Cystic Biliary Atresia: A Wolf in Sheep's Clothing

2013 ◽  
Vol 79 (9) ◽  
pp. 870-872 ◽  
Author(s):  
Sarah J. Hill ◽  
Matthew S. Clifton ◽  
Sarkis C. Derderian ◽  
Mark L. Wulkan ◽  
Richard R. Ricketts
Keyword(s):  
Surgical Treatment ◽  
Obstructive Jaundice ◽  
Biliary Atresia ◽  
Neonatal Jaundice ◽  
Surgical Intervention ◽  
Institutional Review Board ◽  
Significant Delay ◽  
Institutional Review ◽  
Improved Outcomes ◽  
Cystic Biliary Atresia

Neonatal obstructive jaundice is frequently explained by biliary atresia (BA) or the presence of a choledochal cyst (CC). Cystic biliary atresia (CBA) has been a proposed as a subtype of BA with projected improved outcomes. We aimed to characterize these lesions further. We conducted an Institutional Review Board-approved review of all patients treated for obstructive jaundice at our tertiary children's hospital over 10 years. Over the decade we evaluated 91 children with obstructive jaundice: 13 CBA, 52 BA, and 26 CC. Patients with isolated CBA and BA were diagnosed significantly earlier than those with CC (15.9, 54, and 281 days, respectively; P = 0.0001). There was a significant delay between diagnosis and surgical intervention for patients with CBA compared with BA: 17 days versus 5.7 days ( P = 0.004). There was no difference in rate of transplant between CBA and BA (31 vs 50%; P = 0.35). The time from surgery until transplant was 13.9 and 18.6 months for CBA and BA, respectively ( P = 0.62). Although radiographically similar to CC, CBA behaves similarly to isolated BA. Delay in recognition and surgical treatment may affect outcomes and lead to an increased incidence of liver failure. The presence of a cystic biliary malformation in the setting of neonatal jaundice should be regarded as CBA until proven otherwise.

Spontaneous Perforation of the Extrahepatic Bile Ducts in an Infant

PEDIATRICS ◽  
1982 ◽  
Vol 70 (4) ◽  
pp. 601-603
Author(s):  
Dan Andersson ◽  
Ingemar Helin ◽  
Sven-Caspar Nettelblad ◽  
Clas-Göran Cederlund
Keyword(s):  
Surgical Treatment ◽  
Obstructive Jaundice ◽  
Biliary Atresia ◽  
Bile Ducts ◽  
Surgical Intervention ◽  
Rare Condition ◽  
First Year ◽  
Spontaneous Perforation ◽  
Extrahepatic Bile Ducts ◽  
First Year Of Life

Spontaneous perforation of the extrapepatic bile ducts, with an accumulation of bile in the peritoneal cavity, is a rare condition. Nevertheless, it is second in frequency to biliary atresia as the cause of obstructive jaundice during the first year of life.1 Although approximately 60 cases of this condition have been described in the literature, it is only occasionally listed in pediatric textbooks. Nevertheless, it is important that the condition be recognized in time to perform lifesaving surgical intervention. We therefore consider it instructive to present a patient with the classic features of this condition who was cured by surgical treatment on the 29th day of life.

scholarly journals Cystic form of biliary atresia. Treatment experience

2020 ◽  
Vol 10 (1) ◽  
pp. 17-24
Author(s):  
Irina I. Borisova ◽  
Anatoliy V. Kagan ◽  
Svetlana A. Karavaeva ◽  
Aleksey N. Kotin
Keyword(s):  
Surgical Treatment ◽  
Bile Duct ◽  
Common Bile Duct ◽  
Biliary Atresia ◽  
Bile Ducts ◽  
Surgical Intervention ◽  
Ultrasound Examination ◽  
Duct Cyst ◽  
Cystic Form ◽  
The Common

Background. The cystic form of biliary atresia is a rare form of atresia of the biliary tract, which is a relatively favorable variant of the defect and can be diagnosed antenatally. In practice, it is important not only to suspect this diagnosis, but also to differentiate this variant of impaired development of the external bile duct from the cyst of the common bile duct. This is due to the difference in approaches and methods of surgical treatment of choledochal cysts and biliary atresia. Obliteration (atresia) of the bile ducts in the absence of timely surgical intervention quickly leads to the progression of cirrhosis and the development of liver failure. The method of choice in the treatment of AD is Kasai surgery, often palliative in nature, but allowing to delay the time until liver transplantation. The cyst of the common bile duct rarely requires early surgical treatment, and the risk of cirrhosis is significantly lower. Surgical intervention is aimed at removing the cyst and restoring the flow of bile by anastomosing the external bile ducts with the intestines, which is a radical method of treatment and leads to the recovery of the child. External similarity in ultrasound examination of the fetus and newborn baby of the cystic form of biliary atresia of the bile ducts with a cyst of the common bile duct does not always allow differentiation of one defect from another, which can lead to untimely correction of the defect and an unfavorable outcome. Aim. Demonstrate a rare type of biliary atresia. Materials and methods. Between 2001 and 2019, 33 patients with biliary atresia were treated in the Childrens City Multidisciplinary Clinical Specialized Center for High Medical Technologies in St. Petersburg, only two patients had a cystic form. Both children were initially treated as patients with bile duct cyst. Children were operated on at the age of 2 and 3.5 months. The first patient underwent surgery Kasai, the second hepaticoyunoanastomosis. Results. During the observation period (9 years and 4 years), the synthetic function of the liver is normal, and there are currently no indications for transplantation. Conclusion. If a fetus or a newborn with neonatal jaundice is detected during ultrasound examination of a cystic formation in the gates of the liver, it is very important to correctly and quickly make a differential diagnosis between the cystic form of biliary atresia of the biliary tract and the common bile duct cyst.

scholarly journals Transpetrous Clip Occlusion of a Giant Midbasilar Aneurysm Using Hypothermic Cardiac Arrest: 2-Dimensional Operative Video

2020 ◽  
Vol 19 (2) ◽  
pp. E143-E143
Author(s):  
Benjamin K Hendricks ◽  
Robert F Spetzler
Keyword(s):  
Cardiac Arrest ◽  
Surgical Intervention ◽  
Video Recording ◽  
Mass Effect ◽  
Institutional Review Board ◽  
High Morbidity ◽  
Vestibulocochlear Nerve ◽  
Institutional Review Board Approval ◽  
Institutional Review ◽  
Clip Occlusion

Abstract Basilar trunk aneurysms are historically associated with extremely high morbidity. The complexity of circumflex perforator arteries responsible for the perfusion of the brainstem makes surgical intervention for these lesions particularly challenging. This patient had a giant midbasilar aneurysm that encompassed multiple perforators but was associated with progressive mass effect and debilitating morbidity. Therefore, a transpetrous approach was used with transposition of the facial nerve and sacrifice of the vestibulocochlear nerve to permit access to the aneurysm. Hypothermic cardiac arrest was used to permit dome manipulation with a tandem fenestrated clipping of the aneurysm. Postoperative imaging demonstrated a reduction in mass effect attributable to the significantly reduced dome size and persistence of flow via a reconstructed basilar trunk. The patient gave informed consent for surgery and video recording. Institutional review board approval was deemed unnecessary. Used with permission from Barrow Neurological Institute, Phoenix, Arizona.

The registry of anomalous aortic origin of the coronary artery of The Congenital Heart Surgeons’ Society

2010 ◽  
Vol 20 (S3) ◽  
pp. 50-58 ◽  
Author(s):  
Julie A. Brothers ◽  
J. William Gaynor ◽  
Jeffrey P. Jacobs ◽  
Christopher Caldarone ◽  
Anusha Jegatheeswaran ◽  
...  
Keyword(s):  
Young Adults ◽  
Coronary Artery ◽  
Congenital Heart ◽  
Surgical Intervention ◽  
Institutional Review Board ◽  
Successful Implementation ◽  
Cooperative Effort ◽  
Institutional Review ◽  
Children And Young Adults ◽  
The Impact

AbstractThe anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva with interarterial, intramural, and/or intraconal course is a rare congenital anomaly that is associated with a high risk of sudden death in children. The Congenital Heart Surgeons’ Society established the Registry of Anomalous Aortic Origin of the Coronary Artery to help determine the outcome of children and young adults managed with surgical intervention versus observation and to test the hypothesis that subsets of patients with anomalous aortic origin of a coronary artery can be identified in whom the risk of intervention is less than the risk of observation. All institutional members of the Congenital Heart Surgeons’ Society were recruited for participation. The registry consists of a retrospective cohort of patients diagnosed between 1 January, 1998 and 20 January, 2009 and a prospective, population-based cohort of patients newly diagnosed from 21 January, 2009 onwards. Baseline demographics, diagnoses, and results of tests will be obtained through a review of the medical records. Annual follow-up data will be collected. Data will be analysed for different factors of risk at diagnosis, different strategies of treatment, and the impact of both on the outcomes of the patients. As of June 2010, 28 institutions had applied for approval from their institutional review board and 16 institutions had received approval from their institutional review board. Seventy-four patients have enrolled to date. We hope to use the established Pediatric Cardiomyopathy Registry as a guide to successful implementation, with a cooperative effort between institutions. The overall purpose of the Registry of Anomalous Aortic Origin of the Coronary Artery is to determine the outcome of surgical intervention versus observation in children and young adults with anomalous aortic origin of a coronary artery, and to describe the natural and “unnatural” history of these patients over the course of their lifetime. In this report, we describe the creation and design of the Registry of Anomalous Aortic Origin of the Coronary Artery. Data from the registry will be published at a later date.

scholarly journals Multivariate Analysis of Early Surgical Management Factors Affecting Posttraumatic Penoscrotal Avulsion Injury: A Level I Trauma Center Study

2020 ◽  
Author(s):  
MIN JI KIM ◽  
Dong Hwan Lee ◽  
Dong HA Park ◽  
Il Jae Lee
Keyword(s):  
Surgical Treatment ◽  
Scale Score ◽  
Surgical Management ◽  
Trauma Center ◽  
High Speed ◽  
Treatment Algorithm ◽  
Institutional Review Board ◽  
Comprehensive Treatment ◽  
Institutional Review ◽  
Braden Scale

Abstract Background To conduct an accurate evaluation of patients presenting with posttraumatic penoscrotal injuries, and to formulate a treatment algorithm based on this assessment. Methods We conducted a retrospective chart review study. Patients with penoscrotal defects admitted to our level Ⅰ trauma center from 2017 to 2019 were evaluated. The Braden scale score was used for wound evaluation and the Korean patient classification system (KPCS) was used for assessment of severity. Univariate and multivariate analyses were performed for potential risk factors associated with early surgical management. Results In total, there were 58 male patients, and the average Braden scale score was 12.08 ± 2.54, with the scrotum (36.20%), and the penile shaft (32.76%) being popular sites for injuries. The wounds requiring surgical treatment were 20.68% (n=12), with local flaps (33.33%) being most commonly used. The significant predictors of advanced wounds which required surgical treatment were old age (p=0.026, Odds Ratio [OR] 8.238), orthopedic combined injuries (p=0.044, OR 1.088), intubation (p=0.018, OR 9.625), restrain (p=0.036, OR 0.157) and blood transfusion (p<0.001, OR 2.462).Conclusion In multiple trauma patients, penoscrotal defects caused by high-speed trauma are an important matter of concern. Specifically, patients with combined skeletal injuries or requiring respiratory care were prone to advanced wounds. We proposed a five-category algorithm to manage such patients, which included severity of the patient’s condition, respiration, hemodynamic status, comorbidity, and immobilization. Additionally, inter-departmental cooperation and active intervention by plastic surgeons is needed for the comprehensive treatment of such injuries. Trial registration This study was performed in line with the principles of the Declaration of Helsinki. The study and all its protocols were approved by the institutional review board of Ajou Medical Center (approval no. AJIRB-MED-MDB-17-254). The need for informed consent was waived by the institutional review board of our hospital due to the retrospective design of the study.

Fecal Excretion of Rose Bengal I131 in the Diagnosis of Obstructive Jaundice in Infancy with Special Reference to Biliary Atresia

PEDIATRICS ◽  
1971 ◽  
Vol 48 (6) ◽  
pp. 966-969
Author(s):  
João Gilberto Maksoud ◽  
Anneliese Fischer Thom ◽  
Julio Kieffer ◽  
Virgilio A. Carvalho Pinto
Keyword(s):  
Differential Diagnosis ◽  
Obstructive Jaundice ◽  
Biliary Atresia ◽  
Special Reference ◽  
Rose Bengal ◽  
Poor Prognosis ◽  
Surgical Intervention ◽  
Correct Diagnosis ◽  
Fecal Excretion ◽  
The Poor

Despite the poor prognosis of biliary atresia, there are a small number of cases in which early surgery will be of real benefit. Every effort must therefore be made to arrive at the correct diagnosis as quickly as possible so as not to delay surgical intervention. In our experience, FERB I131 has been the most reliable diagnostic test; we are convinced that this test must be done in every case in which the differential diagnosis of neonatal or infantile jaundice is not clear.

scholarly journals Cystic Biliary Atresia: A Delayed Diagnosis of Obstructive Jaundice

2018 ◽  
Vol 26 (2) ◽  
pp. 102-104
Author(s):  
Aishath Azna ALI ◽  
Firdaus HAYATI ◽  
Nornazirah AZIZAN ◽  
Che Ismail CHE NOH ◽  
Marjmin OSMAN
Keyword(s):  
Obstructive Jaundice ◽  
Biliary Atresia ◽  
Delayed Diagnosis ◽  
Cystic Biliary Atresia

scholarly journals Multivariate analysis of early surgical management factors affecting posttraumatic penoscrotal avulsion injury: a level I trauma center study

BMC Urology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Min Ji Kim ◽  
Dong Hwan Lee ◽  
Dong Ha Park ◽  
Il Jae Lee
Keyword(s):  
Surgical Treatment ◽  
Scale Score ◽  
Surgical Management ◽  
Trauma Center ◽  
Treatment Algorithm ◽  
Institutional Review Board ◽  
Comprehensive Treatment ◽  
Institutional Review ◽  
Braden Scale ◽  
Level I

Abstract Background To conduct an accurate evaluation of patients presenting with posttraumatic penoscrotal injuries, and to formulate a treatment algorithm based on this assessment. Methods We conducted a retrospective chart review study. Patients with penoscrotal defects admitted to our level I trauma center from 2017 to 2019 were evaluated. The Braden scale score was used for wound evaluation and the Korean patient classification system (KPCS) was used for assessment of severity. Univariate and multivariate analyses were performed for potential risk factors associated with early surgical management. Results In total, there were 58 male patients, and the average Braden scale score was 12.08 ± 2.54, with the scrotum (36.20%), and the penile shaft (32.76%) being popular sites for injuries. The wounds requiring surgical treatment were 20.68% (n = 12), with local flaps (33.33%) being most commonly used. The significant predictors of advanced wounds which required surgical treatment were old age (p = 0.026, odds ratio [OR] 8.238), orthopedic combined injuries (p = 0.044, OR 1.088), intubation (p = 0.018, OR 9.625), restraint (p = 0.036, OR 0.157) and blood transfusion (p < 0.001, OR 2.462). Conclusion In multiple trauma patients, penoscrotal defects caused by high-speed trauma are an important matter of concern. Specifically, patients with combined skeletal injuries or requiring respiratory care were prone to advanced wounds. We proposed a five-category algorithm to manage such patients, which included severity of the patient’s condition, respiration, hemodynamic status, comorbidity, and immobilization. Additionally, inter-departmental cooperation and active intervention by plastic surgeons is needed for the comprehensive treatment of such injuries. Trial registration This study was performed in line with the principles of the Declaration of Helsinki. The study and all its protocols were approved by the institutional review board of Ajou Medical Center (approval no. AJIRB-MED-MDB-17-254). The need for informed consent was waived by the institutional review board of our hospital due to the retrospective design of the study.

scholarly journals Multivariate Analysis of Early Surgical Management Factors Affecting Posttraumatic Penoscrotal Avulsion Injury: A Level I Trauma Center Study

2020 ◽  
Author(s):  
MIN JI KIM ◽  
Dong Hwan Lee ◽  
Dong HA Park ◽  
Il Jae Lee
Keyword(s):  
Surgical Treatment ◽  
Scale Score ◽  
Surgical Management ◽  
Trauma Center ◽  
High Speed ◽  
Treatment Algorithm ◽  
Institutional Review Board ◽  
Comprehensive Treatment ◽  
Institutional Review ◽  
Braden Scale

Abstract Background To conduct an accurate evaluation of patients presenting with posttraumatic penoscrotal injuries, and to formulate a treatment algorithm based on this assessment. Methods We conducted a retrospective chart review study. Patients with penoscrotal defects admitted to our level Ⅰ trauma center from 2017 to 2019 were evaluated. The Braden scale score was used for wound evaluation and the Korean patient classification system (KPCS) was used for assessment of severity. Univariate and multivariate analyses were performed for potential risk factors associated with early surgical management. Results In total, there were 58 male patients, and the average Braden scale score was 12.08 ± 2.54, with the scrotum (36.20%), and the penile shaft (32.76%) being popular sites for injuries. The wounds requiring surgical treatment were 20.68% (n=12), with local flaps (33.33%) being most commonly used. The significant predictors of advanced wounds which required surgical treatment were old age (p=0.026, Odds Ratio [OR] 8.238), orthopedic combined injuries (p=0.044, OR 1.088), intubation (p=0.018, OR 9.625), restrain (p=0.036, OR 0.157) and blood transfusion (p<0.001, OR 2.462).Conclusion In multiple trauma patients, penoscrotal defects caused by high-speed trauma are an important matter of concern. Specifically, patients with combined skeletal injuries or requiring respiratory care were prone to advanced wounds. We proposed a five-category algorithm to manage such patients, which included severity of the patient’s condition, respiration, hemodynamic status, comorbidity, and immobilization. Additionally, inter-departmental cooperation and active intervention by plastic surgeons is needed for the comprehensive treatment of such injuries. Trial registration This study was performed in line with the principles of the Declaration of Helsinki. The study and all its protocols were approved by the institutional review board of Ajou Medical Center (approval no. AJIRB-MED-MDB-17-254). The need for informed consent was waived by the institutional review board of our hospital due to the retrospective design of the study.

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