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PubMed2003

A case series of gastrointestinal abnormalities in fetuses with echogenic bowel detected during the antenatal period.

OBJECTIVE: The objective was to evaluate the incidence of gastrointestinal abnormalities amongst those fetuses with antenatally diagnosed echogenic bowel (EB). MATERIALS AND METHODS: A retrospective review of all cases delivered from April 2002 to March 2003 with antenatally diagnosed EB was conducted. This was defined as bowel that appeared as echogenic as (if not greater than) the iliac bone on a real-time image. The postnatal outcomes with regard to gastrointestinal abnormalities, till their discharge, were noted. RESULTS: Of the 13,941 patients delivered, there were 70 cases with antenatally diagnosed EB, giving an incidence of 70/13,941 or 0.50%. Of these, 6 defaulted follow-up and 1 had a mid-trimester termination of pregnancy at 21 weeks' gestation for social reasons. Of the remaining 63 cases with EB, 2 were stillbirths at 31 weeks and 35 weeks of gestation, respectively. Three fetuses (3/63 or 4.76%) were diagnosed with gastrointestinal abnormalities. Meconium plug syndrome was diagnosed postnatally in 2 cases, of which, 1 resolved with conservative management while the other required an emergency laparotomy. Intestinal atresia was diagnosed in the postmortem of one of the stillbirths. There was evidence of intrauterine growth retardation (IUGR) in both the stillbirth and the fetus that had required laparotomy. None of the 3 fetuses exhibited clinical features of aneuploidy. CONCLUSION: As the quoted background risk for gastrointestinal pathology is 0.23%, an increased incidence (4.76%) is observed in those fetuses found to have antenatal EB. It is possible that the presence of IUGR is associated with a worse prognosis. Further prospective studies are needed to verify this association.

Abnormalities, Multiple↗
PubMed1999

[Meconium ileus. A clinical contribution].

BACKGROUND: Meconium ileus (MI) is a form of neonatal intestinal obstruction due to an abnormal thickened meconium within the terminal ileum. The aim of this retrospective paper is to review our experience with neonates affected from MI treated at our institute over a twenty year period. METHODS: This report deals with 23 neonates with MI: 14 newborns had uncomplicated MI due to obstruction of the terminal ileum with meconium pellets, while 9 presented with complications (intestinal atresia, volvulus, pseudocyst, peritonitis). RESULTS: A water soluble contrast enema (Gastrografin) was attempted in 12 cases with a success rate of 7/12 (58%). The remaining sixteen neonates underwent laparotomy, with 4 treated by resection and primary anastomosis, 7 by enterostomy (chimney or double-barrelled) and 5 managed with T-tube enterostomy. Survival rate was 93% in uncomplicated MI and 67% in complicated forms. CONCLUSIONS: On the basis of personal experience the authors suggest that the treatment of choice for uncomplicated MI is Gastrografin enema, with T-tube enterostomy to be reserved for enema failure. The surgical treatment of the complicated forms depends on the intra-abdominal findings; nowadays chimney or double barrelled enterostomy is to be reserved in cases where peritonitis, late diagnosis, prematurity or associated anomalies complicate the disease.

Age Factors↗
PubMed1990

An individualized approach to the management of gastroschisis.

A 93% survival rate was achieved in 80 neonates treated for gastroschisis between 1979 and 1986. Uncomplicated gastroschisis occurred in 70 infants (88%); 51% underwent staged silo reduction and 49% had primary fascial closure. Gastroschisis associated with intestinal atresia or volvulus was present in 10 neonates (12%), half of whom had a residual jejunoileum between 10 and 55 cm. Major postoperative complications included gastrointestinal problems (infarction, obstruction, and prolonged dysfunction), wound infection, and catheter-associated difficulties (sepsis, infiltration, and malposition). Three of the six deaths were related to associated conditions (extreme prematurity, trisomy 13, and multiple anomalies) and three were caused by intraoperative hemorrhage, necrotizing enterocolitis, and extensive short-bowel syndrome. No statistical difference in morbidity, mortality, and length of hospitalization was demonstrated between infants treated by silo reduction and primary closure. Safe management of gastroschisis should include an individualized assessment of visceroabdominal disproportion and degree of intraabdominal tension. Vigilant expectation of potentially life-threatening complications is required to decrease postoperative morbidity, irrespective of the technique of abdominal wall closure.

Abdominal Muscles↗
PubMed2001

Bowel resection in children.

BACKGROUND: In developed countries, most bowel resections in children are for congenital anomalies and massive resection and short bowel syndrome are frequent problems. OBJECTIVE: To review indications, morbidity and mortality of bowel resection in children in a developing country. DESIGN: A retrospective study. SETTING: Ahmadu Bello University Teaching Hospital Zaria, Nigeria. SUBJECTS: Forty six children aged < or = 12 years who had bowel resection in a period of five years. RESULTS: The commonest indication was typhoid perforation 25 (54%), 21 (84%) of them aged > 5 years. Intussusception was the indication in eight (17%), six less than one year. Bowel resection was performed for congenital anomalies in nine (18%) patients, seven of them < 1 month (abdominal wall defect in four, intestinal atresia in two, strangulating inguinal hernia in one, strangulating band in one, Meckel's diverticulitis, one). Resection for trauma and neoplastic conditions were few (2.4% each). Twelve patients (26%) developed 17 complications including would infection in seven (15%) anastomotic dehiscence, five (11%), abdominal wound dehiscence, three (14%), intra-abdominal abscess and stitch sinus, one (2%) each respectively. Most of the complications followed right hemicolectomy for intussusception and resection for typhoid perforation. Massive bowel resection was necessary in only one patient. There were nine deaths (20%). The mortality was highest in neonates and infants (38%). CONCLUSION: The indications for bowel resection in this environment differ from that in developed countries. Most of the indications (74%) are preventable by improvements in sanitation and early presentation and treatment. The morbidity and mortality are highest in neonates and infants and meticulous attention to technical details may minimise them.

Child↗
PubMed1989

Idiopathic intestinal perforations in the newborn: an increasingly common entity.

Between 1982 and 1987 seven neonates ranging in age from 24 hours to 1 week were treated for idiopathic intestinal perforations at the Montefiore Medical Center, Albert Einstein College of Medicine. Four of the infants were born prematurely; three were full term. Five were being treated in an intensive care nursery when the perforation was diagnosed. All infants whose perforations were due to necrotizing enterocolitis, appendicitis, Hirschsprung's disease, meconium ileus, intestinal atresias, or drug therapy are excluded from the series. None of the infants had associated anomalies. The sites of the perforations were as follows: two in the jejunum, two in the ileum, one in the cecum, and two in the transverse colon. Six of the perforations were on the antimesenteric aspect of the bowel; one was on the mesenteric aspect. The sizes of the perforations ranged from pinhole to 1 cm in diameter. All the infants did well. Pathologic examination of the resected specimens failed to reveal an etiology for the perforations. There were no cases of duplication or muscular hypoplasia. We believe the etiology of this condition may be ischemic necrosis, secondary to a localized vascular accident in the wall of the affected viscus, but we do not have a good explanation for the upsurge in cases we are seeing.

Colonic Diseases↗
PubMed1984

Colon interposition: an adjuvant operation for short-gut syndrome.

Six infants with short-gut syndrome refractory to medical management underwent isoperistaltic colon interposition (length 11.7 +/- 3.1 cm.). The abdominal catastrophes that required extensive intestinal resection were: volvulus (3), necrotizing enterocolitis (2), and gastroschisis with intestinal atresia (1). No infant had another major congenital anomaly. The average trial of attempted medical therapy prior to colon interposition was 5.5 +/- 3.6 months. There was no perioperative mortality or morbidity associated with the colon interposition. Following the colon interposition, three infants were weaned from total parenteral nutrition (TPN) in 3 +/- 1 months and all survived. In contrast, three infants could not be withdrawn from TPN and they died secondary to complications of TPN (2 from sepsis and 1 from hepatic failure). Long-term survival was associated with a greater length of small bowel remaining after the initial resection (51 +/- 12 cm v 35 +/- 24 cm), colon interposition at a younger age (3 +/- 1 months v 8 +/- 3.5 months), and a shorter duration of medical management prior to colon interposition (2.8 +/- 0.8 months v 6.7 +/- 5.0 months). All survivors are now tolerating a regular diet and having one to four formed stools per day. Normal somatic growth and developmental milestones are being achieved. The follow-up period is from 24 to 84 months. Our experience with the colon interposition in the patient with short gut syndrome has led us to conclude that when a reasonable trial of medical management has failed, a colon interposition is a safe and effective adjuvant to treatment.

Child Development↗
PubMed1996

Prospective evaluation of faecal fatty acid excretion in short bowel syndrome in newborns.

Short bowel syndrome (SBS) in the newborn results in limited intestinal absorptive capacity, leading especially to fatty acid (FA) malabsorption. It is unknown whether adaptation occurs in time in FA absorption, and whether this adaptation is chain-length dependent. The aid of the present study was to prospectively evaluate FA absorption and excretion during SBS in the newborn. Twenty-one neonates who underwent small bowel resection (of variable length) for various reasons (necrotizing enterocolitis, intestinal atresia, meconium peritonitis, cloacal extrophy, etc) were studied. Eight neonates had SBS, defined as a small bowel remnant of less than 50% of the original small bowel length related to gestational age. The mean remaining small bowel length in the SBS group was 34% (24% to 42%). The non-SBS control group consisted of 13 neonates who had only minor small bowel resections. The mean remaining bowel length for the non-SBS group was 95% (70% to 100%). The results show that the total fractional excretion of FA (FE-FA) at 2 weeks and 1, 2, 3, and 4 months postsurgery was 51% +/- 37%, 33% +/- 24%, 51% +/- 65%, 53% +/- 27%, and 7% +/- 2% in patients with SBS, versus 12% +/- 8%, 24% +/- 10%, 9% +/- 3%, 8% +/- 3% and 17% +/- 14% in the non-SBS controls, respectively (P < .05 by ANOVA). There appeared to be an amelioration in time in FA absorption, especially in the SBS group, after 3 months. FE-FA was chain-length related, being considerably less for C10 and C12 than for C14 and longer amounts. An amelioration of absorption occurred in the SBS patients, especially with the longer-chain FA. On the basis of the study data, the authors conclude that in the initial adaptation phase shorter chain lengths are better absorbed than longer chain lengths; however, in the latter FA group, substantial adaptation occurs with time.

Fatty Acids↗
PubMed1996

Fetal death after exposure to methylene blue dye during mid-trimester amniocentesis in twin pregnancy.

Methylene blue dye use during mid-trimester amniocentesis in twin pregnancy is associated with a high risk of small intestinal atresia. It is plausible that the effects of methylene blue as a fetotoxic agent may also lead to fetal death. We conducted a retrospective cohort study of all women who had an amniocentesis during twin pregnancy from 1980 through 1991 in New South Wales, Australia. Women who were exposed to methylene blue dye during the procedure were compared with women who had amniocentesis without dye exposure. Fetal death occurred in 31.8 per cent of pregnancies that had exposure to a high concentration of methylene blue, compared with 14.5 per cent of pregnancies exposed to a low concentration and 4.3 per cent of pregnancies with no exposure to dye. The unadjusted and adjusted risks and 95 per cent confidence intervals (CIs) for fetal death after any exposure to dye were 5.03 (2.12-11.91) and 8.52 (2.28-31.80), respectively. The adjusted odds ratio and 95 per cent CIs for the low and high concentration dye solutions were 4.63 (0.93-23.13) and 14.98 (3.40-66.08), respectively (chi-squared test for trend P < 0.001). Fetus papyraceous was significantly more likely among pregnancies exposed to a high concentration of methylene blue (P < 0.001) than among unexposed pregnancies. These results support the hypothesis that methylene blue dye use during mid-trimester amniocentesis in twin pregnancy increases the risk of fetal death.

Amniocentesis↗
PubMed1990

Epidemiology of Down syndrome in 118,265 consecutive births.

The epidemiology of Down syndrome (DS) was studied in the area which is covered by our registry of congenital malformations. For each of the 139 new DS cases which were ascertained during the period 1979 to 1987 more than 50 factors were studied and compared to those from control infants. The prevalence of DS was 1.17%; 3.6% of the DS cases were stillbirths and 14.4% were induced abortions. Karyotypes were obtained in 137 cases of which all but 7 were 47, + 21, 4 were mosaics (2.8%), and 5 had translocations (3.6%). Interchromosomal effect was a question in 3 cases. The most common types of associated malformations were cardiac anomalies (44.6%) and intestinal atresia. We did not observe seasonality or time/space clusters in spite of the Chernobyl nuclear accident. No paternal age effect was demonstrated. In our material the first-born infants were at lower risk of DS than the later born. Five percent of the mothers of DS had 2 previous spontaneous abortions (controls 2.8%). At birth, the DS infants measured less and their head circumference was lower than in control infants. Weight of placenta was also lower than in control infants. In our material there were 7.9% of consanguineous marriages (P = .010). The pregnancies of the DS children were often complicated by threatened abortions; 6.4% of the mothers of the DS children were diabetic (P = .069). For all other factors studied no statistically significant difference with respect to controls could be demonstrated.

Abortion, Induced↗
PubMed2004

Risks of selected congenital malformations among offspring of mixed race-ethnicity.

BACKGROUND: Little is known about the occurrence of specific congenital malformations among offspring of mixed race-ethnicity. METHODS: Using data from a population-based registry, we explored the occurrence of selected malformation phenotypes in offspring to parents who were of different race-ethnicity. Data were derived from the California Birth Defects Monitoring Program, a population-based active surveillance system for collecting information on infants and fetuses with congenital malformations using multiple source ascertainment. Approximately 2.6 million live births and stillbirths occurred during 1989-2000. Information on parental race-ethnicity (non-Hispanic white, Hispanic, black, and Asian) was obtained from birth certificates and fetal death files. Malformation phenotypes studied were spina bifida, anencephaly, cleft lip, cleft palate, tetralogy of Fallot, d-transposition of great arteries, hypospadias, small intestinal atresia, preaxial polydactyly, microtia, and hypertrophic pyloric stenosis. RESULTS: A total of 11.2% of births were to parents of mixed race-ethnicity. Compared to births of parents who were both white, moderately increased risks (risk ratio >/= 1.7) of anencephaly, polydactyly, and microtia, and decreased risks (risk ratio </= 0.6) of hypospadias and hypertrophic pyloric stenosis were observed among births of several mixed race-ethnicity groups. For anencephaly, polydactyly, and microtia, but not other phenotypes, the risks were different depending on whether maternal versus paternal race-ethnicity was considered. Risks observed between births of a nonwhite parent and a white parent and births of parents who were both nonwhite were similar for most malformation phenotypes. CONCLUSIONS: Some malformation phenotypes appear to vary in their risk based on mixed racial-ethnic groupings.

California↗
PubMed2006

Teratogen update: pseudoephedrine.

Pseudoephedrine is contained in decongestants such as the Sudafed line of products. It is an alpha-adrenergic receptor agonist, which causes blood vessel constriction, including the therapeutic effect of reducing airflow resistance in the nasal cavity. Pseudoephedrine is one of the most commonly used medications in pregnancy, with an estimated 25% of women exposed. It has been demonstrated that alpha-adrenergic receptor agonists slow uterine blood flow, but their effects have not been studied in relation to most reproductive outcomes in animals or humans. Two analyses of health maintenance organization pharmacy data identified 9 malformed infants among 902 first-trimester pseudoephedrine exposures, suggesting no association with birth defects overall; however, studies of such data sets often lack sufficient power to identify risks for specific birth defects. The related compounds, epinephrine, ephedrine, and phenylephrine, have been associated with hemorrhages and cardiovascular and limb malformations in animal models. Risk of ventricular septal defects was associated with decongestant use in pregnant women in 1 recent study. The vasoconstrictive effects of these drugs raise the hypothesis that their use in early pregnancy might increase the risk of vascular disruption defects. Case-control studies, which often do have power to identify risks related to specific birth defects, have explored this hypothesis. Decongestant use in the first trimester has been associated with small increases in risks of 3 defects thought to arise, at least in some instances, from vascular disruption-gastroschisis, small intestinal atresia, and hemifacial microsomia. These findings are somewhat consistent in terms of magnitude of effect and suggest that risks are even greater among women also exposed to the vasoconstrictive effects of cigarette smoking. There are, however, limitations to these studies, including the possibilities of inaccurate recall of exposures and confounding by indication. In addition, the majority of decongestant use is in oral form and the question of whether intranasal formulations carry risk has not been adequately addressed.

Abnormalities, Drug-Induced↗