Large hepatic mesenchymal hamartoma leading to mid-trimester fetal demise.

This report describes a fetus with a large multiloculated cystic liver mass. Two small abdominal cysts were seen on ultrasound at 19 weeks of gestation but the patient was referred to us at 23 weeks, after the mass had grown to 8.0 x 5.6 x 7.0 cm, displacing intra-abdominal organs, heart and diaphragm. There was a small amount of ascites but no hydrops. There was polyhydramnios and a thick hyperechoic placenta. After detailed sonograms and MRI suggested the diagnosis of cystic mesenchymal hamartoma of the liver, cyst decompression was favored and consent was obtained. Unfortunately, absence of fetal cardiac activity was noted on the day of the planned intervention. Autopsy confirmed the diagnosis and demonstrated placental changes consistent with mesenchymal stem villous hyperplasia of the placenta. Large fetal cystic abdominal masses that compress the heart, lungs and other organs may benefit from prenatal decompression. This is the first report of cystic hamartoma of the liver apparent on second-trimester sonography, and the fourth time such a lesion is associated with fetal or neonatal death out of 11 cases diagnosed prenatally.

Reversible encephalopathy associated with tacrolimus in pediatric renal transplants.

Neurological complications post transplant have been described with the use of calcineurin inhibitors. Although tacrolimus may be a better immunosuppressant than cyclosporine, its neurological side effects may be worse. Two children, living-related kidney transplant recipients, were treated with antibody induction, mycophenolate mofetil, prednisone, and tacrolimus. Soon after transplant, they each developed an encephalopathy, which when visualized by magnetic resonance imaging showed that it affected both white and grey matter of the brain. Although the encephalopathy was associated with the use of tacrolimus, there was a complete neurological recovery without cessation of the drug.

Laparoscopic excision of subdiaphragmatic epidermoid cyst: a case report.

Retroperitoneal epidermoid cysts are rare. The authors report a case of an 11-year-old boy with an asymptomatic subdiaphragmatic cyst, which was found incidentally during an investigation for hypertension. At laparoscopy, the cyst was densely adherent to the diaphragm, resulting in a pneumothorax during dissection. Nevertheless, the excision and the diaphragmatic repair could be completed laparoscopically without complication. Microscopic examination showed an epidermoid cyst. No similar case has been reported in the literature.

Symptomatic splenic hamartoma with renal, cutaneous, and hematological abnormalities.

BACKGROUND: There is a rare association between splenic hamartomas and hematological abnormalities with, to our knowledge, only 24 reported cases in the English literature. PATIENTS AND METHODS: We report a case of a splenic hamartoma in a 14-year-old boy associated with membranoproliferative glomerulonephritis, multiple lobular capillary hemangiomas of the skin, hypertension, and anemia. Following imaging with ultrasonography, MRI, and nuclear scans, a hamartoma was suspected, but malignancy could not be excluded. The lesion was removed by partial splenectomy, and pathological examination confirmed the presence of a red pulp splenic hamartoma. RESULTS: The renal, hematological, and dermatological abnormalities resolved following removal of the splenic hamartoma. This is the first reported case of a splenic hamartoma associated with renal, cutaneous, and hematological abnormalities and only the second reported case of a symptomatic splenic hamartoma treated by partial splenectomy.

Ultrasound and a new videobronchoscopic technique to measure the subglottic diameter.

OBJECTIVE: The aim of this study was to evaluate ultrasound (US) and a new videobronchoscopic (VB) technique in the measurement of the subglottic lumen diameter. DESIGN: This blind prospective animal study of 62 recently sacrificed rabbits was conducted at the Montreal Children's Hospital. METHODS: Three different diameter measurements of the subglottis were assessed using B-mode US on each intact animal. The same diameter measurements, as well as a fourth, were estimated with a VB measuring technique. Finally, the subglottic area was surgically exposed allowing for direct caliper measurements of all four corresponding diameters. All three US measurements on each of the 62 rabbits and all four VB measurements on each of the 60 rabbits were compared with the gold standard corresponding caliper measurements. RESULTS: Statistical analysis revealed strikingly convergent values in subglottic lumen diameter measurements using US and calipers. The mean difference between these methods for all 186 observations was 0.11 mm. With 95% confidence, the maximum discordance was less than 0.30 mm on the smallest evaluated lumen. Convergent values were also demonstrated in subglottic lumen diameter measurements using VB and calipers. The mean difference between these methods for all 240 observations was 0.16 mm. With 95% confidence, the maximum discordance was less than 0.37 mm on the smallest evaluated lumen. Pearson's correlation coefficient supported a strong and positive relationship between US and caliper measurements as well as VB and caliper measurements. Simple linear regression model indicated that the subglottic lumen diameter could be predicted by both US and our VB technique. CONCLUSION: This work represents the first attempt to validate B-mode US and a VB technique as measuring tools for the subglottic lumen diameter. Our results have clearly shown that both methods are precise measuring modalities for this purpose. With further studies, these two objective methods of measuring the subglottic diameter could be adopted universally. The precise knowledge of this diameter could help physicians recognize and describe the severity of a narrowed lumen. Reporting results in such a standardized fashion, by either US or VB, could facilitate communication among clinicians and institutions.

Neuromotor spectrum of periventricular leukomalacia in children born at term.

The spectrum of neuromotor abnormalities of term children with periventricular leukomalacia (PVL) has never been specifically defined. We report 12 term children with PVL to delineate its long-term clinical correlates. A retrospective review of a standardized computer database and files of a single pediatric neurologist during a 7-year period was performed. The imaging studies were reviewed independently by two neuroradiologists. The mean age of the patients at the initial neurologic assessment was 24. 4 months (range 5-60); nine were males. The reason for the assessment was developmental delay in 10 (83.3%), seizure in one, and attention-deficit-hyperactivity disorder in one. Three children (25%) had normal motor examinations, three (25%) were hypotonic, three (25%) had spastic diplegia, two (16.7%) had spastic quadriplegia, and one (8.3%) had fine-motor abnormalities. Nine children (75%) had developmental delay (severe global delay in six), and two children (16.7%) had epilepsy; electroencephalograms were abnormal in six (50%). PVL was mild in five (41.7%), moderate in two (16.6%), and severe in five (41.7%) children. Four of eight children with global developmental delay had enlargement of cerebral sulci in addition to white matter changes. PVL in term children presents with a spectrum of neurologic abnormalities, particularly developmental delay and heterogeneous motor findings not limited to classic spastic diplegia. The clinician should consider the diagnosis of PVL in the context of term children with developmental delay and motor abnormalities, even in the absence of perinatal difficulties.

Cow's-milk-induced allergic colitis in an exclusively breast-fed infant: diagnosed with ultrasound.

BACKGROUND: An exclusively breast-fed-8-week-old boy presented with irritability and non-bilious projectile vomiting. He was referred to our Medical Imaging Department to eliminate pyloric stenosis. PATIENT AND METHODS: A diagnosis of colitis was strongly suggested by ultrasound. A more detailed history revealed that the patient also had episodes of colicky pain and bloody stools. An infectious colitis was subsequently excluded and rectal biopsy supported the diagnosis of allergic proctocolitis. RESULTS: The infant responded well to the withdrawal of cow's milk and dairy products from the maternal diet. CONCLUSION: Allergic proctocolitis should be included in the differential diagnosis of infants presenting with vomiting and/or bloody stools.

Septo-optic dysplasia plus: a spectrum of malformations of cortical development.

The authors describe three children with septo-optic dysplasia (SOD)-plus: SOD and an associated malformation of cortical development. All three children had developmental delay, and two of the children had significant associated motor deficits. The associated cortical malformations with SOD include a spectrum of disorders of neuronal organization, not limited, as previously described, to schizencephaly. SOD-plus should be suspected in children with SOD and developmental delay.

Cree leukoencephalopathy: neuroimaging findings.

PURPOSE: To describe the computed tomographic (CT) and magnetic resonance (MR) imaging findings in Cree leukoencephalopathy. MATERIALS AND METHODS: The authors retrospectively reviewed the medical records and neuroimaging studies in 12 infants with Cree leukoencephalopathy (CT in 12 infants, MR in six). The diagnosis was established clinically in six patients and at autopsy in the other six. RESULTS: At CT, extensive, diffuse, and symmetric hypoattenuation was seen in the cerebral and cerebellar white matter in all 12 patients. Hypoattenuation was also seen in the corpus callosum in 11 (92%), internal capsule in 10 (83%), globus pallidus in nine (75%), brainstem in nine (75%), and thalamus in four (33%). The caudate nucleus and putamen were spared. On T2-weighted MR images in six patients, the cerebral and cerebellar white matter, including the subcortical arcuate fibers, was hyperintense as were the internal capsule, corpus callosum, corticospinal tracts, and globus pallidus. The thalamus was affected in four (67%) patients, pons in five (83%), and medulla in four (33%). The caudate nucleus and putamen were not affected. CONCLUSION: Cree leukoencephalopathy causes striking symmetric and diffuse involvement of the cerebral and cerebellar white matter and brainstem with sparing of the caudate nucleus and putamen.

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.

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.

Reliability of frontal chest x-ray in diagnosing pulmonary opacities in children.

RATIONALE AND OBJECTIVES: The World Health Organization recommends that the routine investigation of children with suspected pneumonia should not include lateral chest x-rays. However, the reliability of the frontal view alone in the diagnosis of pulmonary opacities has not been reported. METHODS: The authors studied prospectively 373 consecutive chest x-rays of children, ranging in age from 0 to 17 years, who were examined for suspected pneumonia. One radiologist interpreted the frontal view, and the diagnoses were compared with those of three radiologists who interpreted both frontal and lateral x-rays. RESULTS: William's index for rater reliability was 0.98 for all ages (95% confidence interval: 0.94, 1.00). Thus, a radiologist using only a frontal view would agree with members of an independent group using both frontal and lateral views as often as an isolated member of that group would agree with the other group members. CONCLUSIONS: Detecting a definite pulmonary opacity on the frontal view alone predicts its presence on frontal-lateral views. However, the interpretation of bronchial thickening and peribronchial alveolar confluences as opacities is a problem remaining on either frontal or frontal-lateral views.