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At least 19 recordsLinked to original sources
PubMed1977

Pyloroduodenal deformity due to liver malformation associated wtih omphalocele.

While congenital malformed and malpositioned livers are rare, they are relatively common in patients with omphaloceles. A malpositioned, malformed liver causing pyloroduodenal deformity and apparent pyloric obstruction in an infant or child with an omphalocele has not been previously reported. Thee such cases are described in which symptoms of upper gastrointestinal obstruction developed shortly after primary closure of the omphaloceles. Recognition of this condition at the time of primary closure of an omphalocele may allow consideration of additional procedures to prevent postoperative pyloric obstruction.

Constriction, Pathologic↗
PubMed1975

[Incidence and etiology of omphaloceles (author's transl)].

Omphaloceles are congenital malformations with herniation of the abdominal viscera into the umbilical cord. The incidence in newborns is 1:4300, the sex ratio 1.24:1 in favour to boys. Children with this malformation are neither more frequent at the beginning nor at the end of the sibship; the average maternal age is not increased. In about 50% other different malformations are found. All those omphaloceles, which are found as an obligatory symptom in the EMG syndrome (Wiedemann-Beckwith syndrome) to be probably in most cases autosomal recessively inherited or which are seen within a chromosomal malformation syndrome are of etiologically known origin. Omphaloceles without other malformations are mainly sporadic, less frequent in sibs, possibly due to a polygenic or multifactorial mode of inheritance. Findings in population genetics and animal experiments suggest that exogenous factors, too, can be the cause of malformation syndromes with omphaloceles.

Abdominal Muscles↗
PubMed1979

Gastroschisis and omphalocele.

A series of 100 patients with gastroschisis and omphalocele is presented. It is emphasized that gastroschisis generally has a strikingly homogeneous clinical presentation, while the omphalocele has a more heterogeneous clinical presentation, varying from the smallest to the largest, nearly incorrectable type. The main problem in the two groups is the problem of the closure of the defect. In gastroschisis there are, in addition, two problems related to the length of the intestinal tract and the tendency to peritonitis and septicaemia. In omphalocele, however, the major problem is the associated anomalies. The experience from the present material indicates that greater efforts must be used in the future in trying to avoid hypothermia, both during transportation and during operation. Furthermore, greater efforts must be made in closing the abdominal wall defect primarily in both groups of patients. More liberal use of respirator and total parenteral nutrition, mainly by peripheral veins, has obviously contributed to the improvement in our results from 1 60% mortality rate in the first nine-year period to a 37% mortality rate in the last nine-year period in the omphalocele group. Our best results have been obtained during the last 3 years with 2 deaths in the last 12 patients who all were treated by primary closure. In the gastroschisis group a survival rate of 69% in the whole series and of 74% in those who were primarily completely closed must be looked upon as progress in the light of earlier reports of survival rates of 36% to 43%.

Abdominal Muscles↗
PubMed1975

Treatment of gastroschisis and omphalocele with biological dressings.

Porcine skin grafts and human amniotic membranes have been used as biological dressings for the treatment of gastroschisis or omphalocele in 16 newborn infants. Eleven infants with gastroschisis were managed initially by the Silastic pouch technique but delayed healing resulted in separation of the Silastic sheeting from the wound margins and required use of biologic dressings to achieve complete closure. Biological dressings were used in five patients with intact omphaloceles for all or part of their course. Average time to achieve complete closure was 55 days. Three patients died of associated anomalies. One patient developed an enteric fistula. Biologic dressings appear to be useful adjuncts in the management of some patients with gastroschisis or omphalocele.

Abdominal Muscles↗
PubMed1979

Prognostic factors in omphalocele and gastroschisis.

Size of the abdominal wall defect, viscera exposed or herniated, birth weight, associated medical conditions and congenital anomalies, mode and complications of treatment, and the use of total parenteral alimentation was reviewed in 79 cases of omphalocele and 44 cases of gastroschisis treated in the past 10 yr. Sixty-seven percent of infants with omphalocele and 73% of those with gastroschisis survived. In omphalocele, the most important factors affecting mortality were the presence of other associated abnormalities and low birth weight. The size of the abdominal wall defect, the viscera herniated, and the mode of treatment did not appear to affect mortality. In gastroschisis, the size of the abdominal defect, birth weight, viscera exposed or herniated, and associated anomalies were not significant factors affecting mortality. Mortality was usually secondary to intestinal or wound complications. Statistical analysis could not prove that primary repair resulted in greater survival than the use of a silon pouch, but analysis of complications clearly indicates that the former method is preferable and that silon pouch should be reserved for cases in which primary repair is not possible.

Abdominal Muscles↗
PubMed1978

Amniotic fluid alpha-fetoprotein elevation with fetal omphalocele and a possible mechanism for its occurrence.

Prenatal diagnosis of genetic disease and congenital malformations has become a major area of study in obstetrics. The assessment of amniotic fluid alpha-fetoprotein (AFP) is useful for the diagnosis of neural tube defects. As more patients have been evaluated abnormal increases have been found in other defects, such as omphalocele, duodenal atresia, and congenital nephrosis. Two patients with omphalocele are reported with AFP measurement. A tenfold elevation of AFP was found in the first patient. In the second case a small omphalocele associated with exstrophy of the cloaca was not accompanied by an abnormal AFP increase. The mechanism of AFP elevation is discussed.

Adult↗
PubMed1978

A new nonoperative treatment of large omphaloceles with a polymer membrane.

The management of a huge omphalocele that cannot be primarily closed presents many difficulties. When surgery is technically impossible, the application of a Silon pouch carries a serious risk of infection and painting the sac with tinctures has its own peculiar problems. A newborn baby with a large unruptured omphalocele was successfully treated by covering the sac with a skin-like polymer membrane that is flexible, elastic, and impervious to bacteria and water. The covering did not adhere to the underlying tissues and did not interfere with gastrointestinal function. Infection was not a problem. This material was wrapped around the trunk and covered with a dry sterile dressing. The entire dressing was removed after 3 wk, revealing a small residual area of granulations that subsequently healed at home. This baby was not operated upon. We have also used this technique in a second newborn in whom the sac had ruptured. The rent was repaired and the membrane applied. This method does not eliminate later closure of the large ventral hernia, but ccertainly eliminates the necessity for surgery in the newborn period.

Bandages↗
PubMed1978

Renal malposition associated with omphalocele.

Nine patients with omphalocele had abnormally positioned kidneys; in 8, the kidneys were more cephalad than normal, immediately subdiaphragmatic in position. In one patient the kidneys were more caudal than normal. This renal malposition should be recognized in order to avoid unnecessary imaging procedures in patients with omphalocele.

Adult↗
PubMed1977

A pneumatic reduction device for gastroschisis and omphalocele.

A new preformed pneumatic reduction device is available in two sizes, and it is recommended that a set be available for emergency use in any hospital which deals with the definitive emergency therapy of the newborn. The devices can be washed and flash sterilized as needed and, if properly cared for, can be reused repeatedly. The bag has facilitated the early care of newborns with omphalocele and gastroschisis and has allowed the rapid and effective reduction of eviscerated intestines. These bags can also be used for the controlled ventral hernias necessary in the treatment of infants born with large diaphragmatic hernias. It is hoped that the search for better techniques and materials will be continued, since the dramatic improvement in the survival rate of infants with gastroschisis and omphalocele has occurred not from a better understanding of the defects but by the application of improved techniques and materials in their treatment.

Abdominal Muscles↗
PubMed1979

[Omphalocele and gastroschisis: clinical differences and surgical considerations. A ten year review (author's transl)].

31 infants with anterior abdominal wall defects were treated over the past 10 years. Despite the falling birth rate gastroschisis occurred almost twice as often as omphalocele (19 and 12 cases, respectively). The mortality rate of 71% in omphalocele were related to large defects, rupture of the sac and associated cardiac malformations. The 73% incidence of prematurity, additional malformations and sepsis contributed to the mortality rate of 58% in gastroschisis.

Abdominal Muscles↗
PubMed1979

Omphalocele and partial trisomy 1q syndrome.

A male infant with partial trisomy 1q syndrome (46,XY,der(21),t(1;21)(q25;q22)pat) is described. Clinical findings include small for gestational age, hypoglycemia, ocular hypertelorism, microphthalmia, coloboma of the iris, low-set ears, beak nose, micrognathia, micropenis, cryptorchidism, presacral dimple, flexion contractures of the fingers, bifid thumb, Simian crease, and overriding toes. In addition, he had a large omphalocele not previously seen in trisomy 1q. Postmortem findings include underdeveloped cerebellum, a thymus with moderate depletion of thymocytes, a large PDA, ASD, small adrenal, and fatty change of the liver. The grandfather, father, and a male sibling have translocation t(1;21)(q25;q22). A family history of repeated spontaneous abortions is present.

Abnormalities, Multiple↗
PubMed1979

Parachute mitral valve, coarctation of aorta, radius aplasia, and omphalocele in an infant with the trisomy 18 syndrome.

A premature infant with trisomy 18 had cardiac defects including parachute mitral valve, coarctation of the aorta, and a subcristal ventricular septal defect. The parachute mitral valve was funnel-shaped with a moderately-thick cone of fibrous tissue around the annulus and an eccentric orifice. Many forms of congenital cardiac defects have been described in the 18 trisomy syndrome. However, parachute mitral valve has not, to our knowledge, been part of the spectrum of cardiac defects in trisomy 18. Other anomalies were absent radii, omphalocele, and cleft palate and cleft lip; these have been observed previously in the 18 trisomy syndrome.

Abnormalities, Multiple↗
PubMed1979

Obstruction of the suprahepatic inferior vena cava as a complication of giant omphalocele repair.

Obstruction of the inferior vena cava with resultant Budd-Chiari syndrome after giant omphalocele repair has not previously been reported. Such a case is presented. We hypothesize the mechanism of this blockage to be posterior displacement of the inferior vena cava during reduction of the viscera and closure of the anterior abdominal wall, thereby creating angulation and mechanical blockage of the inferior vena cava at the diaphragmatic hiatus.

Budd-Chiari Syndrome↗
PubMed1975

Technical improvements in the management of omphalocele and gastroschisis.

1. Omphalocele and gastroschisis carried a poor prognosis (50 per cent survival) until the last half decade. 2. The use of a temporary prosthetic covering for the exposed viscera has strikingly altered the outlook in these conditions. 3. Ventilatory assistance, when required, and the ability to monitor blood gases have contributed also to the improved survival rates. 4. In gastroschisis the capability of giving total intravenous nutrition has allowed survival through the long period of bowel dysfunction common in the postoperative period.

Abdominal Muscles↗
PubMed1978

Mercury poisoning from mercurochrome therapy of an infected omphalocele.

A neonate with an infected omphalocele was treated locally with merbromin (mercurochrome) for five days. Extensive skin peeling with bullous lesions, edema, and fever developed three days after mercurochrome therapy. The infant died on the ninth day. Autopsy revealed evidence of heavy metal poisoning of the kidney, excessive mercury levels in the blood, and in tissues of the brain, kidney, and liver.

Fluoresceins↗