Indications and outcomes of duodenum-preserving resection of the pancreatic head in children.

AIM OF STUDY: Duodenum-preserving resection of the pancreatic head (DPRPH) with Roux-en-Y pancreatojejunostomy is a procedure used to remove focal pathological lesions of the pancreatic head. Although predominantly used in adult patients, it is both safe and effective in children. The aim of this study was to review our experience with this procedure, with focus on its indications, complications and long-term outcomes. METHODS: A retrospective analysis of pediatric patients who underwent DPRPH between 1994 and 2015 was performed. Patient files were reviewed for demographic, diagnostic, operative and histological details, postoperative complications. Patients were contacted telephonically and sent questionnaires to determine long-term outcomes. RESULTS: The study cohort consists of 21 patients, 14 girls and 7 boys, with an average age of 11.72 years (range 3 months to 18.6 years), who underwent DPRPH with end-to-end anastomosis of the jejunum to the pancreatic body (Roux-en-Y anastomosis). In four cases the head and also part of the body of the pancreas was resected. In the remaining 17 cases, only the head of the pancreas was resected. Indications for DPRPH were solid pseudopapillary tumor of the pancreas (n = 10), trauma (n = 8), pancreas divisum (n = 1), focal congenital hyperinsulinism (n = 1) and pancreatic cyst (n = 1). The length of follow-up ranged from 1 to 22 years (average 9.66). One patient developed a biliary fistula, which closed spontaneously within 2 weeks after stent insertion. A recurrence of abdominal pain was reported in two patients, occurring at 7 months after the operation in one patient and at 1 year in the other. Pancreatic endocrine insufficiency did not occur in any of the 21 patients. Seven patients currently require a low fat diet, five of which need pancreatic enzyme supplementation. An additional two patients need enzyme supplementation without dietary restriction. CONCLUSION: DPRPH is a safe and effective procedure for the treatment of large focal pathological lesions of the pancreatic head in children. As a less invasive procedure than pancreatoduodenectomy, it is more appropriate for the developing child.

Medically induced CSF rhinorrhea following treatment of macroprolactinoma: case series and literature review.

PURPOSE: Although several reports have addressed cerebrospinal fluid (CSF) rhinorrhea following dopamine agonist (DA) therapy of macroprolactinomas, further study is warranted for this relatively uncommon entity. Toward this aim, our retrospective series and review of literature further clarifies recommendations in treatment of this rare problem. METHODS: We retrospectively reviewed all macroprolactinoma cases in our hospital for a 15-year period. Our systematic search of PubMed identified original articles and reviews of all macroprolactinoma cases with an associated medication-induced CSF leak. RESULTS: Five patients with drug-induced CSF leak were identified; four of these patients received cabergoline therapy an average of 6 weeks before the onset of rhinorrhea and then underwent surgical repair of the CSF leak. Of 35 published studies included, we identified 60 patients with medication-induced CSF leak. Medical therapy included bromocriptine in 34 patients, cabergoline in 21 patients, and use of both DAs in two patients. Three cases did include complete diagnostic and treatment data. Median time from initiation of the DA treatment to occurrence of rhinorrhea was 6 weeks. For CSF rhinorrhea, 49 patients underwent surgical repair (38 by the transnasal approach) and seven patients were treated nonoperatively. CONCLUSION: Baseline skull base erosion in macroprolactinomas in combination with subsequent tumor shrinkage induced by DA therapy may result in spontaneous CSF rhinorrhea. Therefore, such patients should be advised about and monitored for this potential setback. Once CSF leak is diagnosed, prompt treatment must be carried out to avoid infectious complications. Transnasal surgery appears the most effective therapeutic approach.

Pancreas Divisum in Children and Duodenum-Preserving Resection of the Pancreatic Head.

INTRODUCTION: A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives in children with pancreas divisum (PD). MATERIALS AND METHODS: Patients who underwent treatment for PD between 1999 and 2014 at our department were evaluated for sex, age, presenting symptoms, physical examination findings, biochemical markers, diagnostic methods, treatment modalities, and results of treatment during follow-up. RESULTS: Seven patients who underwent treatment of symptomatic PD were included in the study. The median for follow-up period was 8 years (from 26 months to 16 years). Male-to-female ratio was 4:3 and the median age at presentation was 11 years (2-14 years). Presenting symptoms were recurrent episodic epigastric pain. Pancreatitis was documented by elevated amylase or lipase levels. Endoscopic retrograde cholangiopancreatography (ERCP) was the method of diagnosis of PD in all patients. Five patients had complete PD and two had incomplete variants. Three patients improved after ERCP papillotomy. In three patients, papillotomy was unsuccessful but they have only mild episodes of pancreatitis. One patient presented at the age of 4 years with recurrent pancreatitis. She was treated surgically by duodenum-preserving resection of the pancreatic head (DPRPH) because of severe recurrent pancreatitis occurring even after ERCP papillotomy. The patient is 26 months after operation without any reported problems. CONCLUSION: Patients with symptomatic PD are indicated for ERCP papillotomy attempt. If there is not improvement after ERCP, then recurrent bouts of severe pancreatitis are considered as an indication for surgical procedure. DPRPH is a safe and feasible surgical alternative.

Endogenous Heparinoids May Cause Bleeding in Mucor Infection and can be Detected by Nonactivated Thromboelastometry and Treated by Recombinant Activated Factor VII: A Case Report.

Mucormycosis is an aggressive fungal infection, which invades endothelial cells of blood vessels. This condition might lead to destruction of endothelium and release of heparin-like substances to the bloodstream and cause life-threatening bleeding, which is not well described in the literature.We present a patient with mucormycosis who experienced life-threatening bleeding, although no standard laboratory test could detect any coagulopathy.The cause of bleeding-coagulopathy was detected only by nonactivated thromboelastometry (NATEM), which revealed the presence of heparin-like substances. After treatment with recombinant activated FVII rotational thromboelastometry, results improved and the patient stopped bleeding. Regular application of the drug was necessary during acute phase of infection to prevent further bleeding.In this case report, we show that NATEM can detect the presence of heparin-like substances in bleeding patient with mucormycosis infection and that recombinant activated FVII can be used to stop and prevent bleeding until infection resolves.

Dopamine 2 receptor expression in various pathological types of clinically non-functioning pituitary adenomas.

Clinically non-functioning pituitary adenomas account for about one-third of pituitary tumors. The majority of them are pathologically classified as gonadotropinomas or null-cell adenomas without hormonal expression. The rest represent silent corticotroph adenomas and plurihormonal tumors. Conservative therapy with dopamine agonists is effective in some cases only depending on the expression of dopamine 2 receptors (D2R). The aim of this study was to quantitatively estimate D2R expression in clinically non-functioning pituitary adenomas and correlate the results with adenoma type according to pathological classification. Out of the 87 adenomas investigated, 63 expressed gonadotropins, 7 were silent corticotroph adenomas, 7 were plurihormonal tumors, and only 6 did not express any pituitary hormone on immunohistochemical investigation. With the use of the reverse transcriptase PCR technique, D2R mRNA was expressed in all adenomas with very heterogeneous quantity. The expression was very low in corticotroph adenomas (relative median quantity after normalization to housekeeping gene 0.01) and lower in plurihormonal tumors (median 0.4) than in gonadotroph (median 1.3) and null-cell adenomas (median 1.9). The difference between corticotroph adenomas and plurihormonal tumors in comparison with other pathological types was statistically significant. The expression of D2R did not depend on the presence or absence of gonadotropins. We conclude that D2R expression is very low in corticotroph adenomas and significantly lower in plurihormonal tumors. The positivity of gonadotropins does not predict the D2R quantity.

[Indication and radicality of surgical treatment in spinal myeloma]. / Indikace a radikalita chirurgické lécby myelomu v páteri.

In our set of 19 operated patients with spinal myeloma it was in 17 patients (89%) the first manifestation of malignant haematological disease. Indication for admission for surgery were in 18 patients (92%) graphical signs of spinal compression of osteolytic affliction of the vertebra of unclear aetiology, which in 17 (63%) of patients were demonstrated by neurological deficit. On the basis of graphical examinations the most frequent suspicion was on metastatic affliction of the spine at unknown primary focus and the basic diagnosis was determined after the operation at most of cases. In decision about the choice of radicalness of surgery intervention important data about the type of tumour in the spine were usually missing and it especially did not make possible to estimate the life expectation of the patient. When deciding about the radicalness of the surgery, we started from the degree of spinal compression and surgical character of pathological process in the spine. The aim of the operation is to decompress the spinal cord in tumour and to stabilize the afflicted part of the spine. Radical extirpation of tumour followed with stabilization and spine reconstruction we decided to carry out in 7 patients, i.e. 37%, all of them clinically improved and from this group only 2 patients (29%) died till now. We carried out only palliative surgery on the spine in next 12 patients, i.e. in 63%, only 6 patients improved neurologically, i.e. 50% and 10 patients, i.e. 83% died till now.