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Subtotal duodenectomy with jejunal patch for megaduodenum secondary to congenital duodenal malformation.

BACKGROUND/PURPOSE: This report describes a technique devised for two children with megaduodenum consisting of subtotal duodenal resection with the proximal jejunum used as an onlay patch. METHODS: A 2-year-old boy presented after unsuccessful surgical repairs for duodenal stenosis with web. Another 8-year-old boy returned from the United States after several surgical procedures for complicated congenital left diaphragmatic hernia with gastric outlet obstruction. Both boys manifested postprandial right upper abdominal fullness, occasional vomiting, abdominal pain, and failure to thrive. Upper gastrointestinal contrast study findings showed massively dilated duodenums in both patients that were larger than the stomach. During the operative procedures, about 95% of the duodenal wall was resected, leaving the basal portion that was unified with the pancreatic head and the ampulla of Vater as linguiform flap, which was covered with the proximal jejunum as an onlay patch so as to form the anterior wall of the duodenal bulb. RESULTS: The patients showed markedly improved quality of life and catch-up growth after the operations. The duodenal bulbs were of adequate size in follow-up gastrointestinal series. CONCLUSION: This technique will be useful in cases of functional duodenal obstruction with megaduodenum secondary to congenital duodenal malformations leading to blind loop syndrome.

Child↗

Mucous fistula refeeding in neonates with short bowel syndrome.

BACKGROUND/PURPOSE: Neonates with enterostomies commonly suffer from a functional short bowel syndrome (SBS) and have a greater risk of electrolyte and fluid loss with poor weight gain. The authors describe their experience with refeeding stoma effluent into the mucous fistula in neonates. METHODS: A 5-year (1993 to 1997) chart review of neonates with stoma effluent refeeding was undertaken. Demographics, medical history, surgical procedures, timing, and duration of refeedings were reviewed. Enteral and total parenteral nutritional (TPN) requirements, electrolyte, and acid-base disturbances were recorded. RESULTS: Six neonates (gestational ages of 27 to 38 weeks, birth weights of 533 to 3400 g) were identified with nutritional or electrolyte complications before the commencement of refeeding. Enterostomy indications included necrotizing enterocolitis (n = 2), intestinal atresia type 3b (n = 1), complications from ruptured omphalocoele (n = 1), congenital adhesive band obstruction (n = 1), and midgut volvulus after congenital diaphragmatic hernia repair (n = 1). Weight gain during refeeding ranged from 5 to 25 g/kg/d with duration of refeeding lasting 16 to 169 days (two neonates were refed at home) until reanastomoses were done 6 to 44 weeks after the original surgery. There were no complications, and TPN requirements were diminished or eliminated. CONCLUSION: This technique represents a simple and safe method, which lessens the need for TPN and electrolyte supplementation in neonates with enterostomies and SBS before reanastomosis.

Enteral Nutrition↗

Monozygotic twinning and structural defects.

An excess of structural defects occurs in monozygotic twins compared in dizygotic twins or singletons. The excess is composed of three categories of defects. The first includes defects which are part of the MZ twinning, such as conjoined twins and some amorphous twins. In addition, all early embryonic malformations and malformation complexes such as sirenomelia mc. holoprosencephaly mc. and anencephaly mc are increased in MZ twins. The reason for this association is considered to be the common etiology for both the MZ twinning and the early malformation problem. MZ twins provide an excellent model for appreciating the spectra of particular malformation complexes, since the twins often have different gradations in severity of the same type of structural defect. The finding of both discordant and concordant MZ twins with Goldenhar, de Lange, and Rubinstein-Taybi syndromes suggests that these "syndromes" might be early malformation complexes. The other two categories are considered secondary to the MZ twinning process. The most unique category results from any vascular interchange between the MZ twins. Depending on their nature, vascular connections may give rise to reverse flow with acardiac status in one twin during early development, or to vascular disruptions from a deceased co-twin with intravascular coagulation causing embolization in the surviving co-twin. The latter defects may include microcephaly, porencephalic cysts, hydranencephaly, intestinal atresia, aplasia cutis, and limb amputation. Unequal growth may occur as a result of artery to vein placental anastomoses. The final category is deformations due to crowding in utero during late gestation. These do not differ from those in DZ twins.

Cardiovascular Abnormalities↗

Omphalocele.

From a historical curiosity at the turn of the century, today omphalocele and gastroschisis can be corrected with good results, thanks to innovative techniques and better understanding of the newborn. Associated anomalies consist mainly of malrotation, Meckel's diverticulum, intestinal atresia, congenital heart defects, and genitourinary abnormalities. Nonoperative management in certain centers has been sucessful for the very large omphaloceles with an intact sac; however, a secondary procedure is necessary. In the past 10 years, the use of Silastic sheeting and total parenteral nutrition has allowed early repair of even the most severe cases, with excellent end results and low mortality.

Digestive System Abnormalities↗

Newborn surgical emergencies of the gastrointestinal tract.

Intestinal obstruction and necrotizing enterocolitis are two of the most common surgical emergencies of the gastrointestinal tract. This article reviews the signs and symptoms of these conditions and presents guidelines for their management.

Colon↗

Teratogenic potential of cocaine.

Cocaine has been implicated as a potential cause of congenital abnormalities since the mid 1980s. Clinical studies have reported an increased risk of cardiovascular and central nervous system abnormalities as well as an increased incidence of limb reduction defects and intestinal atresias. The published data have not established an unequivocal link between cocaine and these abnormalities. The most compelling evidence for the role of cocaine as a teratogen is the increased risk of genitourinary tract defects. Although animal models have also yielded contradictory conclusions, it is intriguing to note that the abnormalities observed in these models are similar to those seen clinically. This review summarizes the clinical and basic research relating to the teratogenic potential of cocaine.

Abnormalities, Drug-Induced↗

Aplastic anaemia during pregnancy: variable clinical course and outcome.

Aplastic anaemia occurring during pregnancy is a rare event and is associated with high maternal and fetal morbidity and mortality. Prognosis is poorer when aplastic anaemia develops during pregnancy and many such patients have an unsuccessful pregnancy outcome. We report two cases of aplastic anaemia associated with pregnancy. Both the patients had very different antenatal course and outcome. Their etiopathogenesis and management is discussed.

Adult↗

Diseases and surgery of the small colon.

Diseases of the small colon are numerous and they can affect horses of any age. Diseases can be simple obstructions, nonstrangulating obstructions, strangulating obstructions, and congenital defects. American Miniature Horses appear prone to luminal obstruction with impacted intestinal contents, and some ischemic diseases are more common in postpartum mares. Enterotomy and enterectomy have a high success rate in the small colon, provided the affected portion can be exteriorized. The beginning and terminal portions of the small colon can be involved in many diseases and are not readily accessible from standard abdominal approaches. The prognosis is generally more favorable than has been proposed.

Animals↗

Maternal and fetal factors related to abnormal amniotic fluid.

OBJECTIVE: The objective of this study was to identify maternal and infant characteristics related to alteration of amniotic fluid volume at birth. STUDY DESIGN: A series of 27,145 consecutive malformed newborn infants from the Spanish Collaborative Study of Congenital Malformations (ECEMC) was analyzed. From this total, 3.01% were found to have oligohydramnios and 3.69% were found to have polyhydramnios. RESULTS: As expected, renal/urinary tract and lung defects were associated with oligohydramnios, whereas esophageal and intestinal atresias, neural tube defects, and other central nervous system malformations were associated with polyhydramnios. In addition, other defects such as cardiovascular anomalies, hydrocephaly, and microcephaly were also related to abnormalities of amniotic fluid volume. After excluding the defects whose association to oligo- or polyhydramnios is well recognized, we compared the frequency of different variables among them and with infants with a normal volume of amniotic fluid. In comparison with infants with normal amniotic fluid volume, the groups with oligo- and polyhydramnios had lower birth weight, shorter gestational age and umbilical cord, higher parental ages, and a greater frequency of spontaneous abortions. The differences were more marked for weight in newborn infants with oligohydramnios, and for gestational age, umbilical cord length, number of previous pregnancies, and spontaneous abortions in polyhydramnios cases. Placental weight was lower in oligohydramnios cases than in infants with normal amniotic fluid, and higher in polyhydramnios cases. Parental consanguinity and twinning were more frequent in polyhydramnios. Maternal morbidity was higher in both groups with abnormal amniotic fluid volume, especially for acute diseases such as hypertension, diabetes mellitus, and gestational diabetes. Chromosomal aberrations were more frequent in the oligo- and polyhydramnios groups than in cases with a normal volume of amniotic fluid, which supports the suggestion of performing prenatal cytogenetic analysis in any pregnancy complicated by an abnormal volume of amniotic fluid. CONCLUSION: The fact that all of these results are similar in the control group of healthy infants suggests that at least some of the variables associated with abnormal amniotic volume could be considered as causal factors altering the production of fluid.

Birth Weight↗

Antenatal diagnosis of surgically correctable anomalies: effects of repeated consultations on parental anxiety.

OBJECTIVE: To assess whether or not a correlation exists between antenatal consultations and parental anxiety. STUDY DESIGN: In total, 31 couples undergoing prenatal consultation after a diagnosis of a surgically correctable anomaly were asked to fill in a questionnaire (Spielberger State-Trait Anxiety Inventory) measuring anxiety levels (AL) both after the first consultation and at birth. Prenatal counselling was done by a perinatal team including paediatric surgeon, obstetrician, and psychologist. RESULTS: Malformations were: eight diaphragmatic hernias, 10 intestinal atresias, four abdominal masses, nine abdominal wall defects. Each fetus was subsequently followed up at regular intervals from diagnosis to birth. AL at birth were then compared with the number of antenatal consultations. A negative correlation (r=-0.688, p<0.001) was found between the number of consultations and the level of anxiety at birth. Patients having at least two consultations had significantly lower anxiety levels at birth. CONCLUSION: Irrespective of the type of malformation, there is evidence that having more than one antenatal consultation may significantly reduce AL at birth. Therefore, early antenatal diagnosis should be encouraged in order to increase as much as possible chances of repeated consultations for the prospective parents.

Adult↗