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An increasing proportion of congenital choledochal malformation (CCM) are being detected on antenatal ultrasound. However, the actual timing of its surgical correction remains controversial with some series showing an excess of complications the earlier the operation. The aim of this study was to characterize the pathophysiological aspects of this cohort from the perspective of age at surgery in order to inform a more rational basis for clinical decision-making. We analysed a prospective database of CCM acquired over a 26-year period (Jan. 1997 to Dec. 2022) for patient demography; details of pre- and post-natal imaging; age at surgical intervention; operative complications (Clavien-Dindo classification) and outcome. Data are quoted as median (IQR). All comparisons are non-parametric. A P value of 0.05 was accepted as significant. There were 58 (72% female) children with an antenatally-detected CCM from a total of 265 (21.8%) in the series. These were classified as Type 1C (n = 47; 81%), type 4C (n = 3; 5%) and Type 5 (n = 8; 14%). There were no Type 1F lesions in this cohort. Median age at surgery was 113 (IQR 57–198) days. Postnatal cyst (US) size varied from 12 to 130 mm but there was little evidence of a relationship between this and post-natal liver biochemistry (e.g. bilirubin r S = 0.01, P = 0.44; AST r S = 0.14, P = 0.19). Choledochal pressure measurements (n = 46) showed resting pressures of 12 (9–21) mmHg with no significant correlation with age (P = 0.4) or aspartate aminotransferase (P = 0.2) or γ-glutamyl transferase (P = 0.06). The cohort was divided into 2 groups (Early and Late) based on the median age at surgery (all open) (113 days). Biliary obstruction was more common in the Early group (10 vs. 2; P = 0.01). Two perforations occurred, both in the Early group. With a deliberate policy of regular ultrasound-based follow-up we have seen no anastomotic complications (leak, stenosis, persistent intrahepatic biliary dilatation or stones) or post-operative cholangitis in any child [median follow-up 3.42 (1.30–8.05) years]. This is one of the largest series documenting the outcomes of antenatally-detected CCMs, certainly in Europe and North America. Such lesions are invariably cystic in nature, and either Types 1C, 4C or 5. The absence of complications using a policy of early intervention (where possible) in experienced hepatobiliary units was shown. II. [ABSTRACT FROM AUTHOR]

Prior studies of infant ovarian cysts have recommended intervention for those larger than 4 cm. We reviewed the natural history and features of those managed operatively versus nonoperatively. A retrospective study was performed of ovarian lesions in children < 1 year-old from 2000 to 2014. Forty patients were identified. Twenty-eight (70%) underwent operative management, while 12 (30%) were managed conservatively, including one undergoing aspiration. The mean age at surgery was 125 days. All but one patient (96%) had evidence of antenatal torsion intraoperatively or on final pathology. All resected lesions were benign. Ultrasound findings more common in those undergoing surgery included intracystic debris (p < 0.001), fluid–fluid or fluid–debris levels (p = 0.002), absence of Doppler flow (p = 0.014), solid components (p = 0.04), and calcifications (p = 0.001). Cysts managed nonoperatively had an average diameter of 2.5 cm, compared to 5.1 cm in the operative group (p < 0.001). Three of the lesions managed nonoperatively had a diameter greater than 3.5 cm (mean 5.1 cm) and were followed for an average of 153 days until resolution. The majority of infant ovarian lesions were excised, although none were malignant. Aspiration or observation of larger cysts was feasible and safe. Ovarian cysts in this age group should be considered for nonoperative management and closely followed. IIC. [ABSTRACT FROM AUTHOR]

Introduction Choledochal malformations (CMs) are increasingly diagnosed antenatally. There is a dilemma between early surgery to prevent CM-related symptoms and postponing surgery to reduce complications. We aimed to identify the optimal timing of surgery in asymptomatic neonates with antenatally diagnosed CM and to identify predictors for development of symptoms. Methods Using the Netherlands Study group on CHoledochal Cyst/malformation (NeSCHoc) we retrospectively collected demographic, biochemical and surgical data from all Dutch patients with an antenatally detected CM. Results Between 1989 and 2014, antenatally suspected CM was confirmed in 17 patients at a median age of 10 days (1 day–2 months). Four patients developed symptoms directly after birth (24%). Thirteen patients (76%) remained asymptomatic. Two of these progressed to symptoms before surgical intervention at 0.7 and 2.1 months resp. Postoperatively, four patients developed short-term complications and three developed long-term complications. Patients < 5.6 kg (the series median) showed more short-term complications (66%) when compared to patients > 5.6 kg (0%, p = 0.02). Conclusion When not symptomatic within the first days of life, the majority of children with antenatally detected CM remains asymptomatic. Surgery might safely be delayed to the age of 6 months or a weight of 6 kg. Postponing surgery in the clinically and biochemical asymptomatic patient might decrease the complication rate. Levels of evidence Level III. [ABSTRACT FROM AUTHOR]

Aim Antenatal detection of right-sided stomach (dextrogastria) is rare, and its significance in regards to intestinal rotation is unclear. We aimed to review all cases of antenatally-diagnosed dextrogastria in our regional fetal medicine unit over 10 years. Methods A retrospective case-note review of patients identified from a prospectively-maintained database was performed. Results Twenty cases of antenatally-diagnosed dextrogastria were identified from 2004 to 2014. There were 8 terminations and 1 intra-uterine death. One patient has no post-natal information obtainable. Ten infants were live-born, and 2 died secondary to cardiac disease in the neonatal period. All had significant cardiac/vascular anomaly on postnatal assessment, including the 3 neonates in whom dextrogastria was the only antenatal finding. Two neonates developed bilious vomiting and underwent Ladd’s procedure. Operative findings were dextrogastria/malrotation in both. A third child had gastro-oesophageal reflux, and contrast demonstrated stable duodenal/midgut position. This child has not developed symptoms attributable to malrotation and not undergone surgery. All 3 of these infants had asplenia or polysplenia and were managed with antibiotic prophylaxis/immunisation. Five children in the series were not investigated for malrotation and have not come to surgical attention (one is known to be asplenic). Conclusion Antenatally-detected dextrogastria, even if apparently isolated, was always associated with postnatal significant cardiovascular anomaly, splenic abnormality or situs inversus . This may be important for antenatal counselling. We currently recommend postnatal echocardiography and splenic assessment, but reserve GI investigation/intervention for symptomatic malrotation owing to potential significant cardiac comorbidity. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: The purpose of this study is to evaluate (1) the predictive value of fetal bowel dilatation (FBD) for intestinal atresia in gastroschisis and (2) the postnatal management and outcome of this condition. Methods: A retrospective review of all gastroschisis cases diagnosed in our fetal medicine unit between 1992 and 2010 and treated postnatally in our center was performed. Results: One hundred thirty cases had full postnatal data available. Intestinal atresia was found at surgery in 14 neonates (jejunum, n = 6; ileum, n = 3; ascending colon, n = 3; multiple, n = 2). Polyhydramnios and FBD were more likely in the atresia group compared with infants with no atresia (P = .0003 and P = .005, respectively). Fetal bowel dilatation had 99% negative predictive value (95% confidence interval, 0.9-0.99) and 17% positive predictive value (95% confidence interval, 0.1-0.3) for atresia. Treatment of intestinal atresia included primary anastomosis (n = 5), delayed anastomosis (n = 2), and stoma formation followed by anastomosis (n = 7). Infants with atresia had longer duration of parenteral nutrition, higher incidence of sepsis, and cholestasis compared with infants with no atresia (P = .0003). However, the presence of atresia did not increase mortality. Conclusions: Polyhydramnios and FBD are associated with atresia. Absence of FBD in gastroschisis excludes intestinal atresia. In our experience, atresia is associated with a longer duration of parenteral nutrition but does not influence mortality. These findings may be relevant for antenatal counseling. [Copyright &y& Elsevier]

Abstract: Alimentary tract duplications are rare congenital malformations that occur most commonly in the jejunoileal part of the gastrointestinal tract. Management of this pathologic condition is usually drawn up. We report a case of descending colonic communicating duplication in which clinical presentation and anatomopathologic results were unexpected. A slightly echogenic abdominal mass reaching 72 × 36 mm in the left flank was diagnosed in a female fetus during the third trimester ultrasound examination. At birth, volume of the mass rapidly evolved, and despite no intestinal obstruction was observed by compression of the adjacent gastrointestinal tract, abdomen was distended. Abdominal plain film showed a large air collection, and the barium enema demonstrated a slight leak of contrast in the aerated mass, suggesting a communication with the sigmoid colon. No other abnormalities were seen. The patient underwent surgery in emergency. The mass was then totally excised through an antimesenteric resection of the tubular tract joining cystic mass and sigmoid colon. A lateral suture of the colon was subsequently performed. The wall of the duplication is usually composed of a smooth muscle layer covered by an epithelium, mostly of intestinal type. Herein, we describe a descending colonic duplication completely lined with nonkeratinizing squamous epithelium. Therefore, the association of a colonic mucosa (of endodermic origin) and a squamous epithelium (derived from the ectoderm) in our case is an interesting finding and is not explained by the various theories. Furthermore, the clinical characteristics, diagnosis, and treatment of intestinal duplications are discussed with regard to literature. [Copyright &y& Elsevier]

Background/Purpose The management of exomphalos is controversial with many centers in the United Kingdom and elsewhere advocating a conservative nonsurgical approach for the larger examples. Nevertheless, this approach is not without problems or complication. The aim of the study was to ascertain the outcome of all infants with an antenatally diagnosed exomphalos treated recently at our institution using a policy of aggressive abdominal wall closure. Methods This is a retrospective review of all infants with exomphalos treated from January 1995 to September 2002. Results There were 35 infants, all of whom underwent surgery. These were separated into 3 groups: group A (all exomphalos minor) underwent primary closure (n = 11), group B (exomphalos major) underwent primary closure (n = 13), and group C (exomphalos major) underwent staged closure involving a silo (n = 11). Infants in group C had a lower birth weight ( P = .05) and were less mature ( P = .06). They required longer periods of ventilation ( P < .001), a longer hospital stay ( P = .001), and a longer period to achieve full enteral feeds ( P < .001). Overall survival was 34 (97%) of 35 infants. One premature infant who was born with a ruptured exomphalos sac (birth weight, 862 g) died of nonsurgical complications (sepsis and respiratory failure) early after the creation of a silo. Conclusions An aggressive surgical approach in infants with exomphalos is a safe option resulting in effective abdominal wall closure. This requires a skilled multidisciplinary approach and possibly greater resources than other options. [ABSTRACT FROM AUTHOR]