Mesenteric desmoid tumor: De novo occurrence or recurrence following appendectomy?

Desmoid type fibromatosis (DF) is a rare, locally aggressive but benign proliferation of fibrous tissue which produces a fibroblastic mass that can cause a wide range of symptoms secondary to mass effect. When resected, these masses most commonly recur in the first 2 years. We present a case of a 33-year-old male with a history of an appendectomy 2 years prior, though his pathology report did not identify inflammation in the appendix, who presented with gradual onset of abdominal pain, and radiographs that demonstrated a large mass in the right lower abdomen. Given his symptoms the mass was resected and pathologic evaluation revealed a desmoid tumor. This case presents a unique possibility of a recurrent desmoid tumor in which the patient's surgical history and radiographic findings can contribute to the overall management strategy of the patient given the evolving options for treatment of desmoid fibromatosis.

Neurodevelopmental and Cognitive Outcomes in Children With Intestinal Failure.

OBJECTIVES: Recent advances in medical and surgical management have led to improved long-term survival in children with intestinal failure. Yet, limited data exist on their neurodevelopmental and cognitive outcomes. The aim of the present study was to measure neurodevelopmental outcomes in children with intestinal failure. METHODS: Children enrolled in a regional intestinal failure program underwent prospective neurodevelopmental and psychometric evaluation using a validated scoring tool. Cognitive impairment was defined as a mental developmental index <70. Neurodevelopmental impairment was defined as cerebral palsy, visual or hearing impairment, or cognitive impairment. Univariate analyses were performed using the Wilcoxon rank-sum test. Data are presented as median (range). RESULTS: Fifteen children with a remnant bowel length of 18 (5-85) cm were studied at age 17 (12-67) months. Thirteen patients remained dependent on parenteral nutrition. Twelve (80%) subjects scored within the normal range on cognitive testing. Each child with cognitive impairment was noted to have additional risk factors independent of intestinal failure including cardiac arrest and extreme prematurity. On univariate analysis, cognitive impairment was associated with longer inpatient hospital stays, increased number of surgical procedures, and prematurity (P < 0.02). In total, 4 (27%) children demonstrated findings consistent with neurodevelopmental impairment. CONCLUSIONS: A majority of children with intestinal failure demonstrated normal neurodevelopmental and cognitive outcomes on psychometric testing. These data suggest that children with intestinal failure without significant comorbidity may be at low risk for long-term neurodevelopmental impairment.

The impact of perianal disease in young patients with inflammatory bowel disease.

BACKGROUND: Perianal disease is a potentially significant source of morbidity for patients with inflammatory bowel disease (IBD). We sought to identify the impact of perianal disease on IBD outcomes in children, adolescents, and young adults. METHODS: We studied 12,465 inpatient admissions for patients ≤20 years old with IBD in 2009 using the Kids' Inpatient Database (KID). Patients were stratified by their principal diagnosis of ulcerative colitis (UC) or Crohn's disease (CD). Perianal disease (perianal abscess, anal fissure, or anal fistula), complex fistulas (rectourethral, rectovaginal, or enterovesical), and growth failure were defined by ICD-9 codes. Logistic regression was performed adjusting for CD or UC, gender, age, need for surgical intervention, fistulas, or growth failure. RESULTS: Of the 511 (4.1%) patients with perianal disease, 480 had CD (94%, p < 0.001). Girls were less likely to suffer perianal disease (OR = 0.63, CI 0.52-0.76, p < 0.001). Those with perianal disease were more likely to suffer complex fistulas (OR = 3.5, CI 1.98-6.20, p < 0.001) but less likely to suffer enteroenteral fistulas (OR = 0.30, CI 0.15-0.63, p = 0.001) than those without perianal disease. Perianal disease did not increase the incidence of growth failure (p = 0.997) but doubled the likelihood of an operation of any type during admission (p < 0.001). Additionally, patients with perianal disease spent on average 1.29 more days in the hospital (7.45 vs. 6.16 days, p < 0.001) and accrued $5838 extra in hospital charges (p = 0.005). CONCLUSIONS: Perianal disease in younger patients is associated with a longer length of stay, higher hospital charges, and increased rates of both perineal and abdominal operative procedures. These data support the notion that, similar to adults, the presence of perianal disease in pediatric Crohn's patients is associated with a more severe course.

Association of anorectal malformation and intestinal malrotation.

BACKGROUND: Patients born with anorectal malformations (ARM) frequently have other congenital anomalies that are well-defined; however, limited data exist examining the relationship of ARM with malrotation. METHODS: A 10-year retrospective review was performed to examine all patients treated at a regional children's medical center with a diagnosis of ARM. Data were collected to identify malrotation, vertebral, anorectal, cardiac, tracheo-esophageal fistula, renal, radial, limb (VACTERL) anomalies, the type of ARM, operative procedures performed, and long-term bowel management. RESULTS: One hundred forty-six patients were identified. Upper gastrointestinal evaluation was performed in 21 patients (14.4%), while contrast enemas were performed in 22 patients (15.1%). Seven patients were found to have malrotation (4.8%) and 6/7 of these patients had 2 or more VACTERL anomalies. CONCLUSIONS: Patients with ARM and 2 or more VACTERL anomalies should undergo screening for malrotation. Patients with intestinal malrotation, ARM, and poor potential for bowel control should have their appendix preserved during a Ladd's procedure.

Contemporary trends in the use of primary repair for gastroschisis in surgical infants.

BACKGROUND: Gastroschisis is a newborn anomaly requiring emergent surgical intervention. We review our experience with gastroschisis to examine trends in contemporary surgical management. METHODS: Infants who underwent initial surgical management of gastroschisis from 1996 to 2014 at a pediatric hospital were reviewed. Closure techniques included primary fascial repair using suture or sutureless umbilical closure, and staged repair using sutured or spring-loaded silo (SLS). Data were separated into 3 clinical eras: pre-SLS (1996 to 2004), SLS (2005 to 2008), and umbilical closure (2009 to 2014). RESULTS: In the pre-SLS era, 60% (34/57) of infants with gastroschisis underwent primary repair. With the advent of SLS, there was a decrease in primary repair (15%, 10/68, P < .0001). Following introduction of sutureless umbilical closure, 61% (47/77) of infants have undergone primary repair. On multivariate regression, primary repair was associated with shorter intensive care unit stays (P < .001) and time to initiate enteral nutrition (P < .01). CONCLUSIONS: Following introduction of a less invasive technique for gastroschisis repair, most infants with gastroschisis were able to be repaired primarily. Primary repair should be considered in all babies with gastroschisis and favorable anatomy.

The utility of pathologic evaluation of adult hernia specimens.

BACKGROUND: Pathological examination of hernia sac specimens adds additional steps and cost to a surgical procedure but has no proven benefit. Although well studied in pediatrics, there are limited data in the adult literature pertaining to this practice. METHODS: This is a retrospective analysis from a single institution referral center over a 4-year period (2007 to 2011). All inguinal, incisional, ventral, and umbilical hernia repairs greater than 18 years of age were included. RESULTS: A total of 1,216 inguinal (55.4%), incisional (11.4%), umbilical (21.5%), or ventral hernia (11.7%) repairs were included. In 246 cases (20.2%), hernia sac specimens were sent to pathology (open 96.7%; laparoscopic 3.3%). There were no cases in which management of the patient changed because of the final results. CONCLUSION: The rarity of changes in diagnosis and treatment from routine pathologic examination of a hernia sac does not justify this practice and indicates that it may be omitted except in unique circumstances.

Pilonidal disease in a military population: how far have we really come?

BACKGROUND: Pilonidal disease (PD) has a long connection with military personnel, even nicknamed "jeep disease" during World War II. The aim of this study was to identify factors associated with recurrence and complications after surgery in a military population. METHODS: A retrospective cohort analysis of operative therapy for PD at a single institution from 2005 to 2011 was conducted. Patient demographics, disease characteristics, and surgical methods were assessed for the primary outcomes of recurrence and morbidity. RESULTS: A total of 151 patients with PD were identified, who underwent excision (45.7%), excision with primary closure (29.8%), and incision and drainage (9.9%). Overall recurrence and morbidity rates were 27.2% and 34.4%, respectively. Black race, chronic disease, wound infection, and infection and drainage were associated with recurrence (P < .05), and excision with primary closure was associated with increased complications (P < .001). CONCLUSIONS: PD remains a significant source of morbidity and recurrence among military personnel. Certain patient-related and disease-related factors portend a worse prognosis, with black race and operative method the strongest predictors of outcomes.

Pentalysine clusters mediate silica targeting of silaffins in Thalassiosira pseudonana.

The biological formation of inorganic materials (biomineralization) often occurs in specialized intracellular vesicles. Prominent examples are diatoms, a group of single-celled eukaryotic microalgae that produce their SiO2 (silica)-based cell walls within intracellular silica deposition vesicles (SDVs). SDVs contain protein-based organic matrices that control silica formation, resulting in species specifically nanopatterned biosilica, an organic-inorganic composite material. So far no information is available regarding the molecular mechanisms of SDV biogenesis. Here we have investigated by fluorescence microscopy and subcellular membrane fractionation the intracellular transport of silaffin Sil3. Silaffins are a group of phosphoproteins constituting the main components of the organic matrix of diatom biosilica. We demonstrate that the N-terminal signal peptide of Sil3 mediates import into a specific subregion of the endoplasmic reticulum. Additional segments from the mature part of Sil3 are required to reach post-endoplasmic reticulum compartments. Further transport of Sil3 and incorporation into the biosilica (silica targeting) require protein segments that contain a high density of modified lysine residues and phosphoserines. Silica targeting of Sil3 is not dependent on a particular peptide sequence, yet a lysine-rich 12-14-amino acid peptide motif (pentalysine cluster), which is conserved in all silaffins, strongly promotes silica targeting. The results of the present work provide the first insight into the molecular mechanisms for biogenesis of mineral-forming vesicles from an eukaryotic organism.