Outcomes of arterial vascular extremity trauma in pediatric patients.

BACKGROUND: Vascular trauma in children, although rare, carries significant risk for repair. Here we report outcomes from a single trauma center for children with extremity vascular trauma, proximal to the digits. METHODS: Retrospective chart review of patients less than age 18years with an acute, non-iatrogenic traumatic arterial vascular injury of the upper and/or lower extremity between January 2008 and December 2013. Abstracted patient demographics, injury characteristics, surgical management, and disposition were summarized and compared with nonparametric methods. RESULTS: 23 children comprised the study cohort: median age of 8years (IQR: 4.6-12), 61% (n=14) males, 100% survival. Penetrating injuries were the predominate mechanism (n=17, 74%). The median time to presentation was 154min (IQR: 65-330). Acute operations for revascularization included a primary repair (n=15, 65%) or reversed vein graft (n=7, 30%). Fasciotomies were done for 3 (13%) patients. Three amputations were done for failed revascularization. Upper extremity vascular injury (n=15, 65%) was more common. The rate of associated extremity fracture was similar between upper (21%) and lower (33%) extremities (p=0.643). Eight (35%) patients required additional surgery most commonly for debridement, washouts and dressing changes. Three patients' hospital stays were complicated by infection. Impaired function was the most common short- and long-term complication (60%, 75%). CONCLUSION: Pediatric vascular injuries are commonly associated with penetrating injuries and male gender and occurred more frequently in the upper extremities. Overall patency rates after repair were 87%. Fasciotomies were done in 13% of patients, and the overall surgical amputation rate was 13%. There was no mortality in this cohort; however, multiple operations are commonly required, including the return to OR for washouts, debridements and dressing changes. The most common short- and long-term complication was impaired function. Overall good results are achievable in pediatric vascular trauma treated with revascularization.

Outcomes of digital artery revascularization in pediatric trauma.

BACKGROUND: Digit amputation is rare in pediatric trauma but can lead to functional morbidity. The true incidence of digital arterial injury is lacking in the literature, and revascularization techniques are not well-described. METHODS: Retrospective review of a pediatric trauma registry identified patients with a digital artery injury between July 2008 and December 2013. Isolated vein injuries and arterial injuries proximal to the digits were excluded. Descriptive statistics were used. RESULTS: Twenty-five subjects met inclusion. Most were male (n=16; 64%) and the median age was 6.8 (IQR: 2.8, 11.1) years. The most common blunt (n=12) trauma was struck/crushed by object (n=4; 33%) and the most common penetrating (n=13) trauma was because of glass (n=9; 69%). All subjects were managed operatively. Initial operations for arterial repair were primary arterial repair (15; 20%), vein graft (7; 28%), thrombectomy (1; 4%), and amputation (1; 4%). Twelve patients (48%) had reported complications at initial follow-up, but only two (8%) had long-term (>24weeks) sequelae. CONCLUSION: Digital artery injury is rare among pediatric traumas. Functional outcomes after digital artery revascularization are favorable. Primary repair can successfully manage these injuries and vein grafting appears to be a suitable alternative when primary repair is not feasible.

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee.

OBJECTIVE: Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations. METHODS: Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included. RESULTS: Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material. CONCLUSIONS: Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.

Asymptomatic malrotation: Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review.

OBJECTIVE: Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. METHODS: Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. RESULTS: There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. CONCLUSIONS: There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patient's caregivers can help guide a watchful waiting management plan in individual cases.

Prospective observational study with an abbreviated protocol in the management of blunt renal injury in children.

BACKGROUND: There are no published management schemes for blunt renal injuries. We are conducting a 2-center prospective observational study with a fixed management scheme. METHODS: Children with CT proven renal injuries were enrolled with permission. Ambulation is allowed when able regardless of grade. Discharge occurs when tolerating a diet and pain is controlled regardless of hematuria. Urinalysis occurs at follow up in 2-4weeks and repeated as indicated. RESULTS: Between 9/2008 and 9/2012, 70 patients were enrolled. Mean age was 11.8years (3-17), and 70% were male. The mean grade of injury was 2.8±1.1 [1-5]. One nephrectomy (1.4%) was performed for a grade 5 injury. Other renal interventions included an embolization for the hilar bleed and one cystotomy for a clot. Mean LOS was 2.9days±2.4days. In patients without other major injury, LOS was 1.9±1.7days (0.4-8days). There were 5 (7%) readmissions: 3 for pain, 1 for hematuria, and 1 for a bladder clot. 58 patients (83%) gave urinalysis samples at initial follow up (med 18days), where 31 (53%) were positive for blood. CONCLUSIONS: Children with blunt renal injury may benefit from management without strict bedrest guidelines. Hematuria appears to have little influence on recovery.

OHVIRA with a twist: obstructed hemivagina ipsilateral renal anomaly with urogenital sinus in 2 patients.

BACKGROUND: OHVIRA syndrome is a rare diagnosis involving the triad of obstructed hemivagina, uterine anomaly, and ipsilateral renal anomaly. OHVIRA syndrome can be associated with other anomalies due to abnormal embryologic development of the urogenital system. CASES: A 14-year-old female with known left renal agenesis, long-standing urinary incontinence, and history of recurrent urinary tract infections presented with abdominal distention. A 4-year-old female with known right renal agenesis and urinary incontinence was found to have a single common channel at the introitus that communicated with the bladder and a hemivagina on the left. SUMMARY AND CONCLUSION: It is important to identify the presence of a urogenital sinus in the OHVIRA setting, as surgical management for these patients may be affected. In both cases, the urogenital sinus was preserved as the vaginal opening.

Laparoscopic adrenalectomy in children: a multicenter experience.

INTRODUCTION: Laparoscopic adrenalectomy is now being recognized as the standard approach for adrenalectomy for benign lesions in adults. The published experience in children and adolescents has been limited to sporadic small case series. Therefore, we conducted a large multicenter review of children who have undergone laparoscopic adrenalectomy. METHODS: After Institutional Review Board's approval, a retrospective review was conducted on all patients who have undergone laparoscopic adrenalectomy at 12 institutions over the past 10 years. Operative times included unilateral adrenalectomy without concomitant procedures. RESULTS: About 140 patients were identified (70 males [50%]). Laterality included 76 (54.3%) left-sided lesions, 59 (42.1%) right, and 5 (3.6%) bilateral. Mean operative time was 130.2 ± 63.5 minutes (range 43-406 minutes). The most common pathology was neuroblastoma in 39 cases (27.9%), of which 23 (59.0%) had undergone preoperative chemotherapy. Other common pathology included 30 pheochromocytomas (21.4%), 22 ganglioneuromas (15.7%), and 20 adenomas (14.3%). There were 13 conversions to an open operation (9.9%). Most conversions were because of tumor adherence to surrounding organs, and tumor size was not different in converted cases (P=.97). A blood transfusion was required in 2 cases. The only postoperative complication was renal infarction after resection of a large neuroblastoma that required skeletonization of the renal vessels. At a median follow-up of 18 months, there was only one local recurrence, which was in a patient with a pheochromocytoma. CONCLUSIONS: The laparoscopic approach can be applied for adrenalectomy in children for a wide variety of conditions regardless of age with a 90% chance of completing the operation without conversion. The risk for significant blood loss or complications is low, and it should be considered the preferred approach for the majority of adrenal lesions in children.

Umbilical venous catheter complication in an infant with left-sided congenital diaphragmatic hernia: extravasation owing to hepatic vein catheterization.

Infants with left-sided congenital diaphragmatic hernia (CDH) are subject to possible intrahepatic placement of the umbilical venous catheter (UVC) during catheterization. A left-sided CDH containing the left lobe of the liver is subject to repositioned anatomy of the umbilical vein, ductus venosus, and hepatic vasculature. This substantially increases the risk of incidentally cannulating the shifted intrahepatic vessels of the liver when placing a UVC. We present a case of hepatic extravasation with line erosion through the liver capsule secondary to UVC placement in an infant with left-sided CDH containing the left lobe of the liver. The patient underwent successful repair of the diaphragmatic defect and is a healthy youngster without complication from CDH or extravasation of the liver capsule. Cannulation of the hepatic vasculature cannot be ruled out with radiograph in infants with left-sided CDH.

Two-stage basilic vein transposition-a new approach for pediatric dialysis access.

PURPOSE: The fistula first initiative has led to increased efforts to create arteriovenous fistulas (AVFs) as primary dialysis access. Two-stage basilic vein transposition (BVT) allows maturation of smaller veins, often a limiting factor in the pediatric population, before elevation and use. We sought to determine whether using 2-stage BVT improves maturation, use, and patency compared with other AVFs, including arteriovenous (AV) grafts. METHODS: Thirty-one patients underwent AV access creation between 1997 and 2008. Data were collected on types of access, maturation, complications, and patency. RESULTS: Forty-two AV access procedures were performed: 15 (36%) 2-stage BVT, 13 (31%) 1-stage BVT, 6 (14%) radiocephalic, 3 (7%) brachiocephalic, 1 (2%) brachiobrachial, and 4 (10%) AV grafts. Follow-up averaged 20.4 +/- 3.2 months for 2-stage BVT and 47.9+/-4.1 months for other AVFs (P < .001). All 2-stage BVT matured compared with 52% (14/27) of other AVFs (P = .001). More 2-stage BVTs (87%) were used for dialysis than other AVFs (48%, P = .024). Fistula failure occurred in 7% 2-stage BVT compared with 59% other AVFs (P = .001). One-year patency rates were 91% for 2-stage BVT vs 47% for other AVFs (P = .003). CONCLUSIONS: Rates of fistula maturation, use, and patency are higher for 2-stage BVT with lower rates of failure. Two-stage BVT shows great promise as the preferred approach to creation of AVF in pediatric patients, especially those with smaller veins.

Islet hypertrophy following pancreatic disruption of Smad4 signaling.

To investigate the role of transforming growth factor (TGF)-beta family signaling in the adult pancreas, a transgenic mouse (E-dnSmad4) was created that expresses a dominant-negative Smad4 protein driven by a fragment of the elastase promoter. Although E-dnSmad4 mice have normal growth, pancreas weight, and pancreatic exocrine and ductal histology, beginning at 4-6 wk of age, E-dnSmad4 mice show an age-dependent increase in the size of islets. In parallel, an expanded population of replicating cells expressing the E-dnSmad4 transgene is found in the stroma between the enlarged islets and pancreatic ducts. Despite the marked enlargement, E-dnSmad4 islets contain normal ratios and spatial organization of endocrine cell subtypes and have normal glucose homeostasis. Replication of cells derived from primary duct cultures of wild-type mice, but not E-dnSmad4 mice, was inhibited by the addition of TGF-beta family proteins, demonstrating a cell-autonomous effect of the transgene. These data show that, in the adult pancreas, TGF-beta family signaling plays a role in islet size by regulating the growth of a pluripotent progenitor cell residing in the periductal stroma of the pancreas.

Bilateral giant abdominoscrotal hydroceles complicated by appendicitis.

Abdominoscrotal hydrocele is a rare entity, with fewer than 100 cases reported in children. Bilateral abdominoscrotal hydroceles are even less common, with 14 cases reported in children. Various complications of abdominoscrotal hydrocele have been reported in the literature. We present a 4-month-old boy with bilateral giant abdominoscrotal hydroceles who developed appendicitis apparently because of obstruction from the right hydrocele. We discuss the various imaging modalities used to establish the diagnosis and plan the operative approach.

Increased MMP-9 expression and activity by aortic smooth muscle cells after nitric oxide synthase inhibition is associated with increased nuclear factor-kappaB and activator protein-1 activity.

OBJECTIVE: To determine the mechanism underlying increased expression and activity of matrix metalloproteinase 9 (MMP-9) by rat aortic smooth muscle cells (RA-SMC) after inhibition of inducible nitric oxide synthase (iNOS). METHODS AND RESULTS: Treatment of interleukin-1beta-stimulated RA-SMC with aminoguanidine led to an increase of 96% in MMP-9 activity (P = 0.003) by gelatin zymography, a 40% increase in pro-MMP-9 protein (P = 0.018) by Western blot, and a 155% increase in MMP-9 mRNA (P = 0.06) by reverse transcription polymerase chain reaction. Aminoguanidine also caused a 26% decrease in cytosolic IkappaB levels (P = 0.014) by Western blot, as well as a 97% increase in nuclear factor-kappaB binding and a 216% increase in activator protein-1 binding as measured by electrophoretic mobility shift assay. No significant changes were noted in MMP-2 or TIMP-1 expression, protein levels, or activity after aminoguanidine administration. CONCLUSIONS: MMP-9 expression and activity is increased in cytokine stimulated RA-SMCs after iNOS inhibition, coincident with activation of the nuclear factor-kappaB and activator protein-1 pathways. We speculate that local derangements in iNOS may favor MMP-9-dependent vessel wall damage in vivo via an inflammatory cascade mechanism.

Carotid embolectomy in the treatment of a paradoxical embolus.

Development of a paradoxical embolus to the carotid circulation through a patent foramen ovale is uncommon but well documented. Previous surgical experience with this entity is limited. Treatment for this condition typically involves anticoagulation or the use of an inferior vena cava filter with concurrent closure of the patent foramen itself. We report successful surgical treatment of a woman with a paradoxical embolus lodged in her left carotid artery. In addition, we provide a brief review of this rare topic and treatment rationale.

Vaginal reconstruction for ambiguous genitalia and congenital absence of the vagina: a 27-year experience.

BACKGROUND/PURPOSE: Gender assignment to a neonate with ambiguous genitalia is crucial. Patients with an absent vagina require the construction of an artificial vagina. In an effort to improve care, the authors have categorized their experience with this group of children. METHODS: Since 1974, we cared for 114 patients with anomalies of the genitalia. There were 53 genotypic girls with congenital adrenal hyperplasia (CAH), 16 genotypic boys with testicular feminization syndrome (TFS), 13 with mixed gonadal dysgenesis (MGD), 9 with Mayer-Rokitansky syndrome, and 4 true hermaphrodites. The remaining 19 had other genital abnormalities. RESULTS: After 1980, patients with CAH underwent clitoral recession and vaginoplasty. All patients with TFS were raised as girls and underwent orchidectomy. Eleven of the MGD patients were given a female sex assignment and underwent gonadectomy. Twenty-eight patients underwent intestinal vaginoplasty including 8 of the TFS patients, 9 with Mayer-Rokitansky syndrome, 8 patients with cloacal anomalies, 2 patients for rhabdomyosarcoma, and 1 of the MGD patients. CONCLUSIONS: (1) This review emphasizes the range of diagnoses the surgeon must be prepared to address in patients with ambiguous genitalia. (2) Colovaginoplasty is an excellent procedure for replacement of a completely absent vagina. (3) Continued evaluation of this group will delineate appropriate timing and choice of procedure.