Congenital Dilatation Research Articles

Features of diagnosis and treatment of a child with choledochal cyst IС type

Choledochal cysts are congenital cystic dilatations of the extrahepatic and/or intrahepatic bile ducts. Diagnosis and choice of treatment tactics for this pathology often cause difficulties. A case report of a choledochal cyst in a 2-year-old patient with symptoms of recurrent jaundice, cholangitis, and pancreatitis, which required laparoscopic cholecystostomy and cholangiography during preparation for radical surgery, was retrospectively analyzed. The child was re-admitted to the pediatric surgical department with complaints of hyperthermia up to 39°C, resumption of periodic abdominal pain, nausea, and increased icterus of the sclera and skin. Laboratory and ultrasound examination indicated biliary and pancreatic hypertension. Choledochal cyst, cholestasis syndrome, acute cholecystopancreatitis was diagnosed. Owing to the existing clinical symptoms and laboratory and ultrasound data, laparoscopic cholecystostomy was urgently performed. Against the background of conservative therapy, within 2 days after laparoscopic cholecystostomy, clinically, laboratory and according to control ultrasound examination, biliary and pancreatic hypertension were stopped. The child underwent cholangiography through a cholecystostomy without additional anesthesia, which revealed a fusiform dilatation of the common hepatic and bile ducts. After 2 weeks of drug treatment against the background of laparoscopic cholecystostomy, the child underwent excision of the common bile duct cyst, and cholecystectomy with hepatic enteroanastomosis on a Roux-en-Y loop. The postoperative period was smooth. No other complaints or complications were noted within 24 months. In the reported case, recurrent obstructive jaundice was caused by a choledochal cyst. Laparoscopic cholecystostomy enabled the quick and successful relief of biliary hypertension, preparation of the patient for radical surgical treatment, and safe informative cholangiography through cholecystostomy. Hepatic enteroanastomosis with a disconnected loop according to Roux-en-Y was an effective surgical treatment method for choledochal cyst.

Biliary Duct Dilatation: AJR Expert Panel Narrative Review.

Biliary duct dilatation is a common incidental finding in practice, but it is unlikely to indicate biliary obstruction in the absence of clinical symptoms or elevated levels on liver function tests (LFTs). However, the clinical presentation may be nonspecific, and LFTs may either be unavailable or difficult to interpret. The goal of this AJR Expert Panel Narrative Review is to highlight a series of topics fundamental to the management of biliary duct dilatation, providing consensus recommendations in a question-and-answer format. We start by covering a basic approach to interpreting LFT results, the strengths and weaknesses of the biliary imaging modalities, and how and where to measure the extrahepatic bile duct. Next, we define the criteria for biliary duct dilatation, including patients with prior cholecystectomy and advanced age, and discuss when and whether biliary duct dilatation can be attributed to papillary stenosis or sphincter of Oddi dysfunction. Subsequently, we discuss two conditions in which the duct is pathologically dilated but not obstructed: congenital cystic dilatation (i.e., choledochal cyst) and intraductal papillary neoplasm of the bile duct. Finally, we provide guidance regarding when to recommend obtaining additional imaging or testing, such as endoscopic ultrasound or ERCP, and include a discussion of future directions in biliary imaging.

Profiling of right internal jugular vein phlebectasia: a rare case study

Internal jugular vein phlebectasia (IJP) is a congenital non-tortuous dilatation of the internal jugular vein that manifests as a soft, compressible mass in the neck under stress or while doing the Valsalva maneuver. As per a review of several articles, children are more likely to be impacted than adults. It is a benign disorder that often exhibits no symptoms. However, alterations in voice, paralysis of the vocal cords, and/or dysphagia are some symptoms that may be observed. The present case report is that of a 4.5-year-old male child with right IJP and cervical lymphadenopathy, who presented with symptoms of swelling in the neck on straining and pain while swallowing.

Aggressive Pediatric Adrenal Cortical Carcinoma With a Novel Translocation t(20;22) and intergenic fusion of MN1 and ZNF341: A Case Report and Literature Review

Caroli disease involves several different entities in contrast to Caroli syndrome. We herein report two cases of congenital localized dilatation of the intrahepatic bile ducts exhibiting a unique morphology distinct from other types of Caroli disease. Specifically, the dilatation was limited to the second- and third-order intrahepatic bile ducts, and the dilated bile ducts abruptly connected to normal-sized ducts on both the upstream and downstream sides. Both cases are prenatally diagnosed and have been asymptomatic for nearly a decade. Nine other cases were identified in the English and Japanese literature, suggesting that this is a specific subtype of Caroli disease.

Multiple Choledochal Cysts: A Case Report

Background: Choledochal cysts (CC) are congenital dilatations of the biliary tree, characterized by varying degrees of cystic dilatation at various segments of the biliary tract (extrahepatic or intrahepatic), may be detected at any age and in any portion of the bile duct. This study was aimed to present the management of multiple choledochal cyst case.
 Case presentation: A 9 year old boy was brought by his mother to the emergency department at Dr. M. Djamil General Hospital Padang with chief complaint abdominal pain that’s getting worse 2 weeks before admission. Abdominal pain first complained by the patient since age of 4, the pain was felt all over stomach area, not localized, not radiated, not corelated with meal time, colicky, relieved with paracetamol. USG results: Choledochal cyst, Helicobacter pylori infection, elevated liver enzymes due to suspect suppression of choledochal cyst. Abdominal CT-Scan with contrast results: Intrahepatic and extrahepatic choledochal cyst (type IV A).
 Conclusion: Patients was diagnosed with multiple choledochal cysts, diffuse peritonitis, Helicobacter pylori infection, and familial short stature.

Association Among Cystic Volume, Intracystic Pressure, and Histopathological Changes in the Liver in Patients With Choledochal Cyst.

Background Choledochal cyst is a congenital cystic dilatation of the biliary tree. Various aspects of pathophysiology are unclear, particularly with reference to intracholedochal cystic pressure (ICCP) and liver histopathology. This study aimed to determine the relationship among cystic volume, ICCP, and histopathological changes in the liver in patients with choledochal cysts. Methods This cross-sectional study was carried out among 21 patients diagnosed with choledochal cysts, who attended the Department of Pediatric Surgery at Bangabandhu Sheikh Mujib Medical University (BSMMU) from April 2021 to August 2022. Cystic volume was measured pre-operatively using ultrasonography, while ICCP was measured per-operatively with a pressure gauge. Liver histology was assessed through an intraoperative liver biopsy and graded using the meta-analysis of histological data in viral hepatitis (METAVIR) scoring system. The data were analyzed using SPSS version 25.0 (IBM Corporation, Armonk, New York). Frequency and percentages were calculated to present categorical variables, and for quantitative variables, mean, standard deviation (SD), median, and interquartile range (IQR) were calculated. Fisher's exact tests were performed to determine the association between cystic volume, ICCP, and the grading of hepatic fibrosis. A p-value of <0.05 was considered statistically significant. Results The age of the patients ranged from 1 to 12 years, with a mean of 5.0±3.4 years. The male-to-female ratio was 1:4.3. Type I cysts were the most prevalent (71.4%). The median and IQR for cystic volume were 3.4 ml and 1.1-8.2 ml, respectively. The median and IQR for ICCP were 7.46 mmHg and 4.67-9.33 mmHg, respectively. The most frequent grade of fibrosis was F1 (38.1%), followed by F2 (23.8%) and F3 (23.8%). A negative relationship between cystic volume and ICCP was observed, which was statistically significant (p=0.008). A positive relationship was also observed between ICCP and the grading of liver fibrosis, which was statistically significant (p=0.002). Although a negative correlation between cystic volume and grading of liver fibrosis was noted, it was not statistically significant (p=0.198). Conclusions This study reveals significant associations between cystic volume, ICCP, and the grading of liver fibrosis in patients with choledochal cysts. Smaller volume cysts may exhibit higher ICCP, resulting in more pronounced fibrotic changes in the liver.

Choledochal Cyst (CC) Todani type I in two years old girl: a case report

Backgrounds: Choledocal cyst (CC) is a rare congenital cystic dilation of the bile duct and can be associated with severe complications, including malignancy and inflammation of the surrounding anatomy. Approximately 80% of CC is diagnosed in infants and young children in the first decade of life. Complete surgical resection is recommended in patients with choledochal malformations with excellent outcomes. Case Presentation: A two-year-old girl had intermittent abdominal pain in the upper right quadrant. This complaint was accompanied by fever, nausea and vomiting, decreased appetite and drinking. The physical examination revealed jaundice on the sclera and palpable liver, two fingers below the costal arch. Abdominal ultrasound revealed a cystic mass in the right lower lobe suggestive of the hepatic cyst with inflammation of the cystic head of the pancreas and minimal free fluid per lumen. An abdominal C.T. scan without contrast showed a choledochal cyst classified as Todani Type IV. A cyst in the bile duct was found during the surgical procedure Conclusion: Choledocal cyst is one of the differential diagnoses in children with symptoms of abdominal pain. Appropriate surgical measures should be undertaken to avoid complications.

A rare cause of neonatal feeding intolerance: congenital segmental intestinal dilatation—a case report

BackgroundCongenital segmental intestinal dilatation (CSID) is a rare cause of neonatal bowel obstruction. Patients can present in the early newborn period with feeding intolerance and abdominal distension. Diagnosis is challenging due to its rarity and non-specific clinical and radiological findings in a well neonate. Surgical excision remains the mainstay treatment for this disease.Case presentationWe report a case of a newborn male with congenital segmental ileal dilatation, who presented with abdominal distension and feeding intolerance. He was born preterm at 32 weeks and was treated for presumed sepsis due to a premature rupture of the membrane. The patient developed persistent abdominal distension upon initiation of feedings with large amount of gastric aspirates. Otherwise, there was no delay in the passage of meconium. Serial abdominal radiographs showed persistent focal bowel dilatation on the right side. He was initially diagnosed with small bowel atresia. Ultrasound abdomen and lower gastrointestinal contrasted study showed focal ileal dilatation with no evidence of pneumoperitoneum. Intraoperative findings revealed a segmental ileal dilatation with no evidence of mechanical obstruction. The abnormal dilated bowel was excised and sent for histopathological examination. He recovered well after surgery.ConclusionDiagnosis of CSID can be challenging due to its clinical polymorphism and non-specific radiological findings. CSID should be suspected in neonates with feeding intolerance and focal intestinal dilatation without clinical features of sepsis, peritonitis, or mechanical obstruction. Surgical excision is curative with a good outcome.

Embryonic statistical analyses reveal 2 growth phenotypes in mouse models of Down syndrome

Down syndrome is associated with several comorbidities, including intellectual disability, growth restriction, and congenital heart defects. The prevalence of Down syndrome-associated comorbidities is highly variable, and intellectual disability, although fully penetrant, ranges from mild to severe. Understanding the basis of this interindividual variability might identify predictive biomarkers of in utero and postnatal outcomes that could be used as endpoints to test the efficacy of future therapeutic interventions. The main objective of this study was to examine if antenatal interindividual variability exists in mouse models of Down syndrome and whether applying statistical approaches to clinically relevant measurements (ie, the weights of the embryo, placenta, and brain) could define cutoffs that discriminate between subgroups of trisomic embryos. Three commonly used mouse models of Down syndrome (Dp(16)1/Yey, Ts65Dn, and Ts1Cje) and a new model (Ts66Yah) were used in this study. Trisomic and euploid littermate embryos were used from each model with total numbers of 102 for Ts66Yah, 118 for Dp(16)1/Yey, 92 for Ts65Dn, and 126 for Ts1Cje. Placental, embryonic, and brain weights and volumes at embryonic day 18.5 were compared between genotypes in each model. K-mean clustering analysis was applied to embryonic and brain weights to identify severity classes in trisomic embryos, and brain and placental volumetric measurements were compared between genotypes and classes for each strain. In addition, Ts66Yah embryos were examined for malformations because embryonic phenotypes have never been examined in this model. Reduced body and brain weights were present in Ts66Yah, Dp(16)1/Yey, and Ts65Dn embyos. Cluster analysis identified 2 severity classes in trisomic embryos-mild and severe-in all 4 models that were distinguishable using a putative embryonic weight cutoff of <0.5 standard deviation below the mean. Ts66Yah trisomic embryos develop congenital anomalies that are also found in humans with Down syndrome, including congenital heart defects and renal pelvis dilation. Statistical approaches applied to clinically relevant measurements revealed 2 classes of phenotypic severity in trisomic mouse models of Down syndrome. Analysis of severely affected trisomic animals may facilitate the identification of biomarkers and endpoints that can be used to prenatally predict outcomes and the efficacy of treatments.

Robotic Surgery for Pediatric Choledochal Cysts: An American Case Series and Literature Review

IntroductionCholedochal cysts are rare congenital biliary cystic dilations. The US incidence rate varies between 5 and 15 cases per 1,000,000 people. In contrast, Asians, which are a large subset of the population of Hawaii, have an incidence of approximately one in every 1000 births. We report our experience with robot-assisted laparoscopic surgical management with biliary reconstruction of choledochal cysts which to date is the largest American case series to be reported. Materials and MethodsFrom 2006 to 2021, patients diagnosed with a choledochal cyst(s) at a tertiary children's hospital were retrospectively reviewed. Perioperative analysis was performed. Complications were defined as immediate, early, or late. The data underwent simple descriptive statistics. ResultsNineteen patients underwent choledochal cystectomy and hepaticoduodenostomy. Thirteen underwent a robotic approach while the rest were planned laparoscopic. Eighteen of 19 were female with 15/19 of Asian descent. The ages ranged from 5 mo to 21 y. Presenting diagnoses included jaundice, primary abdominal pain, pancreatitis, and cholangitis. Sixty eight percent had type 1 fusiform cysts while the rest were type 4a. Operative time and length of stay for robotic versus laparoscopic were 321 versus 267 min and 8.2 versus 17.3 d, respectively. For the robotic group, there was one immediate complication due to peritonitis. One-year follow-up revealed two patients requiring endoscopic retrograde cholangiopancreatography with dilation/stenting for an anastomotic stricture. There were no anastomotic leaks. ConclusionsRobot-assisted laparoscopic choledochal cystectomy with hepaticoduodenostomy is associated with overall good outcomes with the most common long-term complication being anastomotic stenosis.

Cardiovascular Manifestations of Turner Syndrome: Phenotypic Differences Between Karyotype Subtypes.

Turner syndrome (TS) is a genetic disorder presenting in phenotypic females with total or partial monosomy of the X chromosome. Cardiovascular abnormalities are common, including congenital heart defects (CHD) and aortic dilation. Although mosaic TS is suspected to have less severe phenotype as compared to non-mosaic TS, differences in cardiovascular manifestations between karyotypes are not well studied. This is a single-center retrospective cohort study including patients with TS seen from 2000 to 2022. Demographic data, chromosomal analysis, and imaging were reviewed. Karyotypes were categorized as monosomy X (45X), 45X mosaicism, isochromosome Xq, partial X deletions, ring X(r(X)), TS with Y material, and others. Prevalence of CHD and aortic dilation were compared between monosomy X and other subtypes using Pearson's chi-square test and Welch two-sample t-test. We included 182 TS patients with median age 18 (range 4-33) years. CHD was more common in monosomy X as compared with others (61.4%vs.26.8%, p < 0.001), including bicuspid aortic valve (44.3%vs.16.1%, p < 0.001), partial anomalous pulmonary venous return (12.9%vs.2.7%, p = 0.023), persistent left superior vena cava (12.9%vs.1.8%, p = 0.008), and coarctation of the aorta (20.0%vs.4.5%, p = 0.003). Cardiac surgery (24.3%vs.8.9%, p = 0.017) was more prevalent in the monosomy X group. There was no statistically significant difference for presence of aortic dilation (7.1% vs 1.8%, p = 0.187). Although CHD and need for cardiac surgery are more common in TS with monosomy X as compared to others, all TS subtypes may have similar risk of developing aortic dilation. All TS patients should have similar cardiovascular surveillance testing to monitor for aortic dilation.

Caroli Syndrome Associated with Polycystic Kidney Disease: A Case Report

Introduction: Caroli disease is a fibrocystic liver disease characterized by congenital nonobstructive cystic dilatation of the intrahepatic bile ducts. Its association with congenital hepatic fibrosis defines Caroli syndrome. The more common Caroli syndrome is associated with cystic renal dilatations in 60-80% of patients with polycystic kidney disease and Cacchi-Ricci disease as the most frequently noted renal anomalies. Objective: We want to define this uncommon entity and to describe the radiological characteristics which can, in the absence of their knowledge, be a source of confusion for the radiologist and pose problems of diagnostic delay. Case Report: The patient was a male infant, 1 year and 10 months old, with a history of 2 episodes of urinary tract infections at the age of 3 months and 6 months, and a 13-year-old sister with polycystic hepatorenal disease. The patient was admitted to the pediatric emergency department for acute fever associated with fluid diarrhea evolving for 3 days and irritability and crying at the time of urination. A cytobacteriological examination of the urine showed a turbid appearance of the urine with isolation of Escherichia Coli on culture. Abdominal ultrasound revealed bilateral nephromegaly, with diffuse microcystic hyperechogenicity of medullary pyramids generating multiple comet tail images with a "sponge kidney" appearance as well as a homogeneous splenomegaly and a normal sized liver of heterogeneous echostructure with cystic dilatation of the intrahepatic bile ducts which are centered by hyperechoic punctuations realizing the "Dot Sign". The individualization of this sign at an early age associated with splenomegaly allowed the diagnosis of a Caroli syndrome. While the diffuse medullary hyperechogenicity in the kidney allowed to evoke its association with polycystic kidney disease, especially the recessive one. Discussion and Conclusion: Caroli syndrome is a rare entity of autosomal recessive transmission. ..........

TYPE IV-A CHOLEDOCHAL CYST IN NEWBORN

A congenital cystic dilatation of the bile duct is called a choledochal cyst; it may involve the extrahepatic, intrahepatic, or both biliary radicles. It is an uncommon illness that affects people more frequently in developing nations. We describe a highly uncommon case of a male newborn who has Type IV-A choledochal cyst. The denitive diagnosis was made based on magnetic resonance cholangiopancreatography (MRCP) which showed left sided dilated intrahepatic biliary radicals with fusiform dilatation of common bile duct (CBD) and common hepatic duct (CHD). The decision to follow up with a subsequent surgical plan was taken because there were no complications and the bilirubin level returned to normal after conservative treatment.

Congenital segmental dilatation of the colon in a newborn: a radiologic perspective of an unusual entity.

A newborn with congenital segmental dilatation of the intestine affecting the colon is presented. This rare condition, unrelated to Hirschsprung's disease, may affect any portion of the bowel and is characterized by focal dilatation of a segment of bowel flanked by normal proximal and distal bowel. While reported in the surgical literature, congenital segmental dilatation of the intestine has not been reported in the pediatric radiology literature even though pediatric radiologists may be the first to encounter imaging suggesting the diagnosis. We therefore present the characteristic imaging findings, including abdominal radiographs and images from a contrast enema, and discuss the clinical presentation, pathology findings, associations, treatment, and prognosis of congenital segmental dilatation of the intestine to increase awareness of this unusual diagnosis.

A Case Report of Intraductal Papillary Neoplasm Associated with an Extrahepatic Bile Ducts

Choledochal cysts are congenital cystic dilations of the extrahepatic and/or intrahepatic bile ducts and are rare. Adults with choledochal cysts are at risk of developing biliary carcinoma. Our case is a 31-year-old female patient who presented with right upper quadrant pain. In magnetic resonance imaging/magnetic resonance cholangiopancreatography, there was a fusiform enlargement of the extrahepatic bile duct and cystic duct and it was evaluated as compatible with Type 1 choledochal cyst. Extrahepatic biliary tract excision-cholecystectomy-Roux En Y hepaticojejunostomy procedure was performed. The pathology result was intraductal papillary neoplasm (IPN). IPN is a rare entity in the biliary tract and because it is precancerous, early diagnosis and surgical excision are critical.

Radiological and surgical correlation of pelviureteric junction obstruction in positional anomalies of the kidney in children.

Pelviureteric junction obstruction, also known as ureteropelvic junction obstruction, is a congenital narrowing of the urinary excretory tract at the junction between the renal pelvis and the ureter and is a common cause of congenital pelvicalyceal dilatation. The outcome is variable, from spontaneous resolution to renal parenchymal function loss in cases of untreated high-grade obstruction. Abnormalities in renal ascent, rotation and vascularity can be associated with pelviureteric junction obstruction and easily overlooked radiologically. In this pictorial review, we explore the anatomical, radiological and surgical correlations of pelviureteric junction obstruction in the context of a normal kidney and a spectrum of renal abnormalities, including hyper-rotation (also known as renal malrotation), failed renal ascent, fusion anomalies and accessory crossing renal vessels. For each scenario, we provide technical tips on how to identify the altered anatomy at the first ultrasound assessment and correlation with scintigraphic, cross-sectional and postoperative imaging where appropriate. A detailed ultrasound protocol specifically to assess and characterise pelviureteric junction obstruction in paediatric patients is also offered.

Efficacy of robot-assisted hepaticojejunostomy and laparoscopic-assisted hepaticojejunostomy in pediatric congenital choledochal dilatation: a system review and meta-analysis.

The efficacy of robot-assisted hepaticojejunostomy (RAHJ) and laparoscopic-assisted hepaticojejunostomy (LAHJ) in children with congenital choledochal dilatation has been a topic of much debate and controversy. The purpose of this study was to evaluate the role of RAHJ and LAHJ in pediatric congenital choledochal dilatation. The review program has been prospectively registered (PROSPEROID: CRD42022306868). We searched the PubMed, Embase, Cochrane, CBM, VIP, Web of Science, CNKI databases, and Wanfang databases from March 2021. The Mantel-Haenszel method and a random-effects model were used to figure out the hazard ratio (95% CI). Ten studies evaluated eight hundred and sixty-nine subjects (three hundred and thirty-two in the robotic group and five hundred and thirty-seven in the laparoscopic group), meeting all inclusion criteria. Compared with the laparoscopic group, robotic group demonstrated fewer postoperative complications [p = 0.0009; OR = 0.34 (95% CI, 0.18-0.64); I2 = 3%], shorter postoperative hospital stay [p < 00,001; MD = -2.05 (95% CI, -2.40-1.70); I2 = 0%], and less intraoperative bleeding [p = 0.008; MD = -10.80 (95% CI, -18.80-2.81); I2 = 99%]. There was no significant difference in operative time between the two groups [p = 0.10; MD = 24.53 (95% CI, -5.11-54.17); I2 = 99%]. The same situation happened in short-term complication outcomes [p = 0.06; RR = 0.45 (95% CI, 0.19-1.04); I2 = 0%]. However, children in the RAHJ group had significantly lower levels of long-term complications [p = 0.04; OR = 0.41 (95% CI, 0.17-0.96); I2 = 0%]. Hospitalization costs were significantly higher in the RAHJ group [p < 0.00001; OR = 27,113.86 (95% CI, 26,307.24-27,920.48); I2 = 0%]. For overall complications, subgroup analysis of literature published after 2020 and of literature with high quality scores showed a significant decrease in the RAHJ group. In children with congenital choledochal dilatation, RAHJ is associated with reduced intraoperative bleeding, postoperative complications, and length of stay. Robotic surgery has a bright future in the treatment of pediatric common hepatic duct cysts and deserves to be promoted and popularized.

Choledochal anomalies in adults: A 20-year single-center retrospective cohort experience in the Middle East

Background and study aimsCholedochal cysts are rare congenital cystic dilatations of the bile ducts that occur in fewer than 1% of individuals. The disease is common in East Asia, and most of the literature concerns those populations, but some data about Western populations have been published recently. Long-term reports about the disease in Middle Eastern populations, however, are currently lacking. We report a single-center 20-year experience in diagnosing and managing choledochal anomalies. Patients and methodsParticipants were adult patients in whom choledochal cysts were diagnosed over a 20-year (2000–2019) period at a single tertiary academic care center. Clinical data, including radiologic imaging findings, were retrieved from the patients’ medical records. To describe the baseline characteristics of the population, we calculated descriptive statistics. ResultsCholedochal anomalies were diagnosed in 19 adult patients, whose median age was 30 years (interquartile range [IQR], 23–67 years). Of the choledochal cysts 13 (68.4%) were classified as Todani type I, 4 (21.1%) as Todani type IV, and 3 (15.8%) as Todani type V (Caroli’s disease). No patient had underlying chronic liver disease, and liver synthetic function was preserved in all. Eighteen patients (94.7%) underwent surgery: cyst excision with Roux-en-Y hepaticojejunostomy in 17 and liver transplantation in 1. All 18 survived surgery, and the median postoperative hospital stay was 11 days (IQR, 5–34 days). All 18 were alive 90 days after surgery, and the median follow-up period was 40 months (IQR, 12–140 months). Seven patients (36.8%) developed postoperative surgical complications; 2 patients required rehospitalization, and 1 required reoperation. ConclusionThis description of adults with choledochal cysts is the latest long-term report about this disease in the Middle East. In our 20-year experience, the disease characteristics in our patients were moderately consistent with those described previously.

Cardiovascular risk stratification in Turner syndrome: A study of 173 cases

Turner syndrome (TS) is the most common genetic disorder occurring in 1/2500 live-born girls. Morbimortality is increased in TS and related to cardiovascular (CV) disease such as congenital heart disease (CHD), aortic dilatation and dissection. The main objective of this study was the evaluation of the cardiovascular risk of aortic dissection in pediatric and young patients with TS. A prospective multicentric study was conducted in young female with a Karyotype proven TS. Physical examination and echocardiography were performed for all the patients. Ambulatory monitoring of blood pressure of 24 h for patients over 6 years of age, cardiac MRI or CT-scan for patients over 10 years of age or as needed. In total, 173 patients were included; mean age was 10.5 years. A total of 61.3% had mosaic, 29.5% had monosomy and 9.2% had a structural X chromosome abnormality. The frequency of congenital heart disease was 31.2%. Bicuspid aortic valve was the most common being detected in 22.5% followed by aortic coarctation (4.6%). High blood pressure was detected in 26 patients (15%) with 41.7% of non-dipper profile. Primary hypertension was the most common. Aortic dilatation was found in 4 cases, neither aneurysm nor dissection were found 69.4% of patients were at mild risk of dissection, 28.3% were at moderate risk and 2.3% were at severe risk. BAV, coarctation and monosomy appeared to be risk factors of aortic vasculopathy. A cardiovascular profile should be determined at diagnosis and patient should have a careful monitoring from the age of diagnosis to adulthood in order to identify the patient at risk of aortic dissection and prevent CV complications.

A case series of choledochal cyst with pancreatic divisum: A rare association.

Choledochal cysts (CC) are congenital cystic dilations of the biliary tree usually associated with abnormal pancreaticobiliary ductal junction (APBDJ), but its association with pancreatic divisum has been rarely described. We encountered four cases of CC associated with pancreatic divisum (PD). Three had Type 3 PD and one had Type 1 PD. Two cases presented with pancreatic complications, with one case requiring preoperative minor papilla sphincterotomy for recurrent pancreatitis. The association of CC with PD is infrequent, and the variable presentation alters management strategy. PD may be one of the factors responsible for complications associated with CC.