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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 1979 Cohen et Hayden and in 1983 Wiedemann et al. delineated a syndrome consisting of partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Hitherto the literature pertaining to this syndrome consists of somewhat more than 100 cases of which some, that have been described previously or subsequently under other headings, were rediagnosed as being Proteus syndromes. Of these, more than half show vascular anomalies closely resembling those observed in the Klippel-Trenaunay syndrome, but in the Proteus syndrome appear to be more haphazardly distributed over the integument. We report 3 pediatric patients with the Proteus syndrome, all showing cutaneous angiodysplasias. These patients were initially diagnosed as suffering from "severe or atypical Klippel-Trenaunay syndrome". In one of these, cardiac tumors were observed soon after birth which subsequently showed spontaneous involution and were therefore considered to be rhabdomyomas. In the Proteus syndrome cardiac pathology is rare, and cardiac tumors have not been described previously. Moreover, we observed umbilical hernia in two of our patients, a feature which has hitherto not been reported in patients with the Proteus syndrome. In all our patients a broad thoracic cage resembling a "body-builders chest", asymmetrical and disproportional macrodactyly and broad, flat feet were conspicuous. These broad, flat feet with macrodactyly and large spaces between the first and second digits were designed by the parents of one of our patient as "chimpanzee's feet". Macrodactyly, "chimp's" feet and a broad thoracic cage are considered by us to be clinical hallmarks of the Proteus syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Proteus syndrome. Expansion of the phenotype. Apropos of 3 pediatric cases]. 133 4

Antenatal correction of congenital diaphragmatic hernia in the human fetus is an attractive theoretical possibility that might reverse pulmonary hypoplasia before it becomes too severe for post-natal survival. Initial attempts at correcting these herniae in the human fetus have been beset with many technical problems, one of which has been to prevent acute obstruction to the ductus venosus when the left lobe of the liver is reduced back into the abdomen. We describe a new procedure intended to reduce the liver gradually so that the ductus venosus is never acutely kinked and more normal rib cage mechanics may be maintained.
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PMID:Intrathoracic silo for the potential antenatal repair of diaphragmatic herniae with liver in the chest. 150 52

Lung hernia is an abnormal protrusion of the lung beyond the confines of the thoracic cage. Hernias are classified as cervical, intercostal, or diaphragmatic. Each of these types can be either congenital or acquired. Acquired hernias can be spontaneous, posttraumatic, or pathologic as a result of a neoplastic or inflammatory process. This report describes five cases of lung hernias. Two were congenital cervical hernias, detected as incidental findings on chest radiographs of infants. One was a posttraumatic intercostal hernia detected on a chest radiograph and confirmed with CT. The remaining two were acquired intercostal hernias at the site of prior chest-tube drainage of tuberculous empyemas. Although both of these were clearly shown on CT scans, only one was visible on chest radiographs.
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PMID:Lung hernia: radiographic features. 210 25

Asphyxiating thoracic dystrophy (ATD) is a rare case of autosomal recessive disease. We report a case of full term female infant, who was noted to have small chest cage with severe respiratory distress soon after birth. On physical examination. The chest wall was fixed and small with the narrowest circumference about 29 cm in size, general cyanosis and distended abdomen were noted, there was a umbilical hernia around 1 cm in diameter. Otherwise, no other gross anomalies were found. Radiologic studies revealed short and horizontal ribs, small lung volume but depressed diaphragm, the clavicles and the spine were normal. The abdomen and long bone series all showed negative findings. The patient died of respiratory failure at 18 hours after birth. Autopsy was documented as a case of ATD. According to family history, there was one sibling die in the similar condition, although no autopsy available. Because the disease is transmitted as autosomal recessive trait, so 25% of next sibling will get the same condition. Therefore genetic consulting is necessary.
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PMID:[Asphyxiating thoracic dystrophy: a case report]. 217 59

Seventeen cases of different types of fetal thoracic and intrathoracic abnormalities were diagnosed prenatally by ultrasound: nonimmune hydrops (5 cases), diaphragmatic hernia (3), Potter's syndrome (3), chylothorax (1), exstrophy of the cloaca (1), the extreme form of prune-belly syndrome (2), cystic hygroma (1) and short rib polydactyly syndrome (Majevski type) (1 case). The ultrasonic features of nonimmune hydrops, diaphragmatic hernia, Potter's syndrome and cystic hygroma are well recognized by most experienced sonographers. The ultrasonic prenatal diagnosis of exstrophy of the cloaca, a very rare entity, has not been reported previously. The condition consists of a large infraumbilical anterior abdominal wall defect, lumbosacral myelomeningocele, and fetal ascites. The extreme form of prune-belly syndrome is associated with the absence of the abdominal wall musculature and marked dilatation of the urinary tract, presented ultrasonically as multiple large cysts occupying the distended fetal abdominal cavity. In exstrophy of the cloaca, prune-belly syndrome, Potter's syndrome and short rib polydactyly syndrome the chest abnormality is similar-extreme shortening of the thoracic cage, which has various causes. The differential diagnosis of all these entities and guidelines for their correct prenatal ultrasonic diagnosis are presented.
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PMID:Prenatal ultrasonic diagnosis of fetal thoracic and intrathoracic abnormalities. 352 48

The diaphragm is the most important muscle of respiration. It is believed that the abdominal contents affect diaphragmatic contraction by helping determine its length tension state and by acting as a fulcrum for this muscle to lift the rib cage and thereby increase lung volume. In support of these concepts we describe a patient with severe chronic obstructive pulmonary disease and a large midline hernia of the abdomen who, when standing, had a gastric pressure (Pg) of 4 cm H2O and a maximal transdiaphragmatic pressure (Pdimax) of 14 cm H2O. This was associated with an O2 saturation of 82%, lower thoracic and upper abdominal paradoxical breathing, and severe dyspnea. Once the hernia was reduced there was a rise in Pg to 12 cm H2O, of Pdimax to 27 cm H2O, and of O2 saturation to 89%. There was normalization of the breathing pattern and a decrease in dyspnea. Reduction of this patient's abdominal hernia resulted in an increase in her exercise tolerance.
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PMID:Respiratory consequences of abdominal hernia in a patient with severe chronic obstructive pulmonary disease. 396 6

Between November 1986 and April 1993, 22 cases of intrathoracic abnormality were detected prenatally by ultrasound, and examined postnatally. There were 11 cases of diaphragmatic hernia, 5 cases of cystic adenomatoid malformation of lung, one case of chylothorax, two cases of lung sequestration, and three cases of bronchogenic cyst. The total number of deliveries during that period was 48,281 and the total number of major anomalies at that time was 669 (1.38%). Cases of hydrothorax of various etiology, as well as thoracic cage anomalies were excluded. Prenatal diagnosis allows planned delivery and the assembly of neonatologists and pediatric surgeons.
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PMID:A survey of non-cardiac fetal intrathoracic malformations diagnosed by ultrasound. 804 76

Intercostal pulmonary hernia, a protrusion of lung parenchyma with overlying pleural membranes through an abnormal defect in the thoracic cage, is an uncommon phenomenon. Rib fractures caused by coughing similarly represent an infrequently occurring clinical presentation. Pulmonary herniation through an intercostal defect caused by cough fractures has been described twice in the literature to our knowledge. We present a case of pulmonary herniation secondary to cough fracture in a chronic bronchitic, successfully treated by thoracotomy with application of the basic principles of hernia repair, and a discussion of the mechanisms of injury.
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PMID:Surgical repair of intercostal pulmonary hernia secondary to cough induced rib fracture. 1250 Apr 83

The authors report a case of a lateral chest wall lung hernia, which was presumably congenital. Lung herniations are extremely rare, and congenital herniation of the lung through an apparently intact thoracic cage has not yet been reported. The hernia was repaired by the interposition of a polypropylene mesh. The literature of this rare condition is briefly reviewed.
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PMID:Intercostal lung hernia in a 7-year-old boy. 1615 Mar 63

The authors report their experience with 42 patients in whom anterior lumbar fusion was performed using titanium cages as a versatile adjunct to treat a wide variety of spinal deformity and pathological conditions. These conditions included congenital, degenerative, iatrogenic, infectious, traumatic, and malignant disorders of the thoracolumbar spine. Fusion rates and complications are compared with data previously reported in the literature. Between July 1996 and July 1999 the senior authors (C.I.S., R.P.N., and M.J.R.) treated 42 patients by means of a transabdominal extraperitoneal (13 cases) or an anterolateral extraperitoneal approach (29 cases), 51 vertebral levels were fused using titanium cages packed with autologous bone. All vertebrectomies (27 cases) were reconstructed using a Miami Moss titanium mesh cage and Kaneda instrumentation. Interbody fusion (15 cases) was performed with either the BAK titanium threaded interbody cage (in 13 patients) or a Miami Moss titanium mesh cage (in two patients). The average follow-up period was 14.3 months. Seventeen patients had sustained a thoracolumbar burst fracture, 12 patients presented with degenerative spinal disorders, six with metastatic tumor, four with spinal deformity (one congenital and three iatrogenic), and three patients presented with spinal infections. In five patients anterior lumbar interbody fusion (ALIF) was supplemented with posterior segmental fixation at the time of the initial procedure. Of the 51 vertebral levels treated, solid arthrodesis was achieved in 49, a 96% fusion rate. One case of pseudarthrosis occurred in the group treated with BAK cages; the diagnosis was made based on the patient's continued mechanical back pain after undergoing L4-5 ALIF. The patient was treated with supplemental posterior fixation, and successful fusion occurred uneventfully with resolution of her back pain. In the group in which vertebrectomy was performed there was one case of fusion failure in a patient with metastatic breast cancer who had undergone an L-3 corpectomy with placement of a mesh cage. Although her back pain was immediately resolved, she died of systemic disease 3 months after surgery and before fusion could occur. Complications related to the anterior approach included two vascular injuries (two left common iliac vein lacerations); one injury to the sympathetic plexus; one case of superficial phlebitis; two cases of prolonged ileus (greater than 48 hours postoperatively); one anterior femoral cutaneous nerve palsy; and one superficial wound infection. No deaths were directly related to the surgical procedure. There were no cases of dural laceration and no nerve root injury. There were no cases of deep venous thrombosis, pulmonary embolus, retrograde ejaculation, abdominal hernia, bowel or ureteral injury, or deep wound infection. Fusion-related complications included an iliac crest hematoma and prolonged donor-site pain in one patient. There were no complications related to placement or migration of the cages, but there was one case of screw fracture of the Kaneda device that did not require revision. The authors conclude that anterior lumbar fusion performed using titanium interbody or mesh cages, packed with autologous bone, is an effective, safe method to achieve fusion in a wide variety of pathological conditions of the thoracolumbar spine. The fusion rate of 96% compares favorably with results reported in the literature. The complication rate mirrors the low morbidity rate associated with the anterior approach. A detailed study of clinical outcomes is in progress. Patient selection and strategies for avoiding complication are discussed.
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PMID:Anterior lumbar fusion with titanium threaded and mesh interbody cages. 1691 6


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