Hirschsprung disease in the premature newborn: a population based study and 40-year single center experience.

BACKGROUND/PURPOSE: Understanding of Hirschsprung disease (HD) in premature newborns (PHD) is anecdotal. We have sought in this study to identify the demographic and clinical features of PHD. METHODS: All patients with HD 1970-2011 treated at our tertiary care children's hospital were identified. Patients with biopsy confirmed HD and EGA <37weeks were selected for further review. Prenatal and birth data, demographics, clinical signs, radiologic and pathologic data, and operative interventions were examined. The occurrence of PHD was observed using data from the Utah Department of Health database 1997-2011. RESULTS: 404 patients with HD from 1970 to 2011 were treated. Twenty-seven (6.7%) had PHD. Mean birth weight in PHD was 2196grams and mean gestational age 34 (range 29-36)weeks. Seven patients had Down syndrome. Nonchromosomal anomalies occurred in 25%. Median time from birth to biopsy diagnosis was 42days (range 2-316days). The most common presenting signs were abdominal distension and bilious emesis. The HD incidence in Utah for all births was 1/4322 (0.023%) and for premature infants 1/3885 (0.027%). CONCLUSIONS: PHD are similar to term infants with HD. Diagnosis of HD is often delayed in premature newborns, and associated anomalies are more common.

The effect of ergonomic laparoscopic tool handle design on performance and efficiency.

BACKGROUND: Many factors can affect a surgeon's performance in the operating room; these may include surgeon comfort, ergonomics of tool handle design, and fatigue. A laparoscopic tool handle designed with ergonomic considerations (pistol grip) was tested against a current market tool with a traditional pinch grip handle. The goal of this study is to quantify the impact ergonomic design considerations which have on surgeon performance. We hypothesized that there will be measurable differences between the efficiency while performing FLS surgical trainer tasks when using both tool handle designs in three categories: time to completion, technical skill, and subjective user ratings. METHODS: The pistol grip incorporates an ergonomic interface intended to reduce contact stress points on the hand and fingers, promote a more neutral operating wrist posture, and reduce hand tremor and fatigue. The traditional pinch grip is a laparoscopic tool developed by Stryker Inc. widely used during minimal invasive surgery. Twenty-three (13 M, 10 F) participants with no existing upper extremity musculoskeletal disorders or experience performing laparoscopic procedures were selected to perform in this study. During a training session prior to testing, participants performed practice trials in a SAGES FLS trainer with both tools. During data collection, participants performed three evaluation tasks using both handle designs (order was randomized, and each trial completed three times). The tasks consisted of FLS peg transfer, cutting, and suturing tasks. RESULTS: Feedback from test participants indicated that they significantly preferred the ergonomic pistol grip in every category (p < 0.05); most notably, participants experienced greater degrees of discomfort in their hands after using the pinch grip tool. Furthermore, participants completed cutting and peg transfer tasks in a shorter time duration (p < 0.05) with the pistol grip than with the pinch grip design; there was no significant difference between completion times for the suturing task. Finally, there was no significant interaction between tool type and errors made during trials. CONCLUSIONS: There was a significant preference for as well as lower pain experienced during use of the pistol grip tool as seen from the survey feedback. Both evaluation tasks (cutting and peg transfer) were also completed significantly faster with the pistol grip tool. Finally, due to the high degree of variability in the error data, it was not possible to draw any meaningful conclusions about the effect of tool design on the number or degree of errors made.

Effect of gastrojejunal feedings on visits and costs in children with neurologic impairment.

OBJECTIVE: The objective of the present study was to determine the effect of gastrojejunal tube (GJT) feedings in children with neurologic impairment (NI) on gastroesophageal reflux disease (GERD)- and/or dysfunctional swallowing-related visits and their associated costs. METHODS: The present study is a retrospective cohort study of children with NI and GERD who underwent GJT placement at the study hospital from December 1999 to October 2006. Visits (emergency department, radiology, and hospitalizations) were reviewed from the time of birth until 1 year following GJT placement and classified as either not GERD and/or dysfunctional swallowing related or GERD and/or dysfunctional swallowing related (eg, pneumonias). Incident rate ratios (IRRs) were calculated by dividing the post-GJT visit rate by the pre-GJT visit rate. Other outcomes included associated costs, fundoplications, and deaths. RESULTS: Thirty-three patients met inclusion criteria. The IRR for total visits was 1.78 (95% confidence interval [CI] 1.12-2.81) and for GERD- and/or dysfunctional swallowing-related visits 2.88 (95% CI 1.68-4.94). Feeding tube-related visits (IRR 5.36, 95% CI 2.73-10.51) accounted for the majority. GERD- and/or dysfunctional swallowing-related costs per child per year were low overall, with no difference from pre-GJT versus post-GJT placement ($1851 vs $4601, P = 0.89). Seven (21%) children underwent Nissen fundoplication and 4 (12%) died within 1 year of GJT placement. Two deaths involved jejunal perforation. CONCLUSIONS: Children with NI and GERD who are treated with GJT feedings have significantly more GERD- and/or dysfunctional swallowing-related visits in the following year. The majority of these visits are because of the procedural complications, which are inexpensive. There is, however, mortality associated with the GJT and some children proceed to a fundoplication.

The role of focused abdominal sonography for trauma (FAST) in pediatric trauma evaluation.

PURPOSE: With increasing concerns about radiation exposure, we questioned whether a structured program of FAST might decrease CT use. METHODS: All pediatric trauma surgeons in our level 1 pediatric trauma center underwent formal FAST training. Children with potential abdominal trauma and no prior imaging were prospectively evaluated from 10/2/09 to 7/31/11. After physical exam and FAST, the surgeon declared whether the CT could be eliminated. RESULTS: Of 536 children who arrived without imaging, 183 had potential abdominal trauma. FAST was performed in 128 cases and recorded completely in 88. In 48% (42/88) the surgeon would have elected to cancel the CT based on the FAST and physical exam. One of the 42 cases had a positive FAST and required emergent laparotomy; the others were negative. The sensitivity of FAST for injuries requiring operation or blood transfusion was 87.5%. The sensitivity, specificity, PPV, and NPV in detecting pathologic free fluid were 50%, 85%, 53.8%, and 87.9%. CONCLUSIONS: True positive FAST exams are uncommon and would rarely direct management. While the negative FAST would have potentially reduced CT use due to practitioner reassurance, this reassurance may be unwarranted given the test's sensitivity.

Split abdominal wall muscle flap repair vs patch repair of large congenital diaphragmatic hernias.

PURPOSE: Large congenital diaphragmatic hernias are commonly repaired with a prosthetic patch. We hypothesized that a split abdominal wall muscle flap would reduce the risk of recurrence. METHODS: A retrospective review of neonates with congenital diaphragmatic hernia in whom primary repair was not possible was performed. Kaplan-Meier analyses and Cox proportional hazards modeling were performed. RESULTS: Of 153 patients, 46 could not have repair with primary closure of the diaphragm. Thirty-three survived to discharge and were subjected to analysis for recurrence. Ten underwent repair with a patch, whereas 23 had a muscle flap (internal oblique and transversalis) patch. The groups were similar with regard to demographics, need for extracorporeal membrane oxygenation, repair on extracorporeal membrane oxygenation, and size of the defect. Fifty percent of patch repairs recurred with a median time of 0.5 years. Only one (4.3%) of the patients who had muscle flap patch developed a recurrence. This was significant on Kaplan-Meier analysis (P = .0009) and had a hazard ratio of 14.3 on Cox regression (P = .018). Median follow-up exceeded 4 years. No children required surgery for an abdominal wall hernia. CONCLUSIONS: The split abdominal wall muscle flap allows for closure of large congenital diaphragmatic hernia defects with autologous tissue. This approach is associated with significantly fewer recurrences than patch repairs.

Reflux esophageal stricture--a review of 30 years' experience in children.

PURPOSE: Strictures of the esophagus in children may have multiple etiologies including congenital, inflammatory, infectious, caustic ingestion, and gastroesophageal reflux (peptic stricture [PS]). Current literature lacks good data documenting long-term outcomes in children. This makes it difficult to counsel some patients about realistic treatment expectations. The objective of this study is to evaluate our institutional experience and define the natural history and treatment outcomes. METHODS: A retrospective review of clinical data obtained from children who underwent dilation for PS was performed. RESULTS: Over the past 30 years, 114 children and adolescents received 486 dilations. The most common indications for stricture dilation were PS (42%) and esophageal atresia (38%). Other lesser indications included congenital, foreign body, corrosive, cancer, radiation, allergic, and infectious. This review focuses on the 48 children with PS. Of the children with PS, a congenital anomaly was identified in 23 children; and 12 had neurologic impairment. Average age at presentation was 10.2 years (range, 0.5-18.3 years). Most patients had had symptoms for many months before diagnosis. Peptic stricture was most common in the lower esophagus (n = 39). However, middle (n = 8) and upper (n = 1) strictures were occasionally identified. Noncompliance with medical therapy was a challenge in 12% (n = 5) of children. Children with a PS received a median of 3 dilations, but a subset of 5 patients with severe strictures underwent up to 48 dilations (range, 1-48). Repeated dilations were required for a median of 20 months (range, 1-242 months). Among patients receiving esophageal dilation for PS, 94% required an antireflux procedure (19% required a second antireflux surgery). A subgroup of patients (n = 10) was identified who required extended dilations, multiple surgeries, and esophageal resection. This subgroup had a significantly longer period of symptomatic disease and increased risk of esophageal resection compared with those patients requiring fewer dilations. Surgical resection of the esophageal stricture was ultimately required in 3 children with PS after failure of more conservative measures. CONCLUSION: Children and adolescents presenting with reflux esophageal stricture (PS) frequently require antireflux surgery, redo antireflux surgery, and multiple dilations for recurrent symptoms. We hope that these data will be of use to the clinician attempting to counsel patients and parents about treatment expectations in this challenging patient population.

Infarction of an extralobar pulmonary sequestration in a young child: diagnosis and excision by video-assisted thoracoscopy.

A 4-year-old male presented with abdominal pain. A computed tomography scan of the abdomen was negative, but a pleural effusion and mass was noted in the lower left thorax. Video-assisted thoracoscopic surgery revealed the mass to be a rare case of extralobar pulmonary sequestration that had undergone infarction.

Reflux related hospital admissions after fundoplication in children with neurological impairment: retrospective cohort study.

OBJECTIVE: To examine the impact of fundoplication on reflux related hospital admissions for children with neurological impairment. DESIGN: Retrospective, observational cohort study. Setting 42 children's hospitals in the United States. PARTICIPANTS: 3721 children with neurological impairment born between 2000 and 2005 who had at least one hospital admission at a study hospital before their fundoplication. INTERVENTION: Fundoplication. MAIN OUTCOME MEASURES: Incident rate ratio for reflux related hospital admissions, defined as the post-fundoplication admission rate divided by the pre-fundoplication admission rate. RESULTS: Of the 955 285 children born during the study period, 144,749 (15%) had neurological impairment. Of these, 27,720 (19%) were diagnosed as having gastro-oesophageal reflux disease, of whom 6716 (24%) had a fundoplication. Of these, 3721 (55%) had at least one previous hospital admission and were included in the study cohort. After fundoplication, hospital admissions decreased for any reflux related cause (incident rate ratio 0.69, 95% confidence interval 0.67 to 0.72; P<0.01), aspiration pneumonia (0.71, 0.62 to 0.81; P<0.01), gastro-oesophageal reflux disease (0.60, 0.57 to 0.63; P<0.01), and mechanical ventilation (0.40, 0.37 to 0.43; P<0.01), after adjustment for other patient and hospital related factors that may influence reflux related hospital admissions. Hospital admissions increased for asthma (incident rate ratio 1.52, 1.38 to 1.67; P<0.01) and remained constant for pneumonia (1.07, 0.98 to 1.17; P=0.16). Conclusions Children with neurological impairment who have fundoplication had reduced short term reflux related hospital admissions for aspiration pneumonia, gastro-oesophageal reflux disease, and mechanical ventilation. However, admissions for pneumonia remained constant and those for asthma increased after fundoplication. Comparative effectiveness data for other treatments (such as gastrojejunal feeding tubes) are unknown.

Impact of fundoplication versus gastrojejunal feeding tubes on mortality and in preventing aspiration pneumonia in young children with neurologic impairment who have gastroesophageal reflux disease.

OBJECTIVE: Aspiration pneumonia is the most common cause of death in children with neurologic impairment who have gastroesophageal reflux disease. Fundoplications and gastrojejunal feeding tubes are frequently employed to prevent aspiration pneumonia in this population. Which of these approaches is more effective in preventing aspiration pneumonia and/or improving survival is unknown. The objective of this study was to compare outcomes for children with neurologic impairment and gastroesophageal reflux disease after either a first fundoplication or a first gastrojejunal feeding tube. PATIENTS AND METHODS: This was a retrospective, observational cohort study of children with neurologic impairment who had either a fundoplication or gastrojejunal feeding tube between January 1997 and December 2005 at a tertiary care children's hospital. Main outcome measures were postprocedure aspiration pneumonia-free survival and mortality. Propensity analyses were used to control for bias in treatment assignment and prognostic imbalances. RESULTS: Of the 366 children with neurologic impairment and gastroesophageal reflux disease, 43 had a first gastrojejunal feeding tube and 323 underwent a first fundoplication. Median length of follow-up was 3.4 years. Children who received a first fundoplication had similar rates of aspiration pneumonia and mortality after the procedure compared with those who had a first gastrojejunal feeding tube, when adjusting for the treatment assignment using propensity scores. CONCLUSIONS: Aspiration pneumonia and mortality are not uncommon events after either a first fundoplication or a first gastrojejunal feeding tube for the management of gastroesophageal reflux disease in children with neurologic impairment. Neither treatment option is clearly superior in preventing the subsequent aspiration pneumonia or improving overall survival for these children. This complex clinical scenario needs to be studied in a prospective, multicenter, randomized control trial to evaluate definitively whether 1 of these 2 management options is more beneficial.

Division of the fistula in laparoscopic-assisted repair of anorectal malformations-are clips or ties necessary?

BACKGROUND: Laparoscopic-assisted anorectoplasty (LAARP) was introduced in 2000 by Georgeson (J Pediatr Surg. 2000;35:927-930) and has gained interest because of improved visualization of the rectal fistula and the ability to place the pull-through segment within the levator muscle complex with minimal dissection. Currently, there is no consensus on how the fistula should be managed during LAARP. We postulated that the fistula could be managed with simple division and temporary diversion of urine through a Foley catheter without surgical ligation of the fistula similar to the management of a traumatic urethral injury. METHODS: A retrospective chart review was performed of patients with imperforate anus who underwent LAARP between January 2005 and September 2007. RESULTS: Eight patients were managed with a LAARP. Five male patients had the fistula simply divided. In these 5 patients, the location of the fistula was rectoprostatic (2) and rectobulbar (3). The Foley catheter was left in position until a retrograde urethrogram demonstrated no evidence of a leak (range, 6-40 days). There were no postoperative urethral strictures and one diverticulum. Follow-up has ranged from 10 to 19 months. CONCLUSION: Male patients with a rectourethral fistula at or just below the prostate can be safely and successfully managed with simple division of the fistula.

Quality of life of children with neurological impairment who receive a fundoplication for gastroesophageal reflux disease.

BACKGROUND: Children with neurological impairment (NI) commonly have gastroesophageal reflux disease (GERD) treated with a fundoplication. The impact of this procedure on quality of life is poorly understood. OBJECTIVES: To examine the quality of life of children with NI who have received a fundoplication for GERD and of their caregivers. METHODS: The study was a prospective cohort study of children with NI and GERD who underwent a fundoplication at a children's hospital between January 1, 2005, and July 7, 2006. Quality of life of the children was assessed with the Child Health Questionnaire (CHQ) and of the caregivers with the Short-Form Health Survey Status (SF-36) and Parenting Stress Index (PSI), both at baseline and 1 month after fundoplication. Functional status was assessed using the WeeFIM. Repeated-measures analyses were performed. RESULTS: Forty-four of the 63 parents (70%) were enrolled. The median WeeFIM score was 31.2 versus the age-normal score of 83 (P = .001). Compared with the baseline scores, mean CHQ scores improved over 1 month in the domains of bodily pain (32.8 vs. 47.5, P = .01), role limitations-physical (30.6 vs. 56.6, P = .01), mental health (62.7 vs. 70.6, P = .01), family limitation of activities (43.3 vs. 55.1, P = .03), and parental time (43.0 vs. 55.3, P = .03). The parental SF-36 domain of vitality improved from baseline over 1 month (41.3 vs. 48.2, P = .001), but there were no changes from baseline in Parenting Stress scores. CONCLUSIONS: Parents reported that the quality of life of children with NI who receive a fundoplication for GERD was improved from baseline in several domains 1 month after surgery. The quality of life and stress of caregivers did not improve in nearly all domains, at least in the short term.

Hyperglycemia and outcomes from pediatric traumatic brain injury.

BACKGROUND: The clinical significance of hyperglycemia after pediatric traumatic brain injury is controversial. This study addresses the relationship between hyperglycemia and outcomes after traumatic brain injury in pediatric patients. METHODS: We identified trauma patients admitted during a single year to our regional pediatric referral center with head regional Abbreviated Injury Scale scores > or = 3. We studied identified patients for admission characteristics potentially influencing their outcomes. The primary outcome measure was Glasgow Outcome Scale score. RESULTS: Patients who died had significantly higher admission serum glucose values than those patients who survived (267 mg/dL vs. 135 mg/dL; p = 0.000). Admission serum glucose > or = 300 mg/dL was uniformly associated with death. Admission Glasgow Coma Scale score (odds ratio, 0.560; 95% confidence interval, 0.358-0.877) and serum glucose (odds ratio, 1.013; 95% confidence interval, 1.003-1.023) are independent predictors of mortality in children with traumatic head injuries. CONCLUSION Hyperglycemia and poor neurologic outcome in head-injured children are associated. The pathophysiology of hyperglycemia in neurologic injury after head trauma remains unclear.