The efficacy of double-balloon enteroscopy for intrahepatic bile duct stones after Roux-en-Y hepaticojejunostomy for choledochal cysts

Introduction

Intrahepatic bile duct (IHBD) stones are one of the most complicated morbidities that occur after Roux-en-Y hepaticojejunostomy (RYH); however, the optimal therapeutic approach is controversial.

Methods

Double-balloon enteroscopy (DBE) has been widely and frequently performed even in pediatric patients. We herein report the successful management of IHBD stones by biliary lithotripsy using DBE after RYH for a choledochal cyst (CC). DBE has made it possible to perform endoscopic therapeutic intervention, including balloon dilatation of an anastomotic stricture and removal of IHBD stones, without any major complications.

Conclusion

DBE is a less invasive and safe treatment method for IHBD stones in pediatric patients, which is capable of reaching the bilioenteric anastomosis after RYH for CC.     

Steroid pulse therapy prevents restenosis following balloon dilatation for esophageal stricture

Purpose

This study aimed to evaluate the effectiveness of intravenous steroid pulse therapy following balloon dilatation for esophageal stenosis and stricture in children.

Methods

The study enrolled six children, including three with congenital esophageal stenosis and three with anastomotic strictures after surgery for esophageal atresia, all of whom were treated by balloon dilatation combined with high-dose intravenous methylprednisolone pulse therapy. Methylprednisolone was injected intravenously at a dose of 20 mg/kg/day for 2 days, starting from the day of dilatation, followed by 10 mg/kg/day for 2 days, for a total of 4 days.

Results

Esophageal stricture recurred in all three patients with congenital esophageal stenosis despite repeated balloon dilatation without methylprednisolone. However, the symptoms of dysphagia improved and did not recur after systemic steroid pulse therapy following balloon dilatation. Symptoms also resolved in all three patients with anastomotic strictures following balloon dilatation with systemic steroid pulse therapy. All six patients remained asymptomatic after 6–21 months follow-up, with no complications.

Conclusion

Intravenous methylprednisolone pulse therapy following balloon dilatation is safe and effective for the treatment of esophageal stenosis and strictures in children.

     

Pediatric living donor liver transplantation for biliary atresia with hepatopulmonary syndrome: the gift of a second wind

Purpose

Hepatopulmonary syndrome (HPS) is a progressive, deteriorating complication of end-stage liver disease (ESLD) that occurs in 13?C47% of liver transplant candidates. Although LT is the only therapeutic option for HPS, it has a high morbidity and mortality, especially in patients with severe hypoxemia before transplantation, but the course of HPS after living donor liver transplantation (LDLT), especially for biliary atresia (BA) patients is not well established.

Patients and methods

The present study evaluated 122 patients who received an LDLT for BA and of these, 3 patients had HPS at the time of LDLT in a single-center series.

Results

Two patients of the HPS patients them had biliary and/or vascular complications, but they recovered uneventfully with interventional treatment. None of the patients required supplemental oxygen and had no residual cardiopulmonary abnormalities at a follow-up of more than 24?months.

Conclusion

Although a series of three patients is too small for definitive conclusion and further investigations must be conducted, pediatric LDLT can be a favorable therapeutic option for HPS.     

Stumpfes Bauchtrauma

Background

Developments in the treatment of blunt organ injuries in childhood are focused on conservative measures.

Diagnosis

Ultrasound and computer tomography are used to provide the most sensitive diagnostic basis. Diagnostic laparoscopy, interventional endoscopy (e.g. urologic stents) and interventional radiology (embolization) help to avoid surgery.

Conclusion

Non-operative management is established for spleen and liver injuries and increasingly includes kidney trauma. However, the problems of pancreatic and gastrointestinal injuries are still determined by perioperative complications.     

Lung tattooing combined with immediate video-assisted thoracoscopic resection (IVATR) as a single procedure in a hybrid room: our institutional experience in a pediatric population

Background

Analysis of small pulmonary nodules in children poses an important diagnostic and therapeutic challenge for clinicians.

Objective

To review our experience of lung tattooing with immediate video-assisted thoracoscopic resection (IVATR) performed as a single procedure in a hybrid room for technical difficulties, complications and diagnostic yield of the procedure.

Material and methods

Retrospective analysis of 31 children (16 boys, 15 girls) who underwent lung tattooing of various lesions from January 2001 to July 2011. Data were collected from the Interventional Radiology database, Electronic Patient Chart (EPC) and PACS.

Results

A total of 34 lesions were treated in 31 children. Tattooing was performed on lung lesions with median size 3 mm and median depth 2 mm from pleura. Technical success was 91.1% and diagnostic yield was 100%. In seven children, it was combined with other interventional radiologic procedures. The median procedure time for lung tattooing and IVATR was 197 min.

Conclusion

Lung tattooing with IVATR as a single procedure in a hybrid room is safe and effective in children with several inherent advantages, including avoiding the need to move the child from the interventional radiology suite to the operating room.     

Assessment of coronary artery lesions in children with Kawasaki disease: evaluation of MSCT in comparison with 2-D echocardiography

Background

Transthoracic two-dimensional echocardiography is an effective method for detecting coronary arterial injury in Kawasaki disease. However, its accuracy in the diagnosis of coronary arterial lesions is limited.

Objective

To investigate the value of multislice spiral CT for coronary angiography for observing the coronary arterial injury caused by infantile Kawasaki disease.

Materials and methods

Coronary angiography, using a 64-slice spiral CT scanner, and 2-D echocardiography were performed in 48 children with Kawasaki disease in whom the position, internal diameter, and length of each coronary artery were measured.

Results

MSCT showed coronary artery injury in 15 of the 48 children. Among these 15 children, 20 coronary artery branches showed complications, including the left coronary artery branches in 15 (31.2%) and the right coronary artery branches in 5 (10.4%). Complications in the left coronary artery branches included dilation in 12 (25.0%) and stenosis, calcification and the combination of the two in one each, and the right coronary artery branches showed dilation; two branches also showed beaded changes. MSCT also showed dilation in the left anterior descending arteries in two children. These children showed no abnormality on 2-D echocardiography.

Conclusion

MSCT is a valuable examination method for detecting coronary artery injury in Kawasaki disease.     

Predictive factors affecting the prognosis and late complications of 73 consecutive cases of esophageal atresia at 2 centers

Purpose

While the diagnosis and outcomes of esophageal atresia (EA) have improved, associated anomalies, the management of late complications and growth remain major issues. We analyzed factors that affected the prognosis, late complications and growth.

Methods

We retrospectively reviewed EA patients treated at two centers from 1984 to 2016. Patient characteristics, complications (gastroesophageal reflux [GER], anastomotic stenosis, tracheomalacia, dysphagia) and growth were evaluated.

Results

Seventy-three EA patients were treated (overall survival rate:80.8%). The mean birth weight was 2514?±?509 g in the surviving group, and 2453?±?567 g in the fatal group excluded chromosomal abnormality (p?=?0.76). Cardiac and chromosomal anomalies significantly affected mortality. Postoperative GER and anastomotic stenosis each occurred in 39% of the patients. Only GER was significantly affected by the Gross classification. The standard deviation (SD) values of the EA patients’ growth were all lower than in the normal population. The SD of body weight was significantly lower in patients with extremity anomalies.

Conclusions

Associated cardiac and chromosomal anomalies significantly affected the prognosis. GER and anastomotic stenosis were the most common late complications. The growth of the surviving cases was insufficient. These factors will help optimize the therapeutic strategies and postoperative management for EA.

     

Clinical analysis of liver fibrosis in choledochal cyst

Purpose

Although most patients with choledochal cyst (CC) have a favorable prognosis with prompt treatment, some of them are complicated with liver fibrosis, resulting in prolonged liver dysfunction even after definitive surgery. The aim of this study was to distinguish the high-risk group of liver fibrosis in patients with CC.

Methods

Fifteen patients who underwent liver biopsy during surgery for CC from 1981 to 2012 were enrolled in this study. Liver histology with H&E staining was graded according to Ohkuma’s classification, and the correlation with the clinical characteristics was retrospectively assessed.

Results

Their median age at biopsy was 13.4 months (range 1–42 months), and there were 7 in Ia, 2 in Ic, and 6 in IVa of the Todani classification. There were no significant differences in serum liver function test among those cyst types. The histological grades of liver fibrosis were as follows: grade 0 (no fibrosis) in 7 patients, grade 1 (mild) in 2, grade 2 (moderate) in 3, grade 3 (severe) in 2, grade 4 (cirrhosis) in 1. All 3 patients with grade 3 and 4 had CC with IVa and were under 18-month old. A 3-month-old girl with cirrhosis presented with severe jaundice resulting in living-donor liver transplantation despite bile drainage. A 16-month-old girl with grade 3 suffered from prolonged liver dysfunction and intractable ascites after surgery.

Conclusions

The progression of liver fibrosis is likely to be correlated with IVa, and the postoperative course might be unsatisfactory in advanced cases. The prompt surgical intervention is recommended especially for neonatal and infantile cases with type IVa cyst because irreversible liver cirrhosis could occur as early as in the infantile period.     

Komplizierte Pneumonie im Kindesalter

Background

The term “complicated pneumonia” describes an inflammation of the lung with systemic and/or local complications. The incidence of local complications appears to be increasing.

Diagnosis

In children with pneumonia suffering from persistent fever despite appropriate antibiotics, cough, ventilation inhomogeneity, respiratory distress, chest pain, and weight loss, local complications should be anticipated. The most important diagnostic techniques are chest ultrasound and sectional imaging methods.

Therapy

Empirical therapy is the administration of an antibiotic being effective against the most common causative pathogens (Streptococcus pneumoniae, Staphylococcus aureus). In pleural effusion, empyema, and lung abscess, interventional procedures (pleura drainage with urokinase, CT-guided drainage in lung abscess) may be considered. Surgical interventions should be discussed in cases of failed conservative treatment.

Prognosis

Under appropriate therapy, the prognosis of local complications of pneumonia in childhood is generally good.     

Outcome of 200 pediatric living donor liver transplantations in India

Objective

To describe our experience of pediatric living donor liver transplantation from India over a period of 12 years.

Materials and Methods

A retrospective analysis of 200 living donor liver transplantation in children (18 years or younger) was done for demographic features, indications, donor and graft profile and outcome.

Results

Between September 2004 and July 2016, 200 liver transplants were performed on 197 children. Fifty transplants were done in initial 6 years and 150 in next 6 years. All donors (51% mothers) were discharged with a mean stay of 7 days. The leading indications of liver transplants were cholestatic liver disease (46%) followed by metabolic liver disease (33%) and acute liver failure/acute on chronic liver failure (28.5%). Biliary leakage (8.5%), biliary stricture (9%), hepatic artery thrombosis (4.5%) and portal vein thrombosis (4%) were the most common surgical complications; all could be managed by surgical or interventional radiological measures, except in one child who died. Sepsis, acute rejection and CMV hepatitis in first 6 months were seen in 14.5%, 25% and 17% cases, respectively. Post-transplant lymphoproliferative disease was seen in only 1.5%. Re-transplant rate was 1.5%. The overall 1 year survival rate was 94% and 5 year actuarial survival was 87% with no statistically significant difference between children weight <10 kg vs. >10 kg. Outcome in acute liver failure did not differ significantly between those with acute on chronic liver failure vs. those with chronic liver disease.

Conclusions

Advances in medical and surgical techniques associated with multidisciplinary teams including skilled pediatric liver transplant surgeons, anesthetists, dedicated pediatric hepatologists, pediatric intensivists, interventional radiologists and pathologists resulted in an excellent outcome of living related liver transplants in children. Low age and weight of the baby does not seem to be a contraindication for liver transplantation as outcome were comparable in our experience.

     

Familiarity with current practices of granting and maintaining privileges in pediatric interventional radiology—a worldwide survey of the members of the Society for Pediatric Interventional Radiology (SPIR)

Background

Physician credentialing is a complex process driven by the demand for quality improvement in health care. In the U.S., the Joint Commission Standard of 2007 has tied hospital accreditation to credentialing through mandated use of the Focused Professional Practice Evaluation (FPPE) and Ongoing Professional Practice Evaluation (OPPE).

Objective

To assess pediatric interventional radiologists’ knowledge of how institutions grant them privileges.

Materials and methods

Members of the Society for Pediatric Interventional Radiology (SPIR) were sent a web-based survey regarding credentialing.

Results

Of 122 members from 19 countries, 81 (66%) responded, and of these 81, 59 (73%) were familiar with their hospital’s privileging process. Of 49 U.S. respondents and 32 non-U.S. respondents, 37 (76%) and 17 (53%), respectively, stated that interventional radiology credentialing was different from diagnostic radiology credentialing. Of the 49 U.S. respondents, 24 (49%) reported an OPPE, and of the 32 non-U.S. respondents, 8 (25%) reported an ongoing evaluation. The U.S. OPPE is performed at shorter intervals than its international equivalent.

Conclusion

Four years after the Joint Commission defined the FPPE and OPPE, separate credentialing of pediatric interventional radiology from pediatric diagnostic radiology is more likely in the U.S. than internationally, and U.S. pediatric interventional radiologists are more likely to have a defined ongoing professional evaluation and to be evaluated every 6?months or more frequently. There are many SPIR members who do not know how they obtain privileges and/or are not knowingly subject to an OPPE. This lack of knowledge may affect future education of interventional radiologists as well as the definition of pediatric interventional radiology practices within individual institutions.     

Reconstructing single hepatic artery with two arterial stumps: biliary complications in pediatric living donor liver transplantation

Introduction

Liver grafts can at times have two hepatic arterial stumps. This can result in a dilemma whether to reconstruct single or both the arteries. Hepatic artery (HA) thrombosis is the most dreaded complication in pediatric living donor liver transplantation (LDLT) as it can result in biliary complications and subsequent graft loss. We herein report the feasibility of reconstructing single hepatic artery in pediatric living donor liver transplantation having two arterial stumps in the liver graft.

Materials and methods

From 2008 to 2010, 87 pediatric patients undergoing LDLT were divided into three groups. Group 1 (n = 20): two HA stumps with two HA reconstruction, Group 2 (n = 22): two HA stumps with one HA reconstruction and Group 3 (n = 45): one HA stump with one HA reconstruction. The decision regarding the reconstruction of single or multiple HAs was made depending on the pre-operative radiological and intraoperative assessments.

Results

The incidence of HA thrombosis (p = 0.126) and biliary complications (p = 0.617), was similar in the three groups.

Conclusion

Single HA reconstruction does not increase the risk of biliary strictures in pediatric LDLT recipients having dual hepatic arterial stumps in the liver graft.     

A morphological study of the removed livers from patients receiving living donor liver transplantation for adult biliary atresia

Background

In liver transplantation (LT) for adult biliary atresia (BA), we often encounter a cirrhotic deformation of the native liver. We aimed to investigate a morphological study of the removed livers and the patient??s clinical status.

Methods

We examined 8 BA patients who had undergone LT in adulthood at our hospital. The presence of hypertrophic or atrophic areas of the removed liver was recorded macroscopically. We graded the microscopic findings in the porta hepatis area, a hypertrophic area, and an atrophic area, respectively. Moreover, we investigated the relationship between these morphological findings and the pre-transplant clinical status (MELD score).

Results

Macroscopically, a hypertrophic area existed in central liver in all cases (8/8 cases), while an atrophic area was existed in peripheral liver (7/8 cases). Microscopically, an atrophic area was the most severely impaired, while the porta hepatis and hypertrophic area were relatively intact. The pathological score in a compensatory hypertrophic area was strongly correlated with the MELD score.

Conclusions

This study suggests that the partial shrinking is not uncommon in BA cirrhotic liver. It may be due to the imbalance of bile drainage by the different segment. The patient??s pre-transplant status depends on the compensatory hypertrophic liver.     

Stapled intestinal anastomosis is a simple and reliable method for management of intestinal caliber discrepancy in children

Purpose

Popularity of minimally invasive surgeries has led to the development of stapled intestinal anastomosis for adults. The advanced instruments used in this technique are getting suitable with the small intestinal lumens of neonates and infants. We reviewed and compared the intraoperative and postoperative results of stapled and hand-sewn anastomoses in children.

Methods

The operative data of children who underwent stapled and hand-sewn anastomoses between March 2005 and December 2011 were collected and analyzed retrospectively. Furthermore, we compared patients who underwent anastomoses for colostomy closure of anorectal malformation (4 stapled, 9 hand-sewn) and those who underwent anastomoses for treatment of ileal atresia (3 stapled, 11 hand-sewn).

Results

In the 47 patients who underwent stapled anastomosis, no intraoperative complications were observed and postoperative complications included wound infection (n?=?3), delayed gastric emptying (n?=?1), and ileus (n?=?1). No complications suggesting anastomotic dilatation were identified. It was observed that patients who underwent stapled anastomosis for colostomy takedown with caliber discrepancy had significantly shorter surgery time than those who underwent hand-sewn anastomosis.

Conclusion

Our results suggest that stapled anastomosis is safe and effective for various surgical diseases in neonates, infants, and children.     

Bowel perforation after pediatric living donor liver transplantation

Purpose

Bowel perforation after liver transplantation (LT) is a rare, but highly lethal complication with a poor prognosis. Here, we report the outcome of cases of bowel perforation after pediatric LT in our department.

Patients and methods

The study subjects were 148 patients who underwent pediatric living donor liver transplantation. The 114 with biliary atresia (BA) were divided into two groups: those with associated bowel perforation (Group A) and those without (Group B).

Results

Four patients in all (2.5%) suffered bowel perforation. Their original disease was BA and emergency surgery was performed in all cases, with a mortality rate of 50.0%. Comparison of Groups A and B revealed significant differences in the patient age, body weight, duration of surgery, cold ischemic time, and blood loss volume. The survival rates in Groups A and B were 50.0 and 99.1%, respectively (p?<?0.01). Duration of surgery was an independent risk factor (p?=?0.05).

Conclusion

Bowel perforation after LT is a potentially fatal complication. LT is a procedure that requires care and precision, and the possibility of bowel perforation should always be borne in mind during post-operative management, when the duration of surgery has been long.     

Primary anastomosis in necrotizing enterocolitis: the first option to consider

Introduction

Necrotizing enterocolitis (NEC) is the most frequent gastrointestinal emergency in preterm newborns. Thirty percent of all cases will require surgical intervention. Following resection of the involved segment, most patients will undergo a diverting enterostomy.

Objective

To describe the safety and effectiveness of primary anastomosis in patients with complicated NEC.

Methods

This study was a retrospective chart review. The study participants were obtained from both public and private health systems between December 2004 and December 2009 in Santiago, Chile. The inclusion criteria were any patient who underwent a laparotomy for necrotizing enterocolitis. The following variables were evaluated: gestational age, birth weight, use of peritoneal drains, macroscopic features of the intestinal segment, number of anastomoses, parenteral nutrition requirements and post-surgical complications.

Results

Seventy patients were identified. Sixty patients (85?%) underwent primary anastomosis. The remaining 10 patients underwent a resection with enterostomy. In the primary anastomosis group (n?=?60), twelve percent weighed <1,000?g and 22?% weighed 1,000–1,500?g. Two anastomoses were required in 18 patients. Post-surgical complications included infection of the surgical wound in three cases and anastomotic dehiscence in only one case. Seven percent developed short bowel syndrome. Overall mortality was 11.6?%, all secondary to sepsis.

Conclusion

In this series, primary anastomosis was a safe alternative in the management of complicated NEC, with low morbidity and mortality, independent of age, weight, intraperitoneal contamination or extent of disease.     

Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience

Purpose

Although achalasia is a rare disorder in children, its symptom may mimic common childhood diseases. This study aimed to assess the diagnosis and management of achalasia in children and adolescents in a Brazilian single center during a 12-year period.

Methods

Patients with achalasia were identified from a database built during the period of January 2000?CJanuary 2012 from a Pediatric Gastroenterology reference center. Information regarding demographic data, clinical symptoms, diagnosis, treatment, and long-term follow-up were described.

Results

Thirteen patients were studied; median age was 7 (1?C14)?years. Most frequent symptoms were vomiting (84.6?%) and dysphagia (69.2?%). Weight loss occurred in 46.0?% of patients and chronic cough in 46.1?%. Associated disorders were Down??s syndrome, Allgrove syndrome, and congenital central hypoventilation syndrome. Achalasia was misdiagnosed with anorexia nervosa. Six patients were previously treated as having gastroesophageal reflux disease and asthma. Five patients had pneumatic balloon dilation as initial therapy whereas five had esophageal myotomy. Finally, 11 patients had surgical therapy with a favorable follow-up.

Conclusion

Achalasia symptoms may mimic common diseases in children, and therefore, may delay the diagnosis. This study emphasizes the importance of the clinical symptoms for the diagnosis of achalasia, mainly in those cases with associated disorders.     

Reappraise the effect of redo-Kasai for recurrent jaundice following Kasai operation for biliary atresia in the era of liver transplantation

Purpose

This study was conducted to reappraise the efficacy of redo-Kasai (or revision) in the era of liver transplantation as a treatment option in those patients with recurrent jaundice after initially successful Kasai procedure.

Methods

We studied ten patients that received redo-Kasai, among a total of 102 patients diagnosed with biliary atresia after receiving Kasai operation from 1986 to 2011.

Results

Kasai operation was done at a median age of 55?days and redo-Kasai at 150?days. The bilirubin levels returned to normal in six patients after the procedure. Four of six enjoyed jaundice-free survival with native liver till the time of last follow-up. Three patients died and three received liver transplantation (LT). Only one out of seven patients with three or more episodes of cholangitis survived with native liver, while all the three patients with 1 or 0 episode survived with native liver. The difference was significant (P?=?0.033). Re-do Kasai did not result in more blood loss or operative time during LT.

Conclusion

Redo-Kasai is still valuable in the era of LT and the episodes of cholangitis are the decisive factors affecting the outcome of the procedure.     

CT features of lymphobronchial tuberculosis in children, including complications and associated abnormalities

Background

Lymphobronchial tuberculosis (TB) is tuberculous lymphadenopathy involving the airways, which is particularly common in children.

Objective

To describe CT findings of lymphobronchial TB in children, the parenchymal complications and associated abnormalities.

Materials and methods

CT scans of children with lymphobronchial TB were reviewed retrospectively. Lymphadenopathy, bronchial narrowing, parenchymal complications and associations were documented.

Results

Infants comprised 51% of patients. The commonest site of lymphadenopathy was the subcarinal mediastinum (97% of patients). Bronchial compression was seen in all children (259 bronchi, of these 28% the bronchus intermedius) with severe or complete stenosis in 23% of affected bronchi. Parenchymal complications were present in 94% of patients, including consolidation (88%), breakdown (42%), air trapping (38%), expansile pneumonia (28%), collapse (17%) and bronchiectasis (9%), all predominantly on the right side (63%). Associated abnormalities included ovoid lesions, miliary nodules, pleural disease and intracavitary bodies.

Conclusion

Airway compression was more severe in infants and most commonly involved the bronchus intermedius. Numerous parenchymal complications were documented, all showing right-side predominance.     

Evaluation of the intraoperative risk factors for esophageal anastomotic complications after primary repair of esophageal atresia with tracheoesophageal fistula

Purpose

The aim of this study is to identify the risk factors for esophageal anastomotic stricture (EAS) and/or anastomotic leakage (EAL) after primary repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) in infants.

Methods

A retrospective chart review of 52 patients with congenital EA/TEF between January 2000 and December 2015 was conducted. Univariate and multivariate analyses were performed to identify the risk factors for anastomotic complications.

Results

Twenty-four patients were excluded from the analysis because they had insufficient data, trisomy 18 syndrome, delayed anastomosis, or multi-staged operations; the remaining 28 were included. Twelve patients (42.9 %) had anastomotic complications. EAS occurred in 12 patients (42.9 %), and one of them had EAL (3.57 %). There was no correlation between anastomotic complications and birth weight, gestational weeks, sex, the presence of an associated anomaly, age at the time of repair, gap between the upper pouch and lower pouch of the esophagus, number of sutures, blood loss, and gastroesophageal reflux. Anastomosis under tension and tracheomalacia were identified as risk factors for anastomotic complications (odds ratio 15, 95 % confidence interval (CI) 1.53–390.0 and odds ratio 8, 95 % CI 1.33–71.2, respectively).

Conclusion

Surgeons should carefully perform anastomosis under less tension to prevent anastomotic complications in the primary repair of EA/TEF.