Wolfgang Pumberger

Volvulus without malposition—a single-center experience

BACKGROUND This is a single-center case series about the rare condition of volvulus without malposition and/or malrotation (VWM) in preterm babies. We focus on diagnostic difficulties, and our results should help to distinguish VWM as a distinct entity different from classical volvulus and segmental volvulus. MATERIALS AND METHODS Medical chart review of infants with VWM from 2003-2012 was used. RESULTS A total of 15 patients were identified. All of them had volvulus in the absence of intestinal malposition or other associated intestinal pathologies. All patients were born prematurely. Emergency laparotomy was necessary in all 15 patients. Two groups were identified. Group 1 includes four patients with typical signs of meconium obstruction of prematurity (MOP). Small bowel resection was only necessary in one of these four patients, all survived without residual intestinal lesions. Group 2 consists of 11 patients without signs of MOP-small bowel resection and temporary enterostomy were necessary in all these children. Four patients presented with pneumatosis intestinalis on the abdominal plain film, suggesting necrotizing enterocolitis. Although two infants died, the survivors showed complete recovery. CONCLUSIONS VWM is a distinct disease of prematurity. When associated with MOP, VWM has a favorable outcome of treatment. In contrast, VWM occurring in the absence of signs of meconium obstruction requires small bowel resection. VWM primarily affects the top of the midgut (ileum). Because of absent malposition, presentation of VWM may be uncharacteristic. Pneumatosis intestinalis in advanced VWM may lead to diagnostic difficulties and a delay in treatment.

Status epilepticus, cardiac resuscitation, and posterior reversible encephalopathy syndrome after ingestion of viscous lidocaine: a plea for more childproof packaging of pharmaceuticals.

Abstract Ingestion of viscous lidocaine in children can lead to potentially lethal neurologic and cardiac effects. We report the case of a 2-year-old boy who developed posterior reversible encephalopathy syndrome 2 days after unobserved ingestion of about 500 mg viscous lidocaine (40 mg/kg of bodyweight). Initially, the child presented with convulsive status epilepticus and subsequent cardiac arrest necessitating cardiopulmonary resuscitation for eight minutes. After 2 days of full recovery, the child presented with progressive disorientation, dizziness, and visual neglect. Lasting for 2 days, these symptoms finally disappeared completely. Combined with the findings on cerebral magnetic resonance imaging, this episode was interpreted as posterior reversible encephalopathy syndrome. Two weeks after the ingestion, no neurologic and visual abnormalities were found. Viscous lidocaine is prescribed routinely for dentition or other painful lesions in the oral cavity in children. Despite the potential hazardousness of the drug, packaging of viscous lidocaine is not childproof. Therefore, physicians have to instruct the parents carefully to minimize the risk of overuse or accidental ingestion. In general, the use of viscous lidocaine should be limited.

Perinatal neuroblastoma of the pancreas

Neuroblastoma is the most common solid tumor in infancy. Arising from the neural crest these tumors are usually located along the sympathetic chain from the neck to the pelvis and in the adrenal medulla. We report the case of a 3-week-old boy presenting with recurrent episodes of colicky pain. After ultrasound examination, magnetic resonance imaging and laboratory data a pancreatic neuroblastoma was suspected. Tumor resection via distal pancreatectomy and histologic investigation confirmed the diagnosis. Surgery is the treatment of choice in children with pancreatic masses, and is usually well tolerated even in the neonatal period. Conclusive diagnosis can be frequently established only by tumor resection or biopsy. Pancreatic neuroblastoma is an extremely rare tumor with only a few cases described in literature.

Haemoptysis in a teenager: late diagnosis of unnoticed foreign body aspiration

Chest X-ray in a 17-year-old boy, presenting with haemoptysis, revealed a radiopaque foreign body (FB) in the right lower lobe. There was no history of aspiration. CT located the needle-shaped FB in the right posterobasal lower lobe segment bronchus. In bronchoscopy, the FB turned out to be a pin, of which the radiolucent plastic head was embedded in the peribronchial tissue. Extraction by flexible and rigid bronchoscopy failed; finally, thoracotomy and bronchotomy had to be performed to remove the pin. In delayed diagnosis of a tracheobronchial FB, CT scan is not only necessary to localise the FB but also to depict or rule out secondary pulmonary changes. Nevertheless, radiolucent components of a metallic FB might be invisible even in CT, leading to underestimation of its size and extension. Late diagnosis complicates removal of tracheobronchial foreign bodies and may even necessitate open surgery, including pulmonary resections.

Recurrent pancreatitis due to an intraluminal duodenal diverticulum : report of a case

We report the investigation and treatment of a 14-year-old girl in whom a detailed assessment of recurrent episodes of pancreatitis revealed a large intraluminal ‘windsock’-like duodenal diverticulum. As the diverticulum was closely attached to the papilla of Vater, it was resected by a transduodenal approach. This report focuses on the significance of rare congenital anomalies of the duodenum (e.g., duplication cyst, intraluminal diverticulum) during the diagnostic workup of recurrent pancreatitis in children.

Cholelithiasis in children with CHD: is it a problem?

BACKGROUND An association of heart disease and its treatment with biliary calculi is popularly accepted. We sought determine the prevalence and risk factors of paediatric gallstone disease in the presence of CHD and analyse the treatment options. We evaluated the role of open-heart surgery in the development of gallstones in patients with CHD. Patients and methods In a 10-year, retrospective, chart review (2005-2014), patients with CHD and cholelithiasis were identified and reviewed. RESULTS In all, 19 of 4729 children with CHD had cholelithiasis (0.4%); eight patients underwent cardiac surgery before diagnosis of cholelithiasis (group 1), whereas 11 of them had not (group 2). The prevalence was 0.3% in group 1 and 0.5% in group 2. In nine asymptomatic patients, gallstones were found incidentally. Children with cholecystolithiasis (n=17) received ursodeoxycholic acid. A resolution of gallstones was found in four cases; two patients underwent biliary surgery, and the others (15/17) were successfully managed non-operatively. CONCLUSION Despite an accumulation of risk factors, prevalence of gallstones is not as high as expected in children with CHD. Open-heart surgery with a heart-lung machine plays a minor role as an aetiological factor. In about half of the cases, cholelithiasis is an incidental finding and patients stay asymptomatic. Prophylactic administration of ursodeoxycholic acid is not indicated in children undergoing open-heart surgery for CHDs. Biliary surgery is reserved for patients with recurrent symptoms or cholestasis. In children with CHD, cholelithiasis is a minor and manageable co-morbid condition.

Dysphagia due to a cystic mediastinal mass.

A previously healthy 2-year-old boy was referred because of progressive vomiting and diarrhea, which were initially interpreted as a gastrointestinal infection. The symptoms started about 10 days before the patient presented at our department and had worsened in the last two days. The boy developed progressive dysphagia which advanced to where he was unable to swallow his own saliva. Physical examination and laboratory investigations showed no abnormal findings. Specifically, no mass was found on examination of the abdomen. A contrast study of the esophagus showed a complete stop in the third portion of the esophagus. The latter revealed hyperperistalsis mimicking some kind of resistance (Fig. 1a, b). Because of a vague episode of foreign body impaction, we performed an endoscopic examination of the upper gastrointestinal tract under general anesthesia. The endoscopic investigation showed no signs of foreign body impaction but did reveal a submucosal tumor in the distal portion of the esophagus. A bluish, gleaming, soft swelling led to almost complete narrowing of the esophagus, with no visible ulceration of mucosa or communication with the lumen (Fig. 2a). Surprisingly, we were able to pass through the constriction with no difficulty. The tumor extended into the stomach and was seen as a swelling at the fundus (Fig. 2b). The rest of the stomach and the duodenum were normal. A subsequent ultrasound examination revealed a dumbbell-shaped cystic lesion in the patient’s right thoracic cavity, passing through the diaphragm and in close contact with the wall of the stomach (Fig. 3). The cyst was filled with hyperdense material and the distal esophagus could not be outlined. The mass had led to displacement of the vena cava inferior and the confluence of the liver veins. An MRI was performed to obtain more information about the mass and its relationship to adjacent structures. The investigation showed the cystic lesion at the right lower mediastinum, next to the spine, extending into the abdominal cavity with close contact between the cyst and the wall of the esophagus and the stomach (Fig. 4).

Acute abdomen due to splenic torsion.

A 14-year-old girl presented to our department for having collapsed after an acute episode of epigastric pain and vomiting. Past medical history was uneventful; in particular no previous abdominal complaints were mentioned. Physical examination showed a palpable tenderness in the epigastric region. Initially, gastroenteritis was suspected. Abdominal ultrasound revealed massive splenomegaly and in Doppler ultrasound splenic vein thrombosis was suspected. CT with CT angiography showed an enlarged, non-enhancing spleen with characteristic signs of splenic torsion (whirl …

Die Biegungsfraktur des Unterarmes im Kindesalter

Im Rahmen eines Unfallgeschehens sind Verletzungen der Knochen des Unterarmes bei Kindern sehr haufig. Der Fallende versucht reflexartig den Sturz mit der Hand abzufangen, um Verletzungen an Kopf, Thorax und Abdomen zu vermeiden. Der Knochen besitzt im Kindesalter gewisse Eigenheiten, die zu speziellen, im Erwachsenenalter nicht mehr vorkommenden Verletzungen fuhren. So stellen die offenen Epiphysenfugen des distalen Unterarms „Sollbruchstellen“ mit meist typischem Verletzungsmuster dar. Grunholzund Wulstfrakturen sind charakteristische Sonderformen von Knochenbruchen bei Kindern. Weniger bekannt sind Biegungsfrakturen der Unterarmknochen, im Englischen treffender als „plastic deformation“ bezeichnet. In Unkenntnis dieses Frakturtyps besteht die Gefahr des Ubersehens mit moglicherweise bleibenden Bewegungseinschrankungen fur das betroffene Kind.

Beitrag der Kinderchirurgie zur Therapie der Mukoviszidose im Neugeborenen- und frühen Säuglingsalter

Das Schwergewicht der Forschung auf dem Gebiet der Mukoviszidose (Cystische Fibrose = CF) hat sich in den letzten Jahrzehnten in den Bereich der Molekulargenetik und der Transplantationsmedizin (Lungentransplantation) verlagert. So wird der Beitrag der Kinderchirurgie im Rahmen der Th erapie der CF bisweilen unterschatzt. Ein kurzer historischer Uberblick mag die Bedeutung der Chirurgie vor allem in der Th erapie des Mekoniumileus (MI) unterstreichen. Bereits 1905 beschrieb K. Landsteiner einen Zusammenhang zwischen eingedicktem Mekonium beim Neugeborenen und Veranderungen des Pankreas. Er dachte, dass ein Enzymmangel zur Pankreasfi brose und zum pathologischen Mekonium fuhrt. G. Fanconi fuhrte als erster 1936 den Begriff der „Cystischen Fibrose“ ein und beschrieb die uns heute bekannte Beziehung zwischen Pankreasinsuffi zienz und chronischer Lungenerkrankung. Zwei Jahre spater stellte D. Anderson eine Beziehung zwischen CF und MI her. E. Glanzmann postulierte erstmals 1946, dass eine pathologische Zusammensetzung des Darmsekretes eine Obstruktion im terminalen Ileum und Kolon verursache und so fur die Entwicklung eines MI verantwortlich sei. Bis zum Jahre 1948 war die Prognose von Kindern mit MI trotz chirurgischer Th erapie schlecht. Erst RB Hiatt und PE Wilson beschrieben die ersten erfolgreichen Behandlungen eines MI, wobei im Laufe der folgenden Jahre unterschiedlichste operative Techniken Anwendung fanden. Ein weiterer Fortschritt war die Einfuhrung der konservativen Th erapie beim nicht komplizierten MI durch HR Noblett im Jahre 1969.