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A new postoperative esophagectomy care pathway was recently implemented at our institution. Practice pattern change among provider teams can prove challenging; therefore, we sought to study the barriers and facilitators toward pathway implementation at the provider level.This qualitative study was guided by the Theoretical Domains Framework (TDF) to study the adoption and implementation of a post-esophagectomy care pathway. Sixteen in-depth interviews were conducted with providers involved with the pathway. Matrix analysis was used to analyze the data.Providers included attending surgeons (n = 6), advanced practice providers (n = 8), registered dietitian (n = 1), and clinic staff (n = 1). TDF domains that were salient across our findings included knowledge, beliefs about consequences, social influences, and environmental context and resources. Identified facilitators included were electronic health record tools, such as note templates including pathway components and a pathway-specific order set, patient satisfaction, and preliminary data indicating clinical benefits such as a reduced anastomotic leak rate. The major barrier reported was a hesitance to abandon previous practice patterns, most prevalent at the attending surgeon level.The TDF enabled us to identify and understand the individuals' perceived barriers and facilitators toward adoption and implementation of a postoperative esophagectomy pathway. This analysis can help guide and improve adoption of surgical patient care pathways among providers.

Morgagni hernia (MH) is a rare diaphragmatic hernia which needs surgical repair. The conventional reconstruction involves reduction of hernia, closure of the defect and placement of an intraperitoneal onlay mesh often using robotic platform for ease of dissection and suturing the mesh (r-IPOM). We propose a novel robotic preperitoneal repair (r-TAPP) of MH in four cases and compare them with conventional r-IPOM technique. Between August 2017 and August 2020 nine patients underwent repair of MH. Five cases underwent repair by r-IPOM (group I). For the other four cases, r-TAPP was used (group II). Among the nine cases, the mean age was 53 years in group I and 55 years in group II, mean defect size was 33 mm in group I and 55 mm in group II. Operative time was longer in group II compared to group I (220 min vs 135 min, p = 0.022). Mean length of hospital stay was 1.3 days in group I compared to group II (4.5 and 4.5 vs 1.3 days, p = 0.03). There was statistically significant difference in reduced post-operative pain and time to return to work in group II compared to group I. There was no difference in complications, 30-day readmissions or recurrence of hernia between the two groups. We conclude that compared to the conventional r-IPOM repair, the r-TAPP technique is associated with less pain, early discharge, and faster return to work, translating into overall cost savings for the hospital.

Background: Endophytic submucosal masses at anatomically difficult locations such as lesser curve of the stomach, juxta-gastroesophageal junction and duodenum are challenging to resect laparoscopically due to proximity of vital structures and difficulty to visualise them. To overcome these limitations, we describe a technique of endoscopic tattooing with indocyanine green (ICG) injection into the lesion allowing easy identification and oncological resection in a minimally invasive manner. Patients and Methods: The technique of endoscopic tattooing of the lesion and robotic transgastric eversion resection technique is described in patients with gastrointestinal tumours at difficult anatomical location. Results: Gastric gastrointestinal stromal tumours at the lesser curve (n = 3) and gastroesophageal junction (n = 1) were resected using this technique successfully. Conclusion: The use of intraoperative ICG tattooing of endophytic submucosal lesions at difficult locations can facilitate minimally invasive oncologic resection. This technique allows the surgeon to be more comfortable to approach complex lesions safely to improve patient outcomes.

The use of bariatric surgery has increased for morbidly obese patients with end stage renal disease (ESRD) for whom listing on the waitlist is often restricted until a certain BMI threshold is achieved. Effective weight loss for this population improves access to life-saving renal transplantation. However, it is unclear whether sleeve gastrectomy (SG) vs Roux-en-Y gastric bypass (RYGB) is a more effective therapy for these patients. A decision analytic Markov state transition model was created to simulate the life of morbidly obese patients with ESRD who were deemed ineligible to be waitlisted for renal transplantation unless they achieved a BMI less than 35 kg/m2. Life expectancy following weight management (MWM), RYGB, and SG were estimated. Base case patients were defined as having a pre-intervention BMI of 45 kg/m2. Sensitivity analysis of initial BMI was performed. Markov parameters were extracted from literature review. RYGB improved survival compared with SG and MWM. RYGB patients had higher rates of transplantation, leading to improved mean long-term survival. Base case patients who underwent RYGB gained 1.3 additional years of life compared with patient’s who underwent SG and 2.6 additional years of life compared with MWM. RYGB improves access to renal transplantation and thereby increases long-term survival compared with SG and MWM. The use of SG may be incongruent with the goal of improving access to renal transplantation for morbidly obese patients.

Dysregulation of toll-like receptor (TLR) signaling within the gastrointestinal epithelium has been associated with uncontrolled inflammation and tumorigenesis. We sought to evaluate the role of TLR4 in the development of gastroesophageal reflux-mediated inflammation and mucosal changes of the distal esophagus. Verified human esophageal Barrett's cells with high grade dysplasia (CPB) and esophageal adenocarcinoma cells (OE33) were treated with deoxycholic acid for 24 hours. Cells were pretreated with a TLR4-specific inhibitor peptide 2 hours prior to deoxycholic acid treatment. Inflammatory markers were evaluated using immunoblotting and enzyme-linked immunosorbent assay. A surgical reflux mouse model was generated by performing a side-to-side anastomosis between the second portion of the duodenum and the gastroesophageal junction. Control animals underwent laparotomy with incision and closure of the esophagus superior to the gastroesophageal junction (sham procedure). Esophageal sections were evaluated using hematoxylin and eosin staining and immunohistochemistry. Deoxycholic acid increased expression of inflammatory markers including intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and interleukin 8. Pretreatment with a TLR4 inhibitor significantly decreased deoxycholic acid-induced inflammatory marker expression. C3H/HeNCrl mice demonstrated a significant increase in mucosal hyperplasia and proliferation following DGEA compared to sham procedure. TLR4 mutant mice (C3H/HeJ) undergoing DGEA demonstrated an attenuated hyperplastic and proliferative response compared to C3H/HeNCrl mice. Inhibition of TLR4 signaling attenuates reflux-induced inflammation in vivo. These findings identify TLR4 inhibition as a potential therapeutic target to halt the progression of pathologic esophageal changes developing in the setting of chronic gastroesophageal reflux disease.

Introduction Approximately 5% of patients who have undergone prior Heller myotomy and Roux en Y gastric bypass progress to end-stage achalasia (ESA). Surgical options for ESA are often limited to esophagectomy for management of severe dysphagia or life-threatening aspiration episodes. Mediastinoscopy-assisted transhiatal esophagectomy (MATHE) by a small left neck incision combined with an abdominal incision, without using a transthoracic approach, has been reported to reduce pulmonary complications. We herein present the first report of MATHE in 2 consecutive patients with ESA and gastric bypass. Materials and methods Between August 2017 and September 2020, 2 patients who had undergone Heller myotomy and Roux en Y gastric bypass underwent MATHE for ESA. Transhiatal esophagectomy with mediastinoscopy-assisted dissection was performed. The remnant stomach was used as the conduit in both cases. The embedded Supplemental Digital Content 1 (http://links.lww.com/SLE/A269) reports our novel technique. Results Both cases were completed laparoscopically without conversion to laparotomy or thoracotomy. Median length of hospital stay was 9 days (range, 6 to 11 d). Postoperatively, 1 patient developed a cervical anastomotic leak, which healed with conservative management. No other major complications were observed. Conclusions MATHE can be safely performed in patients with a history of gastric bypass and ESA without requiring thoracoscopic mobilization of the esophagus. Further studies are required to validate reproducibility of our technique as an alternative to using a thoracic incision.

Introduction Morgagni hernia (MH) is a rare, congenital diaphragmatic hernia. We developed a novel robotic-assisted technique to repair MH which enables dissection into the preperitoneal space, facilitating closure of the diaphragmatic defect and placement of a synthetic mesh. Materials and surgical technique Between August 2017 and August 2020, 8 consecutive patients with MH were repaired by robotic-assisted transabdominal preperitoneal (r-TAPP) approach. A preperitoneal plane is developed at the level of the falciform ligament and extended toward the diaphragmatic defect. The pocket is dissected inferior to the defect to allow 3 to 5 cm overlap of synthetic mesh. Excision of the hernia sac followed by closure of defect is performed. A synthetic mesh is deployed in the preperitoneal space with wide overlap. This technique using the robot provides superior optics and ergonomics for dissection while isolating the mesh from underlying viscera and avoiding the need for suturing or tacking of the mesh. Data of patients who underwent r-TAPP were reviewed. Mean operating time was 113 minutes. Mean pain visual analog scale score was 5/2 on post-operative days 1/7. Average hospital stay was 1.8 days. One patient developed superficial cellulitis related to the abdominal drain. There were no procedure-related complications, 30-day readmissions, or hernia recurrences at a mean follow-up of 10 months. Discussion A robotic-assisted preperitoneal approach is a novel, safe, and anatomically justified alternative technique for MH repair that may lead to improved post-operative outcomes.

Gastroesophageal reflux disease (GERD) and esophageal dysmotility are often disqualifying criteria for fundoplication due to dysphagia complications. A tailored partial fundoplication may improve GERD in patients with severe esophageal motility disorders. We evaluate this approach on GERD improvement in non-achalasia esophageal dysmotility patients. A single-institution prospective database was reviewed (2007–2016), with inclusion criteria of GERD, previous diagnosis of non-achalasia esophageal motility disorder, and laparoscopic partial fundoplication. Diagnosis of previous achalasia diagnosis or diffused esophageal spasm was excluded. Motility studies, pre- and post-upper gastrointestinal imaging (UGI), esophageal symptom scores, antacid, and PPI use were collected pre-op, 6 months, 12 months, and long-term (LT). Statistical analysis was made using SPSS v.23.0.0, α = 0.05. Fifty-two patients met the inclusion criteria. A total of 17.3% had esophageal body amotility, 79.6% had severe esophageal dysmotility. A total of 65.9% women (mean age 64 ± 15.7), mean peristalsis 45.3 ± 32.6%, and failed peristalsis 36.0 ± 32.2%. Mean LES residual pressure was 15.0 ± 18.0 mmHg, and 40.7% had hypotensive LES. Mean follow-up time was 25 months [1–7 years], with significant improvement in symptoms and reduction in PPI and antacid use at all time-points compared to pre-op. A total of 74% had UGI studies at 12 months; all showed persistent dysmotility. Six patients had radiographic hiatal hernia recurrence, with only one being clinically symptomatic postoperatively. Three required dilation for persistent dysphagia. A tailored partial fundoplication may be effective in symptom relief for non-achalasia patients with esophageal motility disorders and GERD. Significant symptom improvement, low HHR, and PPI use clearly indicate this approach to be effective for this population.

Minimally invasive surgery (MIS) use for ventral hernia repair has increased over the last decade. Whether outcomes are improved by robotic assistance remains a subject of debate. The aim of this study is to evaluate outcomes (including cost, complications, length of stay (LOS), and pain medication utilization) in patients who underwent an open (OVHR), laparoscopic (LVHR), or robotic (RVHR) ventral hernia repair (VHR). The Vizient database was queried using ICD-9 procedure and diagnosis codes for patients who underwent VHR from January 2013 to September 2015. Complications, 30-day readmission, mortality, LOS, cost, and intra-hospital opiate utilization were analyzed using IBM SPSS v.23.0.0.0. Median tests with post hoc pairwise comparisons, Fischer’s exact, and Pearson’s chi-squared test with Bonferroni correction were applied where appropriate, with α = 0.05. 46,799 patients (OVHR: N = 39,505, LVHR: N = 6829, RVHR: N = 465) met the criteria and patients in each group had similar demographics (Table 1). OVHR was associated with significant increased overall complications, 30-day readmission, LOS, and postoperative pain use compared to RVHR or LVHR. OVHR had higher mortality and postoperative infection rates than LVHR. RVHR had significantly higher rates of complications and postoperative infections compared to LVHR, although there was no difference in mortality, 30-day readmission, LOS, and postoperative pain medication use. Mean direct cost of surgery was significantly higher for RVHR, followed by OVHR and LVHR. Overall patient outcomes were improved in the LVHR and RVHR groups compared to the open approach. However, RVHR patients did not have significant improvement compared with the LVHR group in either short-term outcomes or opiate medication used. While RVHR surgery was the most expensive modality, OVHR was also significantly costlier than LVHR, which was the least expensive. Long-term data on recurrence could not be evaluated and should be studied to determine the role of robotic surgery in VHR and recurrence rates.

Splenic artery aneurysm is the most common visceral arterial aneurysm. Rupture of aneurysm is a rare event but associated with a high mortality. Endovascular coil embolization of bleeding splenic artery aneurysm has emerged as a promising minimal invasive treatment and considered safer than open surgery in selected patients. Nevertheless, several complications related to coils have been reported, the rarest being coil migration and erosion. We report a case of splenic artery coil migration into the stomach and its successful removal by laparoscopic endoscopy combined surgery.

We report a case of 14-year-old female who was admitted with a rare retroorbital subperiosteal hematoma manifested as sudden orbital pain, progressive proptosis and diminution of vision. Cranial computed tomography showed a biconvex high density mass in the superolateral part of the right orbit, which was recognized to be a subperiosteal hematoma intraoperatively. Infection extending into the orbit was important in causing the hemorrhage.

Background This article briefly discusses about the present status, future strategies, and perspectives of neurosurgery in the eastern region of Nepal. Methods B.P. Koirala Institute of Health Sciences, Dharan, Nepal, is the only tertiary referral center responsible for patient care as well as training of postgraduate students and paramedical staff including health care workers in the eastern region of Nepal. We analyzed the data from January 2006 to January 2007 regarding patient care and their outcomes. At the same time, we also evaluated our limitations regarding resources and their impact on patient care. Strategies are suggested to overcome the barriers to establish effective neurosurgical centers in developing countries. Results At present in the BPKIHS, neurosurgical facilities are being provided as a specialty unit in the department of surgery. From January 2006 to January 2007, a total 1120 patients were treated for different neurological and neurosurgical conditions. At present, there are 8 beds allotted to neurosurgical services in the general surgery ward. In addition, there are 8 ICU beds catering to the needs, including neurosurgery, of the hospital. No vascular, epilepsy surgery or functional neurosurgery has been performed due to lack of resources. The only imaging facility for neurosurgery that is available in the hospital is CT scan. Conclusions The study concludes that in a developing country, the neurosurgical unit can be structured in a manner that will fulfill most of the objectives required for better clinical outcome. This structured program may also benefit substantially in training health professionals as well.

Background The primary aim of this study is to detail the problems, complications, their avoidance, and management with transanal pull-through developed from experience with 65 patients. Methods A retrospective study of 65 patients who underwent transanal pull-through between January 2002 and December 2006 was conducted. Their medical charts and operative notes were reviewed for problems encountered during surgery, postoperative period, and follow-up. Results In 46 patients, a primary transanal pull-through was performed, whereas in 19 with a prior colostomy, followed staged pull-through was done. The minimum follow-up was 6 months, with an average of 22 months after surgery (range, 6-47 months). Sixteen patients (25%) experienced at least 1 complication. These included inadvertent full-thickness mobilization of the rectum in 3 (4.6%), retraction and bleeding of colonic mesenteric vessels in 2 (3.7%), difficulty in mobilizing intraperitoneal colon in 1 (1.5%), and a false-positive frozen section in 2 patients (3%). Early postoperative complications occurred in 7 patients (11%), which included sphincter spasm in 3 (4.6%), anastomotic leak in 1 (1.5%), cuff abscess in 2 (3%), and enterocolitis in 1 (1.5%). Late postoperative complications in 46 patients (70%), occurring from 1 week till 3 months of follow-up included perianal excoriation in 22 (34%), increased stool frequency in 20 (31%), anal stenosis in 3 (4.6%), and enterocolitis in 2 patients (3%). Methodology is detailed for avoidance and management of problems and complications. Individual patient analysis, complications timing, and strategy for management are discussed. Conclusion Patient outcomes for transanal pull-through have improved significantly as a result of combination of experience and the ability to avoid and manage associated complications. Experience, avoidance, and interdiction are key factors in complication management.

INTRODUCTION To describe the operative details and results of a modified posterior sagittal transanorectal approach for the reconstruction of urogenital sinus (UGS) anomalies. TECHNICAL CONSIDERATIONS Six children with UGS anomalies underwent surgery using this technique. In a prone jack-knife position, a midline incision was continued to the puborectalis muscle. A plane of dissection was created circumferentially around the rectum separating it from the underlying UGS. Circumferential transanal mucosectomy and a transanal dissection was carried proximally for 5 to 10 cm. The mucosal tube with the serosal wall was resected, exposing the proximal part of the UGS. The posterior and anterior sphincters, anus, and perineal body were then divided in the midline, completely exposing the UGS. Reconstruction of the urethra and vagina was done. At completion of UGS reconstruction, an endoanal pull through of the rectal tube and a low coloanal anastomosis were performed. The muscle complex and perineal body were closed in layers. The modified technique of posterior sagittal transanorectal approach allowed excellent exposure in all 6 patients. None developed any complications related to suture line leak. Fecal and urinary continence was preserved in patients who were continent before the operation. CONCLUSIONS The modified posterior sagittal transanorectal approach is a safe and effective technique in the treatment of UGS anomalies and can be performed without the need for a protective colostomy.

The present article details a new technique for the repair of rectovestibular fistula. Twenty-five patients with rectovestibular fistula, between 13 days and 4 years of age underwent surgical correction by transfistula anorectoplasty (TFARP). The technique, described in detail, involves mobilization of the fistula and the rectum through the fistula and creation of a new anus in the anatomically normal site by preserving both the perineal skin bridge (skin between the neo-anus and the posterior fourchette) and the levator muscle. The mean operating time was 85 min, and the mean hospital stay was 5 days. Moderate anal stenosis developed in 1 patient and was treated successfully by anal dilatations using Hegar dilators. A diverting colostomy was not required in any patient, and none of the patients developed rectal prolapse. Eleven patients who are now 3 years of age or older have voluntary bowel movements with good fecal continence scores. The 14 neonates and infants, who are still too young to be evaluated for continence, have symmetric anal contraction to stimulation and strong squeeze on digital rectal examination. The average number of bowel movements per day was three to five, without the need for any laxative or enema. Transfistula anorectoplasty is a simple surgical procedure that does not divide the levator muscle or the perineal body. Preservation of these structures contributes significantly toward improvement of the aesthetic appearance of the perineum and of fecal continence.

Tracheal agenesis is an extremely rare, typically fatal congenital tracheal malformation. Lack of prenatal symptoms and emergent presentation usually lead to a failure to arrive at the correct diagnosis and manage the airway properly before the onset of irreversible cerebral anoxia. We report the case history of an infant born with immediate respiratory failure who was diagnosed with tracheal agenesis. The clinical presentation, embryology, classification, and surgical management are discussed.

We report the case of a 6-year-old boy with a progressively enlarging mass in the anterior abdominal wall. Computed tomography showed an intensely enhancing heterogeneous lesion arising from the anterior abdominal wall muscles with intraperitoneal extension. Based on the location of the tumor and the radiological findings we made a provisional diagnosis of rhabdomyosarcoma. However, the findings of fine-needle aspiration and Trucut biopsy were inconclusive for malignancy. We performed complete excision of the mass, including the involved left costal segment. Microscopically, the tumor was composed mainly of spindle-shaped cells without malignant features, and immunohistochemical analysis revealed positive staining for actin. These findings confirmed a diagnosis of an inflammatory myofibroblastic tumor (IMT). To our knowledge, this is the first case report of an extrapulmonary IMT arising from the anterior abdominal wall.

We analyzed the methods and outcomes of urethroplasty in men with complex urethral disruptions. The medical records of 40 men with complex urethral disruptions were analyzed. Surgical methods were individualized according to stricture location severity and length of the stricture bladder neck characteristics and presence of complicating factors. Patientswere divided into four groups based on the above characteristics. End-to-end urethroplasty performed in six patients with short bulbar strictures ( 6 cm). Abdominal transpubic repair was successfully applied to patients with rectourethral fistula or lacerated bladder neck. Success rate of anastomotic urethroplasty was 95% while over all success rate was 85%. Guidelines for urethral reconstruction of complex urethral disruptions are predicated on stricture length location bladder neck characteristics and associated complicating factors. End-to-end urethroplasty with stricture excision is highly reliable for short strictures for which previous operative repair have failed. Elaborated perineal repair is extremely versatile for intermediate and longer strictures with associated complicating factors. Abdominal transpubic urethroplasty is effective for patients with rectourethral fistula or lacerated bladder neck. (authors)

Background: Doppler studies of splanchnic vessels have demonstrated alteration in blood flow in bowel obstruction and strangulation. The aim of this study was to evaluate hemodynamic changes in celiac artery (CA), superior mesenteric artery (SMA) and inferior mesenteric artery (IMA) using pulsed Doppler sonography (PDS) in Hirschsprung’s disease. Material and Methods: Fasting splanchnic flowmetry of CA, SMA, and IMA arteries was performed using PDS preoperatively in 13 patients with Hirschsprung’s disease and 13 healthy age- and sex-matched controls. Diagnostic workup for Hirschsprung’s disease included a barium enema and a rectal biopsy. A primary transanal pull through was performed if the transition zone was at rectosigmoid or midsigmoid. Doppler studies were repeated on the 1st and 7th postoperative day under similar conditions. Mean flow velocity (Vmean) and the pulsatility index (PI) of the three major vessels was measured. Results: Patients with Hirschsprung’s disease showed increased blood flow velocities in CA, SMA, and IMA (p < 0.001), an increased resistance to blood flow in IMA (p < 0.001) and a decreased resistance to blood flow in CA and SMA (p < 0.005 and p < 0.001, respectively). The blood flow velocity for IMA normalized after resection of the aganglionic segment (r = 0.41, p < 0.005, 95% CI: 45.4–52.7). Conclusions: Hirschsprung’s diseaseis associated with alterations in splanchnic vessel hemodynamics which are reversible after corrective surgery. Doppler studies may play an important role in the assessment of bowel function after surgery.

We present our results of and operative principles essential for a successful outcome of complex posterior urethral disruption management.A total of 25 patients underwent abdominal transpubic perineal urethroplasty for complex posterior urethral disruption. Preoperative voiding cystourethrogram with retrograde urethrogram and cystourethroscopy were done to evaluate the stricture and bladder neck. Followup consisted of symptomatic and radiological assessment.Patient age was 22 to 57 years. Average followup was 24 months (range 11 to 39). Four patients had previously undergone failed perineal urethroplasty. A rectourethral fistula was present in 8 patients, of whom 2 required colonic diversion, while there were false passages in 3, a periurethral cavity with abscess in 6 and bladder neck laceration in 20. Mean stricture length +/- SD was 6.5 +/- 2.5 cm (range 4 to 9). Four of the 25 patients had previously undergone failed perineal urethroplasty. The mean period between original trauma/failed repair and definitive repair was 11.5 +/- 4.4 months. Urethroplasty could be achieved through the normal subpubic route in 19 patients, while 6 required supracrural rerouting. A total of 20 patients underwent simultaneous bladder neck repair. In 24 of 25 patients (96%) postoperative cystourethrography showed a wide, patent anastomosis. Postoperatively incontinence developed in 1 of 25 patients (4%). Ten of the 25 patients (40%) were impotent after the primary injury. Potency status in our patients did not change after urethroplasty. The overall urethroplasty success rate was 92%.Hostile conditions in the perineum of patients with complex posterior urethral disruption mitigate against a good result. However, the safety and success of combined abdominal transpubic perineal urethroplasty make it the procedure of choice for these difficult strictures.

Object. Spinal cord dysfunction is associated with an altered neuronal metabolism. The objective of this study is twofold: 1) to compare pre- and postoperative levels of cerebrospinal fluid (CSF) metabolites in patients with spinal dysraphism and in control patients by performing proton magnetic resonance spectroscopy; and 2) to evaluate the use of magnetic resonance (MR) spectroscopy in the assessment of surgical outcomes in patients with spinal dysraphism. Methods. The study group population was composed of patients with meningomyeloceles, lipomeningomyeloceles with tethered cord syndrome, and tethered fatty fila. All patients underwent preoperative clinical and neuroimaging (ultrasonography or MR imaging) examinations and MR spectroscopy analysis of metabolites in their CSF. Excision of the neural placode and detethering of a low-lying cord were performed with or without laminectomy. Two months postoperatively, the investigations were repeated. A comparison of pre- and postoperative CSF metabolites was performed using the Wilcoxon signed-rank test and nonparametric tests. Probability values less than 0.05 were considered significant. High levels of lactate (Lac), alanine (Ala), acetate, glycerophosphorylcholine, and choline were observed in the CSF of patients with spinal dysraphism before surgery; after surgery these levels normalized to those observed in control patients. Patients in whom cord retethering occurred could be identified by increased concentrations of Ala and Lac. Conclusions. The results highlight the potential of MR spectroscopy as a promising tool in the assessment of surgical outcomes in patients with spinal dysraphism.

We present our short-term results of abdominal transpubic perineal urethroplasty for complex posterior urethral disruption.From January 2000 to March 2005, 21 patients with complex posterior urethral disruption underwent abdominal transpubic perineal urethroplasty. Complex disruption was defined as stricture gap exceeding 3 cm or associated perineal fistulas, rectourethral fistulas, periurethral cavities, false passages, an open bladder neck or previous failed repair. Preoperative voiding cystourethrogram with retrograde urethrogram and cystourethroscopy were done to evaluate the stricture and bladder neck. Followup consisted of symptomatic assessment and voiding cystourethrogram.There were 11 adults and 10 prepubescent boys with an average age of 26 years (range 6 to 62). Mean followup +/- SD was 28 months (range 9 to 40). Mean stricture length was 5.2 +/- 1.4 cm. Of the 21 patients 12 had previously undergone failed urethroplasty. The mean period between original trauma/failed repair and definitive repair was 10.2 +/- 4.3 months. Urethroplasty was achieved through the subpubic route in 16 patients, while 5 required supracrural rerouting. In 20 of 21 patients (95%) postoperative cystourethrography showed a wide, patent anastomosis. Postoperative incontinence developed in 2 of 21 patients (9.5%). Seven of the 21 patients (33%) were impotent after the primary injury, while 3 of 14 (21.4%) had impotence postoperatively. There were no complications related to pubic resection, bowel herniation or periurethral cavity recurrence.Combined abdominal transpubic perineal urethroplasty is a safe procedure in children and adults. It allows wide exposure to create a tension-free urethral anastomosis without significantly affecting continence or potency. Complications of pubic resection are now rarely seen.

Background: Epidermal inclusion cysts very rarely occur in the midline in the area of the anterior fontanelle. Case Description: We report the second case of epidermal inclusion cysts in the midline in the area of the anterior fontanelle in world literature of a 7-month female infant who presented with a slow-growing soft mass located in the midline over the vertex of the head. Conclusion: These lesions with clear contents can have all the features of encephalocele on clinical examination, and imaging will show the exact location, but histopathology will confirm the diagnosis.

Ganglioneuroma is a rare benign tumor, usually seen in children and young adults, arising in the central nervous system. Ganglioneuroma of the mesentery is extremely rare; only one case has been recorded. We report the second case of a primary mesentery ganglioneuroma. The patient underwent surgical intervention for diagnostic and therapeutic purposes.

Background and Introduction Constipation following posterior sagittal anorectoplasty (PSARP) is common. We correlated the dimensions of rectal pouch before PSARP with the postoperative bowel habit. Classical PSARP was modified with tapering of rectal pouch by plication of its walls thus preserving the internal sphincter because we believe that this preserves continence and lead to better results. It was observed that a distinct relationship exists between the preoperative size of the rectal pouch and constipation. Aim The aim of this study is to correlate the dimensions of preoperative rectal pouch with postoperative constipation. Materials and Methods PSARP was performed (n: 45) in anorectal malformations using an indigenous muscle stimulator. Before PSARP, a distal cologram via high sigmoid colostomy was performed. All the distal cologram were performed by a single senior radiologist and the pressure was kept constant between 15 and 20 cm of water while filling to rule out the confounding factor related to incomplete filling. Rectum index was calculated as follows: The maximum radiological diameter of the rectum within the pelvis in the sagittal plane was multiplied by the maximum diameter of the rectum in the frontal plane. The result of this calculation was divided by the product of multiplying the distance between the ischial spines and the distance between the posterior surface of the pubic symphysis and the anterior surface of the last sacral vertebrae. Results Symptomatic constipation requiring treatment developed in 25 patients (48%). None of these patients had anal stenosis or stricture. Constipation was managed by dietary measures and laxatives. Fifteen patients (60%) had grade 1 constipation and responded favorably. Eight and two patients had grades 2 and 3 constipation, respectively. Those patients who had a rectal pouch index of less than 0.8 had mild constipation grades 0 and 1, whereas those in whom the rectal pouch index was more than 0.8 had severe degrees of constipation (grades 2 and 3). Conclusion Measuring the rectal pouch index can help in identifying the group which is likely to develop constipation after PSARP. These patients can be put on bowel training early on, after the colostomy closure, instead of waiting.

Gunshot wounds to the head are usually mortal injuries. We present a unique case of intracranial ricocheting of bullet without neurological deficits. Patient was treated conservatively with antibiotics for one week and prophylactic anticonvulsants for six weeks. Patient is doing well at six months follow up. Repeat X-ray skull showed that bullet was lying in the occipital region. It is recommended that deep seated bullets should be left behind as any attempt to remove that bullet may increase the morbidity and mortality. However close follow up of these patients is very important as these patients may come back with brain abscess.JNMA J Nepal Med Assoc. 2008 Jul-Sep;47(171):145-146.

Background This report describes a new technique of sphincter saving anorectoplasty (SSARP) for the repair of anorectal malformations (ARM). Methods Twenty six males with high ARM were treated with SSARP. Preoperative localization of the center of the muscle complex is facilitated using real time sonography and computed tomography. A soft guide wire is inserted under image control which serves as the route for final pull through of bowel. The operative technique consists of a subcoccygeal approach to dissect the blind rectal pouch. The separation of the rectum from the fistulous communication followed by pull through of the bowel is performed through the same incision. The skin or the levators in the midline posteriorly are not divided. Postoperative anorectal function as assessed by clinical Wingspread scoring was judged as excellent, good, fair and poor. Older patients were examined for sensations of touch, pain, heat and cold in the circumanal skin and the perineum. Electromyography (EMG) was done to assess preoperative and postoperative integrity of external anal sphincter (EAS). Results The patients were separated in 2 groups. The first group, Group I (n = 10), were newborns in whom SSARP was performed as a primary procedure. The second group, Group II (n = 16), were children who underwent an initial colostomy followed by delayed SSARP. There were no operative complications. The follow up ranged from 4 months to 18 months. Group I patients have symmetric anal contraction to stimulation and strong squeeze on digital rectal examination with an average number of bowel movements per day was 3–5. In group II the rate of excellent and good scores was 81% (13/16). All patients have an appropriate size anus and regular bowel actions. There has been no rectal prolapse, or anal stricture. EAS activity and perineal proprioception were preserved postoperatively. Follow up computed tomogram showed central placement the pull through bowel in between the muscle complex. Conclusion The technique of SSARP allows safe and anatomical reconstruction in a significant proportion of patients with ARM's without the need to divide the levator plate and muscle complex. It preserves all the components contributing to superior faecal continence, and avoids the potential complications associated with the open posterior sagittal approach.

Background/Purpose The aim of this study was to evaluate the feasibility, results, and cost-effectiveness of totally transanal endorectal pull-through (TEPT) in the management of rectosigmoid and midsigmoid Hirschsprung's disease (HD) in a low-income country. Methods Between March 2004 and December 2005, 19 children underwent totally TEPT procedure. The patients' ages ranged from 6 days to 13 years. The primary diagnosis in all 19 patients was HD confined to the rectosigmoid region in 15 and midsigmoid in 4. None had a preoperative colostomy. Follow-up period ranged from 4 to 20 months (mean, 8 months). Results Ages ranged from 0.25 to 65 months, with a mean of 16.24 months. Weights ranged from 3.4 to 13 kg, with a mean of 6.5 kg. Mean time from diagnosis to pull-through procedure was 26 days (range, 6-39 days). The mean length of rectosigmoid resection was 30 cm (range, 20-50 cm). The mean operative time was 95 minutes (range, 75-140 minutes). Mean intraoperative blood loss was 25 mL (range, 15-40 mL). There was one death unrelated to the procedure. One patient had enterocolitis 3 months postoperatively. Average frequency of defecation was 3 (range, 1-6) stools per day. TEPT was associated with a shorter operating time, less blood loss, early return to feeds, and an overall reduced cost. Conclusion The safety and cost-effective benefits of transanal endorectal pull-through in the treatment of HD are of special interest for a developing country. Our data also suggest that functional outcome following TEPT is highly satisfactory and comparable with other established procedures.

Background/Purpose Short bowel syndrome (SBS) is a global malabsorption syndrome that results from extensive intestinal resection. We report our experience of 10 neonates with short bowel managed by a novel triple tube enteral feeding technique. The aim of this research was to provide justification for enteral nutritional strategies to enhance intestinal adaptation, especially in developing countries where parenteral nutrition is either unavailable or expensive. Methods From March 2004 to January 2006, 10 neonates underwent extensive bowel resection, enterostomy, and mucous fistula for necrotizing enterocolitis or midgut volvulus and were managed postoperatively with triple tube enteral feeding technique. Gestational age, birth weight, primary abdominal pathology, timing of surgery, surgical procedure performed, complications, duration and feasibility of refeeding, and weight gain were recorded. Results The group was composed of 8 male and 2 female neonates with a mean gestational age of 34.2 ± 4.6 weeks and mean birth weight of 2580 ± 993 g. Necrotizing enterocolitis accounted for 7 (70%) and midgut volvulus accounted for 3 (30%) cases of SBS. Mean gestational age at surgery was 35.5 ± 2.2 weeks. Mean residual small bowel length and colon length after resection were 35.5 ± 3.5 and 30.5 ± 1.5 cm, respectively. Weight gain during refeeding ranged from 3 to 6 g/kg per day with duration of refeeding lasting 20 to 156 days. Reanastomoses was done 92 ± 4.2 days after the primary surgery. There were 3 surgery-related complications, but no mortality. Mean period of follow-up was 12 ± 2.4 months. Five of the 10 children are now 19 months old and have a body weight of −1.5 ± 0.64 SD scores and height of −1.75 ± 0.99 SD scores. Conclusion This technique represents a safe and effective enteral nutrition strategy that eliminates the need for total parenteral nutrition for SBS in developing countries.

Background A standard contrast enema for Hirschsprung's disease can sometimes be inconclusive in delineating a transition zone especially in neonates and infants. The aim of this study was to determine the utility and diagnostic accuracy of a plain abdominal radiograph transition zone (PARTZ) in predicting the level of aganglionosis. Methods A prospective observational study of neonates and infants with biopsy proven Hirschsprung's disease was carried out from March 2004 through March 2006. All patients underwent a plain abdominal radiograph and a contrast enema followed by a rectal biopsy. The transition zone on a plain radiograph (PARTZ) and contrast enema (CETZ) were compared with operative and pathology reports. Results were analyzed by chi square test and expressed as their p values and 95% confidence intervals. Results PARTZ and CETZ suggestive of Hirschsprung's disease was seen in 24(89%) and 18(67%) patients respectively. The PARTZ and CETZ matched with the pathologic level of transition zone in 22(92%) and 13(72%) patients, p = 0.001, 95% CI (-1.87 to -0.79). In the 9 (33%) patients in whom contrast enema failed to reveal a transition zone, PARTZ was seen in 6/9(66%) patients and correlated with the pathological level of aganglionosis in 4/6(67%) patients, p = 0.001 95% CI (-1.87 to -0.79). The overall accuracy of PARTZ and CETZ was 96% and 84% respectively, p = 0.008, 95% CI (-6.09 to -3.6). Conclusion A plain abdominal radiographic transition zone is reliable in predicting the level of transition zone in cases of inconclusive contrast enema. It may be particularly helpful developing countries where laparoscopic techniques are not available to accurately identify the transition zone.

We report a case of ileal atresia (IA) caused by an omphalic ring closure anomaly. A 2-day-old male neonate started vomiting bile, accompanied by abdominal distention. Laparotomy revealed that the distal part of the ileum was entrapped within the omphalic ring and that this entrapped segment of ileum was atretic. To our knowledge, this potential mechanism of IA has not been described before.

An 18-day-old female neonate presented with abdominal distention and bilious vomiting for 5 days. Abdominal examination showed hepatomegaly and a mass in the right hypochondrium. Ultrasound showed an extrahepatic cyst with internal echoes and dilated intrahepatic radicals. A contrast computer tomography showed a large cyst arising from the falciform ligament, extending into the liver parenchyma and a dilated portal venous system. Diagnosis of falciform ligament abscess with portal pyemia was made. Excision of the falciform ligament was done. After a follow up of 1 year, the child remains asymptomatic. To our knowledge, a falciform ligament abscess causing intrahepatic portal pyemia in a newborn has not been previously been described.

Juvenile polyposis syndrome is an uncommon hamartomatous disorder with gastrointestinal (GI) manifestations of varying degree and malignant potential. We report the cases of an 8-year-old girl and a 5-year-old girl who suffered massive lower GI hemorrhage. Neither patient had a family history of polyposis. After the patients were stabilized, radiological evaluation, laparotomy, and intraoperative colonoscopy revealed multiple polyps in the colon. Both patients underwent total colectomy, mucosal proctectomy, and ileoanal anastomosis. The diagnosis of nonfamilial juvenile polyposis was based on the histological findings and the absence of a family history. To our knowledge, this presentation of juvenile polyposis has been reported only twice before. We discuss the clinical features and diagnosis of juvenile polyposis and the treatment options. Although juvenile polyposis is a rare condition in children, it should be considered in the differential diagnosis of life-threatening GI hemorrhage.

Chronic otitis media although rare in developed countries is still a major problem in the developing countries. If untreated, it can lead to life-threatening intracranial complications. In the available literature the incidence and risk factors for cerebellar abscess following mastoidectomy is not known. In this case we would like to emphasize that CT scan before mastoidectomy is not possible in all the cases particularly in developing countries. There is a need to find the factors that will make one suspect the possibility of concurrent intracranial lesions and which will identify the patients prone to intracranial complications.

A 15-day male neonate presented with postprandial nonbilious vomiting. Barium meal suggested pyloric obstruction. Ultrasound of the stomach after saline loading revealed an echogenic antral valve. Subsequent laparotomy confirmed the ultrasound findings. Excision of the valve resulted in excellent recovery. To our knowledge, gastric outlet obstruction because of an antral mucosal valve in a neonate has not previously been described.

A 2-year-old male is described who presented with fever, fixed flexion deformity of the right hip and a tender mass in the right iliac fossa. A clinical diagnosis of psoas abscess was made. Abdominal ultrasound was suggestive of an echogenic retroperitoneal cyst but could not accurately determine the nature of the cyst. A contrast CT scan showed a retroperitoneal cyst. At laparotomy, a large retroperitoneal cyst containing turbid fluid was found and was excised completely. Histopathological examination was suggestive of cystic lymphangioma. Clinical presentation of retroperitoneal lymphangioma may be variable and misleading, therefore complex imaging studies are necessary in the evaluation of this condition.

Malignant peripheral nerve sheath tumors (MPNSTs) are the leading cause of death in young adults and are one of the most frequent non-rhabdomyosarcomatous soft tissue tumors in pediatric age. These tumors usually occur in young adults from a previously recognized neurofibroma, neurofibromatosis type 1 (NF1), with a noted change in size and pain. This child reached the age of 10 without the presence of the more commonly seen manifestations of NF1. Pseudoarthrosis in children has a high rate of association with NF1, and in this case diagnosis of NF1 was supported by development of MPNST in a pre-existing plexiform neurofibroma.

Purpose: A significant number of children with posterior urethral valves (PUVs) have chronic renal failure due to tubulointerstitial damage. Activation of the renin-angiotensin system is known to inflict this injury. We investigated the role of plasma renin activity (PRA) in patients with PUVs and sought to establish a relationship between renal damage and PRA. Materials and Methods: The records of patients with PUVs were reviewed regarding the time of valve ablation, serum creatinine, scars, grade of reflux and glomerular filtration rate (GFR). PRA was measured before and after valve ablation. Results: A total of 25 patients had PUVs (mean age 3.2 ± 2.4 years, mean period of observation 4.5 ± 1.2 years). Mean PRA was increased before valve ablation in all patients irrespective of age, and decreased after ablation. Of 25 patients 14 (56%) had renal damage, with a mean PRA of 36 ± 4.5 ng/ml per hour compared to 26 ± 8.2 ng/ml per hour in patients with normal renal function (chi-square 4.2 p = 0.01). Of the 14 patients with renal damage 9 (64%) had normal GFR at age 1 year but increased PRA. Of these 14 patients the accepted criteria for renal damage of increased serum creatinine, high grade reflux, scars and decreasing GFR were present in only 6 (43%), 3 (21%), 2 (14%) and 4 (29%), respectively. PRA was increased in all 14 cases. Conclusions: PRA was increased before valve ablation and decreased after ablation. It is increased in patients with early renal damage. Our preliminary observations indicate that PRA may be helpful in identifying patients with early renal damage.

To evaluate the role of angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism as a risk factor for progressive renal damage in Asian Indian children with congenital uropathies.ACE I/D polymorphism was determined by polymerase chain reaction in 84 children with congenital uropathies and 80 unrelated healthy controls. The study group included primary vesicoureteral reflux (29 patients), pelviureteral junction obstruction (21) and posterior urethral valves (34). Mean patient age was 69.4 +/- 4.5 months, and mean followup period was 7.2 +/- 1.5 years. Serum creatinine, ultrasound, voiding cystourethrogram and dimercaptosuccinic acid scans were done to evaluate renal function.The ACE I/D genotype distribution was similar in the 84 patients, II in 37 (44%), DI in 30 (35.7%) and DD in 17 (20.2%), and 80 controls, II in 36 (45%), DI in 30 (37.5%) and DD in 14 (17.5%), chi-square 0.00, p = 1.0). Renal scarring was seen in 49 of 84 patients (58.3%), with D allele present in 35 of 49 (71.4%), compared to 12 of 84 patients (34.2%) in the nonscarring group (chi-square 4.2, p = 0.02). Progressive scarring and renal failure were seen in 23 (27.3%) and 26 (31%) of patients, respectively, with D allele present in 21 of 23 (91.3%) and 21 of 26 (81%), respectively (chi-square 5.4, p = 0.001). Multivariate analysis showed that D allele is an independent risk factor for renal damage.The presence of D allele in I/D polymorphism of angiotensin converting enzyme gene is associated with progressive deterioration of renal function in congenital uropathies. The D allele was also significantly associated with renal scarring independent of known risk factors such as grade of reflux, age at diagnosis, gender and urinary tract infection.

But: La tomographie d'emission monophotonique (TEMP) est utilisee pour mesurer le debit sanguin cerebral (DSC) et la reserve vasculaire cerebrale (RVC) apres injection d'acetazolamide (ACZ). Cette etude prospective est realisee pour tester l'utilite de la RVC dans l'hydrocephalie congenitale (HC). Methodes: Vingt-et-un patients (âge moyen 6,2 mois) atteints d'HC ont beneficie d'une ultrasonographie (US), d'un fond de l'oeil (FO), d'un examen clinique pour etablir un score neurologique (SN) et d'un TEMP pour evaluer la RVC apres injection intraveineuse d'ACZ. Un controle (US, FO, SN, DSC, RVC) etait effectue tous les mois. Resultats: La RVC etait satisfaisante chez dix patients qui montraient un SN et un FO stables malgre une ventriculomegalie minime ou moderee. Ces patients etaient suivis avec un traitement conservateur. Aucune deterioration n'etait notee au cours des 9,8 mois de suivi. La RVC initiale etait faible chez onze patients, egalement suivis avec un traitement conservateur. Pendant une periode de quatre mois, huit de ces patients montraient une diminution de la RVC a moins de 30% de la valeur normale et une chute du SN malgre une stabilite du FO et de l'US. Chez cinq de ces enfants, une intervention chirurgicale a permis une amelioration de la RCV et du SN pendant une periode de quatre mois. Aucune amelioration de la RVC ni du SN n'etait observee chez les trois autres. Conclusions: Chez les enfants atteints d'hydrocephalie congenitale, le mesure de la RVC par TEMP apres injection d'ACZ est un moyen sensible d'evaluation car les alterations de la RVC precedent celles des autres parametres conventionnels - ventriculomegalie et FO. Il y a peu de deterioration chez les patients avant und RVC normale au moment du diagnostic, permettant un traitement conservateur. En cas de deterioration de la RVC, une intervention chirurgicale est a envisager rapidement.