta↗

Pulmonary function and metabolic physiology of theropod dinosaurs

Ultraviolet light analysis of a fossil of the theropod dinosaur Scipionyx samniticus revealed that the liver subdivided the visceral cavity into distinct anterior pleuropericardial and posterior abdominal regions. In addition, Scipionyx apparently had diaphragmatic musculature and a dorsally attached posterior colon. These features provide evidence that diaphragm-assisted lung ventilation was present in theropods and that these dinosaurs may have used a pattern of exercise physiology unlike that in any group of living tetrapods.

Journal Article↗

[Cerebellar hypoplasia in the newborn: association with respiratory control disorders and mental retardation].

Cerebellum malformations are frequently diagnosed since the advent of the neuroradiological studies (computed tomography, magnetic resonance imaging and ultrasonography). Cerebellar hypoplasia is found in association with a wide variety of neurologic and systemic disorders. Clinical picture in newborn may be different than in other periods of life. Two characteristics are interesting in neonatal period: their relation with abnormal respiratory control and mental retardation. The pathophysiology of cerebellar hypoplasia is uncertain, however experimental studies suggest than an abnormality of the Bergman glia may lead to the observed granulle cell layer deficiency in this malformation. It is tempting to speculate that a similar migrational abnormality in the cerebrum accounts for the intellectual impairment seen in some affected patients. Respiration is a complex neural function requiring precise coordination of numerous neural circuits. Cerebellar hypoplasia may be important in the pathogenetic mechanism of abnormal respiratory control due to cerebellar respiratory control disturbance. Also diaphragmatic dysfunction may occur in association with cerebellar atrophy. We report two newborns with cerebellar hypoplasia (vermis and hemispheres) associated with central respiratory and neurological dysfunction, and mental retardation.

Body Weight↗

[Diaphragmatic electromyogram and respiratory pattern after unilateral and bilateral partial denervation of the diaphragm in the cat].

In the present study we investigated the mechanism of early respiratory compensation of partial paralysis of the sternal and lateral diaphragm due to an unilateral or bilateral section of the C5 rootlet of the phrenic nerves in anesthetized cats. Compensatory effects were evaluated from the recordings of the bilateral diaphragmatic EMGs, neural respiratory pattern and ventilation. The results of the study demonstrate that successive C5 denervation of the diaphragm caused a decrease in the ipsilateral diaphragmatic EMG. Bilateral C5 section evoked an up to 10 percent decrease in minute ventilation. The compensation of the unilateral and then bilateral partial impairment of the muscle function was achieved always by an increase in the neuromuscular projection to the currently contralateral diaphragm. Neural mechanisms of compensation involve a general increase in the respiratory drive, expressed mostly as an increase in the frequency of breathing. The contribution of afferent respiratory muscles to these mechanisms is likely.

Animals↗

Volume reduction surgery in the native lung after single lung transplantation for emphysema.

The natural history of emphysema suggests that progression of disease in the native lung may contribute to late deterioration in respiratory function after single lung transplantation. In this report, we describe our experience with unilateral volume reduction surgery in three single lung transplant recipients with emphysema. Each patient had had a late decline in lung function with a recurrence of symptoms. Chest radiographs demonstrated hyperinflation of the native lungs with encroachment on the grafts. Serial pulmonary function testing documented progressive reduction in expiratory flows with increases in residual volumes. Exercise testing confirmed severe intolerance to maximal exercise. Unilateral volume reduction surgery was undertaken at 36, 39, and 55 months after transplantation without incident. Radiographs obtained after the procedures demonstrated restoration of normal diaphragmatic contour, decreased aeration of the native lungs, and improved inflation of the allografts. Exercise testing at 3 months documented a mean improvement in maximal oxygen consumption of 35%. Expiratory flows improved by a mean of 60%. Quantitative ventilation and perfusion scans, however, were essentially unchanged. This experience suggests that unilateral volume reduction surgery may be considered as an alternative strategy in single lung transplant recipients with emphysema who exhibit clinically significant functional deterioration. Differentiation of the adverse effects of hyperinflation of the native lung from other potential causes of late deterioration might not be necessary but may be predictive of the degree of functional improvement after volume reduction. The relief of thoracic overdistention seems to play a primary role in the improvement pulmonary function.

Aged↗

Altered motor control strategies in subjects with sacroiliac joint pain during the active straight-leg-raise test.

STUDY DESIGN: An experimental study of respiratory function and kinematics of the diaphragm and pelvic floor in subjects with a clinical diagnosis of sacroiliac joint pain and in a comparable pain-free subject group was conducted. OBJECTIVE: To gain insight into the motor control strategies of subjects with sacroiliac joint pain and the resultant effect on breathing pattern. SUMMARY OF BACKGROUND DATA: The active straight-leg-raise test has been proposed as a clinical test for the assessment of load transfer through the pelvis. Clinical observations show that patients with sacroiliac joint pain have suboptimal motor control strategies and alterations in respiratory function when performing low-load tasks such as an active straight leg raise. METHODS: In this study, 13 participants with a clinical diagnosis of sacroiliac joint pain and 13 matched control subjects in the supine resting position were tested with the active straight leg raise and the active straight leg raise with manual compression through the ilia. Respiratory patterns were recorded using spirometry, and minute ventilation was calculated. Diaphragmatic excursion and pelvic floor descent were measured using ultrasonography. RESULTS: The participants with sacroiliac joint pain exhibited increased minute ventilation, decreased diaphragmatic excursion, and increased pelvic floor descent, as compared with pain-free subjects. Considerable variation was observed in respiratory patterns. Enhancement of pelvis stability via manual compression through the ilia reversed these differences. CONCLUSIONS: The study findings formally identified altered motor control strategies and alterations of respiratory function in subjects with sacroiliac joint pain. The changes observed appear to represent a compensatory strategy of the neuromuscular system to enhance force closure of the pelvis where stability has been compromised by injury.

Adult↗

A study of the three-dimensional organization of the human diaphragmatic lymphatic lacunae and lymphatic drainage units.

The peritoneal stomata, lymphatic drainage units and subperitoneal terminal lymphatics, called lymphatic lacunae, form a specialized drainage system in the diaphragm, by which absorption of fluid in bulk, particles and cells is carried out in the peritoneal cavity. The aim of this study is to elucidate the three-dimensional organization and function of the subperitoneal lymphatic lacunae and lymphatic drainage units by using lymphatic casts in the scanning electron microscope (SEM), ODO (OsO4-DMSO-OsO4) freeze fracture, conventional SEM and the transmission electron microscope (TEM). The subperitoneal lymphatic lacuna is unique for its large size and its multiple morphology and can be recognized by its broad, flattened enlargement and the blind-ends of lymphatic vessels, from which extend numerous main lymphatic vessels and side branches. These lymphatic vessels communicate with each other and form a rich lymphatic plexus under the diaphragmatic peritoneum. Two layers of lymphatic networks, i.e. the subperitoneal plexus and the deeper plexus are found in the muscular portion. Only one layer is present in the tendinous portion of the human diaphragm. The lymphatic plexus is denser in the tendinous portion than that in the muscular portion. The lymphatic lacunae occur exclusively in the muscular portion of the human diaphragm. The lumina of lymphatic lacunae are separated from the peritoneal cavity by a barrier consisting of cuboidal mesothelial cells, endothelial cells of the lymphatic lacunae and intervening connective tissue forming a lymphatic drainage unit. All these three components of the lymphatic drainage unit abut upon each other, but are not linked by specialized junctions. The cuboidal mesothelial cells frequently extend valve-like cytoplasmic processes that bridge the subperitoneal channel and make give it a tortuous course. The fibrous layer of the connective tissue is arranged in fiber bundles and gives a three-dimensional network forming the floor of the peritoneal stomata and the roof of the lymphatic lacunae. Via the fibrous network, the cuboidal mesothelial cells and the endothelial cells of the lacunae come into close contact with each other and form short subperitoneal channels which connect the peritoneal cavity with the subperitoneal lymphatic lacunae. The lymphatic drainage units may regulate the material absorption of the peritoneal stomata from the peritoneal cavity. It is suggested that the peritoneal stomata together with the subperitoneal channels, lymphatic drainage units and lymphatic lacunae comprise an important diaphragmatic lymphatic drainage system which plays an important role in the absorption of materials from the peritoneal cavity.

Diaphragm↗