Pilomatricomas in children: imaging characteristics with pathologic correlation

Background Although pilomatricoma commonly occurs in children, there is still a poor understanding of the imaging characteristics of pilomatricoma and lack of agreement regarding its imaging findings and histopathologic features. Objectives To characterize the radiologic appearance of pilomatricomas on US, CT, and MR and to correlate the imaging findings with histopathologic features. Materials and methods The imaging findings of 47 pilomatricomas on US (n = 17), CT (n = 31), and MR (n = 5) were retrospectively evaluated. Pathologic specimens of all cases were reviewed and compared with imaging findings. Results All lesions were well-circumscribed, subcutaneous nodules with partial attachment to the overlying skin. On US, the lesions were mostly hyperechoic with posterior acoustic shadowing and hypoechoic rim. On CT, they appeared as enhancing soft-tissue masses with varying amounts of calcification. MR findings were internal reticulations and patchy areas on T2-weighted images and contrast-enhanced T1-weighted images, corresponding to edematous stroma on pathology. Peritumoral inflammatory changes and connective capsule on pathology were well correlated with imaging findings. Conclusion Pilomatricoma should be considered when US or CT shows a well-defined hyperechoic or calcific nodule in subcutaneous fat attached to the skin in children. MR images may be helpful in diagnosis. Pathologic findings are well correlated with imaging findings.     

Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors

Background: Intracranial atypical teratoid/rhabdoid tumors (AT/RT) are rare and extremely aggressive neoplasms seen primarily in childhood. Imaging features are often considered non-specific. However, correct diagnosis of AT/RT is important because these tumors have a markedly different clinical prognosis and require more aggressive therapy.Objective: To determine the imaging features of AT/RT.Materials and methods: We retrospectively analyzed imaging findings in 11 patients with primary intracranial AT/RT presenting over a period of 5 years. CT (n=11), MR (n=7), clinical (n=11) and pathological (n=11) features were evaluated. FISH analysis showing monosomy of chromosome 22 (absence of bcr 22q11 locus) was available for three patients. Immunohistochemical staining for INI-1 (BAF47) was performed on all tumors. Results: There were 11 patients, 6 boys and 5 girls. The age of presentation varied from 1 month to 15 years (average age 3 years 8 months). Six tumors were located in the posterior fossa and five in the supratentorial compartment. The tumors showed a hyperdense solid component (64%) that showed moderate to marked enhancement with contrast medium. On MR imaging, the predominant signal pattern was isointensity on T1-weighted images (57%) and T2 shortening with heterogeneity on T2-weighted images (86%). All tumors were large in size (average 4.2×3.7 cm), and there was a tendency for calcification (36%), hemorrhage (46%), necrosis (46%) and perifocal edema (100%). There was also a high tendency for subarachnoid dissemination, with five patients (46%) demonstrating brain and/or spinal metastasis. At follow-up (n=7), six patients showed local recurrence. At the time of recurrence, all these patients showed extensive leptomeningeal spread of the disease in both intracranial and intraspinal compartments. Conclusion: There are no specific imaging features for intracranial AT/RT. But a high tendency toward large size, a hyperdense solid component on CT scan with calcification, hemorrhage, necrosis and subarachnoid spread suggest that this tumor should be considered in the differential diagnosis of large pediatric intracranial tumors.     

Leptomeningeal metastases in pediatrics: magnetic resonance image manifestations and correlation with cerebral spinal fluid cytology

Background: Detection of leptomeningeal metastases is fundamental to a complete evaluation of central nervous system (CNS) or non‐CNS tumor with suspected involvement of the neuroaxis. Our purpose was to assess the appearances of different magnetic resonance (MR) sequences in the diagnosis of leptomeningeal metastases and correlate those positive findings with the cerebral spinal fluid (CSF) cytology results. Methods: The authors reviewed the medical records and MR image manifestations of leptomeningeal metastases from 18 children who had positive MR findings and retrospectively correlated them with CSF cytologic results. There was a uniform MR protocol and the patients were examined with the same sequences. Results: The abnormalities included pial‐arachnoid disease (n= 16), disease coating the nerves (n= 12), hydrocephalus (n= 3) and subependymal metastases (n= 2). Enhanced T1 images were better than unenhanced fluid attenuated inversion recovery (FLAIR) and T2 to delineate cranial and spinal leptomeningeal metastases. In our sample, seven out of 18 cases were cytologically negative on a single lumbar puncture. Conclusions: Contrast‐enhanced MR imaging can be invaluable, detecting the false‐negative lumbar punctures. FLAIR and diffusion images can be helpful in diagnosing leptomeningeal metastases of non‐enhancing primary tumors. Prognosis was more related to the primary tumor type than to the leptomeningeal enhancement MR pattern.     

Congenital hydranencephaly/porencephaly due to vascular disruption in monozygotic twins

Monozygotic twinning has been associated with a variety of vascular disruptive defects including congenital hydranencephaly/porencephaly. Data involving 24 cases of congenital hydranencephaly/porencephaly associated with twinning are reported. In these cases, the finding of a preponderance of monozygotic twins and the common association of a deceased co-twin support the hypothesis of a vascular disruptive etiology. These defects are presumed to be secondary to embolic phenomena or thromboplastin release from the deceased co-twin to the survivor via the vascular interconnections of a conjoined monochorionic placenta. In all cases of hydranencephaly/porencephaly, a careful examination of the placenta and membranes for evidence of a deceased co-twin is warranted prior to providing recurrence risk counseling.     

Accuracy of MR imaging for detecting epiphyseal extension of osteosarcoma

Background. Too few patients are receiving epiphyseal-sparing limb salvage procedures for osteosarcoma. Objective. To determine how magnetic resonance (MR) imaging can best predict the epiphyseal extension of osteosarcoma. Materials and methods. Forty children underwent complete pretreatment static and dynamic contrast-enhanced MR imaging (DEMRI). Static MR images [T1-weighted and short tau inversion recovery (STIR)] of the epiphyses were read in three ways: (1) for suspicion of any abnormality (tumor or edema), (2) for suspicion of tumor, excluding suspected edema, and (3) validating the second method by using a scale to rate the likelihood of tumor. Presentation imaging was compared to histopathologic findings after chemotherapy and resection. The receiver operating characteristic (ROC) method was used to analyze the scaled ratings of static MR and DEMRI values. Results. At delayed resection, 20 of 40 children with osteosarcoma had confirmed epiphyseal tumor; however, 32 epiphyses were abnormal on STIR and 28 abnormal on T1. Differentiating suspected tumor from edema increased the accuracy to an A_z (area under the ROC curve) of 0.94 for both T1-weighted and STIR static sequences. T1-weighted MR had better specificity and STIR better sensitivity at any given rating. DEMRI was slightly less accurate (Az = 0.90). Conclusion. Static MR imaging most accurately detected epiphyseal extension of osteosarcoma when readers distinguished suspected tumor from edematous or normal tissue. Received: 20 August 1999 Accepted: 13 January 2000     

Radiological work-up in Peutz-Jeghers syndrome

Background The development of modern imaging studies such as high-resolution US and various MR imaging sequences have enabled their application to the examination of the gastrointestinal tract.Objective To compare radiological contrast studies, US and MRI in patients with Peutz-Jeghers syndrome (PJS).Materials and methods Ten members of two families were evaluated. The patients were divided into three groups according to the presence of perioral pigmentations and current clinical symptomatology. Patients were examined using US, MRI and radiological contrast studies.Results In the disease-free family members (n=3), no pathological findings were found. In asymptomatic (n=4) and symptomatic patients (n=3), polyps were demonstrated using contrast studies in all cases. MRI demonstrated all the polyps. Ultrasound revealed the polyps in three out of six patients. In addition to the polyps, intussusceptions were shown. There was no evidence of malignant transformation on any of the studies.Conclusions Although PJS is a childhood or teenage disease, patients should be closely monitored during adulthood because of the development of gastrointestinal and extragastrointestinal malignancies. Contrast studies remain the gold standard. However, radiological follow-up could be undertaken by US or MRI, which would not impose a radiation burden during life-time follow-up.     

Post-contrast FLAIR MR imaging of the brain in children: normal and abnormal intracranial enhancement

Objective To describe the normally enhancing intracranial structures on fluid-attenuated inversion recovery (FLAIR) MRI and evaluate the usefulness of postcontrast FLAIR images of the brain in the assessment of enhancing lesions by comparing postcontrast FLAIR imaging with postcontrast T1-weighted (T1-W) imaging in children.Materials and methods In 218 children, 249 pre- and postcontrast FLAIR MRI examinations of the brain were obtained consecutively between August 2001 and April 2002. The normally enhancing intracranial structures on FLAIR imaging were assessed in 77 MRI studies of 74 children who showed normal intracranial imaging findings. In 86 MRI studies in 68 children who showed enhancing intracranial lesions, lesion conspicuity on postcontrast FLAIR imaging was compared with that on postcontrast T1-W imaging for all lesions (n=107), intra-axial lesions (n=40), or extra-axial lesions (n=67).Results The normally enhancing intracranial structures on FLAIR MRI were the choroid plexus (99%, 76/77), pituitary stalk (84%, 65/77), pineal gland (71%, 55/77), dural sinuses (26%, 20/77), and cortical veins (9%, 7/77). Of all the enhancing lesions, lesion conspicuousness on postcontrast FLAIR imaging was better than postcontrast T1-weighted imaging in 42, equal in 28, and worse in 37. Of 40 intra-axial lesions, lesion conspicuousness on postcontrast FLAIR imaging was better in 6, equal in 10, and worse in 24. Of 67 extra-axial lesions, lesion conspicuity on postcontrast FLAIR imaging was better in 36, equal in 18, and worse in 13. Conspicuousness of extra-axial lesions was significantly better than that of intra-axial lesions on postcontrast FLAIR imaging (P<0.001).Conclusions The choroid plexus, pituitary stalk, pineal gland, dural sinuses, and cortical veins show normal enhancement on postcontrast FLAIR MRI in children, and postcontrast FLAIR imaging appears better than postcontrast T1-W imaging in the assessment of extra-axial enhancing lesions in children.Presented at the 46th Annual Meeting of the Society for Pediatric Radiology, San Francisco, California, USA, May 2003     

Reversible acute methotrexate leukoencephalopathy: atypical brain MR imaging features

Background: Unusual acute symptomatic and reversible early-delayed leukoencephalopathy has been reported to be induced by methotrexate (MTX). Objective: We aimed to identify the occurrence of such atypical MTX neurotoxicity in children and document its MR presentation. Materials and methods: We retrospectively reviewed the clinical findings and brain MRI obtained in 90 children treated with MTX for acute lymphoblastic leukaemia or non-B malignant non-Hodgkin lymphoma. All 90 patients had normal brain imaging before treatment. In these patients, brain imaging was performed after treatment completion and/or relapse and/or occurrence of neurological symptoms. Results: Of the 90 patients, 15 (16.7%) showed signs of MTX neurotoxicity on brain MRI, 9 (10%) were asymptomatic, and 6 (6.7%) showed signs of acute leukoencephalopathy. On the routine brain MRI performed at the end of treatment, all asymptomatic patients had classical MR findings of reversible MTX neurotoxicity, such as abnormal high-intensity areas localized in the deep periventricular white matter on T2-weighted images. In contrast, the six symptomatic patients had atypical brain MRI characterized by T2 high-intensity areas in the supratentorial cortex and subcortical white matter (n=6), cerebellar cortex and white matter (n=4), deep periventricular white matter (n=2) and thalamus (n=1). MR normalization occurred later than clinical recovery in these six patients. Conclusions: In addition to mostly asymptomatic classical MTX neurotoxicity, MTX may induce severe but reversible unusual leukoencephalopathy. It is important to recognize this clinicoradiological presentation in the differential diagnosis of acute neurological deterioration in children treated with MTX.     

Fetal MRI of conjoined twins who switched their relative positions

Conjoined twinning is a very rare occurrence with no genetic predisposition. Twisting of conjoined twins around the axis of their connecting tissue bridge, close to the third trimester, has not been previously reported. We describe a unique case of in utero twisting of conjoined omphalopagus twins who survived without any adverse effects. Fetal US and fetal MRI played a vital role in the diagnosis and perinatal management of these twins.     

MR imaging of abdominopelvic involvement in neurofibromatosis type 1: a review of 43 patients

Background: Plexiform neurofibromas are a frequent complication of neurofibromatosis type 1. This article discusses MR imaging findings and distribution of plexiform neurofibromas in the abdomen and pelvis. Objective: To define the most prevalent patterns of involvement and MR imaging findings in abdominopelvic neurofibromatosis type 1. Materials and methods: We reviewed the MR appearance of abdominopelvic lesions in 23 male and 20 female patients (median age: 16 years) with type 1 neurofibromatosis. The patients were part of a multi-institutional study of 300 patients. Imaging included coronal or sagittal, and axial short tau inversion recovery images. Results: The most common abdominopelvic involvement was in the abdominopelvic wall (n=28, 65%) and lumbosacral plexus (n=27, 63%). Retroperitoneal involvement was frequent (n=15, 35%). Lesions were less often intraperitoneal (21%) (P=0.001). Pelvic disease (n=27, 63%), neural canal involvement (n=18, 42%), and hydronephrosis (n=4, 9%) were also noted. Target-like appearance of plexiform lesions was noted in more than half the patients. Conclusion: Abdominopelvic involvement in neurofibromatosis type 1 is primarily extraperitoneal. Although lesions are most prevalent in the abdominopelvic wall and lumbosacral plexus, retroperitoneal and pelvic involvement is common and usually affects important organs. MR imaging added information in the initial and follow-up clinical evaluation of these patients.     

Imaging following allograft reconstruction in children with malignant bone tumours

Purpose. To determine the nature of the imaging findings following reconstructive surgery using massive allografts in children with malignant bone tumours. Materials and methods. A retrospective review of the imaging studies and medical charts of 25 consecutive children who received an allograft as part of the management of a malignant bone tumour. Results. Uncomplicated allografts were sclerotic relative to native bone on radiographs and showed a typical ’tramline' appearance on bone scintigraphy. On MR, the medullary canal of the allograft showed low signal, similar to or greater than skeletal muscle, but less than subcutaneous fat, on 91 % of T1-weighted images. On short-tau inversion recovery images, the medullary canal was inhomogeneous and hyperintense to subcutaneous fat in 70 % and hyperintense to muscle in the remainder. Complications occurred in 68 % of patients and included allograft fractures (36 %), recurrent tumour (20 %), infection (8 %), and non-union or delayed union (8 %). The radiographic findings alone permitted accurate diagnosis of most serious complications. Infection and rejection were difficult to distinguish with any technique. All complications were suspected on clinical and/or radiological grounds before being shown by MR or scintigraphy. Conclusions. Allografts, whether normal or complicated, have characteristic imaging findings, except that infection and bone resorption related to rejection and revascularisation are difficult to distinguish. Routine MR and bone scintigraphy appear to contribute little to the management of these patients. Received: 6 October 1998 Accepted: 22 March 1999     

US, CT and MR imaging characteristics of nephroblastomatosis

Objectives. To describe the imaging features of nephroblastomatosis with US, CT and MR, to point out characteristics of differentiation between nephrogenic rests (NR) and Wilms' tumour (WT) and to determine the most appropriate imaging modality. Materials and methods. We reviewed the US, CT and MR images of 29 cases of histopathologically confirmed nephroblastomatosis sent to our department for reference evaluation (German nephroblastoma study). The series included 17 kidneys with NR, 6 kidneys with WT and 32 kidneys with both NR and WT. Results. NR presented as multinodular, peripheral, cortical lesions, the diffuse form of distribution being less common. Foci were homogeneous and of low echogenicity, density or signal intensity. The lesions were most clearly depicted with contrast-enhanced CT and T1-weighted (T1-W) MR images. Lesions smaller than 1 cm were rarely identified by US. The most reliable criterion to differentiate NR from WT was their homogeneity. Conclusions. Contrast-enhanced CT and T1-W MR images are of similar potential and superior to US in the diagnosis of nephroblastomatosis. Due to the significant radiation dose of serial CT, MR imaging should be the method of choice wherever it is available. The cost-effectiveness and availability of US makes it ideal for serial follow-up of known lesions. Received: 6 June 1997 Accepted: 9 January 1998     

Imaging characteristics of two subtypes of congenital hemangiomas: rapidly involuting congenital hemangiomas and non-involuting congenital hemangiomas

Background: Common infantile hemangiomas (COMMON) occur in approximately 10% of infants by the age of 1 year, with a female predominance. Some hemangiomas can be fully developed at birth and are thus called congenital hemangiomas (CH). Within this population, two courses have been identified: rapidly involuting CH (RICH) and non-involuting CH (NICH). Little has been reported on the clinical prognosis and imaging features of these entities. Objective: To describe the imaging characteristics of two subtypes of CH, i.e. RICH and NICH, and to compare them with COMMON. Materials and methods: We retrospectively gathered data on 26 children presenting with CH, i.e. lesions fully developed at birth. These lesions were divided into two groups according to the clinical course: suspected RICH (n=8) and suspected NICH (n=18). We used US, CT or MRI and angiography to identify the gross anatomy and structure and the vascularization. Imaging findings were compared with the clinical course and pathology results, when available. The imaging findings in these patients were compared retrospectively with those in 26 patients with COMMON randomly chosen from the database of our multidisciplinary clinic. Results: When compared with COMMON imaging characteristics, NICH and RICH had distinctive features on US such as being heterogeneous (72% of NICH and 62.5% of RICH vs 42.3% of COMMON), visible vessels (72% of NICH and 62.5% of RICH vs 15.4% of COMMON), calcifications (17% of NICH and 37.5% of RICH vs no case of COMMON). On CT and/or MRI, we compared imaging features such as well-defined limits (67% of NICH and 60% of RICH vs 100% of COMMON), and fat stranding (29.4% of NICH and RICH vs 7.7% of COMMON). Conclusion: Distinctive imaging characteristics are observed in cases of CH with US findings of visible vessels and calcifications statistically significant.     

Radiological features of focal nodular hyperplasia of the liver in children

Background. Focal nodular hyperplasia (FNH) is an unusual hepatic tumour in children and should be distinguished from other hepatic lesions. Objective. To describe the imaging characteristics of FNH in children. Materials and methods. We examined five patients (three boys and two girls, mean age 9.4 years) with pathologically confirmed FNH. The diagnosis was obtained by tumour resection (n = 4) and percutaneous needle biopsy (n = 1). One patient with multiple FNHs showed recurrent lesions after tumour resection. All patients were studied with US (including colour and power Doppler US [n = 3]) and CT. Dynamic enhanced CT scans were available in three patients. MRI (n = 2) or coeliac angiography (n = 1) was performed in three patients. Results. Seven of eight FNH lesions in five patients were demonstrated by imaging. The average size of the lesions was 6.5 cm. Six lesions detected on US showed variable echogenicity with a central hyperechoic scar (n = 2). On Doppler examination, central or peripheral hypervascular areas were seen (n = 3). Six lesions detected on contrast-enhanced CT showed high attenuation (n = 4) or iso-attenuation (n = 2). On early phase scans, all the lesions (n = 3) showed high attenuation. Irregular linear or ovoid central scars were detected in two patients on CT. MR demonstrated three lesions in two patients, one of which had not been detected by US or CT. A central low signal intensity scar (n = 1) was seen on T2-weighted MRI. Coeliac angiography performed in one patient showed a hypervascular mass with homogeneous staining. Conclusion. FNH in children shows a wide spectrum of imaging findings on various radiological examinations and the typical central scar was not always seen on imaging studies. Dynamic enhanced CT obtained in the early phase and colour Doppler studies may be helpful in the diagnosis of FNH by allowing characterisation of tumour vascularity. FNH should be included in the differential diagnosis of liver mass in children. Received: 10 September 1997 Accepted: 17 April 1998     

Whole-body magnetic resonance imaging: a useful additional sequence in paediatric imaging

Background: Whole-body MR (WBMR) imaging allows the acquisition of images of the entire body in a matter of minutes. Its use has primarily been in the evaluation of possible metastases in the setting of a known primary tumour. Objective: To document the value of WBMR imaging in ten children in whom this was added as an additional sequence when the primary diagnosis had not yet been made. Materials and methods: Ten children, age range 4 months–15 years (mean 7 years 4 months) had WBMR imaging after initial MR showed an abnormality that raised the possibility of systemic disease. Initial scanning was of the brain (n=1), spine (n=2), retroperitoneum (n=4), hips (n=1), femur (n=1) and wrist (n=1). Results: Abnormalities were detected in eight patients. Two patients had acute lymphoblastic leukaemia, and another had an anaplastic lymphoma, unsuspected prior to the WBMR. Two patients had a previously undiagnosed neuroblastoma with bone marrow metastases. Two patients had Langerhans cell histiocytosis. Another had multiple bone lesions due to cystic angiomatosis. Conclusions: WBMR imaging may be a useful additional sequence in children in whom a systemic and especially a bone marrow abnormality is suspected.Presented at 40th annual meeting of ESPR, Genoa, June 2003     

MR imaging of simple bone cysts in children: not so simple

Objective. The unicameral bone cyst (UBC) is a common cystic bone lesion seen in children. We review and summarize its MR findings, focusing on their appearance following contrast enhancement, and correlating them to known histologic features of UBC. Subjects and methods. A retrospective review of 20 cases (13 boys, 7 girls; age range, 1–17 years; mean age, 8.9) diagnosed as UBC was conducted. Clinical histories, radiographic and MR features, follow-up data, and available pathologic findings were noted. Results. At initial presentation 18 (90 %) of the patients with UBC had a history of acute or remote pathologic fracture. Cysts with history of fracture displayed heterogeneous fluid signals on T1- (n = 9, 50 %), and T2-weighted (n = 15, 83 %) MR images. Gadolinium-enhanced images were obtained in 15 of the 18 UBC cases with history of previous fracture. All showed enhancement with focal, thick peripheral, heterogeneous, or subcortical patterns. Focal nodules of homogeneous enhancement (diameter > 1 cm) within the UBC (n = 5) correlated with areas of ground-glass opacification on plain film. Other interesting MR features were fluid-fluid levels (n = 11), “fallen-leaf” sign (n = 1), soft-tissue changes (n = 2), and detection of septations not seen on plain film (n = 2). Conclusion. UBCs frequently appear complicated on MR imaging, with heterogeneous fluid signals and regions of nodular and thick peripheral enhancement related to previous pathologic fracture and early healing. Received: 27 December 1999 Accepted: 6 March 2000     

MR imaging of glioblastoma in children: usefulness of diffusion/perfusion-weighted MRI and MR spectroscopy

Background Glioblastoma is relatively uncommon in childhood and maybe difficult to differentiate from other brain tumors such as primitive neuroectodermal tumor, ependymoma, or benign astrocytoma. Objective To describe the characteristic MR features in children with glioblastoma and to evaluate the usefulness of diffusion and perfusion MR imaging and MR spectroscopy in pediatric glioblastoma. Materials and methods MR imaging in 11 children (12 tumors) with biopsy-proven glioblastoma was reviewed retrospectively. In one patient, there was a recurrent glioblastoma. We reviewed CT and MRI imaging for tumor location, density/signal intensity, and enhancement pattern. Routine MR imaging was performed with a 1.5-T scanner. In six patients, diffusion-weighted MR images (DWIs) were obtained with a single-shot spin echo EPI technique with two gradient steps, and apparent diffusion coefficients (ADCs) were calculated. Using the gradient EPI technique, perfusion-weighted MR images (PWIs) were obtained in four patients from the data of dynamic MR images. The maximum relative cerebral blood volume (rCBV) ratio was calculated between the tumor and contralateral white matter in two cases. In three patients, proton MR spectroscopy was performed using a single voxel technique with either STEAM or PRESS sequences. The locations of the tumor were the thalamus and basal ganglia (n=8), deep white matter (n=3), and brain stem (n=1).Results Intratumoral hemorrhage was seen in four tumors. The tumors showed high-signal intensity or DWIs, having a wide range of ADC values of 0.53–1.30 (mean ±SD=1.011±0.29). The maximum rCBV ratios of glioblastoma were 10.2 and 8.5 in two cases. MR spectroscopy showed decreased N-acetylaspartate (NAA) and increased choline in three cases. The MR findings of glioblastoma in children were: a diffusely infiltrative mass with hemorrhage involving the deep cerebral white matter, thalami, and basal ganglia. Conclusion Diffusion/perfusion MR imaging and MR spectroscopy are very helpful in diagnosing glioblastoma, determining the biopsy site, and evaluating tumor recurrence.This paper was presented as a scientific contribution at the 39th Annual Congress of the European Society of Paediatric Radiology, Bergen, Norway, June 2002     

MR cholangiography in children with autosomal recessive polycystic kidney disease

Background. Magnetic resonance cholangiography (MRC) is a relatively new, non-invasive imaging technique of the biliary tree that has shown good correlation with endoscopic retrograde cholangiopancreatography. The liver manifestation of autosomal recessive polycystic kidney disease (ARPKD) is congenital hepatic fibrosis (CHF). CHF may be accompanied by Caroli's disease, which is characterised by a non-obstructive dilation of the intrahepatic bile ducts. Objective. A prospective study was conducted to determine the presence and extent of Caroli's disease in children with ARPKD. Materials and methods. Seven children with ARPKD aged from 3.0 to 10.1 years were examined. CHF was confirmed in all biopsied cases (5 of 7). All children had been followed by repeated abdominal US examinations for many years. The MR examination included a morphological imaging study using a T2-weighted turbo spin-echo sequence and a heavily T2-weighted inversion-recovery turbo spin-echo sequence with three-dimensional maximum intensity projection (MIP) reconstructions for MRC. Results. The diagnosis of Caroli's disease could be made in one case by US; in two other children Caroli's disease was suspected, but the differentiation from hepatic cysts was not possible. By MRC, Caroli's disease could be diagnosed in three of seven children. Furthermore, MRC with MIP reconstructions demonstrated the extent of the disease by showing the entire biliary tree from different angles. Conclusions. MRC is a valuable method to establish the diagnosis and demonstrate the extent of Caroli's disease. Received: 5 January 1998 Accepted: 9 December 1998     

Pathology of Intragestational Intervention in Twin-to-twin Transfusion Syndrome

Selective intervention in multiple pregnancy is being used to enhance the chances of survival of at least one conceptus when the risks for the combined conceptuses and mother are considered too great. These procedures have been applied to induced polyembryonic conceptions (selective continuance) and discordant dichorionic twins (selective birth). We report attempts at selective intervention in three monochorionic twin gestations affected by twin-to-twin transfusion syndrome. In all three cases, both fetuses seemed doomed and the mother was in significant distress. The selected survivor in the first case is doing well; both twins were stillborn in the second case; in the third case, the selected survivor died as a neonate but the other twin survived and is doing well. We suggest possible explanations for the clinical outcome of each case based on detailed pathologic examination of the delivered placentas and autopsy examination of the nonsurviving twins. The shared chorionic circulation is the source of both the clinical disorder and the potential complications of any attempt to alleviate the disorder. This situation is unique to monochorionic twins, and we discuss the implications of this for intrauterine therapy of twin-to-twin transfusion syndrome.     

Pathology of intragestational intervention in twin-to-twin transfusion syndrome

Selective intervention in multiple pregnancy is being used to enhance the chances of survival of at least one conceptus when the risks for the combined conceptuses and mother are considered too great. These procedures have been applied to induced polyembryonic conceptions (selective continuance) and discordant dichorionic twins (selective birth). We report attempts at selective intervention in three monochorionic twin gestations affected by twin-to-twin transfusion syndrome. In all three cases, both fetuses seemed doomed and the mother was in significant distress. The selected survivor in the first case is doing well; both twins were stillborn in the second case; in the third case, the selected survivor died as a neonate but the other twin survived and is doing well. We suggest possible explanations for the clinical outcome of each case based on detailed pathologic examination of the delivered placentas and autopsy examination of the nonsurviving twins. The shared chorionic circulation is the source of both the clinical disorder and the potential complications of any attempt to alleviate the disorder. This situation is unique to monochorionic twins, and we discuss the implications of this for intrauterine therapy of twin-to-twin transfusion syndrome.