Predictors of early lung function in patients with congenital diaphragmatic hernia

Purpose

Long-term pulmonary outcomes of congenital diaphragmatic hernia (CDH) have demonstrated airflow obstruction in later childhood. We examined pulmonary function data to assess what factors predict lung function in the first three years of life in children with CDH.

Methods

This was a retrospective study of patients treated for CDH who underwent infant pulmonary function testing (IPFT) between 2006 and 2012. IPFT was performed using the raised volume rapid thoracoabdominal compression technique and plethysmography.

Results

Twenty-nine neonates with CDH had IPFTs in the first 3 years of life. Their mean predicted survival using the CDH Study Group equation was 63% ± 4%. Fourteen infants (48%) required extracorporeal membrane oxygenation (ECMO). The mean age at IPFT was 85.1 ± 5 weeks. Airflow obstruction was the most common abnormality, seen in 14 subjects. 12 subjects had air trapping, and 9 demonstrated restrictive disease. ECMO (p = 0.002), days on the ventilator (p = 0.028), and days on oxygen (p = 0.023) were associated with restrictive lung disease.

Conclusion

Despite following a group of patients with severe CDH, lung function revealed mild deficits in the first three years of life. Clinical markers of increased severity (ECMO, ventilator days, and prolonged oxygen use) are correlated with reduced lung function.     

Incidence and factors associated with sensorineural and conductive hearing loss among survivors of congenital diaphragmatic hernia

Purpose

The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution.

Methods

We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher’s exact test and continuous variables by Mann–Whitney t-test (p ≤ 0.05).

Results

A total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p = 0.0130), prolonged course of hospitalization (p = 0.0011), duration of mechanical ventilation (p = 0.0046), requirement for tracheostomy (p = 0.0013), and duration of loop diuretic (p = 0.0005) and aminoglycoside therapy (p = 0.0003).

Conclusions

We have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language.     

O valor preditivo do peptídeo natriurético tipo‐B em resultados de crianças com hipertensão pulmonar submetidas à cirurgia cardíaca congênita

Background and objectives

In children undergoing congenital heart surgery, plasma brain natriuretic peptide levels may have a role in development of low cardiac output syndrome that is defined as a combination of clinical findings and interventions to augment cardiac output in children with pulmonary hypertension.

Methods

In a prospective observational study, fifty‐one children undergoing congenital heart surgery with preoperative echocardiographic study showing pulmonary hypertension were enrolled. The plasma brain natriuretic peptide levels were collected before operation, 12, 24 and 48 h after operation. The patients enrolled into the study were divided into two groups depending on: (1) Development of LCOS which is defined as a combination of clinical findings or interventions to augment cardiac output postoperatively; (2) Determination of preoperative brain natriuretic peptide cut‐off value by receiver operating curve analysis for low cardiac output syndrome. The secondary end points were: (1) duration of mechanical ventilation ≥72 h, (2) intensive care unit stay >7days, and (3) mortality.

Results

The differences in preoperative and postoperative brain natriuretic peptide levels of patients with or without low cardiac output syndrome (n = 35, n = 16, respectively) showed significant differences in repeated measurement time points (p = 0.0001). The preoperative brain natriuretic peptide cut‐off value of 125.5 pg mL‐1 was found to have the highest sensitivity of 88.9% and specificity of 96.9% in predicting low cardiac output syndrome in patients with pulmonary hypertension. A good correlation was found between preoperative plasma brain natriuretic peptide level and duration of mechanical ventilation (r = 0.67, p = 0.0001).

Conclusions

In patients with pulmonary hypertension undergoing congenital heart surgery, 91% of patients with preoperative plasma brain natriuretic peptide levels above 125.5 pg mL‐1 are at risk of developing low cardiac output syndrome which is an important postoperative outcome.     

Prenatal MRI fetal lung volumes and percent liver herniation predict pulmonary morbidity in congenital diaphragmatic hernia (CDH)

Purpose

The purpose of this study was to determine whether prenatal imaging parameters are predictive of postnatal CDH-associated pulmonary morbidity.

Methods

The records of all neonates with CDH treated from 2004 to 2012 were reviewed. Patients requiring supplemental oxygen at 30 days of life (DOL) were classified as having chronic lung disease (CLD). Fetal MRI-measured observed/expected total fetal lung volume (O/E-TFLV) and percent liver herniation (%LH) were recorded. Receiver operating characteristic (ROC) curves and multivariate regression were applied to assess the prognostic value of O/E-TFLV and %LH for development of CLD.

Results

Of 172 neonates with CDH, 108 had fetal MRIs, and survival was 76%. 82% (89/108) were alive at DOL 30, 46 (52%) of whom had CLD. Neonates with CLD had lower mean O/E-TFLV (30vs.42%; p = 0.001) and higher %LH (21.3 ± 2.8 vs.7.1 ± 1.8%; p < 0.001) compared to neonates without CLD. Using ROC analysis, the best cutoffs in predicting CLD were an O/E-TFLV < 35% (AUC = 0.74; p < 0.001) and %LH > 20% (AUC =0.78; p < 0.001). On logistic regression, O/E-TFLV < 35% and a %LH > 20% were highly associated with indicators of long-term pulmonary sequelae. On multivariate analysis, %LH was the strongest predictor of CLD in patients with CDH (OR: 10.96, 95%CI: 2.5–48.9, p = 0.002).

Conclusion

Prenatal measurement of O/E-TFLV and %LH is predictive of CDH pulmonary morbidity and can aid in establishing parental expectations of postnatal outcomes.     

Outcome of CDH infants following fetoscopic tracheal occlusion — influence of premature delivery

Purpose

To evaluate the mortality and morbidity of infants with congenital diaphragmatic hernia who had undergone fetal endoscopic tracheal occlusion (FETO) and whether this was influenced by premature birth.

Methods

The gestational age at delivery, lung–head ratio (LHR) pre and post FETO, neonatal outcomes, and respiratory, gastro-intestinal, neurological, surgical, and musculoskeletal problems at follow up of consecutive infants who had undergone FETO were determined. Elective reversal of FETO was planned at 34 weeks of gestation.

Results

The survival rate of the 61 FETO infants was 48%, with 84% delivered prematurely. Thirty-one delivered < 35 weeks of gestation. Their survival rate was 18%. Twenty-three of 24 infants who had emergency balloon removal were born < 35 weeks of gestation. Survival was related to gestational age at delivery (OR 0.55, 95% CI 0.420, 0.77, p < 0.001) and the duration of FETO (OR 0.73, 95% CI 0.59, 0.91, p < 0.005). Infants born prior to 35 weeks of gestation compared to those born at ≥ 35 weeks required a longer duration of ventilation (median 45 days versus 12 days, p < 0.001), and a greater proportion had surgery for gastro-oesophageal reflux (50% versus 9%, p = 0.011).

Conclusion

These results emphasize the need to reduce premature delivery following FETO.     

Maternal medical and behavioral risk factors for congenital diaphragmatic hernia

Purpose

Maternal factors contributing to the etiology of congenital diaphragmatic hernia (CDH) remain unclear. We hypothesized that specific maternal medical conditions (pregestational diabetes, hypertension), and behaviors (alcohol, tobacco) would be associated with CDH.

Methods

We conducted a population-based case–control study using Washington State birth certificates linked to hospital discharge records (1987–2009). We identified all infants with CDH (n = 492). Controls were randomly selected among non-CDH infants. Maternal data were extracted from the birth record. Logistic regression was used to adjust for covariates.

Results

Cases and controls were generally similar regarding demographics, although CDH infants were more likely to be male than controls (58.5% vs. 52.5%). Isolated and complex (multiple-anomaly) CDH had similar characteristics. Each of the exposures of interest was more common among case mothers than among control mothers. In univariate analysis, alcohol use, hypertension, and pregestational diabetes were each significantly associated with the outcome. After multivariate adjustment, only alcohol use (OR = 3.65, p = 0.01) and pregestational diabetes (OR = 12.53, p = 0.003) maintained significance. Results were similar for both isolated and complex CDH.

Conclusions

Maternal pregestational diabetes and alcohol use are significantly associated with occurrence of CDH in infants. These are important modifiable risk factors to consider with regard to efforts seeking to impact the incidence of CDH.     

Bowel symptoms in children with anorectal malformation — a follow-up with a gender and age perspective

Background

Gender specific outcome for children with anorectal malformations (ARM) is rarely reported although it is important for medical care and in parent counseling.

Purpose

To assess bowel function according to the Krickenbeck system in relation to ARM-subtype, gender and age.

Method

All children born with ARM in 1998–2008 and referred to two centers in two different countries were followed up. The bowel function in 50 girls and 71 boys, median age 8 years, was analyzed.

Results

Among those with a perineal fistula, incontinence occurred in 42% of the females and in 10% of the males (p = 0.005) whereas constipation occurred in 62% of the females and 35% of the males (p < 0.001). No bowel symptoms differed between the females with perineal and vestibular fistulas (p > 0.3 for every symptom). Sacral malformations were associated with incontinence only in males with rectourethral fistulas. Constipation among the males differed between the age groups: 58% versus 26% (p = 0.013). Bowel symptoms did not change with age among the females.

Conclusion

Gender differences in outcome for children with ARM must be considered. Males with perineal fistulas had less incontinence and constipation than the females with perineal fistulas. The females with perineal and vestibular fistulas had similar outcomes.     

Antithrombin III administration in neonates with congenital diaphragmatic hernia during the first three days of extracorporeal membrane oxygenation

Purpose

To evaluate the effect of Antithrombin III (ATIII) on blood product requirement in neonates receiving extracorporeal membrane oxygenation (ECMO).

Methods

A retrospective case control study of neonates presenting with or without congenital diaphragmatic hernia (CDH) requiring ECMO between 2006 and 2010 was performed. Patient demographics, laboratory data, and information on blood products administered were compared in patients treated before (n = 37) and after (n = 38) a protocol for antithrombin-III (ATIII) administration was implemented.

Results

During the first three days on ECMO patients with CDH received less fresh frozen plasma (FFP) and platelets after ATIII administration was introduced (78.1 ± 19.2 ml/kg vs. 27.8 ± 6.2 ml/kg, p < 0.007 and 67.8 ± 8.6 ml/kg vs. 47.8 ± 8.4 ml/kg, p = 0.05 respectively), while FFP and platelet administration in patients without CDH was not different between the two periods. Patients both with and without CDH received less packed red blood cell (PRBC) transfusions after the ATIII protocol was introduced (230 ± 51.5 ml/kg vs. 73.8 ± 9.7 ml/kg, p < 0.002 and 173.2 ± 22.2 ml/kg vs. 66.0 ± 6.6 ml/kg, p < 0.001, respectively). Finally, cryoprecipitate administered was not different in patients with and without CDH between the two periods (13 ± 2.9 ml/kg vs. 15.9 ± 7.2 ml/kg, p = NS and 6.1 ± 1.8 ml/kg vs. 3.4 ± 0.6 ml/kg, p = NS, respectively).

Conclusions

Introduction of routine ATIII administration was associated with decreases in FFP, platelet, and PRBC exposure in neonates with CDH and decreases in PRBC transfusions in neonates without CDH during the first three days of ECMO support.     

Anorectal malformations with good prognosis: Variables affecting the functional outcome

Backgroud/Purpose

The purpose of this study was to investigate the outcome of patients operated for anorectal malformations (ARMs) with good prognosis.

Methods

Thirty patients underwent clinical evaluation by Rintala score and anorectal manometry recording anal resting pressure (ARP), rectoanal inhibitory reflex (RAIR), and rectal volume (RV). The results were analysed with regard to sex, type of ARM, surgical timing of posterior sagittal anorectoplasty (PSARP), neurospinal cord dysraphism (ND), neonatal colostomy, and institution where they underwent surgery.

Results

6/30 (20%) presented ND despite normal sacrum. 17/30 (57%) patients had a normal Rintala score. ND and neonatal colostomy were significantly associated with a pathologic score (p = 0.0029 and p = 0.0016). Patients with ND had significantly lower ARP compared to patients with normal spine (23.5 ± 7.2 mmHg vs 32 ± 7.9 mmHg, p = 0.023). ARP was significantly lower in patients with neonatal colostomy compared to patients with primary repair (25.22 ± 10.24 mmHg vs 32.57 ± 6.68 mmHg, p = 0.026). RAIR was present in only 2/6 (33%) patients with ND, while in 21/24 (87.5%) without ND (p = 0.015) and in 4/9 (44%) patients with neonatal colostomy, while in 19/21 (90.5%) patients submitted to primary repair (p = 0.014).

Conclusions

Neurospinal cord dysraphism may be present despite normal sacral ratio. From a clinical point of view, patients with good prognosis ARMs are not completely comparable to healthy children. Neurospinal cord dysraphism and neonatal colostomy seem to worsen the clinical and manometric (ARP and RAIR) outcomes of these patients.     

Vesicoureteral reflux and febrile urinary tract infections in anorectal malformations: A retrospective review

Background

Multiple studies document a correlation between anorectal malformations (ARMs) and vesicoureteral reflux (VUR), development of urinary tract infections (UTIs), and subsequent renal disease. We aimed to determine which patient characteristics are associated with VUR and UTI in this population.

Methods

A retrospective review of ARM patients at a free-standing children’s hospital from January 1996 to December 2011 was performed. Logistic regression was used to investigate the associations between VUR and UTI and ARM classification and co-morbid diagnoses.

Results

Of 190 patients, 41 (31%) received a diagnosis of VUR. Thirty-one of the 190 patients had at least one febrile UTI (16%). Of these, only 16 (51%) had a diagnosis of VUR. On multivariable logistic regression, the only patient variable associated with VUR was having an ectopic kidney (p = 0.026). Similarly, the presence of GU malformations was the closest variable associated with developing a UTI (p = 0.073).

Conclusions

In ARM patients, VUR as well as UTIs are associated with the presence of GU malformations. Thus, voiding cystourethrogram (VCUG) testing should be pursued when there are other caudal and GU abnormalities, regardless of fistula location. Antibiotic prophylaxis for UTI should be considered in children with ARM and any GU malformation, not only VUR.     

Fetal MRI lung volumes are predictive of perinatal outcomes in fetuses with congenital lung masses

Purpose

The purpose of this study was to evaluate fetal magnetic resonance imaging (MRI) as a modality for predicting perinatal outcomes and lung-related morbidity in fetuses with congenital lung masses (CLM).

Methods

The records of all patients treated for CLM from 2002 to 2012 were reviewed retrospectively. Fetal MRI-derived lung mass volume ratio (LMVR), observed/expected normal fetal lung volume (O/E-NFLV), and lesion-to-lung volume ratio (LLV) were calculated. Multivariate regression and receiver operating characteristic analyses were applied to determine the predictive accuracy of prenatal imaging.

Results

Of 128 fetuses with CLM, 93% (n = 118) survived. MRI data were available for 113 fetuses. In early gestation (< 26 weeks), MRI measurements of LMVR and LLV correlated with risk of fetal hydrops, mortality, and/or need for fetal intervention. In later gestation (> 26 weeks), LMVR, LLV, and O/E-NFLV correlated with neonatal respiratory distress, intubation, NICU admission and need for neonatal surgery. On multivariate regression, LMVR was the strongest predictor for development of fetal hydrops (OR: 6.97, 1.58–30.84; p = 0.01) and neonatal respiratory distress (OR: 12.38, 3.52–43.61; p ≤ 0.001). An LMVR > 2.0 predicted worse perinatal outcome with 83% sensitivity and 99% specificity (AUC = 0.94; p < 0.001).

Conclusion

Fetal MRI volumetric measurements of lung masses and residual normal lung are predictive of perinatal outcomes in fetuses with CLM. These data may assist in perinatal risk stratification, counseling, and resource utilization.     

Thoracoscopic anatomic pulmonary segmentectomy for the treatment of congenital lung malformation in children

BackgroundLung-sparing surgery has been used to treat congenital lung malformation in children, and segmentectomy has been advocated as a lung-preservation strategy. However, thoracoscopic pulmonary segmentectomy has gained limited popularity considering the complications, the potential for residual lesions, and the technical difficulties associated with this procedure. Therefore, this study aimed to investigate the safety and feasibility of pediatric thoracoscopic anatomic pulmonary segmentectomy for the treatment of congenital lung malformations.MethodsWe conducted a retrospective review of the medical records of 568 patients who were treated at West China Hospital, Sichuan University, from January 2014 to January 2020. The patients were divided into segmentectomy and lobectomy groups according to the surgical procedures they underwent. Clinical and follow-up outcomes were compared between the two groups.ResultsThe segmentectomy and lobectomy groups included 206 and 361 cases, respectively. The mean intraoperative blood loss was significantly higher in the segmentectomy group (6.9 mL vs. 4.5 mL; p = 0.03). The mean surgical time was also significantly longer in the segmentectomy group, (55.6 min vs. 41.5 min; p = 0.018). However, the incidence of complications did not differ significantly between the two groups (2.9% vs. 1.1%, p = 0.21). Patients in both groups did not require reoperation or show residual lesions during hospitalization and follow-up.ConclusionsThoracoscopic anatomic pulmonary segmentectomy is a safe and feasible definitive lung-sparing treatment for specific cases of congenital lung malformation, and has a complication rate comparable to that of thoracoscopic lobectomy.     

Outcomes of pulmonary metastases in hepatoblastoma — is the prognosis always poor?

Background

Hepatoblastoma is a rare tumour accounting for approximately 1% of all paediatric malignancies. Hepatoblastoma complicated by pulmonary metastatic disease continues to cause management difficulties due to a lack of robust evidence and treatment guidelines.

Method

This series is the experience of a tertiary paediatric referral centre. Patients were prospectively enlisted, and their charts were retrospectively reviewed.

Results

Thirty-seven patients were treated for hepatoblastoma from 1995 to 2012 inclusive. The overall survival was 34/37(91.9%). Eight patients had lung metastases at diagnosis (LMD) and twenty-nine did not (NLMD). Two-year EFS was 62.5% in the LMD group and 89.3% in the NLMD group (p = 0.078). Overall survival in the LMD and NLMD groups was 100% and 89.7%, respectively (p = 0.389). Two patients in the LMD group required multiple thoracic resections to achieve cure. Within the NLMD group, two patients developed lung metastases whilst on treatment, and both of these patients died.

Conclusion

In this series, children presenting with lung metastases had a higher risk of relapse but excellent overall survival. However, children who developed pulmonary disease during treatment had a poor prognosis. We advocate aggressive surgical treatment of pulmonary hepatoblastoma to achieve cure.     

Surgical treatment of type IV-A choledochal cyst in a single institution: Children vs. adults

Background

The treatment of type IV-A choledochal cyst is particularly difficult and remains a challenge because of the rareness and the various presentations of the disease involving not only the extrahepatic but also the intrahepatic biliary tract. The purpose of this study is to analyze our clinical experience for surgical treatment of type IV-A choledochal cyst, and compare between children and adults.

Methods

During a 10-year period of time (2000–2010), clinical data of 81 consecutive patients with type IV-A choledochal cyst were retrospectively analyzed. We divided these patients into two groups, the child group (age ≤ 18 years) and the adult group (age > 18 years). According to whether the patient received additional liver resection, patients were divided into a extrahepatic cystectomy (EHC) group and an additional liver resection (LR) group. The long-term outcomes after surgery were evaluated in two groups.

Results

Of all 81 patients, there were 17 children and 64 adults; 16 children and 35 adults belonged to EHC group, one child and 29 adults belonged to LR group. The morbidity of biliary stricture and/or lithiasis in the adults was significantly higher than that in the children (p = 0.041 < 0.05). In the EHC group, the reoperation rate of adults was significantly higher than that of children (p = 0.019 < 0.05). For adult patients, the morbidity of biliary stricture and/or lithiasis and the reoperation rate in EHC group was significantly higher than that in LR group (p = 0.037 < 0.05 and p = 0.026 < 0.05 respectively). Five adults were found to have cholangiocarcinoma within a follow-up period, while no child was found to. However, for adult patients, no significant discrepancy was observed between EHC group and LR group (p = 0.366 > 0.05).

Conclusions

The present study suggests that the children have better outcomes than adults for patients with type IV-A choledochal cyst after EHC, while LR brings better outcomes than EHC for adult patients.     

Non-operative management of early,acute appendicitis in children: Is it safe and effective?

Purpose

The purpose of this study was to determine if early, acute appendicitis in children can be safely and effectively managed with antibiotics alone.

Methods

A retrospective review was performed of children (< 18 yrs) treated non-operatively (NOM) for early, acute appendicitis since May 2012. These were compared to patients treated with appendectomy between January 2011 and October 2011 (OM). Inclusion criteria included: (a) symptoms < 48 h, (b) localized peritonitis, and (c) ultrasound findings consistent with early, acute appendicitis.

Results

Twelve patients (66% female, mean age 12.2,SD = 4.2 yrs) were treated non-operatively, while 12 (50% female, mean age 12.5,SD = 3.2 yrs) were treated operatively. Two NOM children (16.7%) required initial appendectomy. One patient developed recurrent appendicitis requiring appendectomy 7 months post-discharge. Four other NOM patients returned with symptoms but did not require admission or surgery. Two OM patients (8.3%) had hospital visits and admissions related to surgical site infections. Mean length of stay (LOS) for the first visit was 1.5 days (SD = 1.0d) (NOM) vs. 1.3 days (SD = 0.5d) (OM) (p = 0.61). Including first and subsequent admissions, mean LOS was 1.8 days (SD = 1.1d) (NOM) vs. 1.7 days (SD = 1.5d) (OM) (p = 0.97).

Conclusion

Early acute appendicitis in appropriately selected children can be successfully treated non-operatively. Randomized trials with longer follow-up are required.     

Perinatal management of congenital oropharyngeal tumors: The ex utero intrapartum treatment (EXIT) approach

Purpose

To present our experience in the perinatal management of fetuses with large oropharyngeal tumors by ex utero intrapartum treatment (EXIT).

Methods

We performed a retrospective chart review of all patients with congenital oropharyngeal tumor who underwent an EXIT procedure between May 2006 and June 2012.

Results

Four patients were included in the series, three females and one male. The diagnoses were epignathus (n = 2) and congenital epulis (n = 2). Three EXITs were done at term and one at late preterm due to premature rupture of membranes. Median maternal time under anesthesia was 185 min (range: 166–281) and median maternal operative time was 99 min (range: 85–153). Median maternal blood loss was 550 ml (range: 350–2000); one mother required a blood transfusion. Mean maternal hospital stay was 4 days. Median hysterotomy-to-cord clamp time was 24 min (range: 18–66). Mean fetal birth weight was 2.7 kg (range: 2.4–3). The airway was successfully accessed and secured under placental circulation in all cases. In the two patients with congenital epulis the tumors were resected at the base of their pedicles and the airway accessed via direct laryngoscopy before the umbilical cord was clamped. One patient with epignathus underwent a retrograde tracheal intubation under placental circulation and had the tumor resected thereafter. The second patient with epignathus had a tracheostomy done under placental circulation and then had tumor debulking immediately after the EXIT. The maternal morbidity was minimal and there were no mortalities.

Conclusions

We conclude that the EXIT procedure is the ideal delivery strategy for fetuses with prenatally diagnosed oropharyngeal tumors and potential airway obstruction at birth. Patients with prenatally diagnosed oropharyngeal tumors should be promptly referred to a fetal treatment center with a dedicated multidisciplinary team and EXIT capabilities.     

Initial bladder closure of the cloacal exstrophy complex: Outcome related risk factors and keys to success

Purpose

This study examines a large single-institution experience with cloacal exstrophy patients, analyzing patient demographics and surgical strategies predictive of bladder closure outcomes.

Methods

One hundred patients with cloacal exstrophy were identified. Complete closure history including demographics, operative history, and outcomes was available on 60 patients. Twenty-six patients with a history of failed initial bladder closure were compared to 34 with a history of successful initial bladder closure. Univariate logistic regression analysis was used to compare the two groups.

Results

Median follow up time after initial closure was 9 years (range: 13 months-29 years). A 1 cm increase in pre-closure diastasis resulted in a 2.64 increase in the odds of initial closure failure (p = 0.004). Protective strategies against failure included delaying closure (per month) (OR = 0.894, p = 0.009), employing pelvic osteotomies (OR = 0.095, p < 0.001), and applying external fixation (OR = 0.024; p = 0.001). Among patients who underwent osteotomy (31% of patients in the failed group, 82% in the successful group), a longer delay between osteotomy and closure (OR = 0.033; p = 0.005) was also protective against failure.

Conclusion

Patients with a large diastasis are more likely to fail initial closure. Delaying initial closure for at least 3 months, performing pelvic osteotomy, and using an external fixation device post-operatively are strategies that improve closure success.     

Ileal pouch anal anastomosis in pediatric familial adenomatous polyposis: A 24-year review of operative technique and patient outcomes

Background

Total proctocolectomy with ileal pouch anal anastomosis (IPAA) is the operative procedure of choice for familial adenomatous polyposis (FAP) patients. We review 24 years of operative experience and outcomes in pediatric patients with FAP.

Methods

Patients with FAP, age < 20 years, presenting to a single institution between 1987 and 2011 were included. Operative technique and outcomes were reviewed retrospectively. Primary outcomes included postoperative complications (30 days), long-term bowel function, and polyp recurrence at the anal anastomosis.

Results

95 patients with FAP underwent IPAA. Mean age at IPAA was 15.5 years with a mean follow-up of 7.6 years. 29 patients underwent 1-stage IPAA, 65 patients had a two-stage IPAA, and 1 patient underwent a 3-stage procedure. 67 patients had an open procedure, 25 underwent a laparoscopic approach, and more recently 3 patients underwent single incision laparoscopic IPAA. Patients with 1-stage IPAA demonstrate better long term bowel control vs. 2-stage IPAA patients (10.7% vs. 36.0% occasional incontinence, p = 0.018). However, 1-stage IPAA patients suffered increased short-term complications, such as anastomotic leak (17.2% vs. 0%, p = 0.002) and reoperation (20.7% vs. 4.6%, p = 0.02) compared to 2-stage IPAA. Anal anastomosis polyp recurrence occurred in 22.7% of 1-stage patients and 10.0% of 2-stage patients. Short-term complications, polyp recurrence, or long-term continence were equivalent between open and laparoscopic cases.

Conclusion

Single-stage IPAA in children with FAP is associated with better bowel control but increased anastomotic leak, reoperative rate, and polyp recurrence. In experienced hands, laparoscopic IPAA is equivocal to open IPAA.     

Determinants of wait time for infant inguinal hernia repair in a Canadian children’s hospital

Background

Longer wait time for infant inguinal hernia (IH) repair is associated with higher complication rates. We wished to determine if socioeconomic and demographic factors influence wait times for IH repair.

Methods

Children < 2 years old with IH at a Canadian children’s hospital were retrospectively reviewed. Days from diagnosis to surgical consultation (W1) and from consultation to repair (W2) were collected along with demographic, medical, and socioeconomic data. Linear regression analysis was performed.

Results

A total of 131 patients were appropriate for analysis (82.4% male). Median distance to hospital was 27.5 km (IQR = 10.5–50.4) and median income was $34,477 (IQR = 30,127–41,986). Median W1, W2, and Wtotal (W1 + W2) were 24 (IQR = 8–48), 43 (IQR = 21–69) and 79 (IQR = 38–112) days, respectively. Wait times were shorter in infants who were male (p = 0.044), symptomatic (p < 0.001), diagnosed in the ED (p < 0.001), or had an incarcerated hernia (p = 0.006). They were longer for premature infants (p = 0.009) and those with significant comorbidities (p = 0.018). Neither income (p = 0.328) nor distance from hospital (p = 0.292) was associated with longer wait times.

Conclusion

Wait times for IH repair were appropriately influenced by medical risk factors. Income and distance to hospital did not appear to influence wait times. A population-based study is needed to determine if these findings reflect a general trend within the Canadian health care system.     

Transanal endorectal pull-through for classic segment Hirschsprung's disease: With or without laparoscopic mobilization of the rectosigmoid?

Background

It has been suggested that the outcome of transanal endorectal pull-through for classic Hirschprung's disease can be improved by laparoscopically mobilizing the colon before the pullthrough.

Methods

Charts of 43 patients (2005–2009) with proven recto-sigmoid aganglionosis were retrospectively analyzed with respect to postoperative outcomes. Twenty-one had been treated with the transanal endorectal pull through (TERPT) and 22 with the laparoscopically assisted TERPT (LTERPT).

Results

Gender ratio, congenital anomalies, preoperative enterostomy, and follow up did not differ between the groups. More colon was resected in the TERPT group: median 25 cm vs. 15 cm in the L-TERPT group (p < 0.001). The TERPT-procedure took less time: median 153 min. vs. L-TERPT 263 min (p < 0.001). Postoperatively, three patients showed colonic torsions after TERPT (p = 0.07). The long-term clinical outcomes did not differ significantly between both groups. There was a significant association between length of resection and obstructive symptoms (OR = 0.92, p = 0.01).

Conclusion

Postoperative and clinical outcomes are similar using the TERPT or L-TERPT to correct classic segment Hirschsprung's disease. Prevention of colonic torsion should be the prime concern during the TERPT procedure. L-TERPT requires laparoscopic equipment and takes more operation time, whereas TERPT leaves no visible scars. The positive relation between the larger length of resection and obstructive symptoms requires additional research.