Cardiac CT and MRI of cardiac malformations: How to interpret them?

A segmental sequential approach is widely used for the description of congenital heart disease abnormalities in routine reports of computed tomography and magnetic resonance imaging examinations. This consists of three stages as follows: (a) the anatomical description of each segment (viscero-atrial situs, the bulboventricular loop and the position of the great vessels); (b) the relationship between each segment at the atrioventricular and ventriculoarterial levels; and (c) related intra- and intersegmental abnormalities. This article describes the interpretation of computed tomography and magnetic resonance imaging examinations in patients with cardiac malformations using a structured plan.

Hyperammonemia and neonatal cardiac failure.

Severe hyperammonemia (hyperNH3) in neonatal cardiac failure after cardiac surgery is rare. We report a case of a 2470-g female infant born at the week 37 of gestation with complex congenital heart disease (truncus arteriosus type III, interrupted aortic arch and tricuspid valve insufficiency) and hemodynamically non-significant intrahepatic arterio-venous malformation. She developed hyperNH3 (highest NH3 blood level: 467 µmol/L) without severe liver failure (INR of 1.9). The origin of the hyperNH3 was multifactorial including limited capacity of liver detoxification function due to congenital porto-caval shunt, liver ischemia, excessive protein intake and increased protein catabolic rate. HyperNH3 treatment partially succeeded in decreasing ammonia level and included discontinuation of protein intake, administration of phenylacetate and sodium benzoate. This case highlights the fact that NH3 detoxification by the liver has limitations for a neonate with multifactorial causes that decrease liver perfusion.

The place of interventional radiology in Crohn disease in children.

The interventional radiologist plays an important role in the treatment of complicated Crohn disease in children. Interventional radiology includes percutaneous drainage of abscesses and insertion of suitable intravenous lines. A multidisciplinary approach is mandatory for the management of these patients.

Fetal urinoma: two new cases and a review of the literature.

OBJECTIVE: To evaluate the functional prognosis of kidneys affected prenatally by urinomas. METHODS: This was a retrospective review of cases of fetal urinoma reported in the literature, as well as two of our own cases. RESULTS: Twenty-three patients with a prenatal diagnosis of urinoma (five bilateral) were included in the analysis. Postnatal ipsilateral renal function was observed in only six of the 28 renal units (i.e. around 20%). CONCLUSIONS: Although the precise causes of urinomas are still unknown, this review shows that in the event of a fetal urinoma, the probability of a non-functional dysplastic ipsilateral kidney lies at around 80%. In-utero puncture only appears to be justified in cases where fluid accumulation has mass effects on adjacent major structures.

US of the pediatric female pelvis: a clinical perspective.

When investigating pelvic pathologic conditions in female pediatric patients, one needs to be aware of the developmental changes that take place around puberty. The prepubertal uterus is thin, with a fundus equal in size to the cervix. Owing to the hormonal stimulation of puberty, the uterus enlarges and the fundus becomes prominent. The ovaries are demonstrated with ultrasonography (US) at all ages. Ovarian volume increases after 6 years of age. Microcystic follicles are normally seen throughout childhood. US is the modality of choice for imaging the pediatric female pelvis. The main indications for pelvic US in the pediatric age group are pubertal precocity or pubertal delay, pelvic pain or pelvic masses, and ambiguous genitalia. Vaginal bleeding in the prepubertal child can be due to a vaginal foreign body, vaginal rhabdomyosarcoma, or precocious puberty. Common causes of primary amenorrhea in teenagers include gonadal dysgenesis (Turner syndrome) and müllerian (uterovaginal) anomalies. Pelvic pain or pelvic masses in pediatric patients can be due to ovarian torsion, hemorrhagic ovarian cyst, pelvic inflammatory disease, or ectopic pregnancy.

Preoperative manual detorsion of the spermatic cord with Doppler ultrasound monitoring in patients with intravaginal acute testicular torsion.

OBJECTIVE: To assess the effectiveness of preoperative manual detorsion in acute testicular torsion. MATERIALS AND METHODS: Between June 1998 and March 1999, seven patients presenting with testicular torsion underwent manual manipulation under US monitoring in order to restore the flow to the testis prior to surgery (orchidopexy). All detorsions were lateral in direction. The success of the manoeuvre was assessed both clinically and sonographically and confirmed at surgery. RESULTS: Six manoeuvres were successful in restoring flow to the testis. The failed attempt in the seventh patient was due to failure to manipulate beyond an initial 1 1/2 rotations (540 degrees). CONCLUSIONS: Preoperative detorsion is the fastest way to relieve testicular ischaemia. However, manual detorsion of the spermatic cord is not a substitute for surgical exploration and bilateral orchidopexy is still necessary.

Imaging and therapeutic approach of hemangiomas and vascular malformations in the pediatric age group.

Terminology regarding the vascular lesions of the soft tissues remains confusing. A single classification is necessary in order to decide on the proper investigation and the best treatment. At the Workshop on Vascular Anomalies in Rome in June 1996, the membership accepted the Mulliken and Glowacki classification, which differentiates vascular lesions into vascular tumors, including hemangiomas and vascular malformations. At Sainte-Justine, we have set up a multidisciplinary clinic for the discussion of problem patients with vascular anomalies, both in terms of diagnosis and treatment. In this review, we present our experience regarding the classification, the imaging modalities and the treatment of vascular anomalies. In our experience, Doppler ultrasound should be the initial imaging modality for recognizing vascular tumors from vascular malformations. CT scan or magnetic resonance imaging is best to evaluate the extent of the lesions prior to treatment. A multidisciplinary approach is essential to establish a correct diagnosis and define accordingly the appropriate treatment and follow-up.

Soft-tissue venous malformations in pediatric and young adult patients: diagnosis with Doppler US.

PURPOSE: To describe the diagnostic features, appearance, and vascularization pattern of venous malformations (VMs) at Doppler ultrasonography (US). MATERIALS AND METHODS: Between February 1991 and May 1997, 51 soft-tissue VMs were studied with Doppler US in patients between 1 day and 21 years of age (mean age, 9 years). These VMs were located in the maxillofacial region (n = 19), trunk (n = 5), and upper (n = 10) and lower (n = 17) extremities. Twenty-three VMs had venographic confirmation, seven had only histologic confirmation, and 21 had both venographic and histologic confirmation. US was performed with 7.5- or 7-10-MHz linear transducers, a low pulse repetition frequency (mean, 1,680 Hz), and the lowest wall filter (25-50 Hz). RESULTS: At gray-scale US, VMs appeared as hypoechoic, heterogeneous lesions in 82% of cases. All lesions displayed compressibility. In eight lesions (16%), phleboliths were identified, thus confirming the diagnosis of VM. Analysis of vascular flow revealed monophasic, low-velocity flow in 40 VMs (78%), with an average flow velocity of 0.22 kHz. Biphasic flow was noted at the periphery of three lesions, which is indicative of a mixed capillary-venous malformation. The remaining eight lesions did not display any flow. CONCLUSION: In pediatric patients, Doppler US is a noninvasive, easily available, and rapid mode of investigation of vascular lesions and can help confirm the diagnosis of VM when it shows a characteristic flow pattern.

Soft-tissue hemangiomas in infants and children: diagnosis using Doppler sonography.

OBJECTIVE: We describe the sonographic appearance and vascularization of hemangiomas and determine if vessel density and peak systolic Doppler shifts distinguish hemangiomas from other superficial soft-tissue masses. SUBJECTS AND METHODS: Our pilot study included 20 infants and children with hemangiomas who were to undergo biopsy before treatment with interferon alpha-2b. We used Doppler sonography to determine the number of vessels per square centimeter, peak arterial Doppler shift, resistive index, and signs of arteriovenous shunting. All hemangiomas showed high vessel density (more than five per square centimeter) and high Doppler shifts (more than 2 kHz), and these two factors became our diagnostic criteria. A prospective study of 116 patients was then carried out. One hundred sixteen consecutive pediatric patients with superficial soft-tissue masses were examined using Doppler sonography; sonographic findings were compared with the final diagnoses that were established by biopsy, CT, or clinical follow-up. RESULTS: The final diagnoses included 70 hemangiomas, 20 venous malformations, three arteriovenous malformations, three arteriolocapillary malformations, and 20 other masses. Fifty-nine lesions showing high vessel density (more than five per square centimeter) and a peak arterial Doppler shift exceeding 2 kHz were correctly diagnosed as hemangiomas (sensitivity, 84%; specificity, 98%). One arteriovenous malformation showed high vessel density and high Doppler shifts, but none of the other masses that were not hemangiomas did. Eleven patients with hemangiomas who were being treated with interferon at the time of the study fulfilled only one of the two diagnostic criteria. CONCLUSION: High vessel density and high peak arterial Doppler shift can be used to distinguish hemangiomas from other soft-tissue masses.

Peripherally inserted central catheters in infants and children.

PURPOSE: To determine prospectively the feasibility, complications, and mid- and long-term advantages of peripheral insertion of central catheters in infants and children. MATERIALS AND METHODS: During a 15-month period between March 1995 and June 1996, a total of 285 catheter placement attempts were made to peripherally insert central catheters in 183 pediatric patients (89 boys, 94 girls). Phlebographic guidance was used, and the catheters were inserted below the elbow in 99% of cases. Catheter insertion was indicated for prolonged antibiotic therapy in 108 patients (158 catheter placement attempts), hematologic or oncologic care in 24 patients (40 attempts), total parenteral nutrition in 16 patients (46 attempts), and venous access for fluid or blood in 35 patients (41 attempts). The success rate and complications were recorded along with the indication, patient age, and duration of catheter placement. RESULTS: One hundred fifty-two of 158 (96%) catheter placement attempts were successful in outpatients (n = 108), 124 of 127 (98%) in hospitalized patients (n = 75), and 70 of 73 (96%) in patients aged less than 1 year. Infection and pericatheter venous thrombosis were the main complications and were seen in 17 of 276 (6%) and one of 276 (0.3%) catheter placement attempts, respectively. Catheter occlusion occurred in 23 of 276 (8%) catheter placement attempts. CONCLUSION: Peripheral insertion of central catheters was highly feasible in infants and children with this protocol. Such catheters were well tolerated in the pediatric population with a low frequency of complications.

Lymphangiomas in children: percutaneous sclerotherapy with an alcoholic solution of zein.

PURPOSE: To determine the efficacy of percutaneous sclerotherapy with an alcoholic solution of zein in lymphangiomas in pediatric patients. MATERIALS AND METHODS: Fourteen patients with macrocystic and mixed lymphangiomas were treated with use of Ethibloc as a sclerosing agent. The procedure was performed with ultrasonographic and/or fluoroscopic guidance. Follow-up clinical and sequential computed tomographic examinations were performed to evaluate regression of the lesion. RESULTS: Regression was excellent in nine (64%) of the 14 lesions: Regression was complete in four lymphangiomas and was to less than 5% of the initial volume in five. Satisfactory results were obtained in the remaining five (36%) of 14 lesions. The most frequent complication was leakage of Ethibloc without important sequelae in 10 of the 14 lymphangiomas. CONCLUSION: Percutaneous sclerotherapy with Ethibloc was a safe and effective procedure in the treatment of macrocystic and mixed lymphangiomas.

Liver transplantation: review of the literature. Part 2: Vascular and biliary complications.

The first attempted human orthotopic liver transplantation, in 1963, involved a child with biliary atresia, who died on the operating table as a result of uncontrollable coagulopathy. Improvements in immunosuppression, surgical technique, medical imaging and postoperative care, as well as more stringent patient selection, have allowed the development of liver transplantation and its universal acceptance as the treatment for a variety of liver diseases. The radiologist plays a major role in the multidisciplinary transplantation team and must be familiar with each stage of orthotopic liver transplantation and its associated complications. In the first article of this series (Can Assoc Radiol J 1997;48[3]:171-178), the authors reviewed the anatomic features and current concepts relevant to orthotopic topic liver transplantation. In this, the second article, they discuss the vascular and biliary complications of the operation, and the third article will cover the medical complications.

Liver transplantation: review of the literature. Part 1: Anatomic features and current concepts.

The first attempted human orthotopic liver transplantation, in 1963, involved a child with biliary atresia, who died on the operating table as a result of uncontrollable coagulopathy. Improvements in immunosuppression, surgical technique, medical imaging and postoperative care, as well as more stringent patient selection, have allowed the development of liver transplantation and its universal acceptance as the treatment for a variety of liver diseases. The radiologist plays a major role in the multidisciplinary transplantation team and must be familiar with each stage of orthotopic liver transplantation and its associated complications. In the first article of this series, the author reviews the anatomic features and current concepts relevant to orthotopic liver transplantation. Future articles will discuss the vascular, biliary and medical complications of the operation.

Intestinal vascular anomalies in children.

Vascular anomalies are an uncommon cause of gastrointestinal bleeding in childhood. Confusing nomenclature has made objective comparisons of published cases difficult and has interfered with an established consensus regarding diagnosis and therapeutic modalities. The purpose of this study was to clarify the situation by reviewing the records of all children who had intestinal vascular anomalies who were referred to our institution from 1975 to 1995. Thirteen lesions were identified in nine children (five boys and four girls). The median age at clinical onset was 8 years. Only two patients presented with a complex syndrome (Klippel-Trenaunay, 1; Osler-Rendu-Weber, 1). Diagnosis, location, and extension of these anomalies was only possible by angiography, which indicated that seven patients had isolated venous malformations and two had arteriovenous malformations. Because the lesions did not involve the serosa, intraoperative localization was a major problem. The main findings were a few slightly dilated mesenteric veins. Treatment was conservative in four children and surgical in five. Pathological findings on resected bowel demonstrated dilated and abnormal veins in the mucosa and submucosa. Selective angiography should not be delayed in patients with gastrointestinal bleeding if results of all other investigations are negative. Because these lesions are rarely recognizable on operative inspection, precise preoperative angiographic localization of intestinal vascular anomalies is essential to allow for a safe and limited resection of the involved bowel segment. Based on a better understanding of the natural history of these lesions, a classification of vascular anomalies of intestines in children is proposed.

Intrahepatic biliary-enteric bypass for complete extrahepatic biliary obstruction in children.

Intrahepatic biliary-enteric anastomosis is rarely practiced in pediatric surgery. The authors report on two children who have been successfully treated using this method. First described by Longmire and Sandford in 1948, intrahepatic biliary-enteric anastomosis is possible in children. This procedure is only indicated as a last recourse for circumstances in which the extrahepatic biliary tree is completely obstructed.

Placenta percreta: balloon occlusion and embolization of the internal iliac arteries to reduce intraoperative blood losses.

Obstetric hemorrhage is still a potential cause of maternal mortality and morbidity. Angiographic embolization techniques have been described in cases of postcesarean bleeding, vaginal wall hematomas, cervical ectopic pregnancies, and postpartum bleeding to control persistent bleeding from pelvic vessels. We describe two cases of pregnancy complicated with placenta percreta. Balloon occlusion and embolization of the hypogastric arteries were performed during the cesarean section and hysterectomy, resulting in a remarkable reduction in intraoperative blood loss. Balloon occlusion and embolization of the internal iliac arteries significantly reduce intraoperative blood losses.

Echogenic dilated bowel loops before 21 weeks' gestation: a new entity.

OBJECTIVE: The purpose of this study was to determine the outcome at birth for fetuses in which echogenic dilated bowel loops (EDBL) are detected before 21 weeks of gestation. MATERIALS AND METHODS: A retrospective study was undertaken of 45 patients with EDBL. Sonographic criteria included dilated bowel with an echogenic wall. EDBL was divided into two categories: the isolated and the complex (associated with other abnormalities). When the EDBL affected only one quadrant of the fetal abdomen, it was referred to as the local form, and when it was observed in more than one quadrant, it was termed the diffuse form. RESULTS: Intestinal dilatation of 2-8 mm was revealed on sonography in 21 fetuses with the isolated form of EDBL. Follow-up sonography showed resolution of EDBL in 20 cases. The outcome at birth was normal in 19 (90%) cases. The 20th fetus was born with jejunal atresia, and the remaining fetus died after cordocentesis. Twenty-four fetuses with the complex form of EDBL also had intestinal dilatation of 2-8 mm shown on sonography. Nine (38%) of these fetuses were born alive: six had gastroschisis; two had meconium peritonitis; and one had vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia (VATER). In these cases, resolution of EDBL was noticed at sonographic follow-up. Fourteen (58%) of the 24 fetuses were aborted. The remaining fetus died after amniocentesis. The incidence of normalcy is comparable in both local and diffuse dilatation. Amniocentesis was performed in 15 cases. Disaccharidase activity was measured in 13 patients. Low disaccharidase activity was detected in 10 (77%) of 13 cases. We saw cystic fibrosis in none of the 45 fetuses. Infection was detected before and at birth in five (11%) cases. CONCLUSION: EDBL is a new entity that is most likely related to temporary obstruction. A fetus with the isolated form has a good prognosis, whereas a fetus with the complex form has an outcome directly related to the severity of associated abnormalities.

Liver transplantation: review of the literature. Part 3: Medical complications.

The first attempted human orthotopic liver transplantation, in 1963, involved a child with biliary atresia, who died on the operating table as a result of uncontrollable coagulopathy. Improvements in immunosuppression, surgical technique, medical imaging and postoperative care, as well as more stringent patient selection, have allowed the development of liver transplantation and its universal acceptance as the treatment for a variety of liver diseases. The radiologist plays a major role in the multidisciplinary transplantation team and must be familiar with each stage of orthotopic liver transplantation and its associated complications. In the first article of this series (Can Assoc Radiol J 1997;48[3]: 171-178), the authors reviewed the anatomic features and current concepts relevant to orthotopic liver transplantation. In the second article (Can Assoc Radiol J 1997;48[4]: 231-242), they discussed the vascular and biliary complications of the operation. This, the third and final article in the series, covers the medical complications.