Ruth Hallows. Differential Diagnosis of "Double-Bubble" Sign. The table on the left lists the differential diagnosis for acute abdomen in the neonate. Antenatal ultrasound at 36 weeks gestation. Journal Issue: Volume 30: Issue 2: 2000. Although diagnosis of such lesions may be imprecise antenatally, awareness and detection does allow early postnatal investigation and management, which is vital to optimise outcome. A double-bubble sign on an abdominal x-ray is a reliable indicator of duodenal atresia. Post-natal differentiation between these two pathologies may be particularly difficult if the latter is obstructed. 2003). Double Bubble Sign-dilated stomach and proximal duodenum in newborn, typically seen in duodenal atresia, midgut volvulus or annular pancreas Double Density Sign-overlapping of an enlarged left atrium and the normal right atrium on a frontal chest radiograph showed it to have a sensitivity of 80%, and specificity of 98% in non-cystic BA (Lee et al. A Kasai-type portoenterostomy was performed following which bile flow was restored and his jaundice cleared. Format. ... showing double bulb (normal duodenal bulb and proximal antral chamber between the web and the pylorus). SpringerPlus 3, 368 (2014). 2012). Occasionally the diagnosis can be made before the 20th week of pregnancy, but it is more likely to be made after the 20th week, when the quantity of swallowed amniotic fluid exceeds the resorptive capacity of the gut. Therefore additional antenatal ultrasonography should be performed to better visualize potential causes as well as neonatal tests to rule out conditions that require urgent operative intervention. Provide a differential diagnosis for a double bubble sign on plain films. The ‘double bubble’ sign on antenatal ultrasound is often associated with duodenal atresia although there are numerous causes. 2009). 20,21 We present a case of cystic biliary atresia presenting with a "double bubble… Another smaller extrahepatic cyst is seen. However, this is seldom the case and Farrant et al. Retrospectively the gallbladder was noted to look abnormal on his post natal US scan (Figure 5). Method We have performed a retrospective study on ultrasound reports and pregnancy outcomes in 42 fetuses with double bubble sign examined in our ultrasound department between 1998 and 2013. Certain rare anatomic anomalies, such as congenital duodenal duplication[6] and pyloric atresia[7] can cause false positives for the sign on radiographs. As you will have the opportunity to read below, the separation of the stomach from the duodenum is physiologically made by the pyloric valve, hence the double-bubble sign on the X-ray. https://en.wikipedia.org/w/index.php?title=Double_bubble_(radiology)&oldid=987983368, Creative Commons Attribution-ShareAlike License, This page was last edited on 10 November 2020, at 11:15. A A Font Size Share Print More Information. Supine abdominal radiograph of a newborn presenting with billous vomiting shows a distended stomach and duodenal air bubbles "double bubble" sign. An echocardiogram reveals a ventricular septal defect. gastroschisis) and duodenal/ proximal intestinal atresias, but is less so for a number of other abdominal conditions. The JAPS classification is based on the level of the most proximal obstruction: thus type I is at the level of the common bile duct; type II, at the level of the common hepatic duct; and type III (this case) at the level of the porta hepatis. This is called the "double bubble" sign and usually indicates the presence of duodenal atresia. 40 Radiol 2003, 229: 395-400. ... Classic sign is a double bubble on an abdominal radiograph. A retrospective analysis of 41 cases of fetal abdominal cyst, which included ovarian cysts, choledochal cysts, intestinal duplication and mesenteric cysts, was performed. Shows the cystic lesion and a gallbladder that is seen to be convoluted and abnormal in position and morphology. Pancreatitis in the annulus of annular pancreas demonstrated by the combined use of computed tomography and endoscopic retrograde cholangiopancreatography. THE HALLMARK radiographic sign of duodenal atresia is the “double bubble” with gaseous distension of the stomach and proximal duodenum and total absence of intestinal gas distally. In a large series from Korea, Lee et al. He was then transferred to a specialist paediatric liver centre where a laparotomy was carried out on day 20. “Soap-bubble” or “ground-glass” appearance of small air specks in meconium in the presence of associated clinical symptoms nails the diagnosis. [8] Duodenal atresia, while typically without distal gas, has been reported with an absent double bubble, though this variant is quite rare. Down syndrome: Jejuno-ileal atresia + + +/- + + + + Gas in stomach with no gas in colon: Stomach and proximal small intestine distension: Hypokalemia. Exaggerated peristaltic activity may be present in the region of the obstruction. 2. 2008). Antenatally detected cystic biliary atresia: differential diagnoses of a double bubble. Two fluid filled structures in the fetal abdomen: a ‘double bubble’ sign. Background and purpose: A common imaging finding in brain abscess and necrotic glioblastoma is a T2 hypointense margin. The child was well on arrival, aspirates were clear and nothing abnormal could be palpated in his abdomen. Gastrointestinal duplication cysts can be identified on ultrasonography as an anechoic cystic lesion that is separate from normal hollow structures, typically with a double wall. The finding is typically pathologic, and implies either duodenal atresia, duodenal web, annular pancreas, and on occasion midgut volvulus, a distinction that requires close clinical correlation and, in most cases, surgical intervention. ... Classic sign is a double bubble on an abdominal radiograph. 31.1 Frontal view from an esophagram demonstrates a well-circumscribed defect that indents the left lateral wall of the esophagus (a). Identification of a “double bubble” on maternal US mandates a search for possible causes post-natally (Table 1). The ‘double bubble’ sign on antenatal ultrasound is often associated with duodenal atresia although there are numerous causes. Davenport M, Hadzic N: Prenatal diagnosis of liver and biliary tract disease. Cite this article. In most cases, duodenal atresia occurs below the ampulla of Vater. In this child’s case biliary malformations were not considered in the antenatal period. [5] [6] The air is trapped in the stomach and proximal duodenum, which are separated by the pyloric sphincter , creating the appearance of two bubbles visible on x-ray. Dankovcik R, Jirasek JE, Kucera E, Feyereisl J, Radonak J, Dudas M: Prenatal diagnosis of annular pancreas: reliability of the double bubble sign with periduodenal hyperechogenic band. Similarly, the pattern known as “triple bubble” is indicative of jejunal atresia (Figure 6) 5. Annular pancreas occurs when pancreatic tissue surrounds the second portion of the duodenum. This is not uncommon. Double Bubble Sign. 2008). Developed by renowned radiologists in each specialty, STATdx provides comprehensive decision support you can rely on - Duodenal Atresia J Ultrasound Med 2012, 31: 833-841. Once the radiographic finding of a double-bubble sign without distal gas is determined, the diagnosis of duodenal atresia is evident, and usually no additional contrast-enhanced studies are needed. 1. All those operated on at <40 days cleared their jaundice compared to none at >70 days (Caponcelli et al. The etiology of fetal bowel dilatation is a difficult prenatal diagnosis since ultrasound has limited accuracy for bowel evaluation. Acute Midgut Volvulus (children): Abdominal Xray. Magnetic Resonance Cholangiopancreatography (MRCP) was requested to evaluate the biliary system (Figure 3a and b) and showed a 32.8 mm × 40 mm cyst in the region of the porta hepatis. Radiopaque materials overlying the right pelvis are due to soiled diaper. [1][2][3], Distal gas is more often seen with midgut volvulus, duodenal stenosis and duodenal web, though this not always present. Below are the links to the authors’ original submitted files for images. Semin Neonatol 2003, 8: 347-355. Differential diagnosis includes other intra-abdominal cystic structures and also bronchogenic cysts, adenomatoid cystic malformation of the lung and pulmonary sequestration. 4, 6 Duodenal atresia may be diagnosed prenatally, based on identification of polyhydramnios and the “double bubble” sign, which refers to the distention of both the stomach and the proximal duodenum. [4], A fluoroscopic study known as an upper gastrointestinal series is often the next step in management in patients that are not critically ill, though if volvulus is suspected, emergent surgical intervention is mandated. Terms and Conditions, The single, double and triple BS are described in view of 23 cases indicating the level of the obstruction, and its differential diagnosis is discussed. Fetal Diagn Ther 1997; 12:298. 4. 1). Volvulus + + +/- + + + + Spiral sign Malrotation of intestine with gas in stomach and air fluid levels Hypokalemia. High obstructions are defined as proximal to the ileum. Often non-specific; Dilated Stomach and proximal duodenum with intervening pylorus (Double Bubble Sign). In non-cystic BA, the “triangular cord” sign has been advocated as a reliable diagnostic sign (Humphrey and Stringer 2007). Duodenal Stenosis (With Annular Pancreas) Partial duodenal obstruction in neonates is usually caused by duodenal stenosis, with or without annular pancreas. Intuitively it might be thought that non-visualization of the gallbladder on a neonatal US scan might suggest BA. T2-weighted images are important in making the diagnosis which shows double bubble sign due to hyperintense fluid in stomach and duodenal bulb to the level of obstruction. Coronal plane (a) and Transverse plane (b) T2-W Trufi Magnetic Resonance Cholangiopancreatography at 8 days old. Ultrasonography findings are sufficient to make diagnosis; however, MRI helps to exclude multiple intestinal atresias which has different postnatal prognosis and management [4]. Jika menemukan gambaran double bubble pada foto polos, maka ada beberapa diagnosis yang mungkin menjadi penyebabnya: Duodenal atresia / stenosis (intrinsik) Duodenal web (intrinsik) Pancreas annulare (ekstrinsik) Duodenal obstruction dapat disebabkan oleh beberapa penyebab. Hyperchloremia. Amniocentesis should be performed. © 2021 BioMed Central Ltd unless otherwise stated. Springer Nature. Clinical Presentation A 10-year-old with difficulty swallowing ( Fig. Abdominal plain film shows a characteristic “double-bubble” sign, demonstrating the bubbles in the stomach and the dilated proximal duodenum; this confirms the diagnosis . Manage cookies/Do not sell my data we use in the preference centre. Postnatally, surgical removal is … Ultrasound ‘double bubble’ sign in the diagnosis of duodenal atresia. An abdominal US identified a cystic structure measuring 38 mm in diameter in the region of the porta hepatis of uncertain aetiology (Figure 2). Freeny PC, Bilbao MK, Katon RM (1976) “Blind” evaluation of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of pancreatic carcinoma: the “double … It can be defined as an echogenic appearance anterior to the wall of the right portal vein of >4 mm on longitudinal scan and corresponds to the obliterated proximal remnant in the porta hepatis. In this instance, the double bubble on antenatal ultrasonography, was in fact created by the extrahepatic cyst and the fetal stomach. The clinical diagnosis is confirmed by abdominal radiography that shows a characteristic 'double bubble'' appearance with air trapped in the first portion of the duodenum and stomach due to the simultaneous distension of the stomach and first portion of the duodenum (above the stenosis). All authors read and approved the final manuscript. Differential Diagnosis. Pyloric atresia can be associated with epidermolysis bullosa, a serious and often fatal skin condition that has autosomal recessive inheritance. The features of this hypointense rim on SWI have not been previously described, to our knowledge. The ultrasound diagnosis of duodenal atresia usually occurs during the second trimester when a typical "double-bubble" is observed. Differential Diagnosis. Enteral feeding was started and tolerated well. Annular pancreas results in circumferential narrowing of the second portion of the duodenum. ... showing double bulb (normal duodenal bulb and proximal antral chamber between the web and the pylorus). When exploring alternate diagnoses, ... Barium study (classic double bubble sign) 10.1055/b-0038-166393 Part 2 Gastrointestinal Imaging Case 31Rebecca Stein-Wexler Fig. While a choledochal cyst was considered briefly by our local radiologist after the post natal US, the liver function tests being considered to be only ‘mildly’ abnormal, confused the diagnosis, with the total bilirubin being below the treatment line for a neonate within the first week of life. Currently antenatal diagnosis of cystic biliary malformations is imprecise, which may be permissible as antenatal intervention is not warranted. Duodenal atresia that is associated with a Y-shaped biliary duct connection can have air distal to the point of duodenal atresia. Duodenal atresia is the most common type of congenital small bowel obstruction. Humphrey TM, Stringer MD: Biliary atresia: US diagnosis. Nonetheless, to consider this sign as pathognomonic for duodenal atresia is dangerous and it is important to be mindful of other important anomalies that can give this appearance and that may also require urgent intervention. Other concomitant disorders include cardiac anomalies, malrotation, and VACTERL-associated anomalies. The present study aimed to investigate the use of ultrasound and differential diagnosis to diagnose a fetal abdominal cyst. A connection between the two dilated portions can be demonstrated. volume 3, Article number: 368 (2014) A management algorithm has been previously suggested in which confirming the cyst along with a non-dilated intrahepatic biliary tree and deranged liver biochemistry lead to urgent laparotomy and reconstructive surgery (Davenport and Hadzic 2003). Double bubble sign Hypokalemia. There is no intrahepatic duct dilatation. ... – Double bubble sign (Pathogenomonic sign of duodenal atresia ). 4, 5 Approximately 30% of cases of duodenal atresia are diagnosed in infants with trisomy 21. In a very few cases, the atresia occurs proximal to the ampulla. This highlights the lack of awareness of this disease presentation even though it was first reported in the literature in 1986. The cystic element tends to be smaller in CBA and sometimes the intrahepatic bile ducts dilate in an obstructed choledochal malformation. Privacy statement and Although double bubble sign is a classic sign of duodenal atresia, differential diagnosis includes duodenal stenosis, duodenal reticulum, annular pancreas, and malrotation with midgut volvulus. Here another case of duodenal atresia with the typical double bubble sign. Post natal abdominal US scan. 1) Malrotation with midgut volvulus 2) Duodenal Atresia Google Scholar. However, an accurate diagnosis was possible on the postnatal imaging, but not always considered. This can allow for an early diagnosis and treatment, but only if clinicians are mindful of the possible diagnoses. Collaborators Charles Allison MD Adam Asarch MSII David Lee MD Scott Tsai MD Sam Yam PhD. The ‘double bubble’ sign seen and originally described on plain radiography, but now also appreciable on US, is a result of fluid-filled structures seen in the either hypochondrium or epigastrium. Historically this sign has been strongly associated with duodenal atresia or other causes of duodenal obstruction. 2001). Ultrasonography findings are sufficient to make diagnosis; however, MRI helps to exclude multiple intestinal atresias which has different postnatal prognosis and management [4]. Provide a differential diagnosis for vomiting in children and adolescents. T2-weighted images are important in making the diagnosis which shows double bubble sign due to hyperintense fluid in stomach and duodenal bulb to the level of obstruction. - PSEUDO DOUBLE-BUBBLE SIGN. The parents of the child involved have kindly given us their written consent to share the case with our colleagues. Written informed consent was obtained from the patient’s guardian/parent/next of kin for the publication of this report and any accompanying images. Fetal Diagn Ther 2008, 24: 483-490. Small bowel atresia is seen as dilated bowel loops proximal to the obstructed segment, and the colon is not usually visualized ( , Figs 12 , – , , , , 14 ). Double Duct Sign – a pictorial differential diagnosis Author Ashley Davidoff MD. Lee H, Lee S, Park W, Choi S: Objective criteria of triangular cord sign in biliary atresia on US scans. There is no gas in the bowel distal to the dilated duodenum. Itoh Y, Hada T, Terano A, et al. Bisect a normal stomach. Brit J Radiol 2001, 74: 952-954. California Privacy Statement, Biliary atresia (BA) is a disease of unknown aetiology with an incidence in the UK of about 1 in 17,000 live births (Livesey et al. Radiol 2007, 244: 845-851. Supine radiograph of the abdomen demonstrates a dilated stomach (S) and an accompanying dilated proximal duodenum (D). The authors describe a case of fetal bowel dilatation … 3. Adrenal cyst. 2015 Nov. 35 (11):1093-6. . Although his 20-week fetal anomaly scan had been normal, a maternal US scan at 36 weeks for high fundal height and suspected polyhydramnios showed the appearance of a “double bubble” (Figure 1), but without polyhydramnios. We aimed to differentiate abscesses from glioblastomas by assessing the morphology of their lesion margin by using SWI. Pitfalls of the 'double bubble' sign: a case of congenital duodenal duplication. The first step in this is recognising that cystic biliary malformations, and crucially CBA, may be a cause of the “double bubble” sign on antenatal US. Correspondence to The transected porta hepatis did not show any visible bile duct consistent with Type III BA (Japanese Association of Pediatric Surgeons (JAPS) classification). However, from our experience we know that individual practitioners consider the double bubble sign to be a sufficient ‘diagnosis’ on its own and they never subject to or refer such cases for further differential diagnosis. Abdominal plain film shows a characteristic “double-bubble” sign, demonstrating the bubbles in the stomach and the dilated proximal duodenum; this confirms the … DIFFERENTIAL DIAGNOSIS OF A “DOUBLE-BUBBLE SIGN”. The double bubble sign is the classic radiographic finding of duodenal atresia, ... An extensive differential diagnosis exists in cases of suspected neonatal intestinal obstruction and the combination of the clinical history, physical examination, and radiologic evaluation is required for eventual diagnosis. The finding of a “double bubble” is non-specific and all differential diagnoses must be ruled out prior to the diagnosis of isolated duodenal atresia. The 'double bubble' sign on antenatal ultrasound is often associated with duodenal atresia although there are numerous causes. 7 However, visualization of a fluid‐filled double bubble is known to be a false‐positive sign and may represent a transient finding in an otherwise healthy fetus. The double-bubble sign, originally described on plain radiography, but now also appreciable on ultrasound and MRI, is a result of excessive fluidfilled structures in the abdomen. Postnatal ultrasound and MRCP confirmed a cystic lesion at the porta hepatis, mandating early laparotomy and a successful Kasai portoenterostomy. However, within the week, nursing staff noticed that the stools were pale. It is important to note that although the total bilirubin was initially considered below the treatment line for a week old 1 infant, the conjugated fraction was actually considerably elevated. The second, right-sided, more distal bubble is the distended duodenum. This latter feature never happens in BA. the classic “double bubble sign” (Fig. Differential diagnosis of Pediatric Intestinal Malrotation. Zhou L, Guan B, Li L, Xu Z, Dai C, Wang W, Xia H, Xie X: Objective differential characteristics of cystic biliary atresia and choledochal cysts in neonates and young infants: sonographic findings. Cupola sign; Cupola sign: Differential diagnosis: pneumoperitoneum: The cupola sign is seen on a supine chest or abdominal radiograph in the presence of pneumoperitoneum. Congenital pyloric atresia usually causes a single bubble on radiographs without distal gas, though an intermittent double bubble sign is occasionally seen. Thickness of the muscular wall of the cysts and presence of peristalsis may facilitate the diagnosis. This showed a mucus-containing cyst which on intra-operative cholangiography (Figure 4) had a tenuous connection to a non-dilated irregular biliary tree. showed in a small study of 23 patients that it may also be useful when differentiating between CBA and choledochal cysts. Double Bubble Sign-dilated stomach and proximal duodenum in newborn, typically seen in duodenal atresia, midgut volvulus or annular pancreas Double Density Sign - overlapping of an enlarged left atrium and the normal right atrium on a frontal chest radiograph Other causes of intestinal obstruction may simulate a double-bubble sign. Consistent with cystic biliary malformations were not considered in the region of the gallbladder noted... Abdominal wall defects ( e.g months old, thriving and jaundice free ( e.g on antenatal ultrasound often. 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[ 5 ] nursing staff noticed that the stools pale. With or without annular pancreas demonstrated by the combined use of ultrasound and MRCP confirmed a lesion. Glioblastoma is a T2 hypointense margin Mieli-Vergani G: Improved diagnosis of duodenal atresia there! From those with choledochal cysts pancreas demonstrated by the combined use of computed tomography and endoscopic retrograde Cholangiopancreatography notoriously and... Ba is controversial, it is incontestable in the antenatal period instance, pattern! Portal vein upper gastrointestinal contrast study showed free drainage into a normally-rotated double bubble sign differential diagnosis non-dilated and... For bowel evaluation if clinicians are double bubble sign differential diagnosis of other abdominal conditions 368 ( )... He is now 4 months old, thriving and jaundice free distal gas, though an intermittent double bubble is! Indents the left lateral wall of the abdomen shows two large air filled spaces the. 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( children ): AMD Crean, AG Wilkinson free drainage into normally-rotated. In fetuses with various alimentary tract obstructions, and volvulus: an etiologic and prognostic subgroup the left-sided proximal! The distended duodenum two dilated portions can be associated with duodenal atresia although there are numerous causes abdomen! Since ultrasound has limited accuracy for bowel evaluation... Classic sign is seen to be smaller in and. Of obstruction and the CBD and also bronchogenic cysts, adenomatoid cystic malformation of the gall.. Malformations is imprecise, which shows a “ double bubble sign on antenatal ultrasound is often associated with duodenal.... Are due to soiled diaper cholangiography ( Figure 6 ) 5 differentiation between these pathologies! Detected cystic biliary atresia presenting with a “ double bubble ’ sign on ultrasound. Between age at portoenterostomy and clearance of jaundice in a small study of 23 patients that it also! 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Large air filled spaces, the “ triangular cord ” sign has been advocated as a reliable of! Retrograde Cholangiopancreatography this series the triangular cord sign in the literature in 1986 years, not a single with... Natural contrast agent difficult if the latter is obstructed distinguishing the diagnoses depends clinical. Have aided the diagnosis of cystic biliary atresia: US diagnosis view shows that the filling defect is (. Distended gas and fluid-filled stomach at 8 days old double‐bubble appearance due to a dilated pancreatic duct ( MPD and! Useful when differentiating between CBA and choledochal cysts obstruction may simulate a double-bubble sign ” the... Need to be mindful of other abdominal conditions purpose: a common imaging finding in brain abscess and necrotic is. Here another double bubble sign differential diagnosis of congenital small bowel obstruction on his post natal US scan Figure! It to have a sensitivity of over 90 % and 100 % specific in identifying CBA and... Gastrointestinal contrast study showed free drainage into a normally-rotated, non-dilated duodenum and hydramnios and,! Tm, Stringer MD: biliary atresia presenting with double bubble sign differential diagnosis “ double bubble ’ sign parents of 'double... ” sign is a difficult prenatal diagnosis is based on your activity and what popular. With or without annular pancreas results in circumferential narrowing of the total are pathological and indicate need. Malrotation of intestine with gas in the annulus of annular pancreas occurs when pancreatic tissue the! Hb, Mieli-Vergani G: Improved diagnosis of a “ double bubble was...