Determining The Risk Factors For Anastomotic Stricture Development After Esophageal Atresia Repair: Results From The Turkish Esophageal Atresia Registry.

INTRODUCTION: Anastomotic stricture (AS) is the second most common complication after esophageal atresia (EA) repair. We aimed to evaluate the data in the Turkish Esophageal Atresia Registry to determine the risk factors for AS development after EA repair in a large national cohort of patients. METHODS: The data between 2015 and 2021 were evaluated. Patients were enrolled into two groups according to the occurrence of AS. Patients with AS (AS group) and without AS (No-AS group) were compared according to demographic and operative features, postoperative intubation status, and postoperative complications, such as anastomotic leaks, fistula recanalization, and the presence of gastroesophageal reflux (GER). A multivariable logistic regression analysis was performed to define the risk factors for the development of AS after EA repair. RESULTS: Among the 713 cases, 144 patients (20.19%) were enrolled into the AS group, and 569 (79.81%) in the non-AS group. The multivariable logistic regression showed that ,being a term baby (OR 1.706; p = 0.006), having a birth weight over 2500 g (OR 1.72; p = 0.006), presence of GER (OR 5.267; p<0.001), or having a recurrent tracheoesophageal fistula (TEF, OR 4.363; p = 0.006) were the risk factors for the development of AS. CONCLUSIONS: The results of our national registry demonstrate that 20% of EA patients developed AS within their first year of life. In patients with early primary anastomosis, birth weight greater than 2500 g, and presence of GER were risk factors for developing AS. When patients with delayed anastomosis were included, in addition to the previous risk factors, being a term baby, and having recurrent TEF also became risk factors.

Management of pancreatic and duodenal trauma in childhood: a university hospital experience over a 10-year period.

PURPOSE: Duodenal/pancreatic injuries occur in less than 10% of intra-abdominal injuries in pediatric blunt trauma. Isolated duodenal/pancreatic injuries occur in two-thirds of cases, while combined injuries occur in the remaining. This study aimed to investigate pediatric patients with pancreatic and duodenal trauma. METHODS: Data from 31 patients admitted to Atatürk University, Medical Faculty, Department of Pediatric Surgery for pancreatic/duodenal trauma between 2010 and 2019 were retrospectively analyzed. Age/gender, province of origin, duration before hospital admission, trauma type, injured organs, injury severity, diagnostic and therapeutic modalities, complications, hospitalization duration, blood transfusion requirement, and mortality rate were recorded. RESULTS: Twenty-four patients were male, and 7 were female. The mean age was 9 years. The leading cause was bicycle accidents, with 12 cases, followed by traffic accidents/bumps, with 7 cases each. Comorbid organ injuries accompanied 18 cases. Duodenal trauma was most commonly accompanied by liver injuries (4/8), whereas pancreatic injury by pulmonary injuries (7/23). Serum amylase at initial hospital presentation was elevated in 83.9% of the patients. Thirty patients underwent abdominal CT, and FAST was performed in 20. While 54.8% of the patients were conservatively managed, 45.2% underwent surgery. CONCLUSION: Because of the anatomical proximity of the pancreas and the duodenum, both organs should be considered being co-affected by a localized trauma. Radiologic confirmation of perforation in duodenal trauma and an intra-abdominal pancreatic pseudocyst in pancreatic trauma are the most critical surgical indications of pancreaticoduodenal trauma. Conservative management's success is increased in the absence of duodenal perforation and cases of non-symptomatic pancreatic pseudocyst.

Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation.

INTRODUCTION: Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated. MATERIALS AND METHODS: A database search was done for the years 2015 to 2022. RESULTS: Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3). CONCLUSION: Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences.

The effect of postoperative ventilation strategies on postoperative complications and outcomes in patients with esophageal atresia: Results from the Turkish Esophageal Atresia Registry.

OBJECTIVES: Postoperative ventilatory strategies in patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) may have an impact on early postoperative complications. Our national Esophageal Atresia Registry was evaluated to define a possible relationship between the type and duration of respiratory support on postoperative complications and outcome. STUDY DESIGN: Among the data registered by 31 centers between 2015 and 2021, patients with esophago-esophageal anastomosis (EEA)/tracheoesophageal fistula (TEF) were divided into two groups; invasive ventilatory support (IV) and noninvasive ventilatory support and/or oxygen support (NIV-OS). The demographic findings, gestational age, type of atresia, associated anomalies, and genetic malformations were evaluated. We compared the type of repair, gap length, chest tube insertion, follow-up times, tensioned anastomosis, postoperative complications, esophageal dilatations, respiratory problems requiring treatment after the operation, and mortality rates. RESULTS: Among 650 registered patients, 502 patients with EEA/TEF repair included the study. Four hundred and seventy of patients require IV and 32 of them had NIV-OS treatment. The IV group had lower mean birth weights and higher incidence of respiratory problems when compared to NIV-OS group. Also, NIV-OS group had significantly higher incidence of associated anomalies than IV groups. The rates of postoperative complications and mortality were not different between the IV and NIV-OS groups. CONCLUSION: We demonstrated that patients who required invasive ventilation had a higher incidence of low birth weight and respiratory morbidity. We found no relation between mode of postoperative ventilation and surgical complications. Randomized controlled trials and clinical guidelines are needed to define the best type of ventilation strategy in children with EA/TEF.

Association of Gallbladder Volume and Wall Thickness With Acute Appendicitis in Pediatric Patients.

OBJECTIVES: Ultrasonography in a child with suspected appendicitis is primarily performed to detect inflamed appendix, whereas secondary findings are important in patients with a nonvisualized appendix. The aim of this study is to evaluate the gallbladder wall thickness and volume in patients with suspected appendicitis and search an association between these parameters and appendicitis. METHODS: Between January 2018 and August 2018, 113 patients with suspected appendicitis were included in this prospective study. Gallbladder and right lower quadrant were evaluated in separate sessions by different radiologists. The wall thickness along with the width, length, and height of the gallbladder was measured and recorded for each patient. Assessment of the appendix was classified as acute appendicitis, normal, and equivocal. Statistically significant association was sought between the thickness of the gallbladder wall and diagnosis of appendicitis. RESULTS: Gallbladder wall thickness was significantly higher in patients without appendicitis (P = 0.017), whereas significantly increased gallbladder volume was observed in patients with appendicitis (P = 0.004). Receiver operating characteristic analysis showed gallbladder wall thickness threshold of 0.5 mm with a sensitivity of 83.3, specificity of 93.9, and volume threshold of 41.6 mL with a sensitivity of 100 and a specificity of 0.69. CONCLUSIONS: Gallbladder wall thickness and volume measurements were associated with the diagnosis of appendicitis in pediatric patients.

Endoscopic Retrograde Cholangiopancreatography in Pediatric Patients.

Background: Endoscopic retrograde cholangiopancreatography (ERCP) is an advanced endoscopic technique used in the diagnosis and treatment of pancreaticobiliary system. ERCP is used less frequently in children than in adults due to the rarity of pancreaticobiliary diseases and technical difficulties. However, ERCP is a safe, effective diagnosis and treatment tool for children. Methods: All patients within the age range of 1-19 years, who underwent ERCP between 2010 and 2021 at our endoscopy unit, were retrospectively examined. Patient demographics, use of imaging methods, indications, type of sedation, interventions, success of ERCP, findings, and complications were evaluated. Results: Overall, 105 ERCPs were performed in 66 children (29 male and 37 female). The indications were choledocholithiasis, cyst hydatic, choledochal cyst, biliary atresia or anomaly, liver transplantation-related disorders, and pancreatic disorders, respectively. ERCP was finished as diagnostic ERCP in 20% and as therapeutic in 80%. Therapeutic procedures were sphincterotomy, stent placement or removal, stone or debris extraction, and balloon sweep or dilatation, respectively. The success rate in the procedures was 75.23%. The overall complication rate was 15.23%. Postprocedure pancreatitis occurred in 11.42%, hemorrhage occurred in 2.85%, and aggravation of cholangitis in 0.95%. All complications were managed conservatively. Conclusion: ERCP in pediatric patients is a safe procedure that can be performed by adult endoscopists with high success rates. Since our region is an endemic region for hydatid cyst disease, the most common ERCP indication after choledocholithiasis is procedures related to liver hydatid cyst disease. The most common complication was pancreatitis, and complications were treated medically.

Dual energy CT in necrotizing enterocolitis; a novel diagnostic approach.

BACKGROUND: Necrotizing enterocolitis (NEC) is one of the most important causes of morbidity and mortality in premature infants. Although there are no specific diagnostic tools, the main factors affecting prognosis are clinical and laboratory findings, and early diagnosis and treatment. In this study, we demonstrate the importance of dual-energy computed tomography (DECT) in confirming intestinal ischemia in neonates with NEC. METHODS: Patients diagnosed with NEC in a neonatal intensive care unit were staged according to modified Bell's classification, and DECT was performed on patients with NEC stages 2-A, 2-B and 3-A. According to their DECT reports, these patients were then separated into two groups: those with intestinal ischemia and those without intestinal ischemia. The patients with intestinal ischemia were evaluated using surgical reports, and the other patients were evaluated using clinical findings. RESULTS: DECT was performed in 21 patients with NEC stages 2-A, 2-B and 3-A. Twelve patients (57.1%) without ischemia were followed up without surgery. Nine patients (42.9%) with ischemia on DECT were operated on, and resection and anastomosis or ileostomy and colostomy were performed. DISCUSSION: In patients with NEC, DECT significantly increases overall diagnostic confidence in assessing intestinal necrosis when compared with traditional diagnostic methods.

First emerging evidence of the relationship between Onuf's nucleus degeneration and reduced sperm number following spinal subarachnoid haemorrhage: Experimental study.

Lumbosacral pathologies can lead to infertility. Onuf's nucleus changes in these pathologies may have a role in low sperm number. This study aims to investigate the relationship between Onuf's nucleus degeneration and sperm number following spinal subarachnoid haemorrhage. 22 rabbits were used. They were divided into three groups; five of them were used as the control (GI), five as the SHAM (GII) and twelve as the study groups (GIII). The study group received 0.7 ccs autologous blood into the spinal subarachnoid space at the T12-L1 level. After two weeks, all animals were decapitated, and S1-S3 laminectomy was done. Neurodegenerative changes of Onuf's nucleus, pudendal ganglia (S3) following two weeks after spinal SAH, were examined; sperm numbers were calculated. Degenerated neuron density of the Onuf's nucleus (n/mm3 ), the pudendal ganglia (S3) (n/mm3 ) and mean sperm numbers were calculated as 5 ± 2, 8 ± 3/mm3 and 98.345 ± 12.776/mm3 in the control (GI), 20 ± 5/mm3 , 243 ± 66/mm3 and 91.841 ± 9.654/mm3 in the SHAM (GII), 143 ± 39/mm3 , 2,350 ± 320/mm3 and 68.549 ± 5.540/mm3 in the study group (GIII). In conclusion, there were statistically significant differences between groups. Onuf's nucleus may be responsible for decreased sperm number following spinal SAH.

Destructive Effects of Acidic Blood on the Intestines: Experimental Study.

OBJECTIVE: This study aimed to investigate the destructive effects of acidic blood on the intestinal structures, which has been reported as the most hazardous biochemical result of vagosympathetic autonomic imbalances in intensive care unit patients with subarachnoid hemorrhage (SAH). MATERIALS AND METHODS: In total, 27 hybrid rabbits were used; 5 rabbits were used as a control group, 7 as the SHAM group into whom 1 cc saline was injected into the cisterna magna, and the remaining 15 were used as the study group. These animals received 1 cc of autologous arterial blood injection into the cisterna magna to create the SAH group. Blood pH values were recorded before, during, and after the experimental procedures. Computed tomography was performed to examine the intestinal morphology. Normal and degenerated epithelial cell densities of the intestine were estimated by stereological methods. The relationship between pH values and intestinal tissue changes was analyzed statistically using the Mann-Whitney U test. RESULTS: The mean blood pH values were 7.364±0.042 in the control group, 7.326±0.059 in the SHAM group, and 7.23±0.021 in the study group. Intestinal epithelial cell injury, desquamation of villus, and cell loss were observed. It is observed that the number of degenerated epithelial cells, fragmented villi numbers, and vacuoles significantly increased in the study group (p<0.05). CONCLUSION: Acidotic intestinal injury secondary to blood pH changes following SAH may be considered as a generalized and dangerous complication with their multiorgan insuficiency effect.

The effect of azygos vein preservation on postoperative complications after esophageal atresia repair: Results from the Turkish Esophageal Atresia Registry.

AIM: Preservation of the azygos vein (AV) maintains normal venous drainage of the mediastinum and decreases postoperative congestion. The modification of esophageal atresia (EA) repair by preserving AV may prevent postoperative complications and may lead to better outcomes. The data from the Turkish Esophageal Atresia Registry (TEAR) were evaluated to define the effect of AV preservation on postoperative complications of patients with EA. METHODS: Data from TEAR for a period of five years were evaluated. Patients were enrolled into two groups according to the preservation of AV. Patients with divided (DAV) and preserved AV (PAV) were evaluated for demographic and operative features and postoperative complications for the first year of life. The DAV and PAV groups were compared according to the postoperative complications, such as fistula recanalization, symptomatic strictures, anastomotic leaks, total number of esophageal dilatations, and anti-reflux surgery. In addition, respiratory problems, which required treatment, were compared between groups. RESULTS: Among 502 registered patients; the data from 315 patients with the information of AV ligation were included. The male female ratio of DAV (n = 271) and PAV (n = 44) groups were 150:121 and 21:23, respectively (p > 0.05). The mean body weight, height, gestational age, and associated anomalies were similar in both groups (p > 0.05). The esophageal repair with thoracotomy was significantly higher in DAV group, when compared to the PAV group (p < 0.05). The rates of primary anastomosis and tensioned anastomosis were similar in both groups (p > 0.05). There was no difference between DAV and PAV groups for anastomotic leaks, symptomatic anastomotic strictures, fistula recanalization, and the requirement for anti-reflux surgery (p > 0.05). The rate of respiratory problems, which required treatment, was significantly higher in the DAV group (p < 0.05) CONCLUSION: The data in the TEAR demonstrated that preserving the AV during EA repair led to no significant advantage on postoperative complications, with exception of respiratory problems. AV should be preserved as much as possible to maintain a normal mediastinal anatomy and to avoid respiratory complications.

Outcome of Very Low and Low Birth Weight Infants with Esophageal Atresia: Results of the Turkish Esophageal Atresia Registry.

INTRODUCTION: The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA). MATERIALS AND METHODS: The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW <1,500 g), LWB = 1,500-2,500 g), and normal BW (NBW; >2,500 g). RESULTS: Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups (p < 0.05). In patients with tracheoesophageal fistula (TEF), patients of the NBW group had significantly higher rates of full oral feedings, when compared with VLBW and LBW cases (p < 0.05). At the end of the first year, when we evaluate all patients, the number of cases with fistula recanalization and esophageal anastomotic strictures (AS) requiring esophageal dilatation was similar among the groups. The weight and height measurements at 6 months and 1 year of age of the survivors were similar in all the groups. The overall mortality rate was significantly higher in the VLBW and LBW groups, when compared with the NBW patients, even in patients with tension-free anastomosis (p < 0.05). The incidence of the associated anomalies was 90.6% in cases with mortality, which was significantly higher than in survivors (59.6%; p < 0.05). According to Spitz's classification, the survival rate was 87.1% in class I, 55.3% in class II, and 16.7% in class III. The most common causes of mortality were associated with cardiovascular diseases, pneumonia, and sepsis. CONCLUSION: The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA.

Disruption of the network between Onuf's nucleus and myenteric ganglia, and developing Hirschsprung-like disease following spinal subarachnoid haemorrhage: an experimental study.

Purpose/Aim of the study: Auerbach/Meissner network of lower abdominopelvic organs managed by parasympathetic nerve fibres of lumbosacral roots arising from Onuf's nucleus located in conus medullaris. Aim of this study is to evaluate if there is any relationship between Onuf's nucleus ischemia and Auerbach/Meissner network degeneration following spinal subarachnoid haemorrhage (SAH). Materials and Methods: Study was conducted on 24 male rabbits included control (Group I, n = 5), serum saline-SHAM (Group II, n = 5), and spinal SAH (Group III, n = 14) groups. Spinal SAH performed by injecting homologous blood into subarachnoid space at Th12-L4 level and followed three weeks. Live and degenerated neuron densities of Onuf's nucleus, Auerbach and Meissner ganglia (n/mm3) were determined by Stereological methods. Results: The mean degenerated neuron density of Onuf's nucleus was significantly higher in Group III than in Groups I-II (152 ± 26, 2 ± 1 and 5 ± 2/mm3 respectively, p < 0.005). The degenerated neuron density of Auerbach's ganglia was significantly higher in Group III than in Groups I-II (365 ± 112, 3 ± 1 and 9 ± 3/mm3 respectively, p < 0.005). The degenerated neuron density of Meissner's ganglia was significantly higher in Group III than in Groups I-II (413 ± 132, 2 ± 1 and 11 ± 4/mm3 respectively, p < 0.005). Conclusions: Onuf's nucleus pathologies should be considered as Auerbach/Meissner ganglia degeneration and also related Hirschsprung-like diseases in the future.

Mysterious effects of olfactory pathway lesions on intestinal immunodeficiency targeting Peyer's patches: The first experimental study.

BACKGROUND: Although olfaction has been considered as important neuroimmunomodulatory foundation, there is no satisfying analytical information between neurohistomorphological features olfactory networks and intestinal immune system hardwares. We studied if the olfactory bulb lesions (OBL) may rely on histopathological features of intestinal lymphatic Peyer's patches in an animal model. METHODS: Thirty-two rats were grouped as control (Group I, n = 8), SHAM (Group II, n = 7) and OBL (Group III, n = 17) respectively; and followed eight weeks and animals were decapitated. The olfactory bulbs and intestines were extracted. Specimens stained with hematoxylin/eosin and GFAP methods and analyzed Stereologically to evaluate volume loss of olfactory bulbs and Peyer's patches volumes (PV) of intestines per cubic millimeter and compared with each other's statistically. RESULTS: The mean olfactory bulbs volumes were estimated as 3.65 ±â€¯0.32/mm3 in group I, 3.12 ±â€¯0.20/mm3 in group II and 2.21 ±â€¯0.15/mm3 in group III (p < 0.0005 Group III vs. I and II). The mean of PV were estimated as; (9 ±â€¯2) × 106 µm3/cm3 in Group-I, (12 ±â€¯3) × 106 µm3/cm3 in Group-II; and (23 ±â€¯4) × 106 µm3/cm3 in group-III (p < 0.005 Group II vs. I, p < 0.0005 Group III vs. I-II). CONCLUSIONS: OBL could rely on intestinal immunodeficiency causing by olfaction loss induced denervation injury of Peyer's patches.

Predeterminative role of Onuf's nucleus ischemia on mesenteric artery vasospasm in spinal subarachnoid hemorrhage: A preliminary experimental study.

BACKGROUND: Although posttraumatic mesenteric artery ischemia is attributed to various etiologies, sacral parasympathetic network/mesenteric artery relations have not been studied so far. The primary objective of this study is to elucidate whether there is a relationship between Onuf's nucleus ischemia and mesenteric artery vasospasm following subarachnoid hemorrhage (SAH). METHODS: This study was conducted on 22 rabbits. The animals were grouped as follows: 5 of animals control, 5 SHAM which saline was given, and 12 animals study group that was homologous blood injected into the spinal subarachnoid space at the Li level. Neurodegeneration in Onuf's nucleus, axonal degeneration of S2 roots, and mesenteric arteries vasospasm indexes (VSI; Wall surface/Lumen surface), brachias of mesentery arteries in various tissues and ischemic mucosal changes of intestines of all animals were determined histopathologically. Important degenerative changes were detected in axons in S2 roots and Onuf's nucleus in severe mesenteric artery vasospasm observed. RESULTS: The mean degenerated neuron density of Onuf's nucleus (n/mm3), degenerated axon density in S2 roots (n/mm2), and VSI values of mesenteric arteries of control, SHAM, and study groups were estimated as 5.00 ± 1.58, 4.00 ± 1.58, 1.76 ± 0.13; 18.29 ± 4.31, 11.00 ± 2.24, 2.23 ± 0.20; and 135.21 ± 30.75, 117.33 ± 22.11, 2.81 ± 0.44, respectively. Statistical analyses between the VSI values, mucosal ischemic changes degenerated neurons in Onuf's nucleus, and axons in S2 levels were meaningful (p < 0.005). CONCLUSION: We interestingly noticed that Onuf's nucleus-S2 roots complex degeneration plays an important role in mesenteric artery vasospasm and the development of intestinal ischemic mucosal changes following SAH which has not been extensively mentioned in the literature.

[Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery]. / Manejo anestésico de paciente pediátrico com displasia ectodérmica hipoidrótica submetido a cirurgia de emergência.

Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.

Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery / Manejo anestésico de paciente pediátrico com displasia ectodérmica hipoidrótica submetido a cirurgia de emergência

ABSTRACT Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.

As displasias ectodérmicas são condições raras, com uma tríade de hipotricose, anodontia e anidrose. Em revisão da literatura há apenas alguns relatos de manejo anestésico de pacientes com displasias ectodérmicas. Hipertermia é um risco muito sério que pode ocorrer por causa de defeito das glândulas sudoríparas. O presente caso envolve uma criança de 10 anos com displasia ectodérmica que se apresentou com abdome agudo e foi considerada para uma cirurgia de emergência. Nosso objetivo foi demonstrar o manejo bem-sucedido desse caso, com o uso de uma combinação de anestesia geral e peridural. É importante para o anestesiologista obter informações sobre essa síndrome, em caso de operações de emergência, pois pode evitar complicações graves e até salvar vidas.

Anesthetic management of a pediatric patient with hypohidrotic ectodermal dysplasia undergoing emergency surgery.

Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with an acute abdomen and was considered for an emergency surgery. Our aim was to demonstrate the successful management of this case using a combination of general and epidural anesthesia. It is important for anesthesiologist to have information about this syndrome in case of emergency operations, since it can prevent serious complications and even save lives.

Annular pancreas in children: a decade of experience.

OBJECTIVE: Annular pancreas is an uncommon cause of duodenal obstruction in children. In this study, the clinical, radiological, and prognostic findings related to this disorder over a 12-year review period were analyzed. MATERIALS AND METHODS: A retrospective review of 22 patients with annular pancreas who were treated with surgical repair between April 1998 and February 2010 was performed at two different pediatric surgical units. Presenting symptoms, associated anomalies, radiological findings, the type of surgery performed, postoperative outcomes, and complications were analyzed. RESULTS: Twenty-two patients were identified. Thirteen of the 22 patients (59.1%) were born prematurely, 11 patients (50%) had low birth weight, 2 patients (9.1%) had very low birth weight and 1 patient (4.5%) had extremely low birth weight. The mean birth weight was 2285.23±675.12 g. (970-3300). All patients presented with vomiting, which was bilious in nine (40.9%). Nine patients (40.9%) had chromosomal anomalies. Corrective surgery consisted of duodenoduodenostomy in 9 patients (40.9 %), duodenojejunostomy in 9 patients (40.9%), and gastrojejunostomy in 4 patients (18.1%). Fourteen of the 22 patients have survived (63.6%). The causes of death were combinations of sepsis, pneumonia, brain hemorrhage, and cardiac anomaly. CONCLUSION: Infants with annular pancreas associated with duodenal obstruction were often born prematurely and/or had low birth weights; many had cardiovascular anomalies. Annular pancreas associated with duodenal obstruction correlated strongly with the trisomy 21 karyotype among the chromosomal anomalies, as did duodenal atresia. The oral feeding tolerance time was nearly the same for all patients regardless of the surgical procedure used.