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.

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.

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.

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).

[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.

Fatal neonatal Salmonella enteritidis sepsis.

A case history of a fatal neonatal infection caused by Salmonella enteritidis group D is reported. The baby deteriorated rapidly at 24 hours after birth with clinical signs and symptoms of an acute abdomen. Bloody diarrhea led to a tentative diagnosis of midgut volvulus or necrotizing enterocolitis. Autopsy and bacteriologic investigation revealed sepsis by S. enteritidis group D. The same organism was found in cultures taken from stool and vaginal swabs from the mother. This clearly confirmed transmission of the infection during delivery.

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.

Transient pseudohypoaldosteronism in obstructive renal disease.

The cases of two patients with transient pseudohypoaldosteronism due to an up to this point unrecognized obstructive renal disease are reported. Both girls presented with a severe salt-losing episode in early infancy mimicking congenital adrenal hyperplasia. Extensive endocrinologic work-up revealed markedly elevated plasma-aldosterone levels. Clinical and laboratory data were consistent with transient pseudohypoaldosteronism. Sonographic and radiological investigation showed in both children a vesicoureteral reflux of differing grades. After therapy of the electrolyte-imbalance and recovery, one of the children required surgical treatment of vesicoureteral reflux.

BCG osteomyelitis as a rare cause of mediastinal tumor in a one-year-old child.

A one-year-old child with BCG osteomyelitis of the upper spine is reported. The child was admitted because of viral infections of the upper respiratory tract. A lateral x-ray examination of the lung showed what was thought to be a mediastinal tumor. The preoperative diagnostic problems are discussed, because the etiology in our case was unknown until the histological report arrived. The knowledge of the complication, of osteomyelitis after vaccination, is small although the first case was published more than 40 years ago. Since then about 300 cases have been reported in the literature. The disease is seen in the first five years of life in otherwise healthy children. Most cases are localized near the epiphysis of the long bones. A localization in the spine, as described in our case, has been published only three times before. The highest frequency of BCG osteomyelitis is reported in Finland and Sweden with about 40 cases per 1 million vaccinations.

Short-bowel syndrome associated with subtotal necrosis of small intestine after rectal trauma.

We report on a 4-year-old girl who experienced rectal trauma during swimming, sitting on an uncovered draining valve in the swimming pool. This resulted in a powerful suction effect on her rectum, followed by rupture of the sigmoid colon and evisceration of the small intestine. Laparotomy showed a near complete necrosis of the small bowel because of thrombotic lesions and wall lacerations of the superior mesenteric artery (SMA). A subtotal bowel removal associated with a jejuno-ileostoma was carried out, a total length of about 35 cm of the small intestine could be left in situ. Parenteral nutrition was stopped after eight months. At the moment defecation takes place 2-3 times a day, growth and weight gain are quite normal.

[Josef Weinlechner (1829-1906). Pioneer in Vienna pediatric surgery]. / Josef Weinlechner (1829-1906). Wegbereiter der Wiener Kinderchirurgie.

The history of paediatric surgery in Vienna has not been clearly documented so far, especially its development during the second half of the nineteenth century. Josef Weinlechner (1829-1906) is one of the outstanding paediatric surgeons in Vienna during this time. We present an overview of his biography, his position as head of the department of surgery at the St. Anna Children's Hospital, and also discuss his publications concerning various aspects of paediatric surgery. Of particular interest was the discovery of his application for the qualification of a postdoctoral lecturer ("Habilitation") in the archives of the University of Vienna. In this application, Weinlechner refers to his specialisation as that of a paediatric surgeon.

[Congenital oesophageal stenosis due to tracheobronchial remnants]. / Congenitale Oesophagusstenose durch tracheobronchiale Reste.

This paper presents the authors' experience with congenital oesophageal stenosis (= CES) in three patients who had CES due to tracheobronchial remnants. Difficulties with feeding started at 6-9 months of age, which corresponded with the introduction of solids. Contrast studies showed stenosis at the junction of the mid and distal thirds of the oesophagus. Limited resection of the oesophageal stenosis and primary anastomosis was performed in all three patients with good results. CES should be suspected if all other causes of oesophageal stenosis have been excluded by upper gastrointestinal examinations, endoscopy, oesophageal pH monitoring and oesophageal manometry.

Surgical treatment of bilateral Wilms' tumours with special reference to second operations in metachronous disease.

We report on six patients with bilateral Wilms' tumours (among them one pair of siblings) who underwent surgery within a period of 20 years. Wilms' tumours appeared synchronously in three patients and successively in the other three. Case reports are given for three of the six patients. The tumours have a remarkable multilocular appearance, indicating a multilocular genesis. In this context, the phenomenon of nephroblastomatosis is discussed. In addition to surgical treatment, aggressive conservative therapy should be employed, particularly with metachronous disease and the resulting acquired solitary kidney in these patients.