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PubMed1991

Doctors' decisions and prognostications for infants with Down syndrome.

Random samples of pediatricians, family practitioners and pediatric surgeons were surveyed with a questionnaire, which included three questions regarding their decisions about treatment of duodenal atresia in two hypothetical infants with Down syndrome, one with complete trisomy 21 and one with mosaicism. Results showed that doctors are more likely now to advocate surgery than in the past. Pediatricians and family practitioners had similar views, but pediatricians were more assertive about treatment if parents refused. Pediatric surgeons had the most pessimistic outlook and a greater number would not encourage surgery. There was a relationship between the doctors' prognostications and their decisions about treatment. These results emphasize the importance of ensuring that doctors have accurate perceptions about the capabilities of individuals born with Down syndrome.

Attitude of Health Personnel↗
PubMed1987

Rectovestibular fistula associated with colonic atresia.

A complete staged reconstruction has been performed on a 4-year-old girl with imperforate anus, rectovestibular fistula, and colonic atresia. The rectal remnant was a small 10 cm long segment, widely separated from the right colon. An end colostomy had been performed in the neonatal period elsewhere. Associated urinary anomalies were treated first. At the age of 4 years, distal colon remnant dilatations were started using a balloon catheter through the vestibular orifice. Four months later, end-to-end ascending colon-sigmoid anastomosis was done, after colonic mobilization and tapering. A temporary loop ileostomy was left at the left upper quadrant. Three months later a posterior sagittal anorectoplasty was successfully performed. At the age of 5 years she has achieved fecal continence, after the ileostomy closure.

Anus, Imperforate↗
PubMed1993

Autopsy findings in a severely affected infant with a 2q terminal deletion.

We describe a 37-week gestation female infant who was born with a terminal 2q deletion. Both of her parents had normal chromosomes. This infant had multiple anomalies, including hypertelorism, short palpebral fissures, microphthalmia, cleft lip/cleft palate, and abnormal ears. Autopsy documented Dandy-Walker malformation with severe hydrocephalus, aortopulmonary window, secundum atrial septal defect, duodenal atresia, incomplete rotation of the bowel, gonadal dysgenesis, uterus didelphys, and musculoskeletal defects. Compared with the other 6 children with 2q terminal deletion documented in the literature, this patient is the most severely affected. This patient is also the only one documented to have died and undergone autopsy examination. The findings in this case provide more data for the eventual description of a "terminal 2q deletion syndrome" and suggest that some abnormalities, such as gonadal dysgenesis, may be present in living children with this chromosome abnormality.

Abnormalities, Multiple↗
PubMed1986

The late nonfunctioning duodenal atresia repair.

Since 1979, three newborn males had duodenoduodenostomies for duodenal atresia, and all babies had smooth uncomplicated postoperative courses. There were no other anomalies. Between 6 and 18 months postoperatively, each infant developed an obstruction at the anastomosis that was initially treated with prolonged nasogastric suction. Because conservative management did not relieve the obstruction, the first two babies were operated on several times with revision of the anastomosis and bypass procedures, all of which were slow to function and required prolonged intravenous alimentation. It then became apparent that the duodenoduodenostomy was functionally obstructed. Therefore, the third infant was successfully treated with plication only of the dilated atonic proximal duodenum. All three children are now thriving more than 2 years after their surgery.

Duodenal Obstruction↗
PubMed2003

Diaphragmatic duodenal atresia: laparoscopic repair.

Stenosis due to a diaphragm is a type of intrinsic duodenal obstruction in newborns and even in childhood, when obstruction is partial. We present a case of a 13-month-old girl with diaphragmatic stenosis associated with a dilated first and second duodenum. Surgical management consisted of a partial excision of the diaphragm after vertical incision of the anterior part of the second duodenum followed by a transverse suture. This diamond-shaped anastomosis was successfully carried out laparoscopically. No tapering of the duodenum was performed as some authors suggest in cases of megaduodenum. The rapid resumption of peristalsis and fewer adherences than expected after such a minimally invasive approach could make a more invasive procedure unnecessary. Only long-term follow-up and greater experience will show which procedure is most suitable.

Constriction, Pathologic↗
PubMed1985

Ileocaecal atresia.

Meconium ileus is one of the commonest causes of distal small bowel obstruction in neonates. Within the last six years at our hospital two newborns presented with identical signs and symptoms of meconium ileus. Abdominal x-rays showed distal small bowel obstruction with ground glass opacity in the right lower quadrant. Gastrografin enema in both cases demonstrated a microcolon, but there was no reflux of this contrast material into the terminal ileum. Laparotomy in both babies revealed atresia of the ileocaecal valve with dilatation of the terminal ileum loops which contained normal meconium. Ileocolic resection was carried out in both neonates. Sweat chlorides and eventual recovery were normal. Pathology in one of the specimens showed muscularis passing across the atretic area with a telescoped pattern of the muscularis propria suggesting intussusception in utero. Although the presentation of a newborn may be that of meconium ileus, the fact that the Gastrografin enema is unable to enter the terminal ileum in a retrograde fashion should be an indication for immediate laparotomy rather than persisting in this mode of treatment. It is obvious that occasionally the radiological diagnosis of meconium ileus is incorrect.

Cecum↗
PubMed1990

Vancomycin-resistant Pediococcus acidilactici: nine cases of bacteremia.

Pediococci, vancomycin-resistant gram-positive cocci, have been isolated from human specimens, but an association with clinical illness has not been established. Clinical and epidemiologic data were obtained on nine patients who had Pediococcus acidilactici isolated from blood. Patients were eight elderly adults with complicated medical problems and one infant with congenital jejunoileal atresia. Seven patients were hospitalized before P. acidilactici was isolated. Eight had received multiple antibiotics; however, only two had received vancomycin. In all cases there was a delay in correct bacterial identification, and the significance of the isolate was uncertain. There was no clearly identified syndrome associated with P. acidilactici bacteremia. All eight adults had fever and six had pneumonia potentially attributable to other causes. The findings underscore the importance of proper identification of vancomycin-resistant gram-positive cocci. P. acidilactici may be an opportunistic pathogen in severely compromised hosts; however, further observations are necessary to clarify its role in human disease.

Aged↗
PubMed1998

Murine duodenum does not go through a "solid core" stage in its embryological development.

Embryological development of duodenum is believed to go through a "solid core" stage which subsequently vacuolizes. Failure of vacuolization is thought to be the cause of duodenal atresia. This study examines the embryological development of rat duodenum. Thirty-six time-mated Sprague-Dawley rats were studied. Six fetal duodenal specimens were obtained for each gestational day from day 9 to day 20. These specimens were fixed with formalin, sectioned, stained with haematoxylin and eosin and examined under microscope. The duodenal diameters and the lumen diameters at each gestational date were measured. The foregut started to form at the gestation stage of day 10. The lumen was narrowest on days 12 and 13, but the duodenum did not become a "solid core". No vacuolization was detected through out the development of the duodenum. Embryological development of rat duodenum did not go through a "solid core" stage.

Animals↗
PubMed1981

[Gastrointestinal perforations in the newborn (author's transl)].

Between 1958 and 1979, 48 neonates with gastrointestinal perforation were treated in the University Clinic, Mainz. The high rate of premature births (c. 40%) was noteworthy, as were the frequent complications in the perinatal period. An accurate diagnosis was made more difficult by the peculiarities of this age, and the multipilicity of possible causes of perforation. The first clinical symptoms were non-specific. In only 36% of the patients was the so-called "pathognomonic" pneumoperitoneum diagnosed on X-ray. The most common site of perforation was the ileo-coecal region, the commonest causes of perforation were intestinal obstruction (atresias, Hirschsprung's disease) and necrotising enterocolitis. In almost 1/3 of all children, the exact causes of perforation could not be determined from the history, intra-operative findings, or histology. The operative procedure depends on the individual case. It must be suited to the site and cause of the perforation, and to the subsequent disturbances. Of the post-operative complications, those related to paediatric intensive-care proved to be more serious than the directly surgical complications. The total mortality was, at 63%, high. The main cause of death was found to be septicemia, and septic complications. An improvement in the results can only be attained through early diagnosis, immediate operation and peri-operative intensive care.

Female↗
PubMed1977

Diamond-shaped anastomosis for congenital duodenal obstruction.

A retrocolic side-to-side duodenojejunostomy has been a generally accepted standard operative procedure for the correction of congenital duodenal atresia and stenosis. However, this operation has the complication of delayed anastomotic function that often requires a use of transanastomotic feeding tube or intravenous hyperalimentation. A diamond-shaped side-to-side duodenoduodenal anastomosis has been performed in nine consecutive cases of congenital duodenal obstruction, with satisfactory results. A transverse incision is made in the dilated proximal duodenum, and a longitudinal incision in the duodenum distal to the obstruction. The stoma is fashioned by approximating the end of each incision to the appropriate midportion of the other incision. Transanastomotic feeding tubes were not used and oral feedings were easily tolerated. This technique offers the theoretical advantage of providing a more physiological gastrointestinal pathway.

Child, Preschool↗
PubMed2000

[Incidence of urination disorders and erectile dysfunction after surgical treatment of congenital anorectal defects in children].

For assessment of the incidence and characteristics of urination disorders and erectile dysfunction after radical surgical treatment of anorectal congenital defects in children, 60 patients (12 girls, 48 boys) without urination disorders before operation, who have undergone 1-4 proctoplasties were examined. Their was Hirschsprung's disease in 20 patients, anorectal atresia in 40 patients. Iatrogenic urination disorders and erectile dysfunction were revealed in 38 (63.3%) patients, majority of them (24) underwent repeated proctoplasty. The main urination disorders were urinary incontinence (30 patients), infravesical obstruction (10), hyporeflexion of urinary bladder. Erectile dysfunction after operation was in 11 (34.4%) patients from 32. The combination of disorders was revealed in 17 (44.7%) patients. Primary radical operations in children with anorectal defects often cause iatrogenic urination disorders and erectile dysfunction. The number of complications increases progressively after repeated proctoplasty. It is necessary to improve the treatment and the methods of surgical correction bearing in mind the risk of iatrogenic disorders of genitourinary apparatus.

Adolescent↗
PubMed1990

Diamond-shaped anastomosis for duodenal atresia: an experience with 44 patients over 15 years.

During the period between 1974 and 1988 44 patients were treated for congenital intrinsic duodenal obstruction using a diamond-shaped anastomosis (35 atresias, including two multiple atresias and nine stenoses). Neither gastrostomy nor transanastomotic tube was used. Postoperatively, oral feeding was started on days 2 to 6 (3.66 +/- 1.41 day). Intravenous fluid administration was discontinued on days 3 to 20 (7.54 +/- 3.58 day). Fourteen patients died, none related to the operative procedure. Of 30 survivors, 21 patients have been followed from 6 months to 15 years. Body weight was within the normal range at the latest visit. Current barium study performed in 19 patients showed no blind loop, megaduodenum, or anastomotic malfunction. The diamond-shaped anastomosis provides the following advantages: (1) early recovery of anastomotic function, and (2) avoidance of later complications, such as formation of a blind loop or anastomotic stenosis.

Anastomosis, Surgical↗
PubMed1982

[The problem of congenital duodenal obstruction - A report of 123 cases (author's transl)].

The authors report on a consecutive series of 123 children (46 males, 54 females) with congenital duodenal obstruction who were operated on between 1962 and 1980 at the Department of Paediatric Surgery, University of Heidelberg, Surgical Centre. 78 patients (64%) had an extrinsic obstruction; in 36% of the children a membraneous stenosis or complete atresia was observed. 74 children (60%) were mature at birth and had no additional malformations. Out of the remaining 49 children, 25 were mature but had additional malformations. 13 prematurely born children without and 11 prematurely born children with additional malformations were seen. 17 (14%) out of 123 children died. In 74 cases with uncomplicated duodenal obstruction only 5 children (5%) died, as compared to 13 children (27%) out of 49 cases with complicated obstruction. A division of our patients into two time spans (1962 to 1971, 1972 to 1980) shows a total lethality of 25% for the first series, which included 39% cases with complications. In a consecutive series of 54 children operated between 1972 and 1980 no child died.

Down Syndrome↗
PubMed1998

Compound heterozygosity for missense (L156P) and nonsense (R554X) mutations in the beta4 integrin gene (ITGB4) underlies mild, nonlethal phenotype of epidermolysis bullosa with pyloric atresia.

Mutations in the genes encoding the subunit polypeptides of the alpha6beta4 integrin (ITGA6 and ITGB4, respectively) have been previously demonstrated in patients with a lethal form of epidermolysis bullosa with congenital pyloric atresia (OMIM #226730). In this study, we demonstrate for the first time ITGB4 mutations in nonlethal phenotype of epidermolysis bullosa with congenital pyloric atresia. Specifically, the proband was shown to be a compound heterozygote for a missense mutation (L156P) and a nonsense mutation (R554X). The leucine substitution by proline was shown to affect a residue, which was precisely conserved in different human, rodent, and drosophila integrin-beta polypeptides, and consequently disrupts the alpha-helix formation of the polypeptide segment as determined by Garnier alpha-helicity plot. The nonsense mutation in another allele was accompanied by undetectable levels of the corresponding mRNA transcript, as determined by reverse transcription-polymerase chain reaction. The presence of a missense mutation, when combined with a premature termination codon mutation, may explain the milder blistering tendency of the skin in this patient.

Amino Acid Sequence↗
PubMed1997

The Bianchi procedure in a patient with jejunal atresia.

While small-bowel transplantation remains an option for the management of short-bowel syndrome (SBS), every effort must be made to optimize the function of the native bowel. This report describes a patient with SBS who dramatically improved after a Bianchi procedure. The patient was born with type IIIb jejunal atresia, complicated by volvulus of the terminal ileum. He was left with 40 cm of small bowel and his ileocecal valve. During the first 20 months of his life, he was always hospitalized except for 3 months. He was on parenteral nutrition (TPN) and suffered multiple episodes of central line sepsis. Massive small-bowel dilatation caused a functional bowel obstruction with bacterial overgrowth, and villous atrophy of the jejunal mucosa was discovered on biopsies done by colonoscopy. In March 1994, we elected to proceed with a Bianchi procedure. Thirty cm of jejunum were divided longitudinally. During the following year, his enteral tolerance steadily improved to the point where TPN could be discontinued. We conclude that pediatric surgeons should not hesitate to use this procedure in the management of SBS.

Humans↗
PubMed1992

The physician and Down syndrome: are attitudes changing?

A survey of attitudes of pediatricians, child neurologists, and pediatric surgeons toward Down syndrome is compared with a similar study reported in 1975. A contemporary physician would be much more aggressive in treating a child with Down syndrome who has associated anomalies such as duodenal atresia or congenital heart disease. Few would recommend institutionalization of a person with Down syndrome; the majority expect that such individuals could productively spend their adult lives in group homes. Positive changes in physicians' attitudes during the past 15 years have been influenced by parent advocacy groups, court decisions, and studies showing that the ultimate intellectual and social skills of Down syndrome children are greater than was previously believed. The most prominent variable associated with attitudes was the physician's age: the older the physician, the more likely he or she would be nonsupportive of active treatment on behalf of the Down syndrome individual. These findings suggest that ongoing education of medical students and pediatric residents in the field of developmental disabilities and bioethics is required in order to promote well-informed advocacy for the mentally handicapped.

Adult↗
PubMedDate not supplied

Congenital duodenal membrane: a ten-year review.

Traditionally, congenital duodenal membrane (CDM) has been classified as a type of duodenal atresia. To provide a better understanding of this disease entity, the experience of our 13 patients with congenital duodenal membrane treated from April 1988 to November 1998 is reported. Nine were boys and four were girls, including four prematures (31%). Late diagnosis was made in eight babies and led to prolonged hospitalization (mean 20 days), although duodenal obstruction was demonstrated by barium study in all 13 patients. The low frequency of bilious vomiting (46%) and abdominal distention (39%), and lack of definite findings on plain abdominal X-ray and abdominal ultrasonography, have contributed to the delayed diagnosis in our patients. Nevertheless, no major complications developed after web excision with duodenoplasty, and satisfactory weight gain was noted in all patients except the two with Down syndrome. Since CDM is often overlooked, a better understanding of this disease entity can prevent delayed diagnosis, growth retardation and prolonged hospitalization.

Child↗
PubMed1988

Life-saving therapy for newborns: a questionnaire survey in the state of Massachusetts.

The attitudes of Massachusetts pediatricians concerning life-saving therapies for newborns were surveyed. The sample was drawn from the membership list of the Massachusetts Chapter of the American Academy of Pediatrics. Of the sample population of 801 physicians, 449 (56%) responded. Three hypothetical cases were presented with treatment options: (1) An infant with Down syndrome and duodenal atresia, (2) an infant with meningomyelocele and microcephaly and, (3) an extremely low birth weight infant of 700 g and 28 weeks' gestation suffering from birth asphyxia. For case 1, 73% of the physicians recommended surgical correction of the duodenal atresia. Of these, 68% would seek a court order if the parents did not consent to surgery. For case 2, 53% of the physicians recommended surgical repair of the meningomyelocele. For case 3, 90% of the physicians recommended continued resuscitation of the infant and referral to an intensive care unit. Religious affiliation and age were factors influencing these clinical recommendations, with Catholic and younger physicians tending to opt for more aggressive medical intervention. The great majority of physicians (93%) indicated that the economic situation of the family should not affect a decision regarding life-saving therapy. A majority (62%) of the physicians approved of infant care review committees with a primary advisory role. Comparison with earlier attitudinal surveys suggests that pediatricians today are more willing to intervene medically on behalf of infant patients than previously.

Down Syndrome↗