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

In order to better define the outcome of patients with neonatal congenital diaphragmatic hernia (CDH), 17 patients between 3 and 19 years of age, among 34 survivors from 100 CDH have been re-examined clinically. All had a lung radiography, lung function studies, and radionuclide (Technetium 99m, Xenon 133) lung scans. Three patients suffered from asthma, 2 had recurrent bronchitis, 4 poor tolerance to effort, 3 gastrooesophageal regurgitation leading to endobrachyoesophagus and oesophagitis in one, 3 had scoliosis. Lung scans demonstrated hypoperfusion of the herniated side (less than 40%) in 6 patients. Chest films showed hypovascularisation on the herniated side. Lung function studies, performed in 4 of these 6 patients, showed a restrictive syndrome in 1 patient. Our results confirm those in the literature: perfusion is more altered than ventilation. Chest films at one year of age, completed if necessary by radionuclide lung scans, allow identification of children who have important pulmonary hypoplasia. These children need a regular follow-up: respiratory, digestive and orthopedic complications must be treated in order to preserve the respiratory function in adulthood.
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PMID:[Long-term outcome of congenital diaphragmatic hernia. A study of 17 patients]. 179 45

Nineteen survivors of congenital diaphragmatic hernia repair were compared with age and sex matched control children six to 11 years after repair. All subjects were examined clinically and underwent lung function testing. The patients also had individual lung volumes assessed radiographically and had radionuclide (krypton-81 m, technetium-99 m macroaggregates) ventilation-perfusion (V/Q) lung scans. Four patients had pectus excavatum and two had mild scoliosis. Spirometric measurements were lower in the patients than in the control subjects but only the differences in peak expiratory flow and flow at 50% of expired vital capacity were significant. The radiographic left lung volumes in patients surviving left diaphragmatic repair were larger than expected at 49.3% (SD 2%), suggesting alveolar overdistension. V/Q scans showed a mismatch in the ipsilateral lung, mean Q (40% (7%] being significantly lower than mean V (47% (6%)). In seven patients who had required ventilation for four days or more perfusion to the ipsilateral lung was significantly lower (34% (6%)) than values for the 12 patients ventilated for less than four days (43% (6%)). Survivors of right diaphragmatic repair had a better outcome in terms of relative radiographic lung volumes and V/Q distribution. More severely affected children are now surviving repair of congenital diaphragmatic herniation, with residual pulmonary abnormalities that could produce functional impairment in adult life.
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PMID:Pulmonary sequelae in survivors of congenital diaphragmatic hernia. 231 75

Here we report on a girl with a translocation between 1 and 2 and duplication 1p and deletion 2q resulting in a multiple congenital anomaly syndrome including intrauterine growth retardation, microcephaly, hypotelorism, cleft palate, subglottic stenosis, umbilical hernia, scoliosis, anal atresia, bilateral calcaneovalgus, overlapping toes, and vertebral anomalies.
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PMID:A presumptive translocation 1p;2q resulting in duplication 1p and deletion 2q. 265 88

From 1958 to March 1987 we corrected 704 patients with pectus excavatum. The condition occurred more frequently in boys (544 patients) than girls (160 patients). In the majority of patients (86%), the defect was evident at birth or within the first year of life. Musculoskeletal abnormalities were identified in 133 patients (scoliosis, 107; kyphosis, 4; myopathy, 3; Poland's syndrome, 3; Marfan's syndrome, 2; Pierre Robin syndrome, 2; prune belly syndrome, 2; neurofibromatosis, 3; cerebral palsy, 4; tuberous sclerosis, 1; and congenital diaphragmatic hernia, 2). Sixteen patients had associated congenital heart disease. A family history of chest wall deformity was present in 37% of the cases and a history of scoliosis in 11%. Surgical correction was performed using a uniform technique for bilateral subperichondrial resection of the deformed costal cartilages and sternal osteotomy resecting a wedge of the anterior cortex and fracturing the posterior cortex. Anterior displacement was maintained with silk sutures closing the osteotomy defect. In 28 early cases, the sternum was secured by intramedullary fixation with a Steinman pin. All repairs were completed with a low complication rate (4.4%; pneumothorax, 11; wound infection, 5; wound hematoma, 3; wound dehiscence, 5; pneumonia, 3; seroma, 1; hemoptysis, 1; hemopericardium, 1). Six complications were associated with Steinman pin fixation (hemoptysis, seroma, hemopericardium, pneumothorax, 3). Major recurrence occurred in 17 patients (2.7%) and led to revision in 12. Satisfactory long-term results were achieved in the remaining 687 patients, with follow-up ranging from 2 weeks to 27 years. Mean follow-up was 4.3 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical repair of pectus excavatum. 320 60

The Rieger syndrome is characterized by mesoectodermal dysplasia of the iris and cornea, dental defects, in some cases short stature, abnormal external ears, hypertelorism, arachnodactyly, polydactyly, scoliosis, kyphosis, imperforate anus, umbilical hernia, myopathy and in a few cases mental retardation. This report presents a sporadic case of the Rieger syndrome, the findings in which include the characteristic ocular anomalies, shortness of stature, mental retardation, absent dental defects and a peculiar, heretofore unobserved, myopathy.
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PMID:Mesoectodermal dysplasia of the iris and cornea, mental retardation and myopathy: a sporadic case. 500 37

We report on a 31-week fetus with hydrocephalus, hypertelorism, microtia, short neck, vertebral and rib defects, scoliosis, omphalocele, exstrophy of bladder, absent external genitalia and pubic rami, imperforate anus, diaphragmatic hernia, defective lobulation of lungs, single kidney, bicornuate uterus, and flexion deformities of the limbs. Similar extensive anomalies in the rostral and caudal regions were described by Russell et al. [Pediatrics, 67:176-182, 1981] and Stewart et al. [Am J Med Genet, 45:426-429, 1993]. The patients described by them had a combination of the oculo-auriculo-vertebral sequence (OAV) and caudal deficiency sequence, whereas the patient reported here can best be described as a combination of OAV and OEIS (omphalocele, exstrophy of bladder, imperforate anus, spinal defects) complexes. The widespread malformations seen in our patient may be the result of an error during blastogenesis.
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PMID:OEIS complex with craniofacial anomalies--defect of blastogenesis? 780 30

It is often thought that survivors of congenital diaphragmatic hernia (CDH) have an isolated problem related to lung hypoplasia, and little data exist regarding the extrapulmonary problems of high-risk CDH patients who do survive. In 1990, the authors began a multidisciplinary follow-up clinic for CDH patients. Members of the program include representatives from the departments of surgery, pulmonary medicine, development, nursing, and nutrition. Since this program began, the authors have followed up on 33 infants who survived after treatment of high-risk CDH, ie, those who were symptomatic within 6 hours of birth. Twenty patients were treated with extracorporeal membrane oxygenation (ECMO). Neurological problems were common in these patients: seven children (21%) required hearing aids, and seven others had abnormal results with brain-stem auditory evoked response (BAER) testing. Extraaxial fluid collections or enlarged ventricles were present on head computed tomography scans of 10 children, and four children had clinical seizure activity. Fifteen patients had developmental delays, which improved rapidly once the children began to thrive. Six patients required eyeglasses or had strabismus, and one patient is congenitally blind. There were a variety of problems related to growth and nutrition, with six patients needing fundoplications, and 13 patients below the fifth percentile for weight. Of 10 patients with patch repairs, two had recurrent hernias. Six others required surgery for bowel obstruction. Eleven patients had pectus excavatum, usually mild, and four had mild to moderate degrees of scoliosis. There were undescended testicles in five boys, vesicoureteral reflux in two patients, and kidney stones in two patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Congenital diaphragmatic hernia: the hidden morbidity. 817 2

A 12-year-old girl with acute gastric volvulus associated with severe scoliosis and neurological impairment did well initially with nasogastric tube decompression. To prevent recurrence, anterior gastropexy was performed using percutaneous sutures through the abdominal wall and stomach with the gastroscope in place. Simultaneous laparoscopic guidance was used to ensure that the stomach was fixed in a nonrotated position. One year later, volvulus has not recurred. This method of gastropexy is indicated only in children with intermittent gastric volvulus who do not have an underlying abnormality such as diaphragmatic hernia.
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PMID:Laparoscopic-guided gastropexy for intermittent gastric volvulus. 830 16

The authors illustrate a case of right neonatal congenital diaphragmatic hernia (CDH) of Bochdalek with major pulmonary hypoplasia and postoperative massive mediastinal displacement to the right, leading to tracheal compression and recurrent respiratory distress at every attempt to wean the infant from the ventilator. Mediastinal stabilization was obtained by placing an expansion prosthesis (of the cutaneous type) in the right hemithorax, to prevent mediastinal obstruction of the main airways. This procedure may prove to be an original and efficient solution for the critical postoperative respiratory obstruction due to severe pulmonary hypoplasia and mediastinal mass effect in the newborn with CDH. However, this may not prevent progressive scoliosis, a serious problem frequently seen in the absence of one lung.
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PMID:Mediastinal stabilization by an expansion prosthesis in postoperative congenital diaphragmatic hernia with severe pulmonary hypoplasia. 855 35

We report here a case of an asymptomatic lumbar hernia through a congenital defect of the posterior abdominal wall secondary to hypoplasia of the left part of the neural arch of L5 and S1. The bone defect was related to the neurofibromatosis of the patient. Plain radiograms showed a scoliosis and the bone defect. CT scan confirmed the osseous abnormalities and demonstrated a left colonic herniation through the bone defect. Many cases of lumbar hernia secondary to weakness of the abdominal muscles or following bone defects after bone graft harvesting, surgery or trauma have been described. This is, to our knowledge, the first case report of a lumbar hernia secondary to a congenital bone defect.
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PMID:[Colonic lumbar hernia secondary to congenital bone anomalies in a case of neurofibromatosis type I]. 873 10


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