Malformation Repair Research Articles

Comparison of postoperative urinary complications in laparoscopic-assisted anorectoplasty versus posterior sagittal anorectoplasty for anorectal malformation with rectourethral fistula.

Long-term urinary outcomes after anorectal malformation (ARM) repair are affected by surgical approach and sacral anomalies. This study aimed to compare laparoscopic-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) in terms of urinary complications. Between 2001 and 2022, 45 patients were treated with LAARP or PSARP. The rectourethral fistula and inflow angle between the fistula and rectum was confirmed by preoperative colonography. The incidence of urinary complications and treatment were compared between the two groups. Four patients (14%) had remnant fistula and five patients (17%) had neurogenic bladder dysfunction in LAARP group, while three patients (18%) had urethral injury in PSARP group. All patients with remnant fistula were asymptomatic and followed without treatment. The incidence of remnant fistula improved between earlier decade and later decade. In all cases with urethral injury, suture repair was performed and no postoperative leakage was noted. All five patients with neurogenic bladder dysfunction had spine abnormalities that required clean intermittent catheterization (CIC) and two were free from CIC finally. It is important to check inflow angle preoperatively to prevent remnant fistula. For PSARP, meticulous dissection is required when separating fistula from urethra because they create common wall. The most contributing factor to neurogenic bladder is sacral anomalies. Preoperative evaluation and postoperative urinary drainage are important.

Functional Outcomes of Patients Who Underwent Anorectal Malformation Repair Using MRI Guidance

BackgroundDespite the initiation of minimally invasive laparoscopic techniques, the majority of patients who undergo anorectal malformation repair still experience functional bowel issues in childhood, including constipation and fecal incontinence. In this study, we evaluate the functional outcomes of a procedure in which magnetic resonance imaging guidance is used during initial laparoscopic repair to better locate the epicenter of the sphincter muscle complex and pelvic floor with the goal of more accurate placement of the neoanus and improved functional outcomes. MethodsA retrospective chart review evaluated demographic, operative, and outcome details for patients who underwent this procedure. A telephone survey was employed to determine levels of social continence using the validated Baylor Continence Scale and to determine what type of bowel management is used. ResultsTwenty-six patients were included. Median age at operation was 7 months, and median age at follow-up was 4 years old, with a range of 1–9. Bowel management regimen results revealed that 19 % (n = 5) use no bowel management regimen, 58 % (n = 15) use laxatives only, and 23 % (n = 6) use enemas. Enema use was not associated with different spine or sacral anomalies (p = 0.77). Fifteen patients (58 %) answered the Baylor Continence Scale questions and had a median score of 14. No difference was found in scores when accounting for lesion level (p = 0.43), quality of needle placement (p = 0.46), or quality of sphincter muscles (p = 0.75). ConclusionsUsing MRI guidance in the repair of anorectal malformations shows promise in both the qualitative and quantitative functional outcomes of this complex patient population. Level of EvidenceLevel III.

Post-Operative Anorectal Manometry in Children following Anorectal Malformation Repair: A Systematic Review.

Despite surgical correction, children with anorectal malformations may experience long-term bowel dysfunction, including fecal incontinence and/or disorders of evacuation. Anorectal manometry is the most widely used test of anorectal function. Although considerable attention has been devoted to its application in the anorectal malformation cohort, there have been few attempts to consolidate the findings obtained. This systematic review aimed to (1) synthesize and evaluate the existing data regarding anorectal manometry results in children following anorectal malformation repair, and (2) evaluate the manometry protocols utilized, including equipment, assessment approach, and interpretation. We reviewed four databases (Embase, MEDLINE, the Cochrane Library, and PubMed) for relevant articles published between 1 January 1985 and 10 March 2022. Studies reporting post-operative anorectal manometry in children (<18 years) following anorectal malformation repair were evaluated for eligibility. Sixty-three studies were eligible for inclusion. Of the combined total cohort of 2155 patients, anorectal manometry results were reported for 1755 children following repair of anorectal malformations. Reduced resting pressure was consistently identified in children with anorectal malformations, particularly in those with more complex malformation types and/or fecal incontinence. Significant variability was identified in relation to manometry equipment, protocols, and interpretation. Few studies provided adequate cohort medical characteristics to facilitate interpretation of anorectal manometry findings within the context of the broader continence mechanism. This review highlights a widespread lack of standardization in the anorectal manometry procedure used to assess anorectal function in children following anorectal malformation repair. Consequently, interpretation and comparison of findings, both within and between institutions, is exceedingly challenging, if not impossible. Standardized manometry protocols, accompanied by a consistent approach to analysis, including definitions of normality and abnormality, are essential to enhance the comparability and clinical relevance of results.

Effectiveness of Sufentanil-Based Patient-Controlled Analgesia Regimen in Children and Incidence of Adverse Events Following Major Congenital Structure Repairs.

The aim of this study was to evaluate postoperative analgesia effectiveness and patient-controlled analgesia (PCA)-related adverse events. The children who received sufentanil-based PCA following major surgery to repair congenital hip dislocation and hypospadias were divided into 3 groups: sufentanil 4 µg/kg + tramadol 10 mg/kg (ST; n = 301), sufentanil 4 µg/kg (S4; n = 211), and sufentanil 5 µg/kg (S5; n = 451). Analgesics with granisetron 0.2 mg/kg were diluted in 0.9% saline to 100 mL and infused continuously at a basal infusion rate of 1mL/h. A total of 963 children whose average age was 4 years were investigated. The incidence of moderate to severe postoperative pain during rest was significantly lower in the S5 group (0.2%) and ST group (0.3%) compared with the S4 group (3.3%) within 72 hours after surgery (P < .05), and the incidence of moderate to severe pain during activity within 72 hours was also significantly lower in the S5 group (3.5%) compared with the other 2 groups (ST, 21.9%; S4, 33.2%; P < .001). A significant difference was identified among the 3 groups in the total number of PCA administrations, but there was no statistical difference of total usage of opioids among the 3 groups. In terms of PCA-related adverse events, the incidence of postoperative nausea and vomiting in the S5 group was lower compared with the ST group within 72 hours after surgery (P < .001), and there was no significant difference compared with the S4 group (P = .254). There was also no significant difference in the incidence of pruritus and dizziness among the 3 groups. Sufentanil-based PCA can be effectively and safely used in children after major congenital structural malformation repair surgeries. The sufentanil 5 µg/kg dosage in the PCA regimen is superior to sufentanil 4 µg/kg alone or combined with tramadol in lowering moderate to severe pain.

Cardiorespiratory performance and locomotor function of patients with anorectal malformations

The aim of this study was to assess whether adolescents following anorectal malformation repair have a decreased cardiorespiratory performance capacity and impaired motor skills. All eligible children treated for ARMs between 2000 and 2014 were invited to participate in a prospective study consisting of a clinical examination, evaluation of Bowel function and Quality of Life, spirometry, spiroergometry and assessment of the motor activity. The results were compared to a healthy age- and sex-matched control group. There was no statistically significant difference in height, weight, BMI, muscle mass or body fat percentage between the study and the control group. Nine out of 18 patients (50%) had an excellent functional outcome with a normal Bowel Function Score. Spirometry revealed no significant differences between ARM patients and controls, four patients showed a ventilation disorder. Spiroergometry revealed a significantly lower relative performance capacity and the overall rating of the motor activity test showed significantly decreased grades in ARM patients. ARM patients were affected by an impaired cardiopulmonary function and decreased motor abilities. Long-term examinations consisting of routine locomotor function evaluation and spiroergometry are advisable to detect impaired cardiopulmonary function and to prevent a progression of associated complications and related impaired quality of life.

Refinements in surgical techniques for visualized tunnel formation in laparoscopic-assisted anorectoplasty

Laparoscopic-assisted anorectoplasty (LAARP) is becoming a more popular procedure for anorectal malformation (ARM) repair. However, the conventional technique for creating pull-through tunnel between the perineal skin and the pelvic floor has been criticized as being semi-blind. This study aims to present a refined version of our previously reported clamp introduction technique for visualized tunnel formation in the center of the sphincter muscle complex (SMC) for rectal pull- through for ARMs. A retrospective review was performed for ARM patients who underwent LAARP from Jan 2019 to Jun 2020. Longitudinal muscle tube (LMT) tunnel was created using the clamp-dilator introduction technique: a laparoscopic dilator was used to create a pelvic tunnel within LMT in high ARM, and the clamp introduction under the direct vision technique was performed for creating the perineal tunnel of LMT for both high and intermediate ARMs. Seventy patients (1-198days) with high-type (27cases) and intermediate-type (43 cases) ARM underwent LAARP using clamp-dilation introduction technique. No patients suffered from urinary tract injury, recurrent rectourethral fistula, urethral diverticulum and urinary incontinence. One patient suffered from wound infection and rectal retraction which required a redo pull-through on postoperative day 7. Rectal prolapse requiring surgical intervention developed in one patient. Postoperative MRI examination confirmed central placement of the rectum within the LMT in all cases. Our experience demonstrates that a visualized tunnel formation in the LMT center can be achieved by the clamp-dilator introduction technique in LAARP for both high and intermediate ARMs.

Surgical management of sporadic hemangioblastomas located in the posterior fossa of brain.

Hemangioblastomas (HBs) are highly vascular tumors, constituting 2%-3% of central nervous system (CNS) tumors. Surgery is a treatment option for HBs. The purpose of this study was to evaluate the demographic, clinical, and radiological findings of patients with HBs who were operated on via microsurgical technique at our clinic and to determine the findings specific to HBs and evaluate their pathological findings and confirmation. We evaluated age, gender, initial complaints and duration of 100 patients (50 female and 50 male patients), presence of cranial nerve deficits, magnetic resonance imaging, data (localization, presence of hydrocephalus, tumor size, enhancement pattern, and cystic and solid component features), resection rates, pathological findings (grade, Ki-67 ratio, and staining results), recurrence, special conditions of patients, and the final status of patients in this study. Mean age was 39.08 ± 14.77 years and 66.7% of the patients presented with cerebellar findings. Five of the cases were located in the brainstem, five were located in the cerebellopontine angle, and two were in the cerebellar cortex. In all patients, gross total resection was performed using arteriovenous malformation repair. Surgical intervention of small and cystic tumors can be performed safely with en bloc resection along with preoperative detailed imaging, improved microsurgical techniques, and an extensive understanding of anatomical and vascular structures. On the contrary, patients with a brainstem invasion and solid structure present surgical difficulties due to arteriovenous malformation-like vascularizations; therefore, gross total resection is the optimal treatment in HBs.

A New Approach to Cloaca: Laparoscopic Separation of the Urogenital Sinus.

Introduction: Cloaca malformation repair strategy is strongly dictated by common channel and urethral lengths. Mid to long common channel cloacas are challenging and often require laparotomy for dissection of pelvic structures. The balance of common channel and urethral lengths often dictates the approach for reconstruction. Laparoscopy has been utilized for rectal dissection but not for management of the urogenital (UG) structures. We hypothesized that laparoscopy could be applied to UG separation in reconstruction of cloaca malformations. Methods: Records were reviewed for 9 children with cloaca who underwent laparoscopic rectal mobilization and UG separation. Clinical parameters reviewed included demographics, relevant anatomic lengths, operative duration, transfusion requirements, and perioperative complications. Results: Repair was perfomed at a median (interquartile range) age of 12 (7, 15) months. Common channel length as measured by cystoscopy was 3.5 (3.3, 4.5) cm. There were no intraoperative complications. Transfusion requirements were minimal. Postoperative length of stay was 6 (5, 11) days. One patient developed a urethral web and 2 developed vaginal stenosis. One patient later underwent a laparotomy for obstruction due to a twisted rectal pull-through. Conclusions: Laparoscopic rectal mobilization and UG separation in long common channel cloaca are safe and well tolerated. Laparoscopy affords full evaluation of Mullerian structures and enables separation of the common UG wall, which may ultimately enhance long-term urinary continence.

Perioperative blood product transfusion of two different perfusion strategies on pediatric patients undergoing aortic arch surgery.

Simple regional cerebral perfusion (SRCP) or cerebro-myocardial perfusion (CMP) is selectively used in one-stage complex aortic arch malformation repair. This analysis was performed to investigate the effect of CMP and SRCP on perioperative blood product consumption, and to evaluate whether these two strategies have different effects on the clinical outcomes. A retrospective analysis of 284 children with complicated aortic malformation from January 2010 to June 2018 was performed. The overall cohort was divided into SRCP group (n=202) and CMP group (n=82). A comprehensive comparison of perioperative blood product consumption-related indexes was performed. Cardiopulmonary bypass time, cardiac arrest time, cooling, and rewarming time in the CMP group were significantly shorter than those in the SRCP group (P <.05). Chest tube time was 3.82±1.33days in the SRCP group compared to 3.42±0.97days in the CMP group (P = .005). Moreover, intraoperative platelet (PLT) transfusion volume (mL/kg) and rate (%) were significantly lower in the CMP group (P < .001). Multivariate regression analysis found that intraoperative PLT transfusion was significantly negatively correlated with CMP management [OR=0.237 (0.110-0.507), P < .001] and CPB time was independently associated with delayed chest tube removal (>3days) [OR=1.010 (1.001-1.020), P = .031]. In-hospital mortality and early postoperative adverse events were not significantly different between the two groups. In children with on-pump complex aortic arch surgeries, CMP is more preferable than SRCP in blood protection. However, overall prognosis was not remarkably different between these two perfusion groups. They are both safe and feasible.

The effectiveness of digital anal dilatation in preventing anal strictures after anorectal malformation repair

BackgroundAnal dilatation is performed after repair of anorectal malformations(ARMs) and is vital in prevention of anal stricture formation. The ideal protocol utilizes Hegar dilators. In our setup, few parents will acquire Hegar dilators for home based anal dilatation but majority will use fingers as an alternative. The basis of this study was to determine the effectiveness of digital anal dilatations in preventing anal strictures. Materials and methodsThis was a descriptive cross-sectional study carried out at Kenyatta National Hospital (KNH) which recruited children post ARM repair undergoing digital anal dilatation. The diameters of the digits used for anal dilatation were assessed for adequacy using the desired anal size as a reference point. The main outcome variable was the presence of anal strictures. Data were entered in a data collection sheet and analyzed by use of SPSS (V.21.0 Chicago, Illinois). Chi-square test and Fisher's exact test were used to ascertain association among variables. A P-value of less than 0.05 was considered statistically significant. Results50 patients with ARM post repair were recruited in the study. The rate of occurrence of anal stricture was 22%. Among the participants with anal strictures, only 27% were compliant to the dilatation regimen while 73% reported noncompliance. The mean diameter(mm) of the dilating digits at the level of the middle phalanx was Index finger (15.4 ± 0.58), middle finger (15.2 ± 0.61), ring finger (14.2 ± 0.71), small finger (12.5 ± 0.91) and thumb (17.6 ± 0.78). 46% of the participants were dilated with appropriately sized digits. The remainder had dilatations done with larger (22%) or smaller digits (32%). Notably, anal strictures occurred in participants who were dilated with inappropriately sized digits (P = 0.001). ConclusionDigital anal dilatation is safe and effective as a mode of anal dilation in the setting of compliance to a strict dilatation protocol, daily frequency of dilations and use of appropriately sized digits. Owing to the variability in the finger size, we recommend that digits be calibrated and education given to the caregivers on the use of the appropriately sized digit. Level of evidenceTreatment study.Level III.

Prevalence of Active Long-term Problems in Patients With Anorectal Malformations: A Systematic Review.

Anorectal malformations are a spectrum of congenital anomalies of the rectum with high infantile survival rates and variable outcomes. Long-term (>10 years old) active problems associated with this condition have been poorly investigated. The purpose of this review was to systematically define the prevalence of the most common active long-term problems in patients with a history of anorectal malformation repair. MEDLINE, EMBASE, and the Cochrane Library were searched electronically using the OVID search platform. Original articles from August 1, 1994, to October 20, 2015, that included outcome data for patients aged ≥10 years with anorectal malformation. Cloaca was excluded from the study. Prevalence estimates of anorectal malformations were obtained from published articles. CIs were ascertained in the logit scale after transforming prevalence into log odds and were then transformed into the original scale. The same method was used for subgroup analysis investigating high and low anorectal malformations. The overall prevalences of fecal, urinary, and sexual dysfunction were analyzed. Twelve studies including 455 patients with a history of anorectal malformation repair were included for analysis. The range of reported prevalence of long-term active problems was as follows: fecal incontinence, 16.7% to 76.7%; chronic constipation, 22.2% to 86.7%; urinary incontinence, 1.7% to 30.5%; ejaculatory dysfunction, 15.6% to 41.2%; and erectile dysfunction, 5.6% to 11.8%. The study was limited by its retrospective, small size; multiple complex associated anomalies often not reported; and heterogeneous composition of patients with limited stratification analysis. There is an overall high prevalence of active long-term issues in adolescents and young adults with anorectal malformations. Additional multicenter research is needed to define characteristics and predictors of long-term outcome, to implement effective follow-up, and to transition to adult health care.

Effects of Reconstructive Surgery on Bladder Function in Patients with Anorectal Malformations

Bladder dysfunction is common in patients with anorectal malformations and can be congenital or acquired as a consequence of surgery. We investigated the effects of surgical correction of anorectal malformations on bladder function. The charts of all 341 patients who underwent surgery atour center between 1990 and 2010 were retrospectively analyzed for preoperative and postoperative videourodynamics. A total of 52 patients were eligible for study inclusion. Each assessment was scored according to International Children's Continence Society standards. Urodynamic study indicated normal bladder function preoperatively in 36 patients (69%) and postoperatively in 37 (71%). Median bladder emptying efficiency and relative bladder capacity changed significantly after posterior sagittal anorectoplasty. Bladder function according to International Children's Continence Society standards did not change postoperatively in 43 patients (83%). In 1 of 4 patients with deterioration of bladder function the deterioration could be attributed solely to surgery. Clinical outcome was available in 38 patients and showed complete urinary continence with spontaneous voiding in24(63%). Seven of 25 patients (28%) with preoperative videourodynamics indicating normal bladder function demonstrated dysfunctional voiding at latestfollowup. Urodynamic and clinical outcomes after anorectal malformation repair are good, with 63% of patients being continent of urine. Urodynamic studies are of limited value in preoperative settings in these patients. Current techniques of reconstructive surgery for anorectal malformations seem to preserve bladder function in the majority of patients.

STRATOS™ system for the repair of pectus excavatum

Open techniques represent a valid repair option for severe asymmetric pectus excavatum in adults. The use of metal supports is recommended to reduce the risk of recurrence. A wide variety of metal supports have been proposed, with pre-, trans- or retrosternal fixation. A novel open technique using titanium bars fixed to the ribs with clips has been recently introduced (STRATOS™ system) for chest wall reconstruction, rib fracture fixation and chest wall malformation repair. We employed this technique in two adult patients with severe asymmetric pectus excavatum: after sternal mobilization, one bar is passed below the body of the sternum and secured with clips bilaterally to two ribs. In the first case, the results remained excellent 5 years after surgery. In the second case, the initial results were satisfying but the bar ruptured after 30 months: removal of the bars and clips was performed and a subsequent recurrence of the deformity occurred. The experiences reported in literature are still too limited to draw firm conclusions about the use of the STRATOS™ system in pectus excavatum repair, but it seems that the use of two bars may reduce the risk of rupture. At present, we are the only ones who reported long-term results.

Ischemic myocardial injuries after cardiac malformation repair in infants may be associated with oxidative stress mechanisms

Despite advances in pediatric cardiac surgery, perioperative myocardial injury can be the major determinant of postoperative dysfunction after cardiac surgery. This study investigated the pathology-related differences in 29 infants with congenital heart disease that led to death. The infants were treated at the University Hospital of Ribeirão Preto, Brazil. The patients were divided into four groups: Group 1, 16 infants who underwent operations for congenital heart disease on cardiopulmonary bypass; Group 2, four infants who underwent off-cardiopulmonary bypass operations for congenital heart disease; Group 3, nine infants who died from congenital heart disease prior to surgical treatment; and Group 4 (control group), five infants with no congenital heart disease and who died from other causes. The myocardial injuries and oxidative stress mechanisms were assessed by histopathology and immunohistochemistry and were quantified by morphometrical analyses. Contraction band necrosis and dystrophic calcification were found primarily in infants of Group 1. Coagulation necrosis and healing were prominent in Group 2, while infants without repair (Group 3) showed mainly colliquative myocytolysis. Apoptotic cells were more prominent in the operative groups. The control group showed no significant myocardial lesions. Lipid peroxidation was the principal mechanism of oxidative stress accounting for the myocardial lesions. The diversity of the lesions observed in these hearts seemed to indicate a large spectrum of cell damage due to inadequate myocardial perfusion, especially when these infants underwent surgery. Oxidative mechanisms could be a common mediator in the pathogenesis of myocardial injuries, mediated by peroxidation of the membrane phospholipids and resulting in changes in the permeability of the cell membrane, cell death, and intracellular calcium overload. Furthermore, an immature and often hypertrophied myocardium may promote unfavorable conditions, leading to heart failure and a lethal outcome.

Urological Outcomes Following Anorectal Malformation Repair in Childhood

PURPOSE One of the key aims of ARM repair is to maintain renal function. A persistent fistulous communication between the bowel and urinary tract predisposes to infection and prior to modern paediatric surgical techniques was a major cause of morbidity and mortality. MATERIAL AND METHODS This was a cohort study of 75 adult patients with ARM. Participants completed validated questions on urinary, bowel and sexual function. Medical records were retrieved for data on original diagnosis and associated anomalies. RESULTS The age range of the participants was 18-60 years with a median of 25 years. The majority of patients (58/75) are able to spontaneously void. Only 8 had some form of stoma, the majority were continent diversions. In the cases of 9 participants no medical records were available to accurately assess underlying diagnoses. Thirty of the participants (40%) reported that they were incontinent of urine to some degree. The majority of those that were incontinent were wet only on a weekly basis. Urinary tract anomalies were documented in 35/66 (53%) the commonest being vesicouretal reflux. Only 6 of 23 individuals with reflux underwent surgical intervention for reflux. In one case the diagnosis was not made until adolescence and nephrectomy was required. 3 male participants underwent renal transplantation, and 3 were in chronic renal failure. CONCLUSIONS The majority are continent of urine and void spontaneously. Renal tract anomalies are similar to those in other studies. Those who had undergone reconstruction had significant levels of incontinence. The degree of renal anomalies seen is comparable to other studies.

Reproductive and Sexual Outcomes After Anorectal Malformation Repair in Childhood

PURPOSE Anorectal malformations (ARM) are a spectrum of anomalies. Sexual function and fertility may be impaired. This study looks at long-term outcomes. MATERIAL AND METHODS This was a cohort study of 75 adult patients with ARM. Participants completed questionnaires on urinary, bowel and sexual function. Medical records were retrieved for data on original diagnosis and associated anomalies. RESULTS The age range was 18-60 years with a median of 25 years. 18 associated genital anomalies were recorded in 14 women (31%), the commonest being double vagina (6/14) and bicornuate uterus (5/14). In 10 of the 24 (41.67%) males where paediatric medical records were available had 11 documented genital anomalies, the commonest being cryptorchidism (6/24). The mean age of sexual debut for the group was 17.65 years (range 14 – 65 years). Of the 75, 10 were not sexually active (13.3%). In the case of 2 females this was due to vaginal stenosis, both required surgery. 61% of male patients were very or mainly satisfied with their sex lives. Women scored significantly lower than men in terms of sexual esteem and satisfaction. Five males reported poor erections, and 5 reported difficulty in ejaculating. There were 16 pregnancies from 7 females, 10 resulted in live births. Four deliveries were vaginal and 6 Caesarean section. The male participants reported 11 pregnancies in partners resulting in 10 live births. CONCLUSIONS Anorectal malformations have adverse implications for fertility and sexual function. In young adults it is important that issues regarding sexual function and fulfilment are addressed openly.

Posterior Cranial Fossa Box Expansion Leads to Resolution of Symptomatic Cerebellar Ptosis Following Chiari I Malformation Repair

Chiari I malformation occurs when the cerebellar tonsils herniate downward through the foramen magnum into the upper cervical spinal canal. If the posterior fossa craniectomy during corrective surgery leads to excessive enlargement of the foramen magnum, the complication of cerebellar ptosis may result. To treat this difficult problem a posterior cranial fossa box expansion was devised and studied. Patients who developed symptomatic cerebellar ptosis (confirmed by MRI) following cranial vault decompression for Chiari I malformations underwent a posterior fossa box expansion (n=7). This procedure involved coverage of posterior aspect of the enlarged foramen magnum with a split-thickness calvarial bone graft box. The box reconstruction provided separation between the neck musculature, soft tissues from the neural tissue; prevention of cerebellar prolapse; and expansion of the posterior cranial vault. Patients had a mean of 12+/-1 months between initial suboccipital craniectomy and confirmation of symptomatic cerebellar ptosis. Mean age at the time of posterior fossa box reconstruction was 31+/-9 years (range of 14 to 44 years). Preoperative symptoms of headache (7/7, 100%), upper extremity numbness (5/7, 71%) or paresthesias (3/7, 43%), respiratory disturbance (2/7, 29%) and inability to walk (2/7, 29%) improved in all patients postoperatively. At more than 12 month follow-up 6/7 patients (86%) were both disease-free (MRI confirmation) and symptom-free. One out of seven patients had return of headache in a more mild form. Thus, in our patient group the posterior fossa box reconstruction led to resolution of symptomatic cerebellar ptosis following Chiari I malformation repair.

585: What are the Adult Outcomes after Anorectal Malformation Repair?

You have accessJournal of UrologyModerated Poster, Monday, May 22, 2006, 8:00 - 10:00 am1 Apr 2006585: What are the Adult Outcomes after Anorectal Malformation Repair? Melissa C. Davies, Duncan T. Wilcox, Christopher R. Woodhouse, and Sarah M. Creighton Melissa C. DaviesMelissa C. Davies More articles by this author , Duncan T. WilcoxDuncan T. Wilcox More articles by this author , Christopher R. WoodhouseChristopher R. Woodhouse More articles by this author , and Sarah M. CreightonSarah M. Creighton More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(18)32831-3AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "585: What are the Adult Outcomes after Anorectal Malformation Repair?." The Journal of Urology, 175(4S), p. 190 © 2016 by American Urological AssociationFiguresReferencesRelatedDetails Volume 175Issue 4SApril 2006Page: 190 Advertisement Copyright & Permissions© 2016 by American Urological AssociationMetricsAuthor Information Melissa C. Davies More articles by this author Duncan T. Wilcox More articles by this author Christopher R. Woodhouse More articles by this author Sarah M. Creighton More articles by this author Expand All Advertisement PDF downloadLoading ...

Laparoscopically Assisted Colon Resection for Severe Idiopathic Constipation with Megarectosigmoid

Purpose and Background: In a subset of patients with severe idiopathic constipation who require massive amounts of laxatives, overflow pseudoincontinence may develop. In some of these cases, a megarectosigmoid develops while the rest of the bowel remains of normal caliber. These patients may benefit from resection of the dilated colonic segment. Patients and Methods: During a 5-year period, 164 patients were referred for the evaluation of severe constipation. Ninety-three patients had idiopathic constipation, 41 had Hirschsprung disease, and 30 had previously undergone anorectal malformation repair. Among those with idiopathic constipation, five required massive amounts of laxatives for bowel management and also had megarectosigmoid on contrast enema. All patients were first managed with a strict bowel management regimen. They all were continent, but to remain clean, they required massive amounts of laxatives. A laparoscopic sigmoid resection was offered to five patients with constipation refractory to optimal medical management. Our goal was to reduce the therapeutic laxative requirements and improve their quality of life. Results: All five patients presented with incontinence and required fecal disimpaction. With an aggressive bowel management regimen, all were in fact found to have overflow pseudoincontinence, and with high doses of laxatives, they were continent and able to have daily bowel movements. All five patients had a dilated megarectosigmoid, whereas the rest of the colon was of normal caliber. They underwent laparoscopically assisted sigmoid resection. Postoperatively, the laxative requirements of all patients were significantly reduced. All are continent and have daily bowel movements. Conclusion: Laparoscopically assisted sigmoid resection is an ideal approach for the management of intractable idiopathic constipation in a select group of patients.

A remnant tubal pregnancy after cloacal malformation repair

Objective: Report of a remnant tubal pregnancy after cloacal malformation repair. Design: Case report. Setting: A university hospital. Patient(s): A woman with cloacal malformation repair. Intervention(s): Laparoscopic surgery. Main Outcome Measure(s): Remnant tubal pregnancy. Result(s): Laparoscopic right salpingectomy. Conclusion(s): In this patient, ectopic pregnancy was caused by contralateral sperm transmigration to the remnant right tube, which was not connected with the left uterus.