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At least 37 records · Page 2Linked to original sources
PubMed1979

Gastrointestinal anomalies associated with esophageal atresia or tracheoesophageal fistula.

A retrospective analysis of 150 consecutive patients with esophageal atresia and/or tracheoesophageal fistula was undertaken. The incidence and variety of gastrointestinal (GI) anomalies, the method of management, and the cause of death were critically reviewed to outline preferred methods of management. Of these patients, 87 were male and 63 female. There were 62 GI anomalies in 40 patients. The most common GI anomalies included anorectal anomalies in 17 patients, malrotation of the midgut in 13 patients, duodenal atresia or stenosis in ten patients, and annular pancreas and ectopic pancreatic tissue in three patients each. Six patients had the combination of tracheoesophageal fistula, duodenal obstruction, and anorectal anomaly. Of the 150 patients, 23 have died, with 15 deaths occurring in those with associated GI anomalies. The GI anomalies contributed directly to the deaths of five of these 15 patients. Delay in diagnosis and, on occasion, improper initial management resulted in increased morbidity and mortality.

Abnormalities, Multiple↗
PubMed1975

Congenital diaphragmatic hernia (Bochdaleck) with special reference to the prognostic influence of congenital cardiac and gastro-intestinal anomalies.

Of 29 patients with hernia Bochdaleck there were 11 in the non surgical group and 18 in the surgical group. 14 were operated in the first 24 hours of life, and 4 patients after that time. No patients in the non-surgical group survived. In patients who were operated upon in the first 24 hours of life, 5 survived, and after that time 3. In all cases the transthoracic approach was used. The investigation did not permit any safe judgement of the preoperative treatment. On these conditions multiple anomalies combined with prematurity and intrauterine asphyxia had some influence on, whether a patient lived so long, that an operation could take place. Gastro-intestinal anomalies had a certain prognostic significance in the postoperative period. In cured patients the severity of a complicating cardiac failure could be of some importance.

Abnormalities, Multiple↗
PubMed1977

alpha-Fetoprotein levels in pregnancies complicated by gastrointestinal abnormalities of the fetus.

alpha-Fetoprotein (AFP) levels have been measured in maternal serum and amniotic fluid in a variety of gastrointestinal abnormalities of the fetus. Maternal serum AFP levels were consistently elevated in abdominal wall defects of the fetus after 15 weeks gestation and the amniotic fluid levels were raised in 3 of the 4 patients measured. In atresia of the gastrointestinal tract and diaphragmatic hernia, serum AFP levels were usually normal unless there was an associated neural tube defect or multiple pregnancy, although the majority were not measured between 15 and 26 weeks gestation. If elevated amniotic fluid levels of AFP are used in the decision to terminate pregnancy on the assumption of a probable neural tube defect of the fetus, a proportion of terminations will be performed because of abdominal wall defects of the fetus.

Abdominal Muscles↗
PubMed1976

A 94-mm human fetus with the VACTERL association of anomalies.

A spontaneously aborted human male fetus of 94 mm crown-rump length had multiple malformations including atresia of the esophagus with tracheoesophageal fistula, anorectal agenesis, anomalies of the heart and great vessels, kidneys, bladder, and left testis, and a single umbilical artery. This association of defects has not previously been described in a fetus at this immature stage of development.

Abnormalities, Multiple↗
PubMed1977

Conjoined thoracopagus twins.

A case of vaginally delivered stillborn female thoracopagus conjoined twins is presented. Each twin had a cleft lip and cleft palate. They shared a common pericardial sac and a common abdominal cavity. The liver was conjoined, and the small bowel was common to both twins. One twin was asplenic. Although the twins were monozygotic, their viscera were neither identical nor mirror-image to each other, and the visceral anomalies were unique to each twin. The hearts were fused, and communications existed between the circulations at the atrial and ventricular levels. One twin had single atrium, solitary (right) ventricle, malposed great arteries, pulmonary valvular atresia and infundibular stenosis, right aortic arch with aberrant left subclavian artery and with left ductus arteriosus, and atresia of the common pulmonary vein. The other twin had two atrial septal defects of the fossa ovalis and sinus venosus types, partial anomalous pulmonary venous connection, persistent left superior vena cava to coronary sinus, solitary (left) ventricle, malposed great arteries, and left aortic arch with aberrant right subclavian artery and with left ductus arteriosus. There was atrial and ventricular fusion. On hypothetical grounds, and in a comparable anatomic situation, salvage of one twin might be possible by surgical means, while the other twin was sacrificed.

Abnormalities, Multiple↗
PubMed1976

Duplication of the alimentary tract.

Duplications of the intestinal tract as can be seen from the embryologic and anatomic descriptions are varied and frequently complex. The great variety of presentation and pathology encountered requires almost individual treatment for each case. Certainly total surgical excision, or at the very least, good complete internal drainage should be carried out in these cases. Correct therapy should afford relief of the patients's symptoms and prevent future complications.

Abnormalities, Multiple↗
PubMed1976

Patterns of anomalies in children with malformed ears.

Sixteen children with anomalies of the auricle and/or middle ear who presented malformations of the face, mouth, upper airway, spine, limbs, heart, gastrointestinal (GI), and/or genitourinary (GU) systems, were described. While clusters of anomalies suggested syndromes such as the oculo-auriculo-vertebral syndrome of Goldenhar, hamifacial microsomia, mandibulo-facial dysostosis (Treacher Collins syndrome), Pierre Robin, Klippel-Feil, Moebius, Duane, and/or VATER syndromes, many children did not fit what are usually considered even minimal criteria for these syndromes. Several children had malformations which fit the description of more than one syndrome. The importance of investigating the children for unsuspected anomalies, especially of the GU system, was emphasized. Life threatening problems in this group consisted of airway problems, congenital heart disease, and major anomalies of the GI and GU systems. Better management of sucking, swallowing and airway problems might have decreased the early morbidity and mortality (3/16) in this group. Children with multiple defacing anomalies may not be mentally retarded so that aggressive management of their visceral anomalies and hearing problems, and early educational intervention are mandatory. Delay in development may be due to hearing loss, vestibular impairment, ataxia, the consequences of early malnutrition, and multiple hospitalizations rather than to mental retardation. A pessimistic attitude in infancy is unwarranted since it is impossible to predict which children will end up competitive individuals.

Abnormalities, Multiple↗
PubMed1976

Congenital malformations associated with anencephaly and iniencephaly.

The necropsy reports of 294 cases of anencephaly and 50 cases of iniencephaly have been examined, and a tubulated list of associated malformations produced. Cases were divided by sex and the presence or absence of spina bifida. Forty-one per cent of the series had other malformations, and other malformations were more common in those cases with spina bifida than in those without. The most frequent single malformations were: hydronephrosis (8%), cleft palate (8%), diaphragmatic hernia (5%), exomphalos (5%), hare lip (4%), and horseshoe kidney (4%). It is suggested that the presence of other malformations in anencephaly or iniencephaly may imply some aetiological heterogeneity.

Abnormalities, Multiple↗
PubMed1978

Extracardiac malformations associated with congenital heart disease.

The incidence of significant extracardiac malformations was determined in a combined clinical and autopsy study comprising 1000 infants and children with congenital heart disease treated and lost at the Children's Hospital in Helsinki. There were 567 boys and 433 girls. 1/4 of the children had a birthweight of 2500 g or less. 850 children were under 1 yr old. Death occurred during the 1st mth of life in 546 cases. Extracardiac malformations were encountered in 439 children. They were more common in girls than in boys. The incidence of associated malformations was comparatively high in infants with a low birthweight. The noncardiac anomalies were considered main causes of death in 1/3 of the cases. Extracardiac organs were involved in the following order of frequency: alimentary, skeletal, urogenital, central nervous and respiratory system. Of the main cardiac malformations, septal defects were associated with the highest and transposition of the great arteries with the lowest incidence of extracardiac anomalies. An accumulation of some defined noncardiac malformations was observed in patients with certain heart lesions.

Abnormalities, Multiple↗
PubMed1979

[Duplication of the digestive tract in adults. 5 cases (author's transl)].

Five cases of duplication of the digestive tract are reported: oesophagus, stomach, duodenum, and colon. These patients were adults, in whom it is rare (5% of cases) to find such duplications. Two types of image are seen on barium meal examination: opacification of a lumen which may be abnormally blind and which can be easily diagnosed if it is short, but is sometimes more difficult to individualize if the duplication is long (colon); or an extrinsic type of compression which is a part of the wall and much more difficult to diagnose. Knowledge of these different types of radiological images in adults should avoid precipitous investigations and orientate possible surgical interventions.

Adolescent↗
PubMed1975

Congenital heart disease in Liverpool: 1960--69.

The incidence of congenital heart disease (C.H.D.) in Liverpool from 1960 to 1969 inclusive has been determined from the Liverpool Congenital Abnormalities Registry with a follow-up period of 3 to 12 years. The incidence is 6-6 per 1000 total births and this probably represents a very small degree of under-reporting. There is no consistent seasonal variation in the incidence of any of the main congenital heart lesions. In general, infants with C.H.D. tend to be of lower birth weight and born after shorter gestation than controls. This is most conspicuous with patent ductus arteriosus (P.D.A.). Females preponderate in P.D.A. and males in transposition. There is probably also a male preponderance in coarctation and aortic stenosis. Fallot's tetralogy is associated with increased maternal age and parity. Pregnancies leading to the birth of a baby with C.H.D. are complicated by threatened abortion more frequently than are controls. The concordance rate for C.H.D. in twins is low. Monozygotic twins are more liable to C.H.D. than are dizygotic twins. The incidence of C.H.D. in the siblings of affected propositi is 2-3 times that expected. Affected sibs often have the same lesion. About 20 per cent of infants with C.H.D. have associated major defects notably monogolism and defects of the alimentary, skeletal, genito-urinary and nervous systems. These are responsible for the early death of about one quarter of all infants born with C.H.D. The data presented here suggest that environmental rather than genetic factors are predominantly responsible for congenital heart disease.

Abortion, Threatened↗