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At least 1,531 records · Page 85Linked to original sources
PubMed2003

Neonatal Staphylococcal scalded skin syndrome complicating ileal atresia.

Staphylococcal scalded skin syndrome is a toxin mediated Staphylococcal infection, the toxin produced by staphylococcus aureus type 2 phage types (55,71,3A,3B,3C). There is a generalized tender erythema which commences on the head and neck, accompanied by fever, irritability, continuous cry and miserable look. The erythema is followed by cleavage of the upper epidermis in a large sheets mainly in the head, neck and the flexures, with formation of bullae (Nikolsky sign). It is most common in infants and children under 5 years. Most cases respond to antibiotics with other supportive measures. The prognosis is good, and the skin lesions disappear without a residual scar.

Anti-Bacterial Agents↗
PubMed2002

Excellent long-term outcome for survivors of apple peel atresia.

BACKGROUND: Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children. METHODS: The patient charts, operative reports, and office notes of 15 children with apple peel atresia from 6 pediatric surgical centers in the Netherlands were reviewed. Long-term follow-up was assessed through review of office notes and through questionnaires. RESULTS: The median age at the time of operation was 1.5 days. Postoperatively, 53% suffered from cholestasis, and 40% were septic. Three patients died (20%). At follow-up at a median age of 24 months, 1 child showed growth retardation and 2 children suffered from short bowel syndrome. At the time of the questionnaire, all children showed normal growth and development. CONCLUSIONS: Even though children with apple peel atresia often suffer serious morbidity like short bowel syndrome and sepsis during the postoperative course, late morbidity turned out to be low. If the patients survive the operative and direct postoperative period, and survive the morbidity associated with malnutrition and the long-term use of total parenteral nutrition, they have a good chance of having normal bowel function with normal growth and development.

Female↗
PubMed1983

High anorectal atresia - surgical treatment through a sacro-coccygeal-perineal approach.

10 infants with high anorectal atresia had a primary colostomy and later a definitive pull-through operation was performed. This was accomplished through a sacro-coccygeal approach and enabled easy and precise identification of the pubo-rectalis sling of the levator ani muscle. Placement of the rectum inside this sling is the most important factor in achieving post-operative faecal continence in these infants. A follow-up period of 4--10 years showed good bowel control in 9 patients, although occasional slight soiling occurs.

Anal Canal↗
PubMedDate not supplied

Associated congenital anomalies: vestibular fistula, duodenal atresia and obstructive refluxing megaureter.

Congenital gastrointestinal anomalies include spectrum of malformations, simple ones as well as complex, which are associated with anomalies of other organs and systems of organs. Most infants (50-60%) with imperlbrate anus also have genitourinary, cardiovascular, gastrointestinal and vertebral anomalies. This case report is of a female newborn with prenatally diagnosed duodenal atresia and right hydronephrosis. Clinical investigation revealed an imperforate anus with vestibular fistula. A plain abdominal X-ray showed a "double-bubble" sign, characteristic for duodenal atresia. Intraluminal membrane was excised and sigmoid colostomy was performed in general anesthesia on the first day of life. Ultrasound and intravenous pyelography confirmed a hydronephrotic right kidney and obstructive megaureter after Pena. In the fifth month of life, the ureter was tapered and reimplanted. Six months later, posterior sagittal anorectoplasty was performed. In the seventeenth month of life the colostomy was closed This child attained normal growth and development, normal bowel control, fecal continence without soiling, obstipation and urinary infections.

Abnormalities, Multiple↗
PubMed2002

Pregnancy associated aplastic anemia--a series of 10 cases with review of literature.

INTRODUCTION: Pregnancy induced aplastic anemia is a rare entity and the association is not well explained. There are approximately 80 cases in the literature and we are presenting the largest series, so far, of 10 cases. RESULTS: Total of 10 cases had 11 pregnancies. Mean age at presentation was 25.45 years and mean gestation when symptoms first developed was 17.09 weeks. Pallor and bleeding manifestations were the most common presenting complaints. Mean Hb, TLC, ANC and platelets were 4.97 g/dl, 2.74 x 10(9)/l, 1.11 x 10(9)/l and 41 x 10(9)/l, respectively. Bone biopsy cellularity ranged from <5 to 25%. Nine out of 11 (81%) pregnancies were successful of which 7 was full term and 2 were premature. Two babies were small for dates. One spontaneous abortion and one intra uterine death (IUD) were observed. Two out of 11 mothers died due to disease after delivery. Two of the 8 surviving mothers, had spontaneous partial response (22%); 4 mothers were asymptomatic after therapy with immunosuppressives given for 6 months and 3 were lost to follow up without response. Specific therapy (cyclosporin) was tried in two mothers antenatally with partial response in one. One child whose mother was given cyclosporin antenatally had jejunal atresia at birth. CONCLUSION: Pregnancy associated aplastic anemia is a rare association. Spontaneous remission can occur in 25-30% of patients. In the first trimester patients, pregnancy can be terminated while in advanced pregnancy patients can be followed up with stringent supportive care. Cyclosporin may be a safe drug antenatally in such patients. Patients with established aplastic anemia should avoid pregnancy.

Abortion, Spontaneous↗
PubMed1989

Systemic candidiasis with DIC and candida endophthalmitis in a postoperative neonate.

A full-term male neonate, weighing 2540 g at birth, was admitted to hospital on day 2 because of vomiting and severe dehydration. Duodeno-duodenostomy was performed on day 5 for congenital duodenal atresia. The child was well postoperatively until day 9, when he developed fever. Intermittent fever continued despite treatment with several antibiotics. He became seriously ill on day 15 and developed disseminated intravascular coagulation. Treatment with antifungal drugs (amphotericin B and 5-flucytosine) was effective for systemic candidiasis, but candida endophthalmitis developed. There was a persistent vitreous lesion in the left eye, which after cessation of therapy has been improving gradually. Systemic candidiasis and candida endophthalmitis should be considered in neonates who develop signs of sepsis postoperatively.

Candidiasis↗
PubMed2001

Omphalocele, colonic atresia, and Hirschsprung's disease: an unusual cluster of malformations in a single patient.

Omphalocele, colonic atresia (CA), and Hirschsprung's disease (HD) are individually rare congenital malformations. An association between CA and HD has been described, but the co-occurrence of all three malformations has not been previously reported. We present an infant born with all three malformations and review the management issues relevant to this case, with an emphasis on the importance of considering co-existent HD in any infant born with CA.

Abnormalities, Multiple↗
PubMed2000

Choledochal cyst associated with duodenal obstruction.

The association between congenital duodenal obstruction and concomitant choledochal cyst has not been reported, although duodenal obstruction is known to be associated with many other anomalies. The authors describe 2 patients with choledochal cyst with duodenal obstruction. In 1 patient, a diverticulum type of choledochal cyst was found within an annular pancreas. Cyst excision, choledochojejunostomy, and side-to-side duodeno-duodenostomy were performed. The other patient showed separated duodenal atresia and other multiple anomalies including imperforate anus. A choledochal cyst was noted at the time of duodeno-duodenostomy and sigmoid colostomy. Cyst-enterostomy was performed at the age of 8 months, but the patient died of multiple anomalies. Intraoperative cholangiography indicated an anomalous pancreatobiliary ductal junction (APBDJ). In both patients the bile in the cyst contained high levels of amylase, suggesting the presence of an APBDJ. An APBDJ is considered to play an etiologic role in the development of the choledochal cysts associated with duodenal obstruction.

Choledochal Cyst↗
PubMed1979

Duodenal and jejunal atresia with agenesis of the dorsal mesentery: "apple peel" small bowel.

Two newborn infants with duodenal and jejunal atresia and agenesis of the dorsal mesentery represent our surgical experience with "apple peel" small bowel or "christmas tree" demormity. The first patient had the typical appearance of this condition. The postoperative course was complicated by hyperbilirubinemia, septicemia, and disseminated intravascular coagulation. The infant is in satisfactory condition 1.5 years after operation. The second patient had agenesis of the dorsal mesentery without spiraling of the bowel around its vascular stalk. The child died after 1 month, with complete absence of extrahepatic bile ducts as seen at a second laparotomy. Neither child had been subjected to gastrostomy.

Duodenal Diseases↗
PubMed1986

Blind-end atresia coli in two foals.

Blind-end atresia coli was observed in two genetically unrelated foals at about the same time and location. The two foals, one an Appaloosa and the other a Quarterhorse, were born 4 days apart on nearby but separate farms. The Appaloosa foal died after surgical anastomosis of the affected segments of the colon. The Quarterhorse foal was euthanized after the defect was found at laparotomy. Both foals were necropsied and representative tissues were examined histopathologically. Histopathologic results were unremarkable. No cause of the defects could be determined and the timing and geographical location of the two events were considered coincidental.

Animals↗
PubMed1982

Rectal atresia: treatment through a single sacral approach.

Rectal atresia (with a normal anus) is a rare abnormality. A boy was operated upon successfully by using a single stage sacral approach, when he was 1 yr old. In the neonatal period he had a colostomy done for decompression. This colostomy was later useful in demonstrating the distance between the two blind pouches of the anal canal and the rectosigmoid segment. He is now perfectly continent without stenosis, 3 yr after the operation. If this procedure could be done in the neonatal period before the colon becomes very dilated, a long term colostomy might be obviated.

Humans↗
PubMed2005

Double bubble, double trouble.

Duodenal atresia (DA) is not uncommon, either as an isolated anomaly or associated with trisomy 21, malrotation, or cardiac anomalies. It may be diagnosed on antenatal ultrasound by a "double-bubble" sign, which typically persists after birth on a plain abdominal radiograph. DA as a familial association is rare but has been reported with or without other associated anomalies. We report DA in two siblings of nonconsanguineous parents, one case occurring with an annular pancreas in association with gestational diabetes. These two cases suggest possible genetic and environmental components in the aetiology of this anomaly.

Duodenal Obstruction↗
PubMed1982

Gastrointestinal atresia and maternal occupation during pregnancy.

This study originated in observations in cohort studies that women who worked in laboratories during pregnancy may be at increased risk of having an infant with gut atresia. A case-control study has been made of 201 women who had infants with gastrointestinal atresia and 402 women with infants without that malformation but matched for age, parity and time of year for delivery. Maternal occupation during pregnancy was ascertained by interviews of mailed inquiries and only referred to type of occupation; no efforts to obtain detailed information on occupational exposure were made. Occupation of nonresponders was checked using census data. In the case-control study, laboratory work was reported to occur more often among case mothers than among controls, thus verifying the connection. The actual exposure in the laboratory work giving the embryonic damage is not known.

Adolescent↗
PubMed2004

Cloacal exstrophy: morbidity associated with abnormalities of the gastrointestinal tract and spine.

BACKGROUND/PURPOSE: The aim of this study was to review the management of the gastrointestinal tract in cloacal exstrophy and to assess the effect of spinal dysraphism on nutritional outcome. METHODS: Twenty-two patients with cloacal exstrophy were reviewed retrospectively. Gastrointestinal and spinal anomalies were documented. The need for nutritional supplementation was recorded. Weights at 1 and 5 years of age were used to assess growth in childhood. RESULTS: Ten patients were treated initially with ileostomy, 7 with colostomy, and 3 without stoma. Two patients died before surgical reconstruction. Total parenteral nutrition (TPN) was utilized in 8 infants for a median of 10 days (range, 5 to 200). Three patients had complications with colostomies requiring conversion to ileostomy. Patients with an ileostomy required more nutritional supplementation compared with patients with a colostomy (50% v 28%). High-output stoma losses were more common in patients with an ileostomy (40% v 14%). Fourteen patients (67%) with spinal dysraphism had a higher incidence of failure to thrive in the first year of life (69% v 26%) and multiple episodes of enteritis (40% v 25%). Two neonates with duodenal atresia and small bowel deletion died within the first month of life. One patient with short bowel syndrome died of TPN-associated liver disease at 6 months of age. There were no other deaths. CONCLUSIONS: The gastrointestinal tract contributes significantly to the morbidity and mortality in cloacal exstrophy. Nutritional supplementation is more frequently required in patients with an ileostomy. Stoma complications were higher in those with a colostomy. Morbidity is high in patients with spinal dysraphism.

Abnormalities, Multiple↗
PubMedDate not supplied

[Duodenal atresia in a child with Down's syndrome].

A case of Down's syndrome with atresia of the duodenum is reported. The child was the product of a third pregnancy of abnormal course (imminent abortion, hospitalization, cervical suture). The delivery was by caesarean section due to threatening intrauterine asphyxia. The first pregnancy 2 years earlier led to spontaneous abortion, the second one year earlier resulted in stillbirth. Both parents regarded themselves as healthy. The mother was aged 24 years, the father was aged 26 years. Death was caused by circulatory, respiratory failure during bronchopneumonia. Although in the literature the possibility of this anomaly in Down's syndrome is mentioned, but congenital cardiac disease is considered more frequently.

Down Syndrome↗
PubMed1980

[Duodenal stenosis and duodenal atresia in neonates (author's transl)].

Of 129 neonates including 38 premature births who were operated for duodenal atresia or stenosis, 101 survived. 28 children died, not as a result of the operation but primarily from accompanying deformities, or because of subarachnoid and pulmonary hemorrhages associated with coagulation disturbances. 2 babies died of sepsis. 80 of the 101 surviving patients could be surveyed through a questionnaire action. 24 of these children were also examined clinically and radiologically. No noteworthy complaints could be recorded even 16 years after the operation. A retarded development seen during the first 5 years seems to have been well compensated subsequently under suitable living conditions so that the prognosis for later life can be considered favorable.

Abnormalities, Multiple↗
PubMed1981

Reverse smooth muscle plasty: a new method of treating anorectal incontinence in infants with high anal and rectal atresia.

A new technique of treating anorectal incontinence in infants with high anal and rectal atresia is described. In the first stage of this new operation an abdominosacroperineal pull-through procedure according to Rehbein, Romualdi, and Kiesewetter is performed. In the next step of this new method the circular and longitudinal muscle layers of the pulled-through colon are turned back 180 degree and fixed to the pulled-through bowel. The mucosa of the turned back bowel is removed. Then the whole muscle cuff is re-pulled into the puborectalis sling. Pressure studies as well as X-ray investigations showed a good sphincter function of the newly established internal sphincter equivalent.

Anal Canal↗
PubMed2004

Placental vascular compromise in jejunoileal atresia.

BACKGROUND/PURPOSE: The mechanisms of intrauterine vascular disruptions that result in the development of jejunoileal atresia (JIA) are not fully understood. Monochorionic twinning with fetal death of a cotwin is known to be correlated with the development of JIA in the survivor through placental communication. The aim of this study was to evaluate whether other placental vascular compromises might contribute to the development of JIA. METHODS: Forty-five newborns (23 boys and 22 girls) who were treated for JIA at Tsukuba University Hospital from 1978 to 2003 were reviewed. Placental findings were informative in 23 cases. RESULTS: No or slight abnormality of the placenta was found in 19 cases. Significant placental abnormalities were found in 4 patients who also had a low birth weight. One patient with apple peel atresia (APA) had excessive torsion of the umbilical cord (UC), which was inserted at the margin of the placenta, and there was an adjacent area of infarction. One patent with multiple atresia (MA) was a surviving monochorionic twin with intrauterine fetal death of the other. Another case of MA showed marginal insertion of the UC. Severe placental abnormalities including wide infarction, cyst formation, and marginal insertion of the UC were found in 1 case of MA. These 3 cases of MA were complicated with other anomalies including brain anomaly. CONCLUSIONS: Placental vascular compromises were involved infrequently in JIA but might possibly be responsible for the development of JIA as well as associated anomalies and a low birth weight as chronic insults since an early stage of gestation in some cases.

Female↗