Risk stratification in pediatric perforated appendicitis: Prospective correlation with outcomes and resource utilization.

PURPOSE: Despite a wide spectrum of severity, perforated appendicitis in children is typically considered a single entity in outcomes studies. We performed a prospective cohort study to define a risk stratification system that correlates with outcomes and resource utilization. METHODS: A prospective study was conducted of all children operated for perforated appendicitis between May 2015 and December 2016 at a tertiary free-standing university children's hospital. Surgical findings were classified into one of four grades of perforation: I. localized or contained perforation, II. Contained abscess with no generalized peritonitis, III. Generalized peritonitis with no dominant abscess, IV. Generalized peritonitis with one or more dominant abscesses. All patients were treated on a clinical pathway that involved all points of care from admission to final follow-up. Outcomes and resource utilization measures were analyzed using Fisher's exact test, Kruskal-Wallis test, One-way ANOVA, and logistic regression. RESULTS: During the study period, 122 patients completed treatment, and 100% had documented follow-up at a median of 25days after operation. Grades of perforation were: I, 20.5%; II, 37.7%; III, 10.7%; IV, 31.1%. Postoperative abscesses occurred in 12 (9.8%) of patients, almost exclusively in Grade IV perforations. Hospital stay, duration of antibiotics, TPN utilization, and the incidence of postoperative imaging significantly increased with increasing grade of perforation. CONCLUSION: Outcomes and resource utilization strongly correlate with increasing grade of perforated appendicitis. Postoperative abscesses, additional imaging, and additional invasive procedures occur disproportionately in patients who present with diffuse peritonitis and abscess formation. The current stratification allows risk-adjusted outcome reporting and appropriate assignment of resource burden. LEVEL OF EVIDENCE: I (Prognosis Study).

Standardization of care for pediatric perforated appendicitis improves outcomes.

BACKGROUND: The treatment of perforated appendicitis in children is characterized by significant variability in care, morbidity, resource utilization, and outcomes. We prospectively studied how minimization of care variability affects outcomes. METHODS: A clinical pathway for perforated appendicitis, in use for three decades, was further standardized in May 2015 by initiation of a disease severity classification, refinement of discharge criteria, standardization of the operation, and establishment of criteria for use of postoperative total parenteral nutrition, imaging, and invasive procedures. Prospective evaluation of all children treated for 20months on the new fully standardized protocol was conducted and compared to a retrospective cohort treated over 58months prior to standardization. Differences between outcomes before and after standardization were analyzed using regression analysis techniques to adjust for disease severity. RESULTS: Median follow-up time post discharge was 25 and 14days in the post- and prestandardization groups, respectively. Standardization significantly reduced postoperative abscess (9.8% vs. 17.4%, p=0.001) and hospital stay (p=0.002). Standardization reduced the odds of developing a postoperative abscess by four fold. CONCLUSION: Minimizing variability of care at all points in the treatment of perforated appendicitis significantly improves outcomes. TYPE OF STUDY: Prospective Cohort Study. LEVEL OF EVIDENCE: Level II.

Vaginal anomalies and atresia associated with imperforate anus: diagnosis and surgical management.

BACKGROUND: The association of vaginal atresia (or Mayer-Rokitansky-Kuster-Hauser Syndrome) with imperforate anus is rare and can present significant diagnostic and therapeutic challenges. This study describes clinical characteristics, surgical treatment and outcomes in this group of complex children. METHODS: Records of 20 patients were retrospectively analyzed from two pediatric surgical centers. RESULTS: Five patients were excluded from the long-term analysis due to inadequate information, leaving long-term follow-up in 15 patients. Mean follow-up was 10 years (range 1-31.1 years). The diagnosis of vaginal atresia was made pre-operatively in 12 out of 15 patients, and in three patients it was identified during the anoplasty. The anorectal malformations were rectoperineal (N=2), rectovestibular (N=6), recto-bladder neck (N=1) and imperforate anus without fistula (N=6). Satisfactory surgical repair was performed in 13 patients, while one continues to stool through a low perineal fistula awaiting definitive surgery and another underwent a colostomy and mucous fistula. Delayed vaginal reconstruction was due to a failure to identify the problem prior to anoplasty (N=3). Long-term results demonstrated that anorectal continence was much worse than initially appreciated, and many had associated urinary incontinence. Overall stooling score was far lower than in a separate group of children with imperforate anus without vaginal atresia (Levitt and Peña, 2007). CONCLUSIONS: Vaginal atresia with imperforate anus is a rare and an extensive pre-operative workup of females with imperforate anus must include assessment of vagina patency. Vaginal reconstruction and anorectal continuity can be performed in a variety of approaches, but long-term continence is often not optimal. We propose a pathway for management of this difficult genito-anorectal disorder.

Pulmonary hypertension management in neonates.

The management of pulmonary hypertension is multi-faceted, with therapies directed at supporting cardiovascular and pulmonary function, treating the underlying cause (if feasible), and preventing irreversible remodeling of the pulmonary vasculature. Recently, manipulation of signaling pathways and mediators contained within the pulmonary vascular endothelial cell has become a new target. This article will review the pathophysiology of pulmonary hypertension and the broad principles involved in its management, with specific emphasis on pharmacological therapies directed at the pulmonary vascular endothelium.