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Perinatal management of exomphalos diagnosed in late pregnancy
ABSTRACTS
mm skin biopsies from the fetal scalp, trunk, or buttocks without significant bleeding. Biopsy sites seem to heal completely. Some of the diagnoses possible from fetal tissue samplying include ichthyosis, ichthyosiform erythroderma, and epidermolysis bullosa. Fetal blood sampling permits diagnosis of hemoglobinopathies, hemophilias, alpha~ Antitrypsin deficiency, chronic granulomatous disease, congenital neutropenia, red cell antigens, and Duchenne's muscular dystrophy. The procedure has considerable risk and fetal loss rates of about 5% are being reported from amnionitis, fetal trauma or exsanguination, and placental abruption. In addition, an increase in premature delivery of one and one half fold seems likely. In the future diagnostic tests will be increased in number and accuracy and it is possible that cells or other biologic materials may be injected into the circulation to treat the unborn child.--David L. Collins
205
dramnios and no normal kidney or bladders could be demonstrated. These cases were counseled that these anomalies were incompatible with life. In 4 patients unilateral lesions were demonstrated and successfully treated after delivery near term. Three fetuses with bilateral hydronephrosis secondary to urethral obstruction were diagnosed. Two that were born near term died of hypoplastic lungs with end stage hydronephrosis and facial and skeletal deformities. The other electively delivered at 32 wk and had none of the stigmata of Potter's syndrome. Early decompression salvaged sufficient renal function for survival. The authors mentioned they had developed techniques for sonographically guided percutaneous placement of catheters for surgical drainage of the urinary tract. Subsequent to submission of this article they have successfully performed this procedure and it has also been done successfully in San Diego.--David L. Collins
Management of the Fetus With A Correctable Congenital Defect. M. R. Harrison, M. S. Go/bus, and R. A. Filly
J A M A 246:774-777, (August 14), 1981.
Alpha-Fetoprotein: The Diagnosis of Neural-Tube Defects.
B. F. Crandall. Pediat Ann 10:78 85, (February), 1981.
The authors detail a total of 37 conditions which might be diagnosed and managed in the fetus. These are discussed under the following headings: 1. Managed by Selective Abortion---conditions such as anencephaly, inherited chromosomal defects, etc. 2. Detectable in Utero but Best Corrected After Delivery At Term intestinal atresia, and other surgically treatable deformities and tumors. 3. May Require Induced Preterm Delivery for Early Correction Ex U t e r o ~ conditions such as hydronephrosis, hydrocephalus, omphalocele, volvulus, hydrops, intrauterine growth retardation. 4. May Require Cesarean Delivery-monjoined twins, large cysts and tumors, hydrocephalus. 5. May Require Treatment in Utero-~leficient pulmonary surfactant, anemia due to erythroblastosis, hypothyroidism, methylmalonic acidonia (B~z dependent) multiple carboxylase deficiency (biotindependent), nutritional deficiency and intrauterine growth retardation, anatomic lesions that interfere with development, bilateral hydronephrosis (urethral obstruction), diaphragmatic hernia and obstructive hydrocephalus. It is in the last category that the authors are most interested and they have done extensive work in animals reported elsewhere in the creation and treatment of diaphragmatic hernia in utero. Although not reported in this article, they have also treated cases of bilateral hydronephrosis in the human fetus due to urethral valves by inserting a drainage tube into the bladder working percutaneously through the mother's abdominal wall and draining the bladder into the amniotic fluid. This has also been done in San Diego, This whole field may now represent the frontier of pediatric surgery and there is no question that the pediatric surgeons at the University of California, San Francisco are in the forefront of its development. David L. Collins Management of the Fetus with a Urinary Tract Malformation. M. R. Harrison, R. A. Filly, J. T. Parer, M. J. Faet,
J. B. Jacobson, and A. A. deLorimier J A M A 6:635-639, (August), 1981.
Thirteen fetuses are reported with urinary tract malformations diagnosed by prenatal ultrasound. Six had oligohy-
It is stated than an elevated amniotic fluid, alpha-fetoprotein (AFP), in the absence of fetal blood contamination is strong indication of fetal abnormality or complication. About 98% of anencephaly and open spina bifidas can be diagnosed in this way. Closed spina bifida rarely leads to elevated amniotic fluid AFP. Other conditions causing elevated amniotic fluid AFP include threatened abortion, fetal death, congenital nephrosis, omphalocele, esophageal and duodenal atresia, and cystic hygroma. Indications for amniocentesis and measurement of amniotic fluid AFP include: 1. all pregnancies undergoing second trimester amniocentesis for other reasons, 2. birth of a previous child with a neural tube defect (NTD), 3. a parent with NTD, 4. congenital nephrosis, 5. elevated maternal serum AFP which may have been done as part of a screening program to detect NTD, and 6. birth of a previous child with hydrocephalus.--David L. Collins
Perinatal Management of Exomphalos Diagnosed in Late Pregnancy. U. Nicolini, E. Ferrazzi, M. Belotti, G. Pardi,
C. A. dell'Agnola Z. Kinderchir. 33:275 278, (1981).
A case of exomphalos is reported in which the diagnosis was made during late pregnancy by ultrasound. Two hr before caesarian section a needle was inserted through the maternal abdominal wall into the exomphalic sac which was occupying most of the amniotic cavity, and 320 ml of fluid were aspirated. The authors emphasize that this prenatal procedure was able to minimize the risk of rupture of the exomphalic s a c . - - T h o m a s A. Angerpointner
Polydioxanone (POA}. A Novel Monofilament Synthetic Absorbable Suture. J. A. Ray, N. Doddi, D. Regula, J. A.
Williams and A. Melveger Surg Gynecol Obstet 153:497506, (October), 1981.
A new polymer, polydioxanone, has been converted into a uniquely flexible monofilament suture of all sizes. The strength of the polydioxanone suture prior to implantation in
mm skin biopsies from the fetal scalp, trunk, or buttocks without significant bleeding. Biopsy sites seem to heal completely. Some of the diagnoses possible from fetal tissue samplying include ichthyosis, ichthyosiform erythroderma, and epidermolysis bullosa. Fetal blood sampling permits diagnosis of hemoglobinopathies, hemophilias, alpha~ Antitrypsin deficiency, chronic granulomatous disease, congenital neutropenia, red cell antigens, and Duchenne's muscular dystrophy. The procedure has considerable risk and fetal loss rates of about 5% are being reported from amnionitis, fetal trauma or exsanguination, and placental abruption. In addition, an increase in premature delivery of one and one half fold seems likely. In the future diagnostic tests will be increased in number and accuracy and it is possible that cells or other biologic materials may be injected into the circulation to treat the unborn child.--David L. Collins
205
dramnios and no normal kidney or bladders could be demonstrated. These cases were counseled that these anomalies were incompatible with life. In 4 patients unilateral lesions were demonstrated and successfully treated after delivery near term. Three fetuses with bilateral hydronephrosis secondary to urethral obstruction were diagnosed. Two that were born near term died of hypoplastic lungs with end stage hydronephrosis and facial and skeletal deformities. The other electively delivered at 32 wk and had none of the stigmata of Potter's syndrome. Early decompression salvaged sufficient renal function for survival. The authors mentioned they had developed techniques for sonographically guided percutaneous placement of catheters for surgical drainage of the urinary tract. Subsequent to submission of this article they have successfully performed this procedure and it has also been done successfully in San Diego.--David L. Collins
Management of the Fetus With A Correctable Congenital Defect. M. R. Harrison, M. S. Go/bus, and R. A. Filly
J A M A 246:774-777, (August 14), 1981.
Alpha-Fetoprotein: The Diagnosis of Neural-Tube Defects.
B. F. Crandall. Pediat Ann 10:78 85, (February), 1981.
The authors detail a total of 37 conditions which might be diagnosed and managed in the fetus. These are discussed under the following headings: 1. Managed by Selective Abortion---conditions such as anencephaly, inherited chromosomal defects, etc. 2. Detectable in Utero but Best Corrected After Delivery At Term intestinal atresia, and other surgically treatable deformities and tumors. 3. May Require Induced Preterm Delivery for Early Correction Ex U t e r o ~ conditions such as hydronephrosis, hydrocephalus, omphalocele, volvulus, hydrops, intrauterine growth retardation. 4. May Require Cesarean Delivery-monjoined twins, large cysts and tumors, hydrocephalus. 5. May Require Treatment in Utero-~leficient pulmonary surfactant, anemia due to erythroblastosis, hypothyroidism, methylmalonic acidonia (B~z dependent) multiple carboxylase deficiency (biotindependent), nutritional deficiency and intrauterine growth retardation, anatomic lesions that interfere with development, bilateral hydronephrosis (urethral obstruction), diaphragmatic hernia and obstructive hydrocephalus. It is in the last category that the authors are most interested and they have done extensive work in animals reported elsewhere in the creation and treatment of diaphragmatic hernia in utero. Although not reported in this article, they have also treated cases of bilateral hydronephrosis in the human fetus due to urethral valves by inserting a drainage tube into the bladder working percutaneously through the mother's abdominal wall and draining the bladder into the amniotic fluid. This has also been done in San Diego, This whole field may now represent the frontier of pediatric surgery and there is no question that the pediatric surgeons at the University of California, San Francisco are in the forefront of its development. David L. Collins Management of the Fetus with a Urinary Tract Malformation. M. R. Harrison, R. A. Filly, J. T. Parer, M. J. Faet,
J. B. Jacobson, and A. A. deLorimier J A M A 6:635-639, (August), 1981.
Thirteen fetuses are reported with urinary tract malformations diagnosed by prenatal ultrasound. Six had oligohy-
It is stated than an elevated amniotic fluid, alpha-fetoprotein (AFP), in the absence of fetal blood contamination is strong indication of fetal abnormality or complication. About 98% of anencephaly and open spina bifidas can be diagnosed in this way. Closed spina bifida rarely leads to elevated amniotic fluid AFP. Other conditions causing elevated amniotic fluid AFP include threatened abortion, fetal death, congenital nephrosis, omphalocele, esophageal and duodenal atresia, and cystic hygroma. Indications for amniocentesis and measurement of amniotic fluid AFP include: 1. all pregnancies undergoing second trimester amniocentesis for other reasons, 2. birth of a previous child with a neural tube defect (NTD), 3. a parent with NTD, 4. congenital nephrosis, 5. elevated maternal serum AFP which may have been done as part of a screening program to detect NTD, and 6. birth of a previous child with hydrocephalus.--David L. Collins
Perinatal Management of Exomphalos Diagnosed in Late Pregnancy. U. Nicolini, E. Ferrazzi, M. Belotti, G. Pardi,
C. A. dell'Agnola Z. Kinderchir. 33:275 278, (1981).
A case of exomphalos is reported in which the diagnosis was made during late pregnancy by ultrasound. Two hr before caesarian section a needle was inserted through the maternal abdominal wall into the exomphalic sac which was occupying most of the amniotic cavity, and 320 ml of fluid were aspirated. The authors emphasize that this prenatal procedure was able to minimize the risk of rupture of the exomphalic s a c . - - T h o m a s A. Angerpointner
Polydioxanone (POA}. A Novel Monofilament Synthetic Absorbable Suture. J. A. Ray, N. Doddi, D. Regula, J. A.
Williams and A. Melveger Surg Gynecol Obstet 153:497506, (October), 1981.
A new polymer, polydioxanone, has been converted into a uniquely flexible monofilament suture of all sizes. The strength of the polydioxanone suture prior to implantation in