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Query: UMLS:C0019270 (hernia)
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This report describes a 7-year experience with acute peritoneal dialysis in 31 neonates and infants less than 60 days of age. There were 20 boys and 11 girls, ages 3 to 60 days. Tenckhoff catheters of modified length were placed in the newborn intensive care unit (ICU), pediatric ICU, or surgery suites, and hourly exchanges (20 cc/kg) were started immediately postoperatively. Diagnoses included congenital metabolic disorders (11), acute tubular necrosis (6), postcardiopulmonary bypass with renal failure (5), renal cortical necrosis (5), obstructive uropathy (2), renal agenesis (1), and bilateral renal dysplasia (1). Complications included: peritonitis (4), bowel perforation (1), exit site infection (3), leaking dialysate (4), catheter obstruction (2), inguinal hernias (3), umbilical hernia (1), and retroperitoneal hemorrhage (1). There were 19 deaths (61.3%) from 1 to 90 days postinsertion in this high risk group. The (1), and post liver transplant (1). Effective dialysis (lowering of blood urea nitrogen (BUN) or ammonia, correction of acidosis, decrease in fluid overload) was possible in all cases. Five of the 12 survivors remain on chronic dialysis awaiting renal transplantation. Peritoneal dialysis is effective in the newborn period in the management of metabolic disturbances as well as renal failure. Morbidity and mortality (61.3%) is related to the near-morbid condition of the baby at the time of insertion and the severity of the complex underlying diagnosis often associated with multiorgan failure.
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PMID:Peritoneal dialysis in the first 60 days of life. 229 35

Accurate fetal diagnosis became possible by the steadily increasingly complex techniques of amniocentesis, ultrasound, and ultrasound-guided fetal blood sampling and chorion villous sampling. A high degree of diagnostic accuracy for a wide variety of structural and metabolic anomalies is required. The field of fetal diagnosis has been extended to the point that a journal dedicated to this subject alone is a viable proposition. It is becoming apparent, however, that lesions that were well known and well understood when recognized in neonatal life appear in general to have a worse prognosis if the lesion is diagnosed in utero. Fetal surgery began with attempts to perform in utero transfusions for babies with erythroblastosis fetalis. For a while, there was competition between open surgical procedures and the percutaneous placement of blood through catheters introduced into the fetal peritoneal cavity from outside the mother's abdomen. For fetal transfusion, closed techniques proved far safer and just as efficacious. There has been a worldwide interest in shunting of hydrocephalus and obstructive uropathy. The results of shunting hydrocephalus have been disappointing, with most of the patients surviving, but most of the survivors being severely handicapped. The results of shunting obstructive uropathy were that only about 50 per cent of the babies survived, but it appeared that those that did survive did well. Other lesions that have been shunted have been hydrothoraces or fetal ascites. A limited number of open procedures have been carried out in the last few years in San Francisco, and it may well be that diaphragmatic hernia (in appropriately selected patients) will be a lesion that can be corrected by in utero surgery. The future of this field is exciting, but before this form of treatment becomes routine, the ethical implications of the possibility of fetal surgery must be defined much more clearly than is currently the case.
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PMID:Fetal diagnosis and fetal surgery. 265 57

Oligohydramnios, whatever the cause, results in lungs that are 'hypoplastic' or at least smaller than normal. The fine structure in such lungs has not been reported previously. Three fetal lambs had both ureters ligated at 60, 72 and 58 days of gestation. They were sacrificed at 117, 143 and 142 days of gestation. The lungs were fixed by tracheal perfusion at 15 mm Hg. Individual lobar volumes were determined, and the lungs were sampled for electron microscopy. Total lung volumes for 2 of the lambs were very close to those previously reported for lungs of equivalent gestation with diaphragmatic hernia. The third lamb had a twin that had been unrecognised at the time of the original procedure. This lamb's lung volumes were normal. The morphological appearances of the lungs at the electron microscopy level are not normal but dramatically different from those previously reported in lambs with diaphragmatic hernia, phrenic nerve avulsion, and fetal tracheostomy. The lungs of the lamb with bilateral ureteric ligation and a normal twin were essentially normal. These preliminary findings point to the need for more studies of lung structure in oligohydramnios/obstructive uropathy models. However, the normal lungs in a lamb with grossly dysplastic kidneys and a normal twin argue against any important renal trophic factor in lung development in the fetal lamb. An interesting sidelight to this investigation was the unequivocal demonstration that early ureteric ligation results in true renal dysplasia in the fetal lamb. The kidneys of the lamb sacrificed at 117 days were smaller than normal and grossly dysplastic. The kidneys of the lambs that underwent ureteric ligation at 60 and 72 days were structurally different, with the ureters ligated at 60 days being small and dysplastic, while those with the ureters ligated at 72 days were larger and appeared almost polycystic.
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PMID:Development of fetal lamb lung and kidney in obstructive uropathy: a preliminary report. 307 15

Experience in fetal invasive therapeutic procedures is currently nearly entirely based on animal experimental research. Structural defects in the human fetus which are potentially amenable to intrauterine surgical treatment are obstructive uropathy, hydrocephaly and diaphragmatic hernia. The results from closed procedures such as shunting of urine or CNS fluid to the amniotic fluid compartment under ultrasound monitoring is as yet not very encouraging. This is partly determined by technical inadequacies, and partly due to insufficient information available on the possible presence of associated anomalies and functional aspects of the affected organ systems. The risks of invasive procedures for the mother and in particular the fetus must be weighed up against the advantages for the fetus if surgery has been successful. It seems that the development of open surgical procedures will determine the future of fetal surgery.
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PMID:[Prenatal diagnosis and therapeutic intervention]. 367 83

The increased use of ultrasonography in the management and evaluation of pregnancy has provided a unique opportunity to observe the anatomy of the developing fetus from 12 weeks gestation until term. Twenty-eight surgically important anatomic abnormalities have been diagnosed in utero by sonographic examinations at our affiliated institutions over the past three and a half years. These include ascites (five cases), gastroschisis (four cases), omphalocele (three cases), sacrococcygeal teratoma, cystic hygroma, hydrocele, duodenal atresia, multicystic kidney (two cases each), and one each of jejunal atresia, conjoined twins, ureteropelvic junction obstruction, urethral valves, urethral agenesis, and hydronephrosis secondary to reflux. Prenatal diagnosis by ultrasonographic examination has signifcantly improved perinatal management. Elective caesarean section has benefited infants with lesions causing dystocia, such as sacrococcygeal teratoma, omphalocele, and conjoined twins. Advance notification of surgeons and neonatalogists has reduced the delays of postnatal evaluation and treatment that contribute, significantly, to complications and death. In addition, transfer of the pregnant mother carrying an infant with a significant surgical anomaly to a center with facilities for neonatal surgery and specialized postoperative care can be properly planned for in advance. In the near future, intrauterine fetal surgery or palliative intervention may provide increased salvage of patients with obstructive uropathy and diaphragmatic hernia, both of which carry high mortality rates secondary to in utero damage. Sonography has proven useful in following the dilatation of either intestinal or urinary tract structures in utero. In our hands, maternal sonography has improved the surgical care of the newborn and may open a new frontier of intrauterine fetal surgery in the future.
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PMID:Maternal ultrasonography for the antenatal diagnosis of surgically significant neonatal anomalies. 645 75

Two patients with trisomy 11p15 and features of Beckwith-Wiedemann syndrome are reported. The first is a female infant with gigantism, macroglossia, abdominal hypotonia with umbilical hernia, moderate mental retardation, malformative uropathy, and atrial septal defect. Trisomy 11p15 was due to de novo duplication. The second patient was a stillborn (32-33 weeks pregnancy) with an abnormal tongue, posterior diaphragmatic eventration, inner organ congestion mainly of the adrenals. Trisomy 11p15 was due to a t(4;11)(q33;p14)pat. The association of trisomy 11p15 and Beckwith-Wiedemann syndrome is discussed with regard to cytogenetic data and the gene content of 11p, notably the genes coding for insulin and predisposition to Wilms tumour.
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PMID:Trisomy 11p15 and Beckwith-Wiedemann syndrome. A report of two cases. 674 43

The authors report a study on 148 female children demonstrating clinical evidence for inguinal hernias. The diagnosis was evaluated using Ducharme-Bensoussan's procedure for herniography hernia was documented in 143 cases, so which 44% revealed genital tract content. Eight cases produces unrelated pathological findings, especially crural hernia, nephro-uropathy diaphragmatic hernia. However, the main interest of the procedure is in evaluating the controlateral inguinal canal. Whereas three infants presented with bilateral symptoms, bilateral hernia was proven in 66 cases (46,2%) by herniography. In this way, the procedure is a sure way to ensure that hernia is really unilateral and to prevent any controlateral recur in the coming months.
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PMID:[Value of herniography in the therapeutic management of inguinal hernias in young girls. Apropos of 148 cases]. 685 Sep 71

Maternal ultrasound is commonly used for the evaluation and followup of pregnancy. Increasing experience in this field has led to the accurate sonographic description of both normal and abnormal fetal anatomy and to the antenatal diagnosis of increasing numbers of surgically correctable conditions. Antenatal diagnosis has the potential of greatly benefiting obstetrical care, the counseling and advising of parents, and perinatal surgical management. The use of intrauterine fetal surgery and/or manipulation may provide increased salvage of fetuses with diaphragmatic hernia and obstructive uropathy in the future. With the known safety and noninvasiveness of maternal sonography it may become a useful screening procedure for all pregnancies.
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PMID:Maternal ultrasound in neonatal surgery. 715 51

The success of balloon dissection techniques has given laparoscopic surgeons the option of a direct extraperitoneal approach to urologic disease. Unfortunately, these techniques are not without risks. We report on four cases of complications secondary to balloon dilation. In two instances, balloon misplacement caused dissection between muscle layers. This resulted in conversion to an open procedure in one instance and to a postoperative flank hernia in another. Balloon rupture with loose fragments occurred in two patients. In one of these cases, the peritoneum was lacerated, with balloon fragments displaced within the peritoneal cavity.
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PMID:Complications of extraperitoneal balloon dilation. 887 38

Ultrasonic diagnosis is indispensable in perinatal medicine. The applications are; 1) diagnosis of early pregnancy, 2) diagnosis of fetal life, 3) diagnosis of pregnancy weeks by fetal crown rump length, 4) evaluation of fetal growth by biparietal diameter, femur length, abdominal size, or estimated fetal weight, 5) detection of early abnormalities in blighted ovum, fetal death, hydatidiform mole, ectopic pregnancy, etc., 6) diagnosis of fetal anomalies, e.g. anencephaly, hydrocephaly, neck hygraoma, diaphragmatic hernia, congenital heart disease, intestinal obstruction, renal anomalies, obstructive uropathy, etc., 7) diagnosis of fetal diseases, e.g. hydrops fetalis, 8) diagnosis of placenta previa, hydramnios, oligohydramnios, cord coiling, excessive cord twisting, 9) detection and prediction of fetal compromise by fetal and uterine blood flow velocity wave forms, 10) further precise diagnosis by transvaginal sonography, 11) interventional ultrasound in genetic diagnosis with amniocentesis and chorionic villi sampling, fetal blood sampling with cordocentesis, 12) detection of fetal hypoxia by ultrasonic fetal monitor, fetal actocardiogram, and ultrasonic Doppler flowmetry.
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PMID:Ultrasound in fetal diagnosis and therapy. 1012 69


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