Imaging of chronic inflammatory bowel disease with 18F-FDG PET in children and adolescents.

Standard for diagnosis of inflammatory bowel disease (IBD) is the endoscopy of the stomach and the intestine. Aim of this study was to determine the value of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in pediatric patients with mild to moderate IBD.We included 23 children and adolescents between 8 and 17 years (median 15 years, 13 boys, 10 girls) in this retrospective study in a routine clinical setting. Diagnoses were Crohn's disease in 19 and ulcerative colitis in 4 cases.3 children had a conventional FDG-PET, 20 patients a combined FDG-PET-computed tomography exam. All children had upper and lower intestinal endoscopy with biopsy and a Hydro-MRI exam to assess the jejunum and proximal ileum. The gastrointestinal tract was divided in 7 segments: Stomach plus duodenum, jejunum and proximal ileum, terminal ileum, cecum plus ascending colon, transverse colon, descending colon, and rectosigmoid.Superficial gastric lesions were missed, gastric ulcerations were detected. For the stomach, the sensitivity was 0.25, the specificity was 1.00, the positive predictive value was 1.00, for the lower intestine (terminal ileum and colon) the values were 0.74, 0.88, and 0.96; for the terminal ileum 0.89, 0.75 and 0.94, respectively.The sensitivity and specificity for of ileal and colonic lesions is high. FDG-PET has to be discussed as a tool for the determination of extent and degree of inflammation, especially in those parts of the small bowel that are not accessible to endoscopy. This has to be weighed against the additional radiation exposure administrated.

Standard value of ultrasound elastography using acoustic radiation force impulse imaging (ARFI) in healthy liver tissue of children and adolescents.

PURPOSE: Ultrasound elastography by acoustic radiation force impulse imaging (ARFI) is used in adults for non invasive measurement of liver stiffness, indicating liver diseases like fibrosis. To establish ARFI in children and adolescents we determined standard values of healthy liver tissue and analysed potentially influencing factors. MATERIALS AND METHODS: 132 patients between 0 and 17 years old were measured using ARFI. None of them had any liver disease or any other disease that could affect the liver secondarily. All patients had a normal ultrasound scan, a normal BMI and normal liver function tests. The mean value of all ARFI measurements was calculated and potentially influencing factors were analysed. RESULTS: The mean value of all ARFI elastography measurements was 1.16 m/sec (SD ±â€Š0.14 m/sec). Neither age (p = 0.533) nor depth of measurement (p = 0.066) had no significant influence on ARFI values, whereas a significant effect of gender was found with lower ARFI values in females (p = 0.025), however, there was no significant interaction between age groups (before or after puberty) and gender (p = 0.276). There was an interlobar difference with lower values in the right liver lobe compared to the left (p = 0.036) and with a significantly lower variance (p < 0.001). Consistend values were measured by different examiners (p = 0.108), however, the inter examiner variance deviated significantly (p < 0.001). CONCLUSION: ARFI elastography is a reliable method to measure liver stiffness in children and adolescents. In relation to studies which analyse liver diseases, the standard value of 1.16 m/sec (±â€Š0.14 m/sec) allows a differentiation of healthy versus pathological liver tissue.

[Dupuytren's disease in children - case report and literature review]. / Morbus Dupuytren im Kindesalter - Fallbericht und Literaturrecherche.

Dupuytren's disease in childhood and adolescence is extremely rare. As in adults most patients are male. We report on a girl at the age of 7 years with a cord in the palm.

Partial splenic embolization as an alternative to splenectomy in hypersplenism--single center experience in 16 years.

PROBLEM: In young patients with hypersplenism splenectomy implies a lifelong increased risk for post-splenectomy infection. Especially in children, whose immune system is not yet completely matured, the risk for some bacterial infection may increase after splenectomy because the spleen helps to defend against encapsulated bacteria like pneumococci, meningococci and haemophilus influenzae. We present partial splenic embolization as an alternative to surgical splenectomy. METHOD: Partial splenic embolization was performed in 17 patients from 1-31 years with hypersplenism of various etiologies and was achieved by selective catheterization of splenic arteries and injection of 150-355 µm polyvinyl alcohol particles (Ivalon (®)). After the intervention the patients received an intensified analgesic regimen and antibiotics to avoid concurrent infectious complications. RESULTS: Partial splenic embolization represented between 30-60% of the splenic volume and was followed in general by an immediate increase of all blood cells and symptoms of hypersplenism were reduced. In 2 patients the procedure was repeated because the result of the first embolization was insufficient in one patient and became necessary in another in the long run. Post-procedural side effects included fever, abdominal pain, ascites and pleural effusions. There were no acute infections in any patient. CONCLUSION: Our monoinstitutional experiences over 16 years offer, partial splenic embolization in patients with hypersplenism from miscellaneous reasons as a low-risk alternative to surgical splenectomy. The procedure can be repeated as necessary, but it is always a temporary palliation depending on the underlying disease which often leads to liver transplantation. Using intensive analgesia and antibiotics side effects were tolerable, and patients could be discharged after a few days.

An inflammatory myofibroblastic tumor of the stomach as a rare cause of gastric outlet obstruction in an 8-month-old infant.

Inflammatory myofibroblastic tumors (IMT) are a rare entity. Localization in the stomach is extremely seldom and almost exclusively seen in children. Invasive growth of IMT may lead to irresectability or recurrence. In an 8-month-old girl presented with repetitive vomiting for several days. Complete surgical resection of a gastric IMT was possible.

Ultrasound in diagnosis of fractures in children: unnecessary harassment or useful addition to X-ray?

PURPOSE: Handling children with trauma is often very difficult, as the signs and the exact location of the fracture are sometimes unclear. The aim of this study was to compare ultrasound and X-ray for the identification of fractures. MATERIALS AND METHODS: In this study 653 patients from newborn to 17 years old (mean age 4.4 years) with nonspecific clinical signs or clinically indistinct fracture location were examined initially with ultrasound. After identification of the fracture by ultrasound, an X-ray exam of the predefined region followed. In cases of negative ultrasound an X-ray exam was performed not focused on a predefined region. RESULTS: Examinations of 726 parts of the body revealed 308 fractures. 266 fractures were identified by both ultrasound and X-ray, 20 exclusively by ultrasound, 21 exclusively by X-ray. One maxillary fracture was missed. The sensitivity was 92.9% for ultrasound and 93.2% for X-ray, and the specificity was 99.5% (ultrasound) and 99.8% (X-ray). Ultrasound was superior to X-ray on the clavicle, but the opposite was true in the lower limb. In 8.4% of the patients ultrasound helped us to reduce the number of X-rays. CONCLUSION: Ultrasound is comparable to X-ray for the detection of fractures. Ultrasound should be the first imaging method in children with trauma and nonspecific clinical signs or indistinct location of pain, followed by X-ray exams of the predefined region.

[Trabecular bone mineral density measured by quantitative CT of the lumbar spine in children and adolescents: reference values and peak bone mass]. / Trabekuläre Knochendichte der Lendenwirbelsäule bei Kindern und Jugendlichen in der quantitativen CT: Referenzwerte und Peak Bone Mass.

PURPOSE: The aim of this study was to assess bone density values in the trabecular substance of the lumbar vertebral column in children and young adults in Germany from infancy to the age of peak bone mass. MATERIALS AND METHODS: We performed quantitative computed tomography (QCT) on the first lumbar vertebra in 28 children and adolescents without diseases that may influence bone metabolism (15 boys, 13 girls, mean ages 11 and 8 years, respectively). We also measured 17 healthy young adults (9 men, 8 women, mean ages 20 and 21 years). We used a Somatom Balance Scanner (Siemens, Erlangen) and the Siemens Osteo software. Scan parameters: Slice thickness 1 cm, 80 kV, 81 or 114 mAs. We measured the trabecular bone density and the area and height of the vertebra and calculated the volume and content of calcium hydroxyapatite (Ca-HA) in the trabecular substance of the first lumbar vertebra. RESULTS: Prepubertal boys had a mean bone density of 148.5 (median [med] 150.1, standard deviation [SD] 15.4) mg/Ca-HA per ml bone, and prepubertal girls had a mean density of 149.5 (med 150.8, SD 23.5) mg/ml. We did not observe a difference between prepubertal boys and girls. After puberty there was a significant difference (p < 0.001) between males and females: Mean density (male) 158.0, med 162.5, SD 24.0 mg/ml, mean density (female) 191.2, med 191.3, SD 17.7 mg/ml. The Ca-HA content in the trabecular bone of the first lumbar vertebra was 1.1 (med 1.1, SD 0.5) g for prepubertal boys and 1.1 (0.9, 0.4) g for prepubertal girls. For post-pubertal males, the mean Ca-HA content was 3.5 g, med 3.5, SD 0.5 g, and for post-pubertal females, the mean content was 2.8, med 2.7, SD 0.4 g. CONCLUSION: The normal trabecular bone mineral density is 150 mg/ml with a standard deviation of 20 mg/ml independent of age or gender until the beginning of puberty. Peak bone mass (bone mineral content) in the trabecular substance of the lumbar vertebral column is higher in males than in females, and peak bone density is higher in young female adults than in young male adults.

[Assessment of bone density in children and adolescents by quantitative CT of one lumbar vertebra only]. / Messung der Knochendichte bei Kindern und Jugendlichen mittels quantitativer CT an nur einem Lendenwirbelkörper.

AIM: The aim of this study was to determine whether measuring the trabecular bone density in one lumbar vertebra (L) instead of three has an effect on the assessment of bone density in children. MATERIALS AND METHODS: Bone density was measured in 70 patients (38 male, 32 female), ages 1 to 22 years (mean age 12.4, median 13 years) using a Somatom Balance Scanner (Siemens). The trabecular bone density was measured (1-cm slice, 80 kV, 81 mAs) in L1 through L3. RESULTS: The mean bone density in the lumbar vertebrae was 139.9 mg/ml calcium-hydroxylapatite per ml bone in L1, 133.9 in L2, and 131.7 in L3, and the mean of all three vertebrae was 135.2 mg/ml. Measurements in L1 were 4.7 mg/ml (standard deviation [SD] 4.7 mg/ml) above the mean value of the three upper lumbar vertebrae. The density in L2 was 1.3 mg/ml below the mean (SD 3.1 mg/ml). On average, L3 was 3.5 mg/ml below the mean (SD 4.6 mg/ml). This minor systematic deviation in the measurement values for the individual vertebrae from the mean value was so low that there was no statistical evidence for a deviation from an ideal regression line. CONCLUSIONS: When using an adequate technique, especially accurate analysis of the topogram (scout view) and the tomogram, measurement of the bone density of one lumbar vertebra provides satisfactory information regarding the trabecular bone density in children, but the values differ in the three upper lumbar vertebrae. The bone density of L2 is close to the mean of the upper three vertebrae. In the case of pathologic values or a pathologic trabecular pattern, three vertebrae should be measured.

[Sonography of the optical nerve -- experience in 483 children]. / Die Sonographie des Nervus opticus -- Erfahrungen bei 483 Kindern.

INTRODUCTION: In case of increased intracranial pressure (IICP), the inflow of cerebrospinal fluid widens the space between the optic nerve (ON) itself and the surrounding dura mater leading to the sonographic appearance of increased diameter of the ON. The purpose of the study was to gain clinical experience in children and to determine (a) the mean values for patients without proven IICP and (b) pathologic values of those with proven IICP. MATERIALS AND METHODS: Ultrasound of the optic nerve was performed in 483 children with symptoms consistent with IICP, comprising 287 males and 196 females at an age ranging from 4 days to 24 years with a mean age of 7.5 + 5.1 years. The measured diameter of the ON of both eyes and the morphologic criteria concerning nerve sheath and papilla were evaluated retrospectively. RESULTS: Most of the investigated patients (n = 466) had no IICP as confirmed by neurological examination, EEG, sometimes CCT and/or MRI and clinical follow-up. The typical morphological findings in patients with normal intracranial pressure (ICP) were a clear and longitudinally extended demarcation of the ON with a well-delimited nerve sheath and without prominent papilla. The mean diameter of the ON in patients with normal ICP was 3.4 mm + 0.7 mm. In 17 patients with proven IICP, the mean diameter of the ON increased to 5.6 mm + 0.9 mm. Typically, the nerve sheath was indistinguishable from the hypoechoic ON, often but not invariably associated with a prominent papilla. In patients with IICP, the diameter of the ON alone matches the diameter of the ON plus nerve sheath in patients with normal ICP. CONCLUSION: IICP was detected with a high sensitivity by ultrasound using the diameter and the morphological criteria of an indistinguishable nerve sheath. A diameter of more than 4.5 mm is definitely pathologic and requires further investigation. Prominence of the papilla is an unreliable criterion for acute IICP.

X-linked lissencephaly with abnormal genitalia associated with renal phosphate wasting.

X-linked lissencephaly with abnormal genitalia (XLAG) is a rare disorder caused by mutations in the aristaless-related homeobox (ARX) gene. We report on the clinical data of a boy with a 1-bp deletion (790 delC) resulting in a frame shift in the ARX gene and prolonged survival until age 18 months. Similar to other patients, the boy showed postnatal microcephaly, hypothalamic dysfunction, intractable neonatal seizures, and chronic diarrhoea. In addition, he suffered from exocrine pancreatic insufficiency and renal phosphate wasting became apparent from age 5 months, both of which have not been described previously in XLAG. This allows us to speculate that the phenotype of XLAG is more complex than hitherto known and may include renal phosphate wasting which might not have been observed in other patients due to early death.

[Small bowel intussusception in childhood]. / Dünndarminvaginationen im Kindesalter: Diagnostik und Bedeutung.

BACKGROUND: Intussusception is the most common cause of abdominal emergency in early childhood. The majority of cases are ileocolic type of intussusception. Only few reports concerning small bowel intussusception have been reported. PATIENTS AND METHOD: We retrospectively reviewed the clinical records and imaging findings of all patients with the diagnosis of intussusception (comparing small bowel intussusception with ileocolic type of intussusception), which were documented by ultrasound in the period April 1997 to January 2001. The routine ultrasound scans included an evaluation of the entire abdomen using sector and linear transducers of high frequency (5 - 7.5 MHz) and power doppler ultrasound. RESULTS: A total of 22 patients with small bowel intussusception (9 female, 13 male) and 29 patients diagnosed to suffer from ileocolic intussusception (10 female, 19 male) were identified and treated. Children with small bowel intussusception were significant older in comparison to children with ileocolic type of intussusception (median age 50 vs. 11 months). In our series the presenting symptoms of patients with small bowel intussusception consisted of abdominal pain (86 %) and vomiting (36 %). The initial clinical symptoms of patients with ileocolic intussusception were abdominal pain (100 %), vomiting (72 %) and/or rectal fresh blood (35 %). Small bowel intussusception was an incidental finding in 3 asymptomatic patients (14 %). Hydrostatic reduction was attempted in 14 % of children with small bowel intussusception (vs. 93 % of children with ileocolic intussusception), one patient needed operative treatment (vs. 21 %). Outcome in all patients was favorable. CONCLUSION: The high percentage of patients with small bowel intussusception observed may relate to increased use of abdominal ultrasound in children presenting with abdominal pain and improvements in resolution and quality of the images. Small bowel intussusceptions in our series were in the majority of cases short-segmented, self-limited and without a lead point. In comparison to patients with ileocolic intussusception the presenting symptoms of small bowel intussusception are less acute.

[Congenital, solitary hepatic cyst: Observation of the development from birth upon the intervention in the 5th year of life]. / Kongenitale solitäre Leberzyste - Eine Verlaufsbeobachtung von der Geburt bis zur Intervention im 5. Lebensjahr.

BACKGROUND: Sonographic screening helps to discover pathological findings in asymptomatic patients. In these cases the decision about diagnostic and therapeutic consequences is difficult. CASE REPORT: We describe a girl with a congenital small solitary hepatic cyst with rapidly increasing size after the second year, which was interventionally sclerosed at the age of five years. CONCLUSION: Even in cases of small cysts long term follow up investigations are necessary, because the growth rate of the cyst can change.

Esophageal foreign bodies: removal of the new euro coins with a magnet tube.

AIM OF THIS STUDY: We use a magnet tube to extract esophageal coins. It is smaller than the Foley balloon catheter and easier to handle than a forceps. We present the magnet tube maneuver and investigate whether the new euro coins can be removed with a magnet. METHOD: We take radiographs of coins and measure the adhesive force of the magnet and compare with the 10 Pfennig German coin which we most often removed successfully from the esophagus. RESULTS: The 1, 2, and 5 euro cent pieces are magnetic and can possibly be removed with a magnet tube. The non-magnetic pieces can be identified based on the rim and size. DISCUSSION: When an esophageal foreign body is not round and smooth like a coin, the fluoroscopic guided procedure becomes more risky with respect to mucosal injury. When a large object is stuck in the esophagus for more than 24 hours, an endoscopy must be considered to rule out any severe damage to the esophageal wall.

[Solid-pseudopapillary tumors in childhood]. / Solid-pseudopapillärer Pankreastumor im Kindesalter, Ein Bericht über 5 Fälle, Germany.

We report on fife female patients with solid pseudopapillary tumors of the pancreas. The tumors are extremely rare in children. They occur mainly in adolescent and young adult females. The tumors are neoplasms of low malignancy with infrequent metastases, for instance, in the liver or the peritoneum. Although the tumors had reached a large diameter, all of them underwent complete tumor resection. After that the patients have a very good prognosis. Thus, so it is important to distinguish solid-pseudopapillary tumors from other tumors of the pancreas.

[Hereditary deficiency of C1-esterase inhibitor presenting with recurrent abdominal pain]. / Hereditäres Angioödem Typ I durch C1-Inhibitor-Mangel als Ursache von rezidivierenden Bauchschmerzen.

Hereditary deficiency of C1-esterase inhibitor (C1-INH) which clinically manifests as hereditary angioedema is a rare disorder. In previously not diagnosed cases, a fatality rate of up to 30 % has been reported. The diagnosis of C1-esterase inhibitor deficiency should not be missed in patients presenting with angioedema in the face, stem or extremities. We report the case of a 17-year-old girl with recurrent abdominal pain and swelling of the hands.

[Pathophysiology, diagnosis and therapy of three congenital dacryocystoceles]. / Pathophysiologie, Diagnose und Therapie von drei kongenitalen Dakryozystozelen.

BACKGROUND: Congenital dacryocystocele has been rarely described in German literature. Congenital obstruction and distention of the lacrimal sac makes it necessary to differentiate for various causes. PATIENTS AND METHODS: Three infants had congenital dacryocystocele. The diagnosis was made by sonography and was established by probing the lacrimal duct and nasal endoscopy that showed a white prominent tumor below the lowest conch. RESULTS: Directly after endonasal and canalicular opening of the dacryocystocele clear liquid with white detritus drained, and the swelling decreased. No further procedure was needed. CONCLUSION: The diagnosis of dacryocystocele can be made by inspection, probing the lacrimal duct, ultrasound and nasal endoscopy. If probing of the lacrimal duct could not easily open the Hasner's membrane, endonasal opening can be performed. The cooperation with an ear-nose-throat specialist could lead to the diagnosis of dacryocystocele and sufficient treatment without the need for any further radiological examinations.

[Wilson-Mikity syndrome as a cause of respiratory insufficiency of prematurity]. / Wilson-Mikity-Syndrom als Ursache einer respiratorischen Insuffizienz bei einem Frühgeborenen.

The Wilson-Mikity syndrome is a differential diagnosis of chronic lung disease in the neonate and primarily related to immaturity. It is characterized by the absence of typical clinical and radiological findings of the respiratory distress syndrome (RDS). Infectious causes are being discussed.

[Lipoma of the corpus callosum in a newborn infant: misinterpretation of cranial ultrasound by coincidence with thrombocytopenia]. / Lipom des Corpus callosum bei einem Neugeborenen: Fehlinterpretation der Schädelsonographie durch Koinzidenz mit Thrombopenie.

Among the very rare intracerebral lipomas, those of the corpus callosum are the most frequent. Due to the advanced technology and the frequent use of ultrasonography these lesions are diagnosed more and more often. A female neonate was admitted to our hospital because of a progressive thrombocytopenia. Pregnancy was complicated by an autoimmune thrombocytopenia of the mother. While there were no remarkable findings on clinical presentation, a sonogram of the brain revealed an area of increased echogenicity in the midline which was interpreted as an intracerebral hemorrhage. In absence of any respective clinical signs a magnet-resonance-tomography of the brain was performed leading to the hypothesis of a lipoma of the corpus callosum (LCC) that could be verified by a densitometry in a cranial computer tomography (CT). Obviously, the initially performed sonogram was misinterpreted as an intracerebral hemorrhage due to the coincidence with the thrombocytopenia. At last the discrepancy of clinical and ultrasonographical findings led to the diagnosis by magnet-resonance-tomography and CT scan. Knowledge of the typical sonographic appearance of an LCC may be helpful for the differential diagnosis of this rare lesion even in fetal ultrasound.