Assessment of interpretation of paediatric skeletal radiographs in the emergency room.

AIM: To assess the performance of paediatric skeletal radiography interpretation in the emergency department. MATERIALS AND METHODS: In a large paediatric referral centre, data from trauma patients with initially misdiagnosed skeletal radiography was collected from October 2014 to June 2015. Data analysis focused on demographic data, region of the injury and clinical consequences as well as outcome of treatment. RESULTS: In 125 of 2,316 patients aged from 1-17 years an initial misdiagnosis was documented (5.4%). Misdiagnosis was detected and corrected the next day in a routine review attended by an experienced paediatric radiologist. False-negative interpretation (missed fracture) was found in 62 and false-positive (overdiagnosis) interpretation in 63 patients. The highest error rate was found in elbow radiography (12%) followed by wrist (8%), fingers (4.5%), metacarpus (4.2%), and toes (3.5%). The most frequently missed fracture was supracondylar elbow fracture (n=12). In case of initially missed fractures treatment was adjusted delayed the next day. In none of these patients, were repositioning or open surgical procedures necessary. No misdiagnosis resulted in additional morbidity. CONCLUSION: Misinterpretation of paediatric skeletal radiography by paediatric emergency physicians occurred frequently. The majority of diagnostic errors occurred in a few regions. None of these misdiagnoses negatively affected patients' health. A routine review by an experienced paediatric radiologist is mandatory, but may suffice the next day.

Therapeutic step-up strategy for management of hereditary pancreatitis in children.

BACKGROUND/PURPOSE: Various different regimes exist for the treatment of hereditary pancreatitis in childhood. Here, we propose a therapeutic pathway with emphasis on endoscopic and surgical procedures. METHODS: From 2006 to 2013, 12 patients with a diagnosis of hereditary pancreatitis were prospectively included in a therapeutic step-up schema. The treatment outcome was evaluated and correlated to aetiological factors and pathoanatomic findings. RESULTS: After diagnostic work-up (laboratory data, ultrasound examination, magnetic resonance cholangiopancreatography and genetic testing), all 12 patients underwent early endoscopic retrograde cholangiopancreatography (ERCP), which was successfully performed in ten children. Obstructive pancreatitis was found in eight children, and required sphincterotomy, dilation and stenting for 12 months. In two children with unsuccessful ERCP, open surgical drainage procedures were performed. After a mean follow-up of 32 months all children are free of recurrence of pancreatitis without any impairment of everyday activities. CONCLUSIONS: For children with hereditary pancreatitis, a therapeutic step plan with early ERCP and open surgical drainage procedures in case of impossible or insufficient endoscopic treatment prevents recurring pancreatitis and offers a normal quality of life without any major complications.

Early postnatal diagnosis of Costello syndrome.

Costello syndrome is a rare congenital disease with typical craniofacial and musculoskeletal features, cutaneous lesions, cardiac defects and cancer susceptibility. Affected patients show severe feeding difficulties for the first years of life and developmental delay. We present the case of a patient, in whom fetal tachycardia, polyhdramnios and physical characteristics led to an early diagnosis of Costello syndrome. Based on this patient we describe challenges and problems of therapeutic management of infants with Costello syndrome.

Necrotizing enterocolitis after open cardiac surgery for congenital heart defects--a serious threat.

Neonates with congenital heart disease are at risk to develop necrotizing enterocolitis (NEC). Especially in the postoperative period after correction of a congenital heart defect this intestinal inflammatory disease can be a potentially life-threatening complication. The purpose of this case series is to define the specific features of NEC in the postoperative situation, with emphasis on diagnostic difficulties.Medical records of neonates developing severe NEC in the postoperative period after surgical correction of a congenital heart defect from 2008 to 2011 were reviewed and compared.7 neonates with NEC after surgical correction of a congenital heart defect were identified. In all 7 patients typical radiologic findings of NEC were missing. Diagnosis was late in all 7 cases; 5 patients already had intestinal perforation. In 3 patients NEC successfully managed before surgical correction of the congenital heart defect dramatically relapsed in the postoperative period. 2 patients showed intestinal mucormycosis, an entity not clearly differentiated from NEC. Due to progressed disease at diagnosis all patients required extensive surgical procedures; 2 of them subsequently died.In the postoperative situation after surgical correction of a congenital heart defect diagnosis of NEC might be challenging. The general condition of these children complicates clinical examination and characteristic radiologic signs are absent. Late diagnosis and exclusive pathogens cause high morbidity and mortality.

[Closing gastroschisis: a distinct entity with high morbidity and mortality]. / Closing Gastroschisis: eine Sonderform der Gastroschisis mit hoher Morbidität und Mortalität.

PURPOSE: We correlate severe bowel damage in gastroschisis to the rare intrauterine event of narrowing of the abdominal wall around the protruding intestines. We describe this "closing gastroschisis" as a distinct entity. Prenatal ultrasound findings as gastric or bowel dilation were compared to the postnatal findings in order to find markers for an early in utero diagnosis of closing gastroschisis. Early diagnosis could prompt timely delivery to save the compromised bowel and avoid short gut syndrome. MATERIALS AND METHODS: We documented the pre- and postnatal course of our patients with gastroschisis from 2007 to 2009.  Closing gastroschisis was suspected antenatally and confirmed postnatally. We identified 5 out of 18 patients showing closure of the abdominal wall with varying degrees of bowel damage. Prenatal ultrasound findings were correlated to the postnatally confirmed extent of intestinal damage. RESULTS: We could not find consistent ultrasound markers for prenatal diagnosis of closing gastroschisis. In prenatal ultrasound three patients presented significant gastric dilation and then experienced severe courses postnatally due to segmental gut necrosis. One of these three died and the other two developed short gut syndrome. In one case progressive intraabdominal loop dilation with simultaneous shrinking of the extraabdominal loops occurred corresponding to closing gastroschisis with segmental midgut necrosis. CONCLUSION: Closing gastroschisis must be seen as a special form of gastroschisis. Extended intestinal damage is often life-threatening. In longitudinal observation dynamics of fetal ultrasound findings can lead to the diagnosis of closing gastroschisis. Progressive intraabdominal loop dilation is always highly suspicious and must lead to close follow-up and timely delivery.

[Hepatic artery pseudoaneurysm following blunt abdominal injury]. / Arterielles Pseudoaneurysma der Leber nach stumpfem Bauchtrauma.

Posttraumatic hepatic artery pseudoaneurysms are a rare but life-threatening complication of blunt abdominal trauma with liver damage. We report the case of a child who developed a pseudoaneurysm of the right hepatic artery after a bicycle accident with central liver rupture. After an episode of hemodynamically relevant hemobilia due to delayed bleeding, the asymptomatic pseudoaneurysm was diagnosed coincidentally by ultrasound. Because of the progression in size angiographic coiling was performed and led to thrombotic occlusion of the pseudoaneurysm. After a symptom-free period of 1 month the child required surgery because of acute cholecystitis.

[Bleeding into retroperitoneal cavernous lymphangioma following blunt abdominal trauma]. / Blutung in ein kavernöses retroperitoneales Lymphangiom nach stumpfem Bauchtrauma.

The case of a 12-year-old boy with hemorrhage into a previously unknown retroperitoneal lesion following blunt abdominal trauma is reported. Diagnostic work-up of a post-traumatic surgical acute abdomen revealed a giant multicystic tumor in the retroperitoneum, which could be completely removed. Histological examination confirmed the suspected diagnosis of congenital lymphangioma. Attention should be drawn to the possible coincidence of trauma and a preexisting asymptomatic lesion. Knowledge of the characteristic ultrasonographic and computer tomographic features is essential in order to make a correct diagnosis.

Fetal liver magnetic resonance imaging in anterior body wall defects: a study of specimens from the museum of pathology.

PURPOSE: The purpose of the study was to evaluate the appearance of liver anatomy in fetuses with complex anterior body wall defects (ABWD) using magnetic resonance imaging (MRI). METHODS: Nineteen specimens from the National Museum of Pathological Anatomy (Vienna) with a wide range of ABWD were studied on a 1.5-T MR scanner (T1- and T2-weighted sequences) with special focus on the liver and the relationship between the large lesion and the adjacent structures. RESULTS: The contrast of T2-weighted sequences was superior to that of the T1-weighted images. The liver was found in an intraabdominal location in 3 cases and in a completely extraabdominal location in 11 fetuses. Five fetuses had a very special configuration or position of the liver. Furthermore, the images showed an anteriorly located confluence of hepatic veins into the inferior vena cava in 5 specimens. Associated malformations of the complexly structured malformations involved the urogenital tract, the central nervous system (CNS), the cardiac system and the musculoskeletal system. Prenatal fast magnetic resonance imaging (MRI) in one fetus confirmed the liver in a both inside/outside position, whereas the diagnosis at fetal ultrasound scan (US) in this case had been uncertain. CONCLUSIONS: These results show that MRI may play an important part in antenatal diagnosis of ABWD, complementary to prenatal US. Prenatal fast MRI should be considered if fetal US yields ambiguous findings.

[MR imaging in congenital complicated anterior body wall defects]. / MRT von komplizierten angeborenen Bauchwanddefekten.

INTRODUCTION: Aim of this study was to estimate the value of postmortem MR imaging in evaluation of specimen with congenital anterior body wall defects of the museum of pathologic-anatomy. MATERIAL AND METHODS: We examined 19 specimen with a 1.5 Tesla unit by using T 1 - and T 2 -weighted sagittal and coronal sequences. In some specimen additional axial T 2 -weighted images were obtained. We evaluated the site of the bowel, the liver, the heart and presence of associated disorders. RESULTS: The bowels were completely intraabdominal, in two specimen, completely extraabdominal in 12 specimen and in 5 specimen intra- and extraabdominal. The liver was in two specimen completely extraabdominal/ in 12 completely intracorporal, and in 5 specimen intra- and extraabdominal. In 5 cases the heart was located extraanatomically. In 12 specimen we found disorders of the spine and the extremities. Congenital disorders of the kidneys were found in 6 specimen. CONCLUSION: MR imaging is of great value in the assessment of congenital anterior body wall defects. In the light of ultrafast sequences the role of fetal MR imaging in the evaluation of congenital body wall defects may be mandatory in the future.

Severe liver haemorrhage during laparotomy in very low birthweight infants.

UNLABELLED: A review is presented of severe liver haemorrhage as a serious complication in surgery on very low birthweight (VLBW) infants. Clinical data and pathological findings, as well as the outcome of the treatment, of five VLBW infants (<1000 g) who experienced liver haemorrhage during surgical exploration for necrotizing enterocolitis or spontaneous intestinal perforation were reviewed retrospectively. At the time of surgery, all infants had signs and symptoms of impending sepsis. The bleeding was predominantly "spontaneous" without obvious iatrogenic liver damage. In three infants, severe liver haemorrhage could be stabilized by early liver tamponade using absorbable thrombostatic sponges and polyglactin mesh. CONCLUSION: Intraoperative liver haemorrhage potentially life-threatening complication of surgery in preterm infants, which is not frequently investigated. Immediate perihepatic liver packing may be used to achieve adequate bleeding control.

Videofluoroscopy of deglutition in children after repair of esophageal atresia.

PURPOSE: To evaluate the functional disorders of the oral and pharyngeal phases of deglutition after repair of esophageal atresia in children. MATERIAL AND METHODS: 19 children (10 girls, 9 boys, mean age 22 months) underwent videofluoroscopy of deglutition after repair of esophageal atresia. The videofluoroscopic studies were assessed according to functional and morphological changes in the oral, pharyngeal and esophageal phases. The persistence of radiologic findings on videofluoroscopy was determined. RESULTS: The oral phase was normal in all patients. The main functional disorder of the pharyngeal phase was aspiration in 7 (37%) children. A completely normal deglutition in the pharyngeal and esophageal phases was not seen in any patient. CONCLUSION: Videofluoroscopy after repair of esophageal atresia is helpful in differentiation of functional and morphological disorders that can lead to prandial aspiration and have an influence on the decision about continued therapy.

[The intrauterine rupture of the fetal bladder as a complication of low urinary tract obstruction]. / Die intrauterine Blasenruptur als Komplikation infravesikaler Harnentleerungsstörungen beim Feten.

Lower urinary tract obstruction in fetuses causes megacystis, megaureter and hydronephrosis. The spontaneous rupture of the fetal bladder represents a rare complication in cases of total bladder outlet obstruction. We report about two different cases, present pre- and postnatal therapeutic regimes and discuss the possibility of spontaneous healing of a ruptured fetal bladder before delivery.

Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery: a hereditary compound structure in duodenal atresia?

Duodenal atresia predominantly represents an isolated entity, but could also be part of a complexly structured intestinal malformation. We report four children, including two from one family, with duodeno-jejunal atresia associated with malrotation, volvulus, and absent parietal attachment of the mesentery. Gross absence of the mesentery and absence of distal parts of the superior mesenteric artery were the most remarkable findings. The small intestine was supplied retrogradely from the right colic artery. Consideration of embryological theories of this malformation takes into account the important role of the duodeno-jejunal flexure during the process of entry of the small bowel loops into the abdomen. Familial occurrence suggests autosomal recessive inheritance. We propose differentiating the pathoanatomical findings in our patients from classical apple peel small bowel syndrome (APSB).

Limb-body wall complex: a compound anomaly pattern in body-wall defects.

Our presentation of four cases demonstrates the essential features of limb-body wall complex (LBWC), representing a compound anomaly pattern in body-wall defects. The diagnosis of this entity is based on two of the three following characteristics: (1) exencephaly/encephalocele and facial clefts; (2) thoraco- and/or abdominoschisis; and (3) limb defects. A definite association with internal anomalies and severe kyphoscoliosis makes a more distinct concept of the pathogenesis reasonable. Limb-body wall malformations result from a malfunction of the ectodermal placodes involving the early embryonic folding process. The poor prognosis of LBWC calls for early antenatal diagnosis.

Splenosis mimicking tumor recurrence in renal cell carcinoma: detection on selective spleen scintigraphy.

A 2-year-old boy had been operated on for a giant renal cell carcinoma including splenectomy because of disrupture of the splenic capsule. During a follow-up examination, 3 nodules were detected by ultrasound in the splenorenal area. This gave reason to suspect tumor recurrence. Considering the possibility of splenosis, a selective spleen scan using denatured red blood cells was performed as a final diagnostic step. This method confirmed the nodules as representing splenic tissue. Splenosis should be included in the differential diagnosis of solid masses in the postsplenectomy patient.

Proctocolitis in breast fed infants: a contribution to differential diagnosis of haematochezia in early childhood.

Dietary protein induced proctocolitis in exclusively breast fed infants is rarely taken into consideration as a cause of rectal bleeding or blood streaked stool in the neonatal period and early infancy. Eleven babies are presented in whom it is believed that bleeding through the rectum was due to proctocolitis as a result of allergy triggered by cows' milk protein transferred to the infants via the breast milk. Colonoscopy was performed in five infants, revealing benign eosinophilic proctocolitis. Standard treatment was the exclusion of the allergen from the mother's diet. Resolution of visible rectal bleeding took place within 72 to 96 hours after elimination of the offending protein from the mother's diet.

Intraabdominal extralobar pulmonary sequestration exhibiting cystic adenomatoid malformation: prenatal diagnosis and characterization of a left suprarenal mass in the newborn.

An intraabdominal extrathoracic pulmonary sequestration (IEPS) was detected by prenatal ultrasound in a fetus of 19 weeks' gestation. The well-defined echogenic mass, including multiple cystic areas, was located in the left suprarenal region. Knowledge of the characteristic ultrasound appearance helped to differentiate between neuroblastoma and IEPS before surgical treatment. Histologic examination showed an association between IEPS and features of cystic adenomatoid malformation type 2.