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Purpose The identification of urachal remnants is occurring more in infancy. Despite evidence that nonoperative management is effective, operative management remains common and has a high complication rate. We sought to determine if the complication rate after urachal resection is associated with age. Methods Patients undergoing urachal remnant resection were identified from ACS NSQIP Pediatric from 2013 to 2017. Exclusion criteria included emergent operations, contaminated wounds, and any additional procedures. Patients were compared based on complication rates, need for reoperation or readmission, and length of stay. Results A complication occurred in 16 of 476 patients (3.3%), 6 (1.3%) had reoperation, and 11 (2.3%) were readmitted. The median age for patients requiring reoperation was lower (0.1 years) than those not (1.3 years; p = 0.004). The median age of those readmitted was lower (0.4 years) than those not (1.4 years, p = 0.03), and a weak trend of longer length of stay in younger patients was identified (ρ = − 0.16, p Conclusions Operative management of younger patients resulted in greater risk of reoperation, readmission, and longer length of stay. Given that nonoperative management is effective, it may be of benefit to delay resection of urachal remnants to after 1 year of age. Study type Treatment study. Level of evidence Level III.

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.

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.

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.

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%).

Purpose: To determine if routine bridling of nasoenteric feeding tubes is a low-morbidity method of decreasing unintended tube dislodgement in pediatric patients. Methods: From November 2012 to June 2015, bridle systems were implemented in 30 pediatric patients with nasoenteric feeding tubes in place for an extended period of time, and data was prospectively collected. Historical cohort controls were 33 tracheoesophageal fistula (TEF) repair patients with taped nasoenteric tubes from 2001 to 2012. A second control group was 20 patients with nasoenteric tubes placed under fluoroscopy from February 2012 to July 2013. Analysis compared bridled children with these 2 groups to look at differences in total number tube dislodgements and tube dislodgements per 100 days. Complications related to bridle use were noted. Results: Among the 30 bridled patients, only 4 tube dislodgements were recorded in 1553 total days a tube was in place. During each of these instances, the bridle remained in place. There was a significant association between experimental group and total number tube dislodgements: only 3 of 30 bridled patients experienced tube dislodgements, compared to 18 of 33 TEF patients (p=0.0006) and 9 of 20 patients with fluoroscopically placed tubes (p=0.021). Children with bridled tubes experienced a lower rate of tube dislodgements per 100 days than TEF children with taped tubes (p

Intestinal failure is most commonly treated by the administration of total parenteral nutrition (TPN). In some patients, however, surgical therapy may increase the ability to use the intestine for nutrition and thereby decrease the complications of TPN therapy. A multidisciplinary comprehensive intestinal failure program was initiated at the University of Nebraska Medical Center in October 2000. Here we describe the surgical approaches to patients with short bowel syndrome and the subsequent impact on the need for TPN and on survival. Fifty patients (children = 30, adults = 20) underwent surgical procedures to restore intestinal continuity (n = 5), repair enterocutaneous fistulas (n = 5), resect dysmotile or strictured/obstructed bowel segments or mesenteric desmoid tumors (n = 7), stricturoplasty (n = 2), Bianchi tapering and lengthening (n = 20), serial transverse enteroplasty (n = 8), and other operations (n = 8). Of these 50 patients, three patients did not require TPN after surgical intervention and seven had remnant small bowel anatomy that precluded TPN weaning (e.g., end duodenostomy) and were listed for transplantation or continued on full TPN support. Of the 40 remaining patients, most received the majority of calories fromTPNat the time of referral, i.e., mean calories fromTPN _ 90%. Subsequent to the surgical and medical therapy, 26 (65%) have been completely weaned off TPN. In addition, 10 had substantial decreases in their TPN requirements (i.e., from 85% of calories from TPN at onset decreased to a median 35% of required calories at most recent follow-up). Four patients remained on the same amount of TPN support. Four of the seven patients listed for transplantation underwent successful transplantation. Despite the complications of short bowel syndrome, 86% (n = 43) of the patients are alive and well at a mean follow-up of 2 years. Patient deaths occurred primarily in those listed or eligible for transplantation and were related to advanced liver disease (n = 3), gastrointestinal hemorrhage (n = 1), or line sepsis (n = 1). Two other patients died, one from influenza A infection and one from unknown cause at home, months after complete discontinuation of TPN. In this series of patients with short bowel syndrome, surgical intervention led to weaning or discontinuation of TPN support in 85% of patients. An organized multidisciplinary approach to the patient with short bowel syndrome is recommended.

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.

The optimal age at which to perform orchiopexy for cryptorchidism has long been debated. The aim of this study was to determine if age at orchiopexy affected testicular atrophy.A retrospective review of patients undergoing orchiopexy from 2000 to 2010 was conducted. An individual testis, rather than patient, was used as the dependent variable. A total of 349 testicles from 1126 charts (ICD-9=752.51) were identified. Primary study outcome was testicular survival without atrophy.Mean follow up for the study was 25 months. There was postoperative atrophy in 27 testes (7.7%). Intraabdominal testicle was independently associated with increased postsurgical atrophy (p0.0001). The odds of postsurgical atrophy were 15.66 times higher for an abdominal vs. inguinal location (95% CI: 5.5-44.6). Testicular atrophy was highest for orchiopexy at ages 13-24 months (n=16 of 133, 12%) vs. those less than 13 months (n=3 of 64, 5%), and those greater than 24 months (n=8 of 152, 5%) (p=0.0024). After adjusting for location, age was not statistically significant with postsurgical atrophy (p=0.055).From this study we conclude that there is no increase in testicular atrophy in patients less than 13 months.

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.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).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.Pbto(2) monitoring allows for early recognition of low-Do(2) situations, enabling appropriate therapeutic intervention.

Recurrence of Crohn's disease after surgery is a common occurrence, pointing to the need for a strategy to prevent recurrent disease. We report the postoperative course of 10 patients who required intestinal resections for complications related to Crohn's disease.All patients had a Pediatric Crohn's Disease Activity Index score of 10 or greater. Among these patients, 5 began treatment with 6-mercaptopurine in the perioperative period. All 10 had received various combinations of prednisone and salicylate compounds. Patients who were given 6-mercaptopurine did not discontinue the medication until 2 years after the surgery.To date, none of the five patients who were placed on 6-mercaptopurine have had recurrence of their Crohn's disease (mean disease-free period 32.6 +/- 18.4 months). Among those five patients not receiving 6-mercaptopurine there have been three relapses (mean time to relapse 3.7 +/- 1.2 months). Log-rank sum analyses of Kaplan-Meier survival curves show benefit to patients receiving 6-mercaptopurine in preventing relapses after intestinal resection (p0.05).Although the underlying pathophysiologic reasons leading to the high relapse rate after intestinal surgery in Crohn's disease are unknown, we conclude that treatment with 6-mercaptopurine in the perioperative period may be warranted to help prevent the recurrence of Crohn's disease after surgery.

Congenital diaphragmatic hernia (CDH) presents beyond the first hours of life in 10% to 20% of cases. Presenting symptoms may be quite nonspecific, and are often gastrointestinal rather than respiratory in origin. We have recently had experience with five such cases, one in a newborn and four in older children. All presented with symptoms related to gastrointestinal complications of their diaphragmatic defect. In the newborn, gastric perforation had occurred, a complication of this anomaly not previously reported. The chest radiograph showed loops of bowel in the chest in all cases, allowing correct preoperative diagnoses. Urgent operative intervention was undertaken in each case with good results and no long-term morbidity. The risk of intestinal strangulation in the late-presenting CDH patient warrants emergent surgical management, which should be rewarded by uniform survival with few complications. Although preoperative stabilization may decrease the severity of pulmonary vasospasm in the newborn with respiratory failure, delay may increase the risk of bowel infarction in the older child presenting with gastrointestinal symptoms.

Oral contraceptives are one of the most effective and widely used of the reversible contraceptive methods. Thromboembolic disease associated with the use of oral contraceptives has been widely reported. In recent years, attempts to understand the pathogenesis of oral contraceptive-induced thromboembolic disease have found a correlation between larger estrogen doses and increased risk for a thrombotic event. Because the newer triphasic oral contraceptives provide effective contraception with a method of administration that mimics normal hormonal fluctuations during the menstrual cycle, some prescribers may infer that these products are associated with a decreased incidence of adverse effects over alternative oral contraceptives. We present two cases of idiopathic thromboembolism associated with the use of a triphasic oral contraceptive with a discussion of the proposed pathogenic mechanisms for these events.2 cases of thromboembolism in young women with no risk factors except use of triphasic oral contraceptives are reported. A 21-year old White woman, Gravida I Para I, presented to the emergency room with a painful, blue, mottled right lower leg after pain in the hip and buttock for 1 week. She had taken a triphasic oral contraceptive containing 35 mcg ethinyl estradiol and 0.5, 0.75, 1 mg norethindrone for 1 month, and had no other related history. Doppler and venogram tests showed thrombosis of the ileal, femoral, popliteal and infrapopliteal veins. She was treated with heparin, streptokinase, and urokinase without success and recovered after ileal, femoral and popliteal thrombectomy. The 2nd case was a 30-year-old Gravida III Para I Black woman who had taken a pill containing 50 mcg ethinyl estradiol and 500 mcg norgestrel for 13 years and had recently switched to the triphasic pill described above. She had dull midepigastric pain, nausea, vomiting, diarrhea and chills, for 1 week. Physical exam was negative except for abdominal tenderness and a heart murmur. Abdominal ultrasound revealed portal venous thrombosis extending to the splenic and superior mesenteric veins. She was treated with transhepatic urokinase without effect and celiotomy was performed. She was discharged with an occluded right branch of the portal vein. These cases point out the fact that the estrogen dose in triphasic pills is not lower than that in low dose combined oral contraceptives.

Raynaud's phenomenon is a prominent manifestation of systemic sclerosis (scleroderma) affecting the hand. The resulting digital ischaemia may progress to digital tip ulceration or gangrene. Four patients with scleroderma, presenting with severe unremitting unilateral pain in the hand, were evaluated by arteriography and plethysmography. In addition to the usual changes of narrowing and occlusion of the digital arteries themselves, arteriography revealed more proximal occlusion of the radial and ulnar arteries at the wrist and the superficial palmar arch. Plethysmography confirmed virtual absence of pulsatile digital blood flow. Two patients underwent microsurgical reconstruction of the radial and ulnar arterial inflow into the hand and the superficial palmar arch using reversed interposition vein grafts, with immediate subjective resolution of their severe pain and rapid healing of the digital ulcers. Both remain pain free 1 year post-operatively, and pulse-volume recordings have confirmed objectively the restoration of pulsatile blood flow to the fingers.