Esophageal obstruction-prenatal detection rate and outcome.

OBJECTIVE: Prenatal diagnosis of esophageal obstruction is believed to improve the outcome for the affected newborn. However, the prenatal detection rate is only 10-40%, the diagnosis is usually not made before the third trimester and the false-positive rate has been high. This study investigated the prenatal detection rate and time of prenatal diagnosis at our center and its influence on outcome. In addition, incidence, detection rate and accuracy of the diagnosis in a large non-selected population were determined. METHODS: All cases diagnosed pre- or postnatally with esophageal obstruction and examined prenatally by ultrasound at the National Center for Fetal Medicine in Norway during 1987-2004 were evaluated. RESULTS: Of 48 cases with esophageal obstruction, 21 (44%) were diagnosed prenatally (median, 32 + 0 weeks). All 21 had a small or empty stomach, 20/21 (95%) had polyhydramnios and 9/21 (43%) had a visible esophageal pouch. Associated anomalies were present in 38/48 cases (79%). The karyotype was abnormal in 11/48 cases (23%). Ten (21%) pregnancies with lethal fetal conditions were terminated. Two fetuses died in utero. Ten infants with associated anomalies died within 3 months after birth. The 26/48 (54%) survivors included 16/21 cases with a prenatal diagnosis of esophageal obstruction and 9/10 cases with isolated esophageal obstruction. CONCLUSIONS: The clinical signs of polyhydramnios were the most important factors for prenatal detection of esophageal obstruction. Consequently, the time of diagnosis was late and the detection rate was low (44%). An increased awareness of the possibility of esophageal obstruction, leading to targeted examinations whenever the suspicion is raised during pregnancy, might improve the prenatal detection rate and thereby provide a possibility to improve the outcome. Of the cases with isolated esophageal obstruction, 90% survived, irrespective of prenatal diagnosis.

Nordic consensus on treatment of undescended testes.

AIM: To reach consensus among specialists from the Nordic countries on the present state-of-the-art in treatment of undescended testicles. METHODS: A group of specialists in testicular physiology, paediatric surgery/urology, endocrinology, andrology, pathology and anaesthesiology from all the Nordic countries met for two days. Before the meeting, reviews of the literature had been prepared by the participants. RECOMMENDATIONS: The group came to the following unanimous conclusions: (1) In general, hormonal treatment is not recommended, considering the poor immediate results and the possible long term adverse effects on spermatogenesis. Thus, surgery is to be preferred. (2) Orchiopexy should be done between 6 and 12 months of age, or upon diagnosis, if that occurs later. (3) Orchiopexy before age one year should only be done at centres with both paediatric surgeons/urologists and paediatric anaesthesiologists. (4) If a testis is found to be undescended at any age after 6 months, the patient should be referred for surgery--to paediatric rather than general surgeons/urologists if the boy is less than one year old or if he has bilateral or non-palpable testes, or if he has got relapse of cryptorchidism.

Surgical treatment of undescended testes.

UNLABELLED: The mainstay of therapy for undescended testes is operative treatment within the first years of life in order to avoid ongoing testicular degenerative changes. The surgical therapy for the palpable undescended testis is orchiopexy and when the testis is non-palpable, a supplementary laparoscopic approach. Success of orchiopexy for inguinal testes has been >95% and for abdominal testes >85-90% in most series. CONCLUSION: Operation within the first year of life is a safe therapy for undescended testes.

Imperforate anus: A relatively common anomaly rarely diagnosed prenatally.

OBJECTIVE: Despite the relatively common occurrence of imperforate anus, prenatal diagnosis is rarely reported. In this study, we investigated the presence and diagnosis of imperforate anus along with strategies for improving prenatal diagnosis of the condition. PATIENTS AND METHODS: Fetuses and infants with imperforate anus who had been examined prenatally by ultrasound at the National Center for Fetal Medicine (NCFM) from 1987 to 2004, were evaluated. RESULTS: Of 69 cases with imperforate anus, only 11 (15.9%) were diagnosed prenatally, at a median gestation of 18 + 4 (range, 15 + 6 to 35 + 6) weeks. In all 11, dilatations of the rectum or lower part of the bowel were seen. Additional anomalies, most of them diagnosed prenatally, were present in 59/69 (85.5%) of the cases. The most frequent additional anomalies were urogenital (53.6%). The karyotype was abnormal in nine cases (13.0%). A retrospective evaluation of available videotapes of 22 cases of imperforate anus that were not diagnosed prenatally revealed that it was possible to suspect the diagnosis in 11/22 (50%) cases. Sixteen infants were born with imperforate anus without prenatal diagnosis of any abnormality. In total, 31/69 (44.9%) cases were terminated, two (2.9%) died in utero and 12 (17.4%) died postnatally. Twenty-four (34.8%) infants survived, including all 10 with isolated imperforate anus and seven of eight cases with only one additional anomaly. CONCLUSIONS: The prenatal detection rate of imperforate anus was only 15.9%. Imperforate anus is often associated with other anomalies; in this study, 85.5% had additional anomalies. Prenatal diagnosis makes prenatal counseling possible and facilitates optimized postnatal care. We believe that the prenatal detection rate of imperforate anus could be improved. Examiners should intensify their search for typical findings of imperforate anus especially when other anomalies that frequently accompany this condition are present.

Characteristics and outcome of 90 cases of fetal omphalocele.

OBJECTIVE: The aim of this study was to describe the outcome of a case series of fetuses with omphalocele. METHODS: Ninety fetuses with omphalocele at the National Center for Fetal Medicine (NCFM) between January 1985 and January 2004 were followed from the time of prenatal diagnosis. Follow-up times ranged from 6 months to 17 years. Omphaloceles were subdivided into epigastric, central and hypogastric types based on their location on the abdominal wall. RESULTS: There were 58 central and 32 epigastric omphaloceles. Abnormal karyotype was found in 40/58 (69%) of the central and in 4/32 (12.5%) of the epigastric omphaloceles. Trisomy 18 was the most frequent abnormality associated with omphalocele. Among the fetuses with normal karyotype, 89% of the central and 71% of the epigastric cases had other anomalies. 38 (66%) of the fetuses with central omphalocele were terminated and 12 (21%) died during pregnancy or after delivery. 11 (34%) of the fetuses with epigastric omphalocele were terminated and eight (25%) died during pregnancy or after delivery. Of the 90 cases followed from the time of diagnosis there were 21 (23%) survivals. Of eight survivals with central omphalocele, only two were considered healthy while six had other anomalies and/or substantially impaired development. Of 13 survivals with epigastric omphalocele, six were considered healthy and seven had other anomalies and/or substantial impairment. CONCLUSIONS: Fetal central and epigastric omphaloceles may be different entities: central omphaloceles are more strongly associated with abnormal karyotype (69%) than are epigastric omphaloceles (12.5%). The outcome of fetuses with omphalocele is poor irrespective of the type of omphalocele, with only eight of the 90 (9%) being alive and healthy at present. The results emphasize the importance of identifying both those fetuses with a potentially good prognosis and favorable outcome and those which are likely to have a fatal outcome.

Surveillance and outcome of fetuses with gastroschisis.

OBJECTIVES: Infants with gastroschisis have a high survival rate. However, the rate (10-15%) of intrauterine fetal death (IUFD) is considerable, and the association with fetal distress is well known. The aim of this study was to describe the outcome of fetuses with a prenatal diagnosis of gastroschisis. The impact of correct prenatal diagnosis, surveillance and signs of complicating risk factors were evaluated. METHODS: All fetuses with prenatally diagnosed gastroschisis at the National Center for Fetal Medicine from January 1988 to August 2002 were registered and prospectively evaluated with regular ultrasound examinations. From 34-36 completed gestational weeks the fetuses were monitored daily to every second day with cardiotocography (CTG). RESULTS: Gastroschisis was diagnosed in 64 fetuses at a mean gestational age of 19 + 2 weeks. All had normal karyotype. Associated anomalies were found in four cases (6.3%). Three pregnancies (4.7%) were terminated, all for reasons other than gastroschisis. One fetus (1.6%) died in utero. Thirteen fetuses (22%) had abnormal CTG leading to subsequent Cesarean sections. Mean gestational age at delivery was 36 + 1 weeks. Mean birth weight was 2586 g. Thirteen infants (22%) were small for gestational age (SGA). In 15 cases (25%) meconium-stained amniotic fluid was found; 14 of these had abnormal CTG and/or were SGA. Small bowel atresia was found in four infants (6.7%). Four infants died postnatally at the age of 0-9 months. CONCLUSIONS: CTG surveillance of fetuses with gastroschisis may improve the outcome through detection of fetal distress thereby reducing the risk of IUFD. Other clinical situations of importance that are associated with gastroschisis are described and discussed.

Gastric outlet obstruction caused by heterotopic pancreas.

Heterotopic pancreas (HP) in the stomach is a relatively well-known entity, but there are not many symptomatic cases reported in children. We report on a 9-year-old boy presenting with nausea and vomiting. The first gastroscopic examination showed a crater-like lesion in the antrum, but at follow-up gastroscopy a few weeks later the lesion was polypoid, obstructing the pylorus. Endoscopic biopsy was not diagnostic, but histological examination after open excision showed HP. It is unclear why the lesion changed so markedly in appearance in just a few weeks. HP is a rare cause of gastric outlet obstruction in children.

Extubation failure due to phrenic nerve injury.

A 26 week gestation infant had an increasingly elevated right hemidiaphragm following drainage of bilateral pleural effusions and failed extubation on numerous occasions. Electric stimulation of the phrenic nerves revealed absent activity on the right, indicating phrenic nerve injury from chest tube drain insertion. Diaphragmatic plication was performed and the infant successfully extubated four days later.

Fetal duodenal obstructions: increased risk of prenatal sudden death.

OBJECTIVES: The aim of this study was to describe the outcome of fetuses with duodenal obstruction diagnosed prenatally and to compare the outcome with the results of studies of newborns. METHODS: All fetuses with a prenatal diagnosis of duodenal obstruction were registered and evaluated prospectively from January 1985 to December 2000. RESULTS: Duodenal obstruction was found in 29 fetuses at a mean gestational age of 29+2 weeks. Polyhydramnios was found in 24 cases (83%). Six fetuses (21%) had trisomy 21. Associated anomalies, including trisomy 21, were found in 18 cases (62%). Four fetuses with normal karyotype died in utero at 31-35 gestational weeks. Two of them had associated anomalies, but the anomalies could not explain the prenatal deaths and the deaths occurred suddenly and unexpectedly. Three infants died postnatally; all three had associated anomalies. Four infants with normal karyotype had neurological impairment suggesting that they might have had intrauterine asphyxia. CONCLUSIONS: The present study indicates that duodenal obstruction is a more serious condition than previously believed, with an increased risk of prenatal asphyxia and death, even when the karyotype is normal and no associated anomalies are present. We consider the possibility that it could be caused by bradycardia/asystole following vagal overactivity due to distension of the upper gastrointestinal tract.

Closed gastroschisis: Antenatal and postnatal features.

Four cases of antenatally diagnosed gastroschisis are described in whom there was significant closure of the abdominal wall defect around the prolapsed midgut. In 2 cases this resulted in near-total midgut infarction and short bowel syndrome. In a further case there was entry and exit jejunal and colonic atresia and significant midgut damage but some viability of extrabdominal bowel. In the final case, although there was complete closure of the ring around the base of the midgut, no intestinal loss had occurred. In the latter 2 cases, serial antenatal ultrasound imaging had shown development of intrabdominal bowel dilatation prompting early preterm delivery. Three of these infants currently are alive and well. The authors suggest the use of the term closed (or closing) gastroschisis to describe this phenomenon. J Pediatr Surg 36:1834-1837.

[Laparoscopic examination of non-palpable testis]. / Laparoskopisk undersøkelse ved ikke-palpabel testikkel.

BACKGROUND: Approximately 20% of undescended testes are impalpable. They can be located intraabdominally, in the inguinal canal, in ectopic position, or they can be missing. Traditionally, surgical groin exploration has been carried out in such cases, sometimes followed by laparotomy if no testis or spermatic cord could be found in the groin. This approach often involves unnecessary and sometimes too extensive surgery. Laparoscopy facilitates the identification of the testicle or proves the lack of it in a less invasive way. MATERIAL AND METHODS: From 1986 to 1999, 77 boys aged one year five months to 13 years 7 months underwent laparoscopy for unilateral or bilateral nonpalpable testis in our department. RESULTS: In 72 boys (94%), the findings were conclusive in the form of intraabdominal testis in 26, an intact intraabdominal spermatic cord entering the deep inguinal ring in 27, and a missing testis in 19 boys. INTERPRETATION: Laparoscopy identified the boys who did not require further surgery because the testis was missing, Furthermore, when a testis was found, laparoscopy was helpful in choosing the most appropriate method of orchiopexy. It is concluded that at present, laparoscopy is the preferred method of investigation in boys older than one year of age with nonpalpable testis.

The Vågå Study: an outline of the design.

We carried out an epidemiological study of headache in a rural parish in the mountainous region of southern Norway. During a 2-year period from October 1995, 1838 parishioners in the age range 18-65 (or 88.6% of the target group) were examined in a structured interview based on a questionnaire. The questionnaires were validated in two ways: 100 records were re-checked, with a consistency of 98%, and a re-check of 41 parishioners was carried out > 2 months after the 1st examination. The details of the latter control study will be reported later. Only one result of the study is given: namely, idiopathic stabbing headache ("jabs and jolts syndrome") was present in > 30% of parishioners.

Glucocorticoid and ACTH regulation of rat peritoneal phagocyte chemiluminescence and nitric oxide production in culture.

In order to study the adrenocortical regulation of monocyte/macrophage functions further, leucocytes from the rat peritoneum were incubated in vitro with glucocorticoid concentrations up to 10 mumol L-1 and with adrenocorticotropic hormone (ACTH) up to 100 micrograms mL-1. The monocyte/macrophage production of reactive oxygen molecules was measured by luminol amplified chemiluminescence, and the production of nitric oxide (NO) was measured as nitrite (NO2-). Dexamethasone in vitro in nanomolar concentrations inhibited monocyte/macrophage chemiluminescence and also nitric oxide production; the potency was dexamethasone > methylprodnisolone > prednisolone. ACTH enhanced both activated chemiluminescence and endotoxin-induced nitric oxide production, but only at concentrations about 20-100 micrograms mL-1, and there was no significant effect of physiological concentrations. In summary, the results of the present study further confirm and substantiate that glucocorticoids in low pharmacological concentrations have a general inhibitory effect on monocyte/ macrophage production of reactive oxygen molecules through the specific glucocorticoid receptors, while the stimulatory effect of ACTH is only observed by very high, non-physiological concentrations. Furthermore, since low concentrations of glucocorticoids inhibited the production of these reactive oxygen molecules in vitro, indirect mechanisms involving hormones and other elements outside the immune system are not essential for the effect of glucocorticoids on monocytes/macrophages.

Early hospital discharge after direct angioplasty for acute myocardial infarction.

To determine the feasibility and safety of early hospital discharge after myocardial infarction, we reviewed a 3-yr experience with direct angioplasty: 204 patients had direct angioplasty with in-hospital mortality of 3.4%. Of these patients, 125 were discharged < 5 days after infarction and 98% of these were available for 30-day follow-up. There was one early death (0.8% mortality), two early readmissions without complications, and three late readmissions. Thus early hospital discharge a mean of 3.4 days after infarction can be achieved in > 60% of patients undergoing direct angioplasty with no significant early complications and excellent 30-day survival (99.2%).

[The incidence of acute complications in children with diabetes mellitus]. / Insidensen av akutte komplikasjoner hos barn med diabetes mellitus.

The incidence of acute complications in children with diabetes mellitus was investigated from April 1992 to April 1993. During this period 27 of 66 children (40%) had experienced at least one episode of serious hypoglycemia, and six (9%) had been hospitalized because of hyperglycemia or ketoacidosis. Children whose parents were divorced or single, experienced more episodes of serious hypoglycemia than children of married or cohabitant parents. The children who had experienced serious hyperglycemia or ketoacidosis had higher HbA1c, used a relatively higher daily dose of insulin and were older than the other children.

[Diabetes mellitus in children. Therapeutic control, social conditions and self care]. / Diabetes mellitus hos barn. Behandlingskontroll, sosiale forhold og egenomsorg.

We present a descriptive study of children with diabetes mellitus in Sør-Trøndelag county. The study included 66 children. Eight of these children had at least one other chronic disease in addition to diabetes. 31 children used more than two insulin injections daily. The median value of HbA1c was 8.6%. HbA1c was positively correlated with duration of diabetes. However, for the children who were out of remission, HbA1c was positively correlated with age and negatively with number of meals per day. Children whose parents were divorced or single had higher HbA1c than children whose parents were married or lived together. HbA1c was also higher among children who received special lessons at school than among children who did not receive such lessons. Thus, age, duration of diabetes and psychosocial factors were the most important determinants for metabolic control in our study.

[Duplication of the small intestine. Unusual cause of abdominal pain]. / Duplikasjon av tynntarm. Uvanlig årsak til magesmerter.

Duplications of the alimentary tract are rare. Since 1982 only three patients with such anomalies have been treated in the Department of Paediatric Surgery at the University Hospital in Trondheim, Norway. We describe the last patient treated, where duplication of the ileum was found upon laparotomy, performed because of exaggerated recurrent abdominal pain. Duplications may be found anywhere along the alimentary tract and diagnosis can be difficult. The duplications can be found upon laparotomy for suspected appendicitis or bowel obstruction. The condition may also be suspected upon antenatal ultrasonography and in the event of abdominal symptoms after birth.