Cecostomy button for antegrade enemas: survey of 29 patients

Objective

This study evaluated the Trap-door button use (Cook Medical, Bloomington, IL) for antegrade enemas in children.

Methods

Since 2002, patients with fecal incontinence or encopresis and constipation underwent percutaneous cecostomy under laparoscopy using a button.Technical details are described. Age at surgery, operative time, hospital stay, diagnosis, indications for cecostomy, and duration of follow-up were recorded. A survey was proposed via a questionnaire that was sent to the patients. Patients wearing the button for less than 1 month were excluded from this evaluation.The survey concerned volume and frequency of enemas, difficulties encountered, benefits and disadvantages of this method, and assessment of the antegrade enemas in continence.

Results

Twenty-nine patients, 18 males and 11 females, aged 3 to 21 years (mean, 8.5 years) underwent laparoscopic Trap-door button placement. The indications for all the patients were intractable fecal incontinence in 24 cases and constipation with encopresis in 5 cases. Incontinence was because of myelomeningocele (n = 10), anorectal malformations (n = 11), caudal regression syndrome (n = 1), 22q11 syndrome (n= 1), and Hirschsprung disease with encephalopathy with convulsions (n = 1). Constipation with encopresis was because of sacrococcygeal teratoma (n = 1), cerebral palsy (n = 1), and acquired megarectum with psychiatric and social disorders (n = 3). A total of 26 cecostomy button placements and 3 sigmoidostomy button placements were successful with no intraoperative complication. The mean operative time was 25 minutes (10-40 minutes), and the hospital stay was 2.5 days (1-4 days). Twenty-two parents or patients answered the questionnaire. At the time of this survey, 2 patients had improved their fecal continence and had had the button removed. A mean of 4 weekly enemas was enough to improve fecal continence troubles (range, 1 daily to 1 for 2 weeks). The volume for enemas was 250 to 1000 mL (mean, 700 mL). The time required for the irrigation of the bowel by gravity took from 5 to 60 minutes (mean, 25 minutes) for 20 patients. Before surgery, 14 patients needed a diaper, day and night, and 6 needed sanitary protection. Soiling was a very significant inconvenience for all the patients. After surgery, only 5 patients needed a diaper (cerebral palsy, 22q11, cloacal malformation, myelomeningocele, bladder exstrophy) because of moderate results or urinary incontinence and continued soiling. Patients were asked to give an assessment (null = 0, bad = 1, fair = 2, good = 3, very good = 4). None of the patients felt there had been no changes or a bad result. There were 5 patients who felt they had an average result, 5 a good result, and 12 a very good result. The mean grade was 3.44 (17.2/20). A total of 3 patients had hypertrophic granulation tissue formation around the cecostomy button, and 12 had tiny leakage.

Conclusion

Percutaneous placement of a cecostomy button under laparoscopic control is an easy and major complication-free procedure. The use of the Trap-door device by the patients or with the help of the parents for antegrade enemas is effective and satisfactory. It improves the quality of life and is reversible.     

Colonic manometry as predictor of cecostomy success in children with defecation disorders

Purpose

The aim of this study was to define the predictive value of colonic manometry and contrast enema before cecostomy placement in children with defecation disorders.

Methods

Medical records, contrast enema, and colonic manometry studies were reviewed for 32 children with defecation disorders who underwent cecostomy placement between 1999 and 2004. Diagnoses included idiopathic constipation (n = 13), Hirschsprung's disease (n = 2), cerebral palsy (n = 1), imperforate anus (n = 6), spinal abnormality (n = 6), and anal with spinal abnormality (n = 4). Contrast enemas were evaluated for the presence of anatomic abnormalities and the degree of colonic dilatation. Colonic manometry was considered normal when high-amplitude propagating contractions (HAPC) occurred from proximal to distal colon. Clinical success was defined as normal defecation frequency with no or occasional fecal incontinence.

Results

Colonic manometry was done on 32 and contrast enema on 24 patients before cecostomy. At follow-up, 25 patients (78%) fulfilled the success criteria. Absence of HAPC throughout the colon was related to unsuccessful outcome (P = .03). Colonic response with normal HAPC after bisacodyl administration was predictive of success (P = .03). Presence of colonic dilatation was not associated with colonic dysmotility.

Conclusion

Colonic manometry is helpful in predicting the outcome after cecostomy. Patients with generalized colonic dysmotility are less likely to benefit from use of antegrade enemas via cecostomy. Normal colonic response to bisacodyl predicts favorable outcome.     

Functional outcome after operation for Hirschsprung disease—transanal vs transabdominal approach

Background

It has been hypothesized that the extensive transanal dissection in transanal endorectal pull-through (TEPT) for Hirschsprung disease (HD) can impair the anal sphincters in neonates and thereby cause incontinence. Theoretically, transabdominal endorectal pull-through might have less impact on the sphincters. The aim of this study was to compare functional outcome in HD patients operated with either TEPT or laparotomy-assisted endorectal pull-through (LEPT) with particular focus on soiling and fecal incontinence.

Patients and Methods

Anorectal function in 52 children older than 3 years is reported. The patients were operated for HD with either TEPT (n = 28) or LEPT (n = 24) and followed prospectively. Functional outcome was recorded by standardized interviews. The Krickenbeck criteria were used to classify voluntary bowel movements, soiling, and constipation.

Results

The median age at follow-up was 5.7 years (3.1-13.2) for TEPT and 10.1 years (7.7-16.2) for LEPT. Twenty-nine patients reported soiling at final follow-up. There was no difference in the rate of soiling between children operated with TEPT (54%) or LEPT (58%). Constipation was reported in 11 children (TEPT, 25%; LEPT, 17%).

Conclusions

The functional outcome and in particular the rate of soiling did not differ between patients operated with LEPT or TEPT.     

Antegrade enemas for defecation disorders: do they improve the colonic motility?

Purpose

The aim of the study was to describe the changes in colonic motility occurring after chronic antegrade enema use in children and young adults.

Methods

Colonic manometry tracings of patients who had used antegrade enemas for at least 6 months and were being evaluated for possible discontinuation of this treatment were retrospective reviewed.

Results

Seven patients (median age of 12 years, range 3-15 years) met our inclusion criteria. Four patients had idiopathic constipation, 2 had tethered cord, and 1 had Hirschsprung disease. Colonic manometry before the use of antegrade enemas showed dysmotility in 6 (86%) children, mostly in the distal colon. None of the patients underwent colonic resection between the 2 studies. All the patients had colonic manometry repeated between 14 and 46 months after the creation of the cecostomy. All patients with abnormal colonic manometry improved with the use of antegrade enema with a complete normalization of colonic motility in 5 (83%) patients.

Conclusion

Use of antegrade enema alone, without diversion or resection, may improve colonic motility.     

Comparison of volume-controlled and pressure-controlled ventilation in steep Trendelenburg position for robot-assisted laparoscopic radical prostatectomy

Study Objective

To compare the effects of volume-controlled ventilation (VCV) and pressure-controlled ventilation (PCV) on respiratory mechanics and hemodynamics in steep Trendelenburg position.

Design

Prospective, randomized clinical trial.

Setting

University hospital.

Patients

34 ASA physical status 1 and 2 patients undergoing RLRP.

Interventions

Patients were randomly allocated to either the VCV (n = 17) or the PCV group (n = 17). After induction of anesthesia, each patient's lungs were ventilated in constant-flow VCV mode with 50% O₂ and tidal volume of 8 mL/kg; a pulmonary artery catheter was then inserted. After establishment of 30° Trendelenburg position and pneumoperitoneum, VCV mode was switched to PCV mode in the PCV group.

Measurements

Respiratory and hemodynamic variables were measured at baseline supine position (T1), post-Trendelenburg and pneumoperitoneum 60 minutes (T2) and 120 minutes (T3), and return to baseline after skin closure (T4).

Main Results

The PCV group had lower peak airway pressure (AP_peak) and greater dynamic compliance (C_dyn) than the VCV group at T2 and T3 (P < 0.05). However, no other variables differed between the groups. Pulmonary arterial pressure and central venous pressure increased at T2 and T3 (P < 0.05). Cardiac output and right ventricular ejection fraction were unchanged in both groups.

Conclusions

PCV offered greater C_dyn and lower AP_peak than VCV, but no advantages over VCV in respiratory mechanics or hemodynamics.     

Remifentanil for the insertion and removal of long-term central venous access during monitored anesthesia care

Study Objective

To determine the analgesic efficacy of three different rates of remifentanil infusion in patients undergoing insertion or removal of long-term central venous access devices during monitored anesthesia care and local anesthetic field infiltration.

Design

Double-blinded, randomized, controlled study.

Setting

Operating theatre of an University hospital.

Patients

44 unpremedicated, ASA physical status 1 and 2 patients, aged 18-65 years, undergoing insertion or removal of a Port-a-Cath or Hickman catheter.

Interventions

Patients sedated with a propofol target-controlled infusion were randomly allocated to three groups: Group R25 (n = 14), Group R50 (n = 15), and Group R75 (n = 15), to receive remifentanil 0.025, 0.05, and 0.075 μg/kg/min, respectively. Rescue remifentanil 0.5 μg/kg was administered for pain scores > 3. The remifentanil infusion rate was maintained constant unless respiratory and/or cardiovascular unwanted events occurred, whereupon the rate was adjusted in 0.01 μg/kg/min decrements as necessary.

Measurements

Pain scores (primary outcome), sedation, and movement scores (secondary outcomes) were assessed during local anesthetic infiltration of the anterior chest wall and 5 other procedural steps.

Main Results

All infusion rates had equal analgesic efficacy, as shown by comparable pain scores, number of rescue boluses, and number of patients requiring rescue analgesia. Excessive sedation was associated with the highest remifentanil rate such that Group R75 patients were significantly more sedated than Groups R25 or R50 at selective procedural steps (P < 0.05). More Group R75 patients (6/15) required remifentanil rate reduction than did patients from Group R50 (1/15) or Group R25 (0/14), P < 0.01, most commonly because of respiratory depression.

Conclusions

For the insertion or removal of long-term central venous access devices, all three remifentanil infusion rates proved to be equally analgesic-efficient. However, the excessive sedation and tendency to respiratory and cardiovascular events associated with the highest remifentanil infusion rate renders such a rate less desirable for this purpose.     

Therapeutic strategies for idiopathic chylothorax

Study Objectives

The objectives of the study were to present our institutional experience of idiopathic chylothorax in children and to propose therapeutic strategies.

Design

This was a retrospective, single-center study.

Patients

Patients were 6 children (4 boys, 2 girls) presenting with an idiopathic chylothorax diagnosed from the presence of a chylous pleural effusion with triglycerides greater than 1.2 mmol/L and a cellularity greater than 1000 cells/mL with a predominance of lymphocytes.

Results

Median age of onset was 7 years (range, 2-14 years). Initial symptoms included cough (n = 4), tachypnea (n = 4), asthenia (n = 5), abdominal pain (n = 2), and bronchitis (n = 1). Chest radiography showed 2 left, 2 right, and 2 bilateral pleural effusions. Serum biology assessment was normal in all children. Respiratory function assessment at diagnosis revealed a decrease in functional residual capacity in 3 children and a decrease in lung diffusing capacity in 2 children. Initially, all patients received a medium-chain triglyceride diet for 29 months (range, 10-50 months). Total parenteral nutrition was required for 4 patients (for 1-4 months), and somatostatin was tried in one child. Two children required pleuroperitoneal shunting, bilateral in one case. During the follow-up (median duration, 6 years; range, 2-16 years), chylothorax stabilized in all patients and 5 patients were able to return to a normal diet.

Conclusion

A medium-chain triglyceride diet associated in some cases with total parenteral nutrition may stabilize idiopathic chylothorax in children. In cases where conservative treatment has failed, pleuroperitoneal shunting may be useful.     

Childhood phaeochromocytoma and paraganglioma: 100% incidence of genetic mutations and 100% survival

Introduction

The aim is to identify the incidence of genetic mutations and outcome of children presenting with phaeochromocytoma/paraganglioma (PGL) to a single paediatric surgical service to determine the need for genetic counselling in associated kindreds.

Methods

A retrospective case note review was undertaken of all cases treated between 1998 and 2008 with particular reference to presentation, management, and predisposing genetic conditions.

Results

Seven cases (4 male, 3 female) were identified (median age, 13 years; interquartile range, 9-16). Three cases had a family history of phaeochromocytoma/PGL. All presented with neurologic symptoms related to hypertension, including headaches (n = 5), hemiparesis (n = 2), facial palsy, and hemianopia. All underwent surgical resection. Five patients had meta-iodobenzylguanidine (MIBG) therapy for apparently malignant features. All cases were found to have a predisposing genetic mutation: von Hippel-Lindau (n = 3), succinate dehydrogenase mutations (n = 3), and multiple endocrine neoplasia (n = 1). All patients are alive after a median follow-up of 5 (interquartile range, 2-7) years.

Conclusions

All 7 cases had a familial genetic mutation identified, and none arose de novo. We advocate genetic counselling for all families of children diagnosed with phaeochromocytoma/PGL with lifelong surveillance tailored to the underlying syndrome because of the increased risk of synchronous and metachronous tumours associated with these genetic syndromes.     

Transanal rectosigmoid resection for severe intractable idiopathic constipation

Introduction

Idiopathic constipation is a source of significant morbidity in children. A subset of patients is refractory to medical therapy and requires surgical intervention. We present a novel surgical technique for the management of these patients.

Methods

We reviewed the records of 288 patients with severe idiopathic constipation and soiling. Patients who were refractory to medical management and had a megarectosigmoid underwent a transanal full-thickness rectosigmoid resection with a primary colo-anal anastomosis.

Results

Fifteen patients underwent a transanal rectosigmoid resection. The preoperative contrast enema demonstrated an enormously dilated rectosigmoid in 14. An average of 43 cm (range, 8-98 cm) of rectosigmoid was resected. Of 14 patients with more than 3 months of follow-up, the preoperative laxative dose was 68 mg of senna/d (range, 52-95 mg), which decreased to 8.6 mg postoperatively (P < .001). Nine patients are clean without soiling, 1 is more prone to diarrhea, but is clean. Two patients soil occasionally, but are noncompliant, and 2 were lost to follow-up.

Conclusion

Transanal rectosigmoid resection for medically intractable idiopathic constipation resulted in a dramatic reduction or elimination in laxatives use while preserving continence. It is a useful alternative to surgical options such as other colonic resections, antegrade enemas, and stomas.     

Early Humoral-Mediated Graft Injuries in ABO-Incompatible Kidney Transplantation in Human Beings

Introduction

Acute humoral rejection is the most important risk factor for early graft loss in ABO-incompatible (ABO-i) renal transplantation (RTx) and is present from the early period after RTx. However, the characteristics of early humoral-mediated graft injury are pathologically uncertain.

Objective

To analyze tissue from 10 protocol graft biopsies performed in 10 patients within 30 days post-RTx to clarify the pathologic features of early humoral-mediated graft injuries in ABO-i RTx.

Methods

Pathologic findings were examined using light and electron microscopy and immunofluorescence studies for C4d. Protocol biopsies were performed within 30 days after RTx in the absence of an episode of dysfunction (creatinine concentration 1.21-1.81 mg/dL).

Results

The immunofluorescence study demonstrated C4d deposition in peritubular and glomerular capillaries. Acute glomerulitis with infiltration of mononuclear cells and neutrophils was observed in 3 patients. Furthermore, glomerulitis was accompanied by endothelial cell injuries, widening of subendothelial spaces with a double-contoured glomerular basement membrane, and mesangiolysis.

Conclusion

In ABO-i RTx, early humoral-mediated graft injuries were observed in approximately 30% of patients despite normal graft function. They were characterized by C4d deposition and glomerular capillary injury. These findings suggest that renal glomeruli are the first site of graft injury by anti-A or anti-B blood type antibody with complement activation in ABO-i RTx.     

Complications of recombinant activated human coagulation factor VII

Background

Recombinant factor VIIa (rFVIIa) frequently is used for treatment of life-threatening hemorrhage in trauma.

Methods

A retrospective review of injured patients receiving rFVIIa at an American College of Surgeons-verified Level 1 trauma center was performed. Controls were matched for age, sex, Injury Severity Score, and traumatic brain injury. Thrombotic complications in patients administered rFVIIa, including deep venous thrombosis (DVT), pulmonary embolus, acute myocardial infarction, ischemic stroke, mesenteric ischemia, arterial thromboembolism, and death, were determined.

Results

Thirty-six patients were given rFVIIa, of whom 5 (13.8%) had thrombotic complications. Indications for rFVIIa were life-threatening intracranial bleeding in the presence of pre-injury anticoagulation or hemorrhage. The incidences of DVT (n = 4) and acute myocardial infarction (n = 1) were noted. In the control group, there were fewer thrombotic complications (DVT, 1; pulmonary embolus, 1). The mortality rate (52.8%) was higher in patients receiving rFVIIa compared with the control group (22.2%; P = .014). Pre-injury anticoagulation was common in the treatment group.

Conclusions

Pre-injury anticoagulation is frequently the indication for rFVIIa administration. Thrombotic complications occur with rFVIIa administration. The mortality rate of injured patients who receive rFVIIa is high.     

Pain on injection of propofol: a comparison of methylene blue and lidocaine

Study Objective

To investigate whether methylene blue, given before injection of propofol, was effective in reducing the frequency and severity of pain associated with propofol injection.

Design

Prospective, randomized, double-blinded clinical study.

Setting

Operating room of a university hospital.

Patients

90 adult, ASA physical status 1 and 2 patients undergoing elective surgery.

Interventions

Patients were randomly allocated to one of three groups of 30 patients each. Group I received 50 mg of methylene blue, Group II received 40 mg of lidocaine, and Group III, the control group, was given normal saline. All drugs were given as a 2.0 mL bolus 45 seconds before propofol administration.

Measurements

Injection pain using vocal responses, facial grimacing, arm withdrawal, tears, and questioning of the patient were noted. A 4-point scale was used for documenting pain.

Main Results

Pain frequency was 90% in the saline group, whereas the frequencies were significantly lower in the lidocaine and methylene blue groups (26.7% and 40%, respectively).

Conclusions

Intravenous pretreatment with methylene blue appears to be effective in reducing the pain during propofol injection.     

Factors influencing rat survival in a warm renal ischemia model: time to adapt the protocols

Introduction

Survival in warm renal ischemia models is not only dependent on the treatment or surgical technique being evaluated, but also on factors inherent to the model itself. Use of rats of various strains in previous studies makes interstudy comparison difficult when trying to design an appropriate model control that would yield intermediate survival. In this study, impact of rat strain on survival after prolonged warm renal ischemia in the setting of delivery-controlled inhalational anesthesia was evaluated.

Materials and methods

Under general delivery-controlled inhalation anesthesia with isoflurane, Dahl salt-sensitive, Wistar-Furth, Sprague-Dawley, and spontaneously hypertensive rats (n = 66 rats) were subjected to 150 minutes of unilateral renal warm ischemia time, subsequent reperfusion, and contralateral nephrectomy. Animals were followed up for 1 month, after which survivors were euthanized and morphologic changes in kidneys were scored.

Results

Thirty-day survival was: Dahl salt sensitive, 78%; Wistar-Furth, 67%; Sprague-Dawley, 55%; and spontaneously hypertensive rats, 0% (P < .0001). Histologic acute injury scores were higher for non-survivors versus 30-day survivors (P < .0001).

Conclusion

Our data strongly suggest that rat strain is a major factor influencing survival and that strain and warm ischemia time selections must be considered together when designing a model control yielding intermediate survival. Further study is warranted in order to compare the effect of delivery-controlled inhalational versus historical anesthesia methods on animal survival.     

Primary anorectoplasty in females with common anorectal malformations without colostomy

Background/Purpose

The objective of this study is to assess the feasibility of primary posterior sagittal anorectoplasty in vestibular fistula without a covering colostomy.

Methods

Girls presenting from July 1997 to July 2005 with vestibular fistula were included prospectively in the study, in a nonrandomized manner, after excluding those with megarectosigmoid and pouch colon. All underwent primary posterior sagittal anorectoplasty after total gut irrigation with normal saline. They were kept nil per oral until the fifth postoperative day. No patient was started on anal dilatation. Patients were assessed for immediate and delayed complications as well as voluntary bowel movements and continence.

Results

A total of 72 patients with an age range of 1.5 months to 8 years (median, 9 months) were studied after excluding 7 with pouch colon and 3 with megarectosigmoid. Of the 72, 3 had undergone previous surgery. Follow-up ranged from 7 months to 8 years. No wound dehiscence or recurrence of fistula was noted. There were 5 mild wound infections. At 1 month postoperative, all patients had 1 to 3 stools per day with no episodes of soiling. None required anal dilatations, laxatives, or enemas.

Conclusions

Primary posterior sagittal anorectoplasty in vestibular fistula can be performed without a covering colostomy provided fecal contamination of the wound can be kept to the minimum in the first postoperative week. We achieve this by thorough total gut irrigation preoperatively and keeping the child nil per oral for the first 5 postoperative days. Continence rates are excellent and postoperative constipation is unlikely if megarectosigmoid and pouch colon are ruled out before surgery. Anal dilatation is not required after surgery.     

Midterm follow-up study of high-type imperforate anus after laparoscopically assisted anorectoplasty

Purpose

The aim of this study was to evaluate postoperative anal function of high-type imperforate anus after laparoscopically assisted anorectoplasty (LAARP).

Methods

Between 2000 and 2002, LAARP was performed in 13 patients with high-type imperforate anus. Clinical data of the LAARP group were compared with the posterior sagittal anorectoplasty (PSARP) group (n = 7) operated before 2000. All patients were treated with initial colostomy in the newborn period. After anorectoplasty, daily glycerin enemas were given for postoperative bowel management. Anorectal function of these patients was evaluated using the Kelly score and manometry at the age of 3 to 5 years (LAARP) and 5 to 6 years (PSARP).

Results

Age at evaluation in the LAARP group was younger than that in the PSARP group (51 ± 10 vs 73 ± 12 months, P < .01). Kelly score was 3.8 ± 1.3 vs 3.4 ± 0.8, respectively. Resting pressure of internal sphincter was 31 ± 11 vs 33 ± 10 cm H₂O, respectively. Relaxation reflex of the internal anal sphincter was observed in 62% (8/13) and 29% (2/7), respectively.

Conclusions

Midterm follow-up study revealed that satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectoplasty may be a good alternative in this patient population. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.     

Combined Application of Blocking Antibodies and MicroRNA Interference in Inhibiting CD44 Expression

Background

We sought to explore the effect of CD44 targeting on the tolerance to memory cell-mediated graft rejection.

Methods

We developed a cardiac transplantation model in nude mice and administered anti-CD44 monoclonal antibodies (mAbs) to these mice. Then, we used anti-CD44 mAb and CD44-interfering microRNA (miRNA) to inhibit CD44 expression in vitro.

Results

The median survival time (MST) associated with multiple intraperitoneal injections was >100 days, whereas that associated with CD4⁺ Tm cells blocked CD44 and that associated with a single intraperitoneal injection of anti-CD44 mAb was 11 and 10.3 days, control group was 5.5 days. The inhibition effect of the anti-CD44 mAb in 3T3 cells significantly reduced with cell proliferation. Used CD44 miRNA in 3T3 cells, the most obvious inhibition effect of mRNA appeared at 48 hours after transfection and the inhibition decreased subsequently. In combination, antibody-mediated blocking and miRNA showed some synergistic effects.

Conclusion

The inhibition of CD44 can significantly prolong the MST in memory models. The inhibition effect of combined application showed limitations with regard to cell proliferation and duration of action, but the short-term synergistic effect of the combined approach was stronger than the effects of individual approaches.     

Late-presenting congenital diaphragmatic hernia

Background

Late-presenting congenital diaphragmatic hernia (CDH) is a rare subset of CDH, most of the information derived from small series or case reports. The aim of this study was to document the clinical manifestations of late-presenting CDH using a large multicenter database.

Methods

Information about late-presenting CDH (diagnosed at later than 30 days of age) was identified from the database of the CDH Study Group (3098 cases collected during 1995-2004) and reviewed retrospectively.

Results

Seventy-nine cases (2.6%) from 30 centers met the inclusion criteria. Seven cases had a Morgagni hernia. There were 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (32 days to 15 years). Major associated anomalies (10 cardiac and 7 chromosomal abnormalities) were identified in 12 cases (15%). Presenting symptoms were respiratory in 20 (43%), gastrointestinal in 15 (33%), both in 6 (13%), and none (asymptomatic) in 5 (11%). The hernia was left-sided in 53 (69%), right-sided in 21 (27%), and central or bilateral in 3 (4%). Patients with gastrointestinal symptoms invariably had left-sided hernias (n = 19), whereas patients with respiratory symptoms (n = 24) seemed equally likely to have right- or left-sided lesions. A primary repair without patch was done in all cases with 100% survival.

Conclusions

Presenting symptoms of late-onset CDH can be respiratory or gastrointestinal, but presentation with gastrointestinal problems was more common in left-sided hernias, whereas respiratory symptoms predominated in right-sided lesions. The prognosis is excellent once the correct diagnosis is made.     

Long-term disease-specific quality of life in adult anorectal malformation patients

Background

Fecal and urinary incontinence may differently influence various aspects of quality of life (QOL). The main aim of the present study is to determine whether fecal and urinary incontinence measured at time 1 of the study will predict QOL at time 2 (after 4 years), above and beyond the prediction already explained by fecal and urinary incontinence at time 2.

Methods

Thirty-six adult patients from the Italian Parents' and Patients' Association for Anorectal Malformations answered items about urinary and fecal incontinence at time 1 of the study and completed the Hirschsprung Disease/Anorectal Malformation Quality of Life questionnaire after 4 years from the first questionnaire. Two sets of hierarchical regression analyses were conducted with fecal and urinary incontinence serving as predictors of QOL and the different areas of QOL from the Hirschsprung Disease/Anorectal Malformation Quality of Life serving as outcome variables.

Results

The principal findings indicated that fecal continence is a strong predictor of QOL in the areas of social functioning, emotional functioning, and body image and that urinary incontinence predicted sexual functioning.

Conclusions

It seems that one's past experience with fecal incontinence is extremely relevant to current QOL, especially for body image. Urinary incontinence contributed less in explaining QOL in our patients, but because it is very relevant for sexual functioning, it should not be disregarded.     

Renal angiomyolipoma in Japanese tuberous sclerosis patients

Purpose

Renal angiomyolipoma (AML) is a benign neoplasm that may grow massive in tuberous sclerosis (TS) patients. The aim of this study was to document the characteristics of renal AML in Japanese TS patients.

Methods

Medical records of 29 TS patients followed up at the authors’ center were reviewed for the presence, size, symptom, and treatment of renal AML.

Results

Twenty-four patients screened for renal AML were subdivided into 4 groups: group 0 (n = 8), no mass; group 1 (n = 5), AML less than 1 cm in diameter; group 2 (n = 4), AML 1 to 4 cm in diameter; group 3 (n = 7), AML greater than 4 cm in diameter. When present, AML always affected both kidneys and were multiple. All patients in groups 1 and 2 were symptom free, and the tumors seemed stable in size. All tumors in group 3 grew progressively causing various symptoms. Total or partial nephrectomy or transarterial embolization was performed in 5 patients with limited success.

Conclusions

AML in TS patients can be stable or aggressive. Pediatric surgeons aware of this problem should be involved in a follow-up program.     

Extracorporeal membrane oxygenation as a bridge to definitive tracheal reconstruction in neonates

Purpose

Infants born with severe tracheal anomalies may not survive beyond the first few hours of life without aggressive cardiopulmonary support and/or emergent airway surgery. The purpose of this study was to review our experience with critically ill neonates supported on extracorporeal membrane oxygenation (ECMO) before tracheal reconstruction.

Methods

A retrospective review of a single institution ECMO registry was conducted. Outcomes of neonates requiring tracheal repair were examined.

Results

Three children with tracheal anomalies (complete tracheal rings [n = 2]; bronchogenic cyst [n = 1]) underwent definitive airway reconstruction. All were placed on ECMO (venovenous [n = 2]; venoarterial [n = 1]) within 24 hours after birth. Tracheoplasties (tracheal resection with end-to-end anastomosis [n = 1]; slide tracheoplasty [n = 1]; carinal resection and reconstruction [n = 1]) were performed at 3.7 ± 2.2 days of life. There were no hemorrhagic or thrombotic complications for an ECMO time of 117.3 ± 60.1 hours. The postoperative durations until extubation and hospital discharge were 12.0 ± 3.2 and 34.3 ± 11.6 days, respectively. All children remain alive and well without cardiopulmonary and neurologic sequelae at a mean follow-up of 4.5 years.

Conclusions

Excellent clinical outcomes can be achieved in neonates born with severe tracheal anomalies using ECMO as a bridge to definitive tracheal reconstruction.