[Multidisciplinary approach to surgical disorders of the pancreas in children]. / Multidisciplinární prístup k chirurgickému onemocnení pankreatu v detském veku.

INTRODUCTION: Surgical diseases of the pancreas in children are not common and may be associated with significant morbidity and potential mortality. A multidisciplinary approach is essential for correct diagnosis, surgical strategy and postoperative as well as follow-up care. METHOD: Retrospective analysis of patients operated on due to a pathological lesion of the pancreas focused on diagnostics, operating procedures, postoperative complications, and long-term results. Between 1991 and 2016, eighty-nine children were treated in our department for a pathologic lesion of the pancreas. 39 of them were boys and 50 were girls. RESULTS: Mean age of the patients was 9.3 years (1 month-18.4 years). Patients were followed from the operation to the age of 19, after which they were referred for follow-up to adult specialists. The indications for surgery were trauma in 34 children, solid pseudopapillary tumor in 23 children, biopsy in 10, hyperinsulinism in 8, chronic pancreatitis in 4, pancreatic cyst in 3, insulinoma in 3, carcinoma in 2, and serous cystadenoma and pancreas divisum in one patient. The most frequent procedures performed on the pancreas were distal pancreatectomy in 35 cases, the duodenum-preserving pancreatic head resection in 23 cases, pseudocystogastroanastomosis in 11 cases, 9095% pancreatic resection in 5 cases, Whipple operation in two cases, Puestow procedure in one case, tumor enucleation in one case, and tumor biopsy for cancer in one case. In 5 patients after major pancreatic injury, ERCP and papillotomy with insertion of a stent into the pancreatic duct was performed. 3 patients died, one after a polytrauma with severe pancreatic injury and two patients with pancreatic cancer. CONCLUSION: Pancreatic surgery in children is not a common operation, and individual as well as institutional experience remains limited. After more than 20 years of experience with pancreatic surgery, we believe that close cooperation with surgeons, pediatric gastroenterologists, radiologists, anesthesiologists, intensivist, pathologists and ERCP specialists is necessary for successful diagnosis and treatment of pancreatic disease in children.Key words: pancreas pancreatic surgery in children duodenum preserving head resection of the pancreas.

Extremely hypotrophic newborn of mother with systemic lupus erythematosus and antiphospholipid syndrome.

The case presented describes a high-risk pregnancy of a woman with systemic lupus erythematosus (SLE) with multiple lesions of central nervous system (CNS), vasculitis, secondary epilepsy and antiphospholipid syndrome (APS). At gestational age 28 weeks and 3 days the pregnancy was urgently terminated via caesarean section and an extremely hypotrophic immature newborn with a birth weight of 580 g was born. The high disease activity in the mother at the time of conception and the histologically proven chronic placental insufficiency due to APS are presumably the causes for the extensive hypotrophy of the neonate. The significant comorbidity of the newborn, including respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, osteopathy of prematurity, transient hypothyroidism and hypocortisolism, vesicoureteral reflux, and hypertonic-hyperexcitation syndrome complicated his three-month stay in NICU. A positive titre of transplacentally transferred anticardiolipin and anti-ß2 glycoprotein antibody was detected in the child and persisted through the following 30 months. During the three-year follow-up, significantly delayed neuropsychological development with microcephaly (-4 SD) and short stature of the child was observed. Finally, the authors discuss possible causes of neuropsychological consequences in children of mothers with SLE and APS and emphasize the need for long-term monitoring and specialized care to improve development of these children.

Occurrence of selected antiphospholipid antibodies in offspring born from mothers with autoimmunity.

Autoimmune diseases can be accompanied by presence of various antiphospholipid antibodies (aPL). The laboratory criteria of antiphospholipid syndrome are based on detection of anticardiolipin, lupus anticoagulant or to antiß2- glycoprotein I but currently a significance of other multiple aPL is being discussed. Because of their vascular and neuroinflammatory effect aPL, if being transplacentally transferred, might inflict damage in developing organism. The aim of our study was to determine the occurrence of eight selected aPL in offspring of mothers with proven autoimmune disease with aPL positivity. The possible influence of aPL presence on clinical, ultrasound and laboratory outcome of children was observed as well. The prospective study included 38 women: 17 women with primary antiphospholipid syndrome and 21 women with other diagnosed autoimmune disease with detected aPL. Also included were 39 children born to the above mentioned mothers between January 2009 and April 2010 in Perinatology Centre in Pilsen, Czech Republic. The control group consisted of 30 mothers without AD and their 30 healthy singletons. Preliminary results of the study showed the presence of aPL in 42.1% neonates of aPL positive mothers with autoimmune disease, six month later aPL were present in only 37.5 % of these children. Observed occurrence of aPL positivity at 6 months of age in originally negative offspring could be attributed to vaccination or food exposure. Psychomotor development of children has proceeded without major deviations. The follow-up study continues and will evaluate both groups of children at two years of age.

Transvenous Removal of Glue from Obstructed Straight Sinus during Transarterial Embolization.

SUMMARY: Inadvertent embolization of the veins that drain arteriovenous intracranial shunts is likely to precipitate passive hyperemia with subsequent hemorrhage and brain edema. The mainstay of therapy for this complication is to decrease the flow through the shunt as much as possible and maintain the patient's blood pressure below baseline. We herein report our successful attempts at glue removal from partially obstructed straight sinus in two cases using a microsnare. This complication occurred during intracranial arteriovenous shunt embolization.

[Visual function in hypophyseal tumors]. / Zrakové funkce u hypofyzárních tumoru.

Visual acuity measured using the Snellen method in 29 patients with hypophyseal tumors amounts to normal or nearly normal values (6/9-6/6), with mean value of 0.75. Measuring of visual acuity with the more accurate "log MAR" optotype tables yields acuity values which decline with the severity of the disease from 1.04 to 0.87 with a mean value of 0.97. Not withstanding, in all of these patients markedly lowered contrast sensitivity values were found in all of the frequencies tested (1.15-29.25 c/deg). The use of the visual acuity determinations only is insufficient for early detection of functional visual disturbances in compressions of the visual pathway.

Epilepsy associated with low-grade brain glial neoplasms.

In 1991-1993, 52 patients underwent surgery for low-grade supratentorial glioma. In 37 of them (astrocytoma 22, oligodentrocytoma 12, oligodendroglioma 2) seizures, often refractory to drug therapy, appeared as the first symptom. These cases were retrospectively analyzed. The patients had partial seizures: simple, complex, or secondarily generalized (preoperative duration: from 3 days to 17 years (mean 2 years); frequency: between 1 and 2/year and over 10/day). Neurological examination either revealed slight focal changes or was normal. Conventional craniotomy and resection of a tumor, without intraoperative electrocorticography, was performed. Partial resection was performed in 73%, subtotal in 5%, "total" in 22% of the cases. Postoperatively, 27 patients had focal radiotherapy, 3 of them in combination with chemotherapy. Two patients were reoperated. Out of 33 alive (89%), about two-thirds appear normal by neurological examination and are seizure-free at present (mean follow-up period 28 months). Most remain on antiepileptic drugs at lower doses. Histological and immunohistochemical analyses of resected tissue together suggest that the peripheral zone of cortical tumor infiltration may participate on epileptogenesis.

[Long-term results after surgical treatment of head injuries]. / Dlouhodobé výsledky po chirurgicky resených poraneních hlavy.

It outflows from a survey of 174 cases operated during five years for skull injuries that the resulting state depends of preoperative level of consciousness. In injuries with the loss of consciousness, there is a close correlation between the type of motor-topain response and the resulting state. It was stated from the delay for up to 5 years after surgery that the independence from the day life has been achieved in 65% of injured patients which experienced preoperatively a mild loss of consciousness as they localized the pain stimulus or produced unintelligible sounds in response to pain. The similar condition has been achieved in only 14% cases of preoperatively deep loss of consciousness, which responded to pain or not with decerebrative extension, or unconsciously flexion of extremities. The resulting state is less dependent of operative diagnosis. Epidural hematomas show better results, where the independence of the day life has been achieved in 62% of cases. The majority of them was able to perform their previous working activities later on. In contrast, the worst results were achieved in association with brain contusions, where only 18% of injured patients sustained the independence of environs with no return to the previous work. Prognostically, the factor of age is the most important. In the age group over than 60 years, the mortality represented 58%, whereas in 20-40 years of age it was only 24%.

Fibrinolytic activity of human brain.

Authors studied the fibrinolytic human brain activity using the fibrin film in order to determine the location of tissue plasminogen activators. An amount of 20 human brains has been evaluated issued from cadavers with no signs of cerebral disease when alive. Most high concentration of plasminogen activators was evidenced in samples from plexus chorioideus and pia mater, while the fibrinolytic activity of grey and white matter showed uniformly low values. Large amount of plasminogen activators may be released into cerebro-spinal fluid, what can lead to a rapid fibrin clot dissolution around the aneurysm, so the rebleed may be consequential to it.