Study of acetylcholinesterase activity in rectal suction biopsy for diagnosis of intestinal dysganglionoses: 17-year experience of a single center

Although the utility of the acetylcholinesterase (AChE) histochemistry on rectal suction biopsy in diagnosing Hirschsprung`s disease (HD) has been documented, few reports address a great number of biopsies and patients. Our aim is to present a 17-year experience on the method of rectal suction biopsy and AChE histochemical staining for diagnosis of intestinal dysganglionoses. Between August 1989 and July 2006, 297 children suspected of having HD were submitted to rectal suction biopsies that were evaluated by the same two surgeons. There were 18 complications (6.0%), namely one self-limited rectal bleeding and 17 (5.7%) inadequate procedures that were repeated. A total of 157 patients (52.8%) showed no increased AChE activity and the remaining patients (140-47.2.0%) presented patterns of increased AChE activity confirming the diagnosis of HD or neuronal intestinal dysplasia. Among the 140 cases suspected as having HD, in 131 children the diagnosis of HD was confirmed and they were operated on. The histological studies showed that 111 children presented the classic form of HD or a long spastic segment. Sixteen children presented total colonic aganglionosis and four children proved to have intestinal neuronal dysplasia, according to histological and radiological criteria. Nine (6.6%) newborns were identified as false-positives and no false-negative results were verified. The rectal suction biopsy combined with AChE staining is advantageous for the differentiation between normal bowel and intestinal dysganglionoses. The rectal suction method is simple and can easily be performed by experienced surgeons. The histological evaluation is very objective and can be performed by a non-pathologist.

Fast-track rehabilitation for lung cancer lobectomy: a five-year experience

Objective: Fast-track rehabilitation is a group of simple measures that reduces morbidity, postoperative complication and accelerates postoperative rehabilitation reducing hospital stay. It can be applied to lung cancer lobectomy. Fast-track rehabilitation cornerstones are: minimally invasive surgical techniques using video-assisted and muscle sparring incisions, normovolemia, normothermia, good oxygenation, euglicemia, no unnecessary antibiotics, epidural patient-controlled analgesia, systemic opiods-free analgesia, early ambulation and oral feeding. Our objective is to describe a five-year experience with fast-track rehabilitation for lung cancer lobectomy. Patients and methods: A retrospective non-controlled study including 109 consecutive patients submitted to fast-track rehabilitation in the postoperative care of lung cancer lobectomy was performed. Only collaborative patients who could receive double-lumen intubation, epidural. catheters with patient-controlled analgesia, who had Karnofsky index of 100, previous normal feeding and ambulation, absence of morbid obesity, diabetes or asthma, were eligible. Postoperative oral feeding and aggressive ambulation started as soon as possible. Results: Immediate postoperative extubation even in the operation room was possible in 107 patients and oral feeding and ambulation were possible before the first hour in 101 patients. Six patients could not receive early oral feeding or ambulate due to hypnosis secondary to preoperative long effect benzodiazepines. Two patients could not ambulate immediately due to epidural catheter misplacement with important postoperative pain. Ninety-nine discharges occurred at the second postoperative day, four of them with a chest tube connected to a Heimlich valve due to air teak. No complication of early feeding and ambulation was observed. Postoperative hypnosis due to long duration benzodiazepines or pain does not allow early oral feeding or ambulation. Avoiding long duration preoperative benzodiazepines, immediate postoperative extubation, regional thoracic PCA and early oral feeding and ambulation were related to a lesser frequency of complication and a shorter hospital stay. Conclusion: Fast-track rehabilitation for lung cancer lobectomies can be safety performed in a selected group of patients if a motivated multidisciplinary group of professionals is available and seems to reduce postoperative complication and hospital stay. (C) 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

A 65-year experience treating acne, including 26 years with oral isotretinoin

This special article is based on the contents of a lecture given by Sampaio and was written together with Bagatin. it is a summary of the authors` experience treating various forms of acne before and after the introduction of oral isotretinoin, which revolutionized the management of the disease. Nowadays, this drug is considered the unique option to obtain a prolonged remission or cure of acne due to its action in all etiopathogenic factors involved in the disease. Therefore it should be prescribed in accordance to current recommendations, as well as for difficult cases, including those showing tendency to scars or serious psychological and social repercussions. Other topical and/or systemic therapeutic modalities should be considered in the initial approach and for mild and moderate cases.

Ten-Year Experience with Sevelamer and Calcium Salts as Phosphate Binders

Most patients with chronic kidney disease experience abnormalities in serum calcium, phosphorus, parathyroid hormone, and vitamin D metabolism. These can lead to vascular calcification (VC), which has been associated with increased risk for cardiovascular disease and mortality. Although hyperphosphatemia is believed to be a risk factor for mortality and VC, no randomized trial was ever designed to demonstrate that lowering phosphate reduces mortality. Nonetheless, binders have been used extensively, and the preponderance of evidence shows that sevelamer slows the development of VC whereas calcium salts do not. Four studies have demonstrated a slower progression of VC with sevelamer than with calcium-containing binders, although a fifth study showed nonsuperiority. Conversely, the results on mortality with sevelamer have been variable, and data on calcium-based binders are nonexistent. Improved survival with sevelamer was demonstrated in a small randomized clinical trial, whereas a larger randomized trial failed to show a benefit. In addition, preclinical models of renal failure and preliminary clinical data on hemodialysis patients suggest a potential benefit for bone with sevelamer. Meanwhile, several randomized and observational studies suggested no improvement in bone density and fracture rate, and a few noted an increase in total and cardiovascular mortality in the general population given calcium supplements. Although additional studies are needed, there are at least indications that sevelamer may improve vascular and bone health and, perhaps, mortality in hemodialysis patients, whereas data on calcium-based binders are lacking. Clin J Am Soc Nephrol 5: S31-S40, 2010. doi: 10.2215/CJN.05880809

Twenty seven-year experience with sternal cleft repair

Congenital sternal cleft is a rare disease, and primary repair in the neonatal period is its best management. In 1998 we presented three surgical techniques for sternal cleft correction, but since 1999 we have elected one of them as our procedure of choice. Our latest results are now presented. It is a review of 15 patients operated from October 1979 to December 2007. Surgical repair consisted of 3 sliding chondrotomies, 10 reconstructions basec on a `posterior sternal wall`, 1 reconstruction combined to Ravitch operation for pectus excavatum and 1 associated with total repair of Cantrell`s pentalogy. Data concerning epidemiological features, surgical reconstruction, aesthetic results, postoperative major complications, mortality and hospital stay, were collected from hospital charts. Follow-up ranged from 4 months to 27 years. All patients submitted to surgical, correction had a good aesthetic and functional result. Neither postoperative mortality nor major complication was observed. Two patients had subcutaneous fluid collection that prolonged the drainage duration. The mean hospital stay was 6 days. In conclusion, reconstructing sterna. cleft with a `posterior periosteal flap from sternal bars and chondral graft` is an effective option with good aesthetic and long-term functional results. (C) 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Growth after ventricular septal defect repair: does defect size matter? A 10-year experience

Aim: To evaluate whether the ventricular septal defect (VSD) size, along with the degree of preoperative growth impairment and age at repair, may influence postoperative growth, and if VSD size can be useful to identify children at risk for preoperative failure to thrive. Methods: Sixty-eight children submitted to VSD repair in a Brazilian tertiary-care institution were evaluated. Weight and height measurements were converted to Z-scores. Ventricular septal defect size was normalized by dividing it by the aortic root diameter (VSD/Ao ratio). Results: Twenty-six patients (38%) had significantly low weight-for-height, 10 patients (15%) had significantly low height-for-age and 13 patients (19%) had both conditions at repair. Catch-up growth occurred in 82% of patients for weight-for-age, in 75% of patients for height-for-age and in 89% of patients for weight-for-height. Weight-for-height Z-scores at surgery were significantly lower in patients who underwent repair before 9 months of age. The VSD/Ao ratio did not associate with any other data. On multivariate analysis, weight-for-age Z-scores and age at surgery were independent predictors of long-term weight and height respectively. Conclusion: The VSD/Ao ratio was not a good predictor of preoperative failure to thrive. Most patients had preoperative growth impairment and presented catch-up growth after repair. Preoperative growth status and age at surgery influenced long-term growth.

Small Bowel Endoscopy Using the Double-Balloon Technique: Four-Year Results in a Tertiary Referral Hospital in Brazil

Background: Double-balloon enteroscopy (DBE) allows evaluation and therapy for various small bowel diseases. In this series the outcome of a 4-year experience in a tertiary hospital school in Brazil is reported. Methods: A total of 457 consecutive DBE were performed in 418 patients from August 2004 to August 2008. 93 patients with several indications, whose aim was not the evaluation of suspected diseases of the small bowel mucosa, were excluded, therefore leaving 364 DBE in 325 patients for analysis. Data were retrospectively collected with regard to clinical, endoscopic findings, therapy and complications. Results: Among the 364 DBE performed in 325 patients, 143/325 were males (44%) and 182/325 females (56%) with a mean age of 48.6 +/- 15.7 years (range 17-89). Mean investigation time was 64 +/- 22 min (range 35-135). The depth of insertion beyond the ligament of Treitz was 230 +/- 85 cm (range 30-500) by the antegrade approach and 140 +/- 75 cm (range 0-320) by the retrograde approach. Total enteroscopy was achieved in 41.66% of the attempts (30 of 72 patients). Overall diagnostic yield was 54.95% (200 of 364 procedures) ranging from 0 to 100% in this series, depending on the indication. Angiodysplasia was the main diagnosis in 24.5% (49 of 200 procedures) and endoscopic treatment, including biopsies, hemostasis, tattooing and polypectomy were performed in 65.38% (238 of 364 procedures). No major complications were reported. Conclusions: DBE is a feasible, safe and well-tolerated procedure allowing endoscopic therapy. Selection of indications increases its diagnostic yield. Copyright (C) 2009 S. Karger AG, Basel

Management of brachial plexus region tumours and tumour-like conditions: relevant diagnostic and surgical features in a consecutive series of eighteen patients

Tumours of the brachial plexus region are rare and most publications are case reports or studies with a small series of patients. The aim of this study is to present our experience in managing these lesions. We review 18 patients with tumours in the brachial plexus region submitted to surgical treatment in a 6 year period, including their clinical presentation, neuro-imaging data, surgical findings and outcome. The tumours comprised a heterogeneous group of lesions, including schwannomas, neurofibromas, malignant peripheral nerve sheath tumour (MPNST), sarcomas, metastases, desmoids and an aneurysmal bone cyst. The most common presentation was an expanding lump (83.33%). Eleven tumours were benign and 7 were malignant. Neurofibromatosis was present in only 2 patients (11.11%). Gross total resection was achieved in 14 patients and sub-total resection in the others. Only 3 patients presented with new post-operative motor deficits. The incidence of complications was low (16.5 %). The majority of tumours were benign and most of them could be excised with a low incidence of additional deficits. Some of the malignant tumours could be controlled by surgery plus adjuvant therapy, but this category is still associated with high morbidity and mortality rates.

Blindness as a complication of monobloc frontofacial advancement with distraction

The monobloc frontofacial osteotomy provides aesthetic and functional improvement in the treatment of various craniofacial deformities. This procedure, through highly complex, has had some significant associated complication, such as cerebrospinal fluid leakage, hematoma, infection, and bone resorption. Distraction has been successfully used to gradually elongate bone and soft tissue. This method seems to provide improved results over conventional surgery, with less morbidity. We present a case of a patient with Apert syndrome who underwent monobloc advancement using the Rigid External Device (RED) device and who developed a transient bilateral amaurosis on the fourth postoperative day before distraction. A second procedure was performed to push back the frontal bandeau, maintaining the device in position. The blindness was resolved with this procedure as well as treatment with systemic steroids. The distraction was started thereafter, and the desired improvement was acquired. To our knowledge, this is the first case of transient bilateral amaurosis in a patient undergoing monobloc distraction.

Tessier No. 4 Facial Cleft: Evolution of Surgical Treatment in a Large Series of Patients

Background: Tessier no. 4 facial cleft is a rare, complex, and challenging craniofacial malformation. The present article aims to describe different clinical features evidenced in 21 cases of this malformation, discussing a 20-year experience with and evolution of its surgical treatment. Methods: Some demographic data, clinical features, and reconstructive results were evaluated retrospectively. These patients have been evaluated and treated in three specialized Brazilian craniofacial centers. Nineteen were already operated on, with a mean follow-up of 3.5 years (range, 1 to 20 years). Results: Sex distribution showed a male prevalence (2: 1). The average age of initial treatment was 5.4 years. Four cases were affected on the right side of the face, seven on the left, and 10 bilaterally. Six patients had other rare associated facial clefts, including nos. 5 (three patients), 7, 9, and 10. Cleft upper lip was evidenced in all patients, and maxillary hypoplasia was present in five and maxilla cleft in eight. Lower eyelid coloboma was seen in almost every case (19 patients); 10 of these had medial canthus dystopia. Four patients had amniotic bands in the limbs. Surgical repair was individualized to each patient. Surgical experience gained with these patients allowed the authors to develop some technical modifications, which have improved aesthetic results, camouflaging scars into natural folds and anatomical units, without compromising functional outcomes. Conclusions: The great majority of Tessier no. 4 facial clefts can be appropriately treated using local flaps. Classic techniques are extremely useful, but long-term results could be improved if the technical modifications described were adopted. (Plast. Reconstr. Surg. 122: 1505, 2008.)

Assessment of immediate conservative breast surgery reconstruction: A classification system of defects revisited and an algorithm for selecting the appropriate technique

Background: Although various techniques have been used for breast conservation surgery reconstruction, there are few studies describing a logical approach to reconstruction of these defects. The objectives of this study were to establish a classification system for partial breast defects and to develop a reconstructive algorithm. Methods: The authors reviewed a 7-year experience with 209 immediate breast conservation surgery reconstructions. Mean follow-up was 31 months. Type I defects include tissue resection in smaller breasts (bra size A/B), including type IA, which involves minimal defects that do not cause distortion; type III, which involves moderate defects that cause moderate distortion; and type IC, which involves large defects that cause significant deformities. Type II includes tissue resection in medium-sized breasts with or without ptosis (bra size C), and type III includes tissue resection in large breasts with ptosis (bra size D). Results: Eighteen percent of patients presented type I, where a lateral thoracodorsal flap and a latissimus dorsi flap were performed in 68 percent. Forty-five percent presented type II defects, where bilateral mastopexy was performed in 52 percent. Thirty-seven percent of patients presented type III distortion, where bilateral reduction mammaplasty was performed in 67 percent. Thirty-five percent of patients presented complications, and most were minor. Conclusions: An algorithm based on breast size in relation to tumor location and extension of resection can be followed to determine the best approach to reconstruction. The authors` results have demonstrated that the complications were similar to those in other clinical series. Success depends on patient selection, coordinated planning with the oncologic surgeon, and careful intraoperative management.

In pursuit of prognostic factors in children with pilocytic astrocytomas

This study described a 23-year experience in the treatment of children with pilocytic astrocytomas (piloA) with the aim of identifying putative clinical, histopathological, and/or immunohistochemical features that could be related to the outcome of these patients. Clinical data of 31 patients under 18 years of age with piloA were obtained from 1984 to 2006. The mean age at the time of surgery was 7.8 +/- 4.2 years (1 to 17 years), and the mean follow-up was 5.7 +/- 5.4 years (1 to 20 years). The most common site of tumor formation was the cerebellum (17), followed by brainstem (4), optic chiasmatic hypothalamic region (4), cerebral hemisphere (3), cervical spinal cord (2), and optic nerve (1). Gross total resection (GTR) was achieved in 23 (74.1%), mainly in those with tumors located in the cerebellum and cerebral hemispheres (P = 0.02). The global mortality rate was 6.4%. Nine patients were reoperated. Rosenthal fibers, eosinophilic granular bodies, microvascular proliferation, and lymphocytic infiltration were observed in most cases. The mean Ki-67LI was 4.4 +/- 4.5%. In all cases, Gal-3 expression in tumor cells was observed with variable staining pattern. Aside from GTR, no other clinical, histopathological, or immunohistochemical features were found to be related to the prognosis. We postulate that strict follow-up is recommended if piloA is associated with high mitotic activity/Ki67-LI, or if GTR cannot be achieved at surgery. Tumor recurrence or progression of the residual lesion should be strictly observed. In some aspects, childhood piloA remains an enigmatic tumor.

Total gastric transposition is better than partial gastric tube esophagoplasty for esophageal replacement in children

Whenever the surgeon uses the stomach as an esophageal substitute, either one of two techniques is generally performed: total gastric transposition or gastric tube esophagoplasty. No existing reports compare the complications associated with these two surgical procedures. The purpose of this study is to review the authors` experience with total gastric transposition and verify whether this technique is superior to gastric tube esophagoplasty in children by comparing the main complications with those reported in the publications of gastric tubes esophagoplasties in the English language literature published in the last 38 years. A total of 35 children underwent total gastric transposition according to the classical technique. Most of these patients (27, or 77.1%) had long gap esophageal atresia. The most frequently observed complications were compared to those reported in nine studies of gastric tube esophagoplasty comprising 184 patients. Mortality and graft failure rates were also compared. Seven patients (20.0%) presented with leaks, all of which closed spontaneously. Six children were reoperated, three experienced gastric outlet obstruction secondary to axial torsion of the stomach placed in the retrosternal space and the other three experienced delayed gastric emptying that required revision of the piloroplasty. There were two deaths (5.7%) and no graft failure. Strictures were observed in five patients (14.2%) and all of these were resolved with endoscopic dilatations. Six patients had diarrhea that spontaneously resolved. In the late follow-up period, all patients were on full feed and thriving well. The comparisons with gastric tube patients demonstrated that the total gastric transposition group presented with significantly less leaks and strictures (P = 0.0001 and 0.001, respectively). The incidence of death and graft failure was not statistically different. In conclusion, gastric transposition is as a simple technical procedure for esophageal replacement in children with satisfactory results, and is superior to gastric tube esophagoplasty.

Inequalities in the distribution of dental caries among 12-year-old Brazilian schoolchildren

We assessed the inequality in the distribution of dental caries and the association between indicators of socioeconomic status and caries experience in a representative sample of schoolchildren. This study followed a cross-sectional design, with a sample of 792 schoolchildren aged 12 years, representative of this age group in Santa Maria, RS, Brazil. Guardians answered questions on socioeconomic status and a dental examination provided information on the dental caries experience (DMF-T). Inequality in dental caries distribution was measured by the Gini coefficient and the Significant Caries Index (SiC). The assessment of association used Poisson regression models. Socioeconomic factors were associated with prevalence of dental caries for the whole sample and also for individuals with a high-caries level. Children from low-income households had the highest prevalence of dental caries. The Gini coefficient was 0.7 and the SiC Index 2.5. The percentage of caries prevalence was 39.3% (95% CI: 35.8%-42.8%) and the mean for DMF-T was 0.9 (± SD 1.5). Inequalities in the distribution of dental caries were observed and socioeconomic factors were found to be strong predictors of the prevalence of oral disease in children of this age group.

Narrowing socioeconomic inequality in child stunting: the Brazilian experience, 1974–2007

Objective To assess trends in the prevalence and social distribution of child stunting in Brazil to evaluate the effect of income and basic service redistribution policies implemented in that country in the recent past. Methods The prevalence of stunting (height-for-age z score below \22122 using the Child Growth Standards of the World Health Organization) among children aged less than 5 years was estimated from data collected during national household surveys carried out in Brazil in 1974\201375 (n = 34 409), 1989 (n = 7374), 1996 (n = 4149) and 2006\201307 (n = 4414). Absolute and relative socioeconomic inequality in stunting was measured by means of the slope index and the concentration index of inequality, respectively. Findings Over a 33-year period, we documented a steady decline in the national prevalence of stunting from 37.1 per cent to 7.1 per cent. Prevalence dropped from 59.0 per cent to 11.2 per cent in the poorest quintile and from 12.1 per cent to 3.3 per cent among the wealthiest quintile. The decline was particularly steep in the last 10 years of the period (1996 to 2007), when the gaps between poor and wealthy families with children under 5 were also reduced in terms of purchasing power; access to education, health care and water and sanitation services; and reproductive health indicators.Conclusion In Brazil, socioeconomic development coupled with equity-oriented public policies have been accompanied by marked improvements in living conditions and a substantial decline in child undernutrition, as well as a reduction of the gap in nutritional status between children in the highest and lowest socioeconomic quintiles. Future studies will show whether these gains will be maintained under the current global economic crisis