Neuroendocrine tumor of the pancreas and bilateral adrenal pheochromocytomas. A rare manifestation of von Hippel-Lindau disease in childhood

Background

A 12-year-old girl presenting with intermittent epigastric pains and diarrhea was referred to our clinic. Diagnostic workup revealed nonfunctional bilateral adrenal pheochromocytomas as well as a neuroendocrine tumor of the pancreatic head. This is the first report on the combination of a neuroendocrine pancreatic tumor with adrenal pheochromocytoma in a pediatric patient with von Hippel-Lindau (VHL) disease.

Methods

von Hippel-Lindau disease was confirmed by molecular genetic analysis of peripheral blood lymphocytes, which revealed the mutation VHL c. 695 G > A. The family history showed also VHL disease in the mother who carried the same mutation.

Results and Conclusion

Open laparotomy, organ-sparing enucleation of pheochromocytoma, and pylorus-preserving resection of the pancreatic head tumor were successfully performed. After an uneventful postoperative course, the child fully recovered. She was free of further manifestations of VHL disease 30 months after surgery.     

Conservative hemodynamic surgery for varicose veins

Conservative hemodynamic surgery for varicose veins is a minimally invasive, nonablative technique that preserves the saphenous vein and helps avoid excision of varicosities. It represents a physiologic approach to the surgical treatment of varicose veins based on knowledge of the underlying venous pathophysiology gained through detailed duplex scanning. A change in venous hemodynamics is attained through fragmentation of the blood column by interruption of the refluxing saphenous trunks, closure of the origin of the refluxing varicose branches, and preservation of the communicating veins that drain the incompetent varicose veins into the deep venous system. After surgery, varicose veins regress through a reduction in hydrostatic pressure and efficient emptying of the superficial system by the musculo-venous pump. Obvious advantages of this technique are that it is done in an ambulatory setting, minimizes the risk of surgical complications, and permits a rapid return to full activity. The long-term hemodynamic improvement and recurrence rate of this technique remain to be established.     

Early antibiotic treatment for severe acute necrotizing pancreatitis: a randomized, double-blind, placebo-controlled study

BACKGROUND & AIMS: In patients with severe, necrotizing pancreatitis, it is common to administer early, broad-spectrum antibiotics, often a carbapenem, in the hope of reducing the incidence of pancreatic and peripancreatic infections, although the benefits of doing so have not been proved. METHODS: A multicenter, prospective, double-blind, placebo-controlled randomized study set in 32 centers within North America and Europe. Participants: One hundred patients with clinically severe, confirmed necrotizing pancreatitis: 50 received meropenem and 50 received placebo. Interventions: Meropenem (1 g intravenously every 8 hours) or placebo within 5 days of the onset of symptoms for 7 to 21 days. Main Outcome Measures: Primary endpoint: development of pancreatic or peripancreatic infection within 42 days following randomization. Other endpoints: time between onset of pancreatitis and the development of pancreatic or peripancreatic infection; all-cause mortality; requirement for surgical intervention; development of nonpancreatic infections within 42 days following randomization. RESULTS: Pancreatic or peripancreatic infections developed in 18% (9 of 50) of patients in the meropenem group compared with 12% (6 of 50) in the placebo group (P = 0.401). Overall mortality rate was 20% (10 of 50) in the meropenem group and 18% (9 of 50) in the placebo group (P = 0.799). Surgical intervention was required in 26% (13 of 50) and 20% (10 of 50) of the meropenem and placebo groups, respectively (P = 0.476). CONCLUSIONS: This study demonstrated no statistically significant difference between the treatment groups for pancreatic or peripancreatic infection, mortality, or requirement for surgical intervention, and did not support early prophylactic antimicrobial use in patients with severe acute necrotizing pancreatitis.     

The 16 golden hours for conservative treatment in children with postoperative small bowel obstruction

Background

Conservative treatment of postoperative small bowel obstruction in children is widely accepted, provided that there are no clinical signs of bowel strangulation. However, the length of time surgery can be safely deferred remains unclear.

Purpose

The aim of this study was to determine the time limit for observant management of postoperative small bowel obstruction.

Methods

The medical records of 128 children with 174 episodes of postoperative small bowel obstruction were reviewed.

Results

Spontaneous resolution occurred in 63% of cases, 85% within 48 hours of admission. Bowel compromise was noted in 31% of the operated cases, and about half of them underwent bowel resection, accounting for 6% of all admissions. None of the cases treated surgically within 16 hours of admission was associated with bowel strangulation or need for resection.

Conclusions

In children presenting with postoperative small bowel obstruction, prolonging observant treatment for more than 48 hours yields only a small benefit in terms of spontaneous resolution. Bowel strangulation can occur within 16 hours of admission. On the basis of these findings, we suggest raising the index of suspicion for compromised bowel after 16 hours and making the decision for surgery at around 48 hours.     

Pediatric renovascular hypertension: 132 primary and 30 secondary operations in 97 children

PURPOSE: This study was undertaken to characterize the contemporary surgical treatment of pediatric renovascular hypertension. METHODS: A retrospective analysis was conducted of the clinical data of 97 consecutive pediatric patients (39 girls, 58 boys), aged from 3 months to 17 years, who underwent operation at the University of Michigan from 1963 to 2006. All but one patient had refractory hypertension not responsive to contemporary medical therapy. Developmental renal artery stenoses accounted for 80% of the renal artery disease, with inflammatory and other ill-defined stenoses encountered less frequently. Splanchnic arterial occlusive lesions affected 24% and abdominal aortic coarctations, 33%. RESULTS: Primary renal artery operations were undertaken 132 times. Procedures included resection beyond the stenosis and implantation into the aorta in 49, renal artery in 7, or superior mesenteric artery in 3; aortorenal and iliorenal bypasses with vein or iliac artery grafts in 40; focal arterioplasty in 10; resection with reanastomosis in 4; operative dilation in 4; splenorenal bypass in 2; and primary nephrectomy in 13 when arterial reconstructions proved impossible. Bilateral renal operations were done in 34 children, and 17 underwent celiac or superior mesenteric arterial reconstructions, including 15 at the time of the renal operation. Thirty patients underwent abdominal aortic reconstructions with patch aortoplasty (n = 19) or thoracoabdominal bypass (n = 11). Twenty-five of the aortic procedures were performed coincidently with the renal operations. Thirty secondary renal artery procedures were done in 19 patients, including nine nephrectomies. Hypertension was cured in 68 children (70%), improved in 26 (27%), and was unchanged in three (3%). Follow-up averaged 4.2 years. No patients required dialysis, and there were no operative deaths. CONCLUSION: Contemporary surgical treatment of pediatric renovascular hypertension emphasizes direct aortic implantation of the normal renal artery beyond its stenosis and single-staged concomitant splanchnic and aortic reconstructions when necessary. Benefits accompany carefully executed operative procedures in 97% of these children.     

Epidemiology of osteoporotic hip fractures in Spain

We conducted a multicentre study, divided into a retrospective and a prospective portion. The retrospective study evaluated osteoporotic hip fractures that occurred during 2002. The prospective study evaluated osteoporotic hip fractures that occurred during May 2003. The study was conducted in 77 hospitals in Spain and comprised patients 60 years of age and over. In the retrospective study we registered 13,195 hip fractures. Of the patients, 74% were women and 26% were men. The mean age was 80.7+/-8.4 years. The average incidence was 6.94+/-0.44 hip fractures per 1,000 inhabitants/year (95% CI, 6.07-7.82). In the prospective study, we registered 1,399 hip fractures. This represents a monthly incidence of 0.60+/-0.04 hip fractures per 1,000 inhabitants/year (95% CI, 0.51-0.69). Of the subjects, 74% were women and 26% were men. The mean age was 81.4+/-8.1 years. Using these data, we calculated the average annual prevalence in 2003 to be 7.20 fractures per 1,000 inhabitants. Thirty-three percent had previously suffered a hip fracture. Prior to the fracture, only 18% had received medical treatment for osteoporosis. After discharge from the hospital, only 26% were receiving pharmacological treatment for osteoporosis.     

Endoscopic endonasal approach to the ethmoidal planum: anatomic study

The endoscopic endonasal technique is currently used by otolaryngologists for the management of different extradural lesions located below the ethmoidal planum. The cooperation between ENTs and neurosurgeons has recently pushed the use of such approach also in the removal of some intradural lesions, which has promoted the interest for an anatomic study to identify the anatomical landmarks and the dangerous points during the endoscopic approach to this area. In six fresh cadaver heads, unilateral and bilateral measurements between the main landmarks of the approach were performed by means of an endoscopic endonasal approach. A wide exposure of the midline anterior skull base was realized. The maximum of lateral extension was obtained between the two medial orbital walls, at the middle of the cribriform plate (mean distance 25,33 mm), while the mean distance between the anterior and posterior ethmoidal arteries at the level of the lamina papyracea was 16 mm. The endoscopic endonasal route can be considered a minimally invasive technique to approach the ethmoidal planum. It requires adequate anatomical knowledge and endoscopic skill for its realization. Due to the wide window realizable through this corridor, it could be considered in selected cases for the removal of intradural lesions such as meningiomas or estesioneuroblastomas.     

Avoiding Axillary Treatment in Sentinel Lymph Node Micrometastases of Breast Cancer: A Prospective Analysis of Axillary or Distant Recurrence

Background

The need for axillary lymph node dissection (ALND) in breast cancer patients with sentinel lymph node (SLN) micrometastases remains controversial. The aims of the study were to evaluate the locoregional failure and outcome of breast cancer patients with sentinel node micrometastases who did not undergo completion ALND.

Methods

Between November 2000 and December 2006, SLN biopsy was successfully performed in 1178 patients with invasive breast carcinoma. Only patients with macrometastasis (>2 mm) underwent ALND, while patients with negative SLN or micrometastases did not undergo further treatment of the axilla, by either surgery or radiotherapy. Regarding adjuvant therapy decision, patients with SLN-micrometastases (pN1_mi) were considered as node-positive patients.

Results

Of 1,178 patients, 59 (5%) had micrometastases. Of those with micrometastases, 14 (24%) underwent ALND because the intraoperative study of the SLN yielded a positive result. With a median follow-up of 60 (range, 8–94) months, none of the patients with SLN micrometastases in whom ALND was omitted developed an axillary recurrence, while one patient in whom ALND was performed developed infraclavicular lymph node recurrence. One patient, who declined postoperative breast irradiation, developed breast recurrence and distant metastasis.

Conclusions

Breast cancer patients with SLN micrometastases in whom ALND was omitted had a very low locoregional failure rate. This study supports the theory that ALND might be avoided in these patients, providing that adjuvant systemic treatment equal to treatment provided to treat node-positive disease is administered. However, longer follow-up and results of additional prospective studies are needed.     

Clinical outcome at ten years after laparoscopic fundoplication: Nissen versus Toupet

Laparoscopic surgery has become the elective approach for the surgical treatment of gastroesophageal reflux disease in the last decade. Outcome data beyond 10 years are available for open fundoplication, with good-to-excellent results, but few studies report long-term follow-up after laparoscopic fundoplication. We performed a retrospective study of all the patients that underwent laparoscopic Nissen and Toupet fundoplications as antireflux surgery between 1995 and 1998 in our institution. To evaluate the long-term results, a face-to-face interview was performed in 2009. One hundred and six patients were included in the study. Surgical techniques performed were Nissen fundoplication (NF) in 56 patients and Toupet (TF) in 50. Complication rate was 4 per cent in both groups (nonsignificant [NS]). Two patients (4%) of NF required reoperation because of dysphagia. After 10 years, 10 per cent of the patients remain symptomatic in both groups. Fifteen per cent of NF take daily inhibitors of the proton pump versus 14 per cent of TF (NS). Twenty per cent of NF refer dysphagia, all of them without evidence of stenosis at endoscopy or contrasted studies. The satisfaction rate of the patients was 96 per cent in NF and 98 per cent in TF. Laparoscopic Toupet fundoplication seems to be as safe and long-term effective as Nissen, but with a lower incidence of postoperative dysphagia. In our experience Toupet fundoplication should be the elective approach for the surgical treatment of gastroesophageal reflux disease.