Conservative management of urachal anomalies.

PURPOSE: The purpose of this study was to evaluate trends in management of urachal anomalies at our institution and the safety of nonoperative care. METHODS: Based on our experience managing urachal remnants from 2000 to 2010 (reported in 2012), we adopted a more conservative approach, including preoperative antibiotic use, refraining from using voiding cystourethrograms (VCUG), postponing surgery until at least six months of age, and considering nonoperative management. A retrospective analysis of urachal anomaly cases was conducted (2011-2016) to assess trends in practice. Charts indicating anomalies of the urachus were pulled and trends in management (nonoperative versus surgical treatment), VCUG and antibiotic use, and outcomes were reviewed. RESULTS: Data from 2000-2010 and 2013-2016 were compared. Our findings indicate care has shifted towards nonoperative management. A smaller proportion of patients from 2013-2016 was treated surgically compared to 2000-2010. Patients receiving nonoperative treatment exhibited lower rates of complication relative to surgically managed cases. VCUGs were eliminated as a diagnostic tool for evaluating urachal anomalies. Prophylactic preoperative antibiotic use was standardized. No patients with a known urachal remnant presented later with an abscess or sepsis. CONCLUSIONS: We find that a shift towards nonoperative treatment of urachal anomalies did not adversely affect overall outcomes. We recommend observing minimally symptomatic patients, especially those under six months old. STUDY TYPE: Performance improvement. LEVEL OF EVIDENCE: Level IV.

Heterotaxy Syndrome and Intestinal Rotation Abnormalities.

BACKGROUND: Infants with heterotaxy syndrome (HS) have abnormal lateralization of organs along the right-left body axis. Intestinal rotation abnormalities (IRAs) are a potential source of morbidity and mortality. For this study, our objective was to prospectively observe a cohort of infants with HS and determine the incidence and natural history of IRA. METHODS: Infants ≤6 months of age with HS were enrolled in this prospective observational study. Exclusion criteria were other congenital abnormalities that necessitated abdominal surgery. HS was defined as any arrangement of organs that was not situs solitus or situs inversus along with associated congenital heart disease. The investigation for IRA was at the discretion of each participating center. RESULTS: Infants were recruited from January 2012 to December 2016. Thirty-eight infants from 7 institutions were included; 22 infants had right isomerism and 16 infants had left isomerism. Twenty-nine infants (76%) were evaluated for IRAs; 21 of 29 evaluations (72%) were abnormal. Eight infants were investigated because of symptoms, and 21 infants were evaluated routinely. The median age at symptom presentation was 46 days (range: 5-171 days). Seven infants had a Ladd procedure; 4 were prophylactic, with 3 as part of a combined procedure, and 3 were emergent. No child suffered acute midgut volvulus over a median follow-up of 1.6 years (range: 0.06-4.93 years). CONCLUSIONS: IRAs are common in infants with HS. Infants with symptoms presented by 6 months of age. There was no failure of expectant management resulting in midgut volvulus during a median follow-up of 1.6 years.

Neurodevelopmental outcomes of tracheoesophageal fistulas.

PURPOSE: The purpose of this study was to perform a retrospective review of tracheoesophageal fistula (TEF) patients who followed up in a state-sponsored program to assess neurodevelopmental outcomes. METHODS: Records were reviewed retrospectively of children who underwent TEF repair between August 2001 and June 2014. Children discharged from the neonatal intensive care unit were referred to the state-sponsored Developmental Tracking Infant Progress Statewide (TIPS) program. We reviewed TIPS assessments performed before age 24months and noted referral for early school intervention services. Poor outcomes were defined as scores of "failure" on the screening assessment or referral for enrollment in early intervention services by 24months. Children with TEF were compared with case-matched nonsyndromic children of similar gestational age and birth weight. RESULTS: Seventy-eight children underwent TEF repair. Thirty-eight followed up with TIPS. Survival was 93.6%. Predictors of hospital survival were Waterston classification (p=0.001), birth weight (p=0.027), and ventilator days (p=0.013). LOS was the only significant predictor of referral for early intervention services (p=0.0092) in multivariate analysis. There was a borderline significant difference in referral rate between children with TEF and controls. 52.6% of TEF patients were referred, while 34.2% of controls were referred (p=0.071). CONCLUSION: More than half of TEF patients experience neurodevelopmental delays requiring referral for early intervention (53%).

Trends in surgical management of urachal anomalies.

PURPOSE: We have noted an increasing frequency of diagnosed urachal anomalies. The purpose of this study is to evaluate this increase, as well as the outcomes of management at our institution over 10 years. METHODS: A retrospective analysis of urachal anomalies at our institution was performed. Inclusion criteria were Anomalies of Urachus (ICD 753.7) or Urinary Anomaly NOS (ICD 753.9) between January 2000 and December 2010. Exclusion criteria were having an asymptomatic urachal remnant incidentally excised. RESULTS: Eighty-five patients (49 male, 36 female) presented between 0 and 17 years of age (mean 1.5 years). Diagnoses increased from 0 in 2000 to 21 in 2010. Zero was surgically managed in 2000 while 21 were managed in 2010 (p=0.0145). Fifteen patients (17.6%) were observed with 13 (13/15, or 15.3%) resolving without complication while 2 were operated on. Average time to resolution (clinical or radiologic) was 4.9 months (Range: 0.4-12.6). A total of seventy-two patients (84.7%) underwent excision. Thirty-nine (54%) surgical cases were outpatient while 33 (46%) were admitted. Thirteen (18%) had post-operative complications. Ten (77%) of the complications were wound infections. Patients under 6 months of age accounted for 60% (6 of 10) of all wound infections and 52% (17 of 33) of hospitalizations. CONCLUSIONS: Our experience and review of the literature suggest a high complication rate with surgical management in young patients, mostly from infections and support non-operative management of all non-infected urachal remnants in children.

Identifying strategies to decrease infectious complications of gastroschisis repair.

PURPOSE: We describe the infectious complications of gastroschisis in order to identify modifiable factors to decrease these complications. METHODS: Data from 155 gastroschisis patients (2001-2013) were reviewed. Complicated gastroschisis (intestinal atresia, necrotic bowel, or perforation) were excluded, leaving 129 patients for review. Patient demographics, surgical details, postoperative infections and complications, and length of stay were reviewed. We used CDC definitions of infectious complications. RESULTS: The average gestational age of patients was 35.97weeks. Silos were used in 46% of patients (n=59) for an average of 7.4days. Thirty-one patients (24%) acquired an infection within the first 60days of life. Patients who developed an infection were born earlier in gestation (P=0.02), weighed less (P=0.01), required silos more often (P=0.01), and received a sutured repair (P=0.04). Length of stay of patients with an infection was longer than in patients without infection (P=0.01). CONCLUSIONS: Infectious complications following gastroschisis repair are common. Subsets of gastroschisis patients at increased risk of infection include patients with silos, preterm delivery, low birth weight, and sutured repair. Based on our findings, our recommendation would be to carry gastroschisis patients to term and advocate against the routine use of silos, reserving their use for those cases when primary closure is not possible.

Chronic cervical esophageal foreign bodies in children: surgical approach after unsuccessful endoscopic management.

OBJECTIVES: We reviewed the surgical management of chronic cervical esophageal foreign bodies (CCEFBs) in a pediatric population after failed endoscopic retrieval. METHODS: A descriptive analysis via a retrospective chart review of patients with CCEFBs who failed initial endoscopic management was performed between 2008 and 2013. Details were recorded regarding presenting symptoms, time from symptom onset to diagnosis of the CCEFB, surgical approach, and complications. RESULTS: Three patients with CCEFBs unsuccessfully managed with endoscopy were identified. The range of ages at diagnosis was 14 months to 4.5 years. The foreign bodies (FBs) were present for at least 1 month before diagnosis (range, 1 to 10 months). Respiratory symptoms were predominant in all cases. Neck exploration with removal of the FB was performed in each case. Complications included esophageal stricture necessitating serial dilations (patient 1), left true vocal fold paresis that resolved spontaneously (patient 3), and tracheoesophageal fistula with successful endoscopic closure (patient 3). No long-term sequelae were experienced. CONCLUSIONS: A high index of suspicion is required to recognize CCEFBs in children with respiratory distress. Although endoscopic management remains the first-line treatment, it may fail or may not be possible because of transmural FB migration. In this setting, neck exploration with FB removal is a safe and effective alternative.

Cerebral oxygenation in major pediatric trauma: its relevance to trauma severity and outcome.

INTRODUCTION: Trauma is the commonest cause of death in the pediatric population, which is prone to diffuse primary brain injury aggravated by secondary insults (eg, hypoxia, hypotension). Standard monitoring involves intracranial pressure (ICP) and cerebral perfusion pressure, which do not reflect true cerebral oxygenation (oxygen delivery [Do(2)]). We explore the merits of a brain tissue oxygen-directed critical care guide. METHODS: Sixteen patients with major trauma (Injury Severity Score, >16/Pediatric Trauma Score [PTS], <7) had partial pressure of brain tissue oxygen (Pbto(2)) monitor (Licox; Integra Neurosciences, Plainsboro, NJ) placed under local anesthesia using twist-drill craniostomy and definitive management of associated injuries. Pbto(2) levels directed therapy intensity level (ventilator management, inotrops, blood transfusion, and others). Patient demographics, short-term physiological parameters, Pbto(2), ICP, Glasgow Coma Score, trauma scores, and outcomes were analyzed to identify the patients at risk for low Do(2). RESULTS: There were 10 males and 6 females (mean age, 14 years) sustaining motor vehicle accident (14), falls (1), and assault (1), with a mean Injury Severity Score of 36 (16-59); PTS, 3 (0-7); and Revised Trauma Score, 5.5 (4-11). Eleven patients (70%) had low Do(2) (Pbto(2), <20 mm Hg) on admission despite undergoing standard resuscitation affected by fraction of inspired oxygen, Pao(2), and cerebral perfusion pressure (P = .001). Eubaric hyperoxia improved cerebral oxygenation in the low-Do(2) group (P = .044). The Revised Trauma Score (r = 0.65) showed moderate correlation with Pbto(2) and was a significant predictor for low Do(2) (P = .001). In patients with Pbto(2) of less than 20 mm Hg, PTS correlated with cerebral oxygenation (r = 0.671, P = .033). The mean 2-hour Pbto(2) and the final Pbto(2) in survivors were significantly higher than deaths (21.6 vs 7.2 mm Hg [P = .009] and 25 vs 11 mm Hg [P = .01]). Although 4 of 6 deaths were from uncontrolled high ICP, PTS and 2-hour low Do(2) were significant for roots for mortality. CONCLUSIONS: Pbto(2) monitoring allows for early recognition of low-Do(2) situations, enabling appropriate therapeutic intervention.