Gastric outlet obstruction possibly secondary to ulceration in a 2-year-old girl: a case report

Gastric outlet obstruction due to ulceration is extremely rare in childhood. We report a case of gastric outlet obstruction possibly secondary to peptic ulceration and our surgical management. Our approach, without vagotomy or antrectomy, would appear to be a safe and effective

Distraction-Induced Intestinal Growth: The Role of Mechanotransduction Mechanisms in a Mouse Model of Short Bowel Syndrome

Novel strategies are needed to address the problem of patients with short bowel syndrome. We previously demonstrated a three-fold lengthening of pig bowel after 2 weeks of applied distractive forces, but we have not elucidated the mechanisms facilitating this growth. We used a mouse model of distraction-induced enterogenesis. High molecular weight polyethylene glycol (PEG) osmotically stretched an isolated small bowel segment (PEG-stretch). Significant increases in villus height and crypt depth and in intestinal epithelial cell length and numbers suggested epithelial remodeling in addition to proliferation during enterogenesis. LC-MS/MS analysis showed a two-fold upregulation of α-actinin-1 and -4. We also demonstrated that p-focal adhesion kinase (FAK), FAK, α-actinin, and Rac1 were significantly upregulated and that F-actin was relocalized in PEG-stretch versus controls. Blockade of the phosphotidyl inositol 3? kinase pathway failed to influence the increase in proliferation or decline in apoptosis after stretch, suggesting alternative signaling pathways are used, including MEK and P38MAPK, which were both upregulated during enterogenesis. Our data suggests that several known mechanotransduction pathways drive distraction-induced enterogenesis.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/140227/1/ten.tea.2013.0383.pd

Treatment of vesico-ureteral reflux in infants and children using endoscopic approaches

Abstract Vesicoureteral reflux (VUR) represents one of the most significant risk factors for acute pyelonephritis in children. Endoscopic treatment of VUR dates back to 1981 when Matouschek first described injection of the ureteral orifice in an attempt to correct VUR. In addition, also Politano and colleagues and McDonald described successful correction of reflux using endoscopic techniques. After these reports subureteral Teflon injection (STING) came to be appreciated as a viable new way to less invasively correct one of the most common pediatric urologic problems. The technique is technically easy to perform and is usually performed as an outpatient procedure. It is performed in general anesthesia in children and may require repeat injections, particularly in patients with high-grade reflux. As for endoscopic technique, a main problem existed. The success in children with high grade reflux was less than reported for open or laparoscopic reimplant techniques. However, in the past 10 years, newer products have become available that are changing the indications for endoscopic correction. In these review, we analyzed the papers published in the literature on this topic to give to the readers an updated overview about the results of endoscopic treatment of VUR after 30-years of his first description

Congenital diaphragamatic hernia associated with aortic coarctation: a case report

Congenital diaphragmatic hernia associated with cardiac anomalies is a major therapeutic challenge. We report a case of Congenital diaphragmatic hernia associated with coarctation of the aorta

Administration of a dipeptidyl peptidase IV inhibitor enhances the intestinal adaptation in a mouse model of short bowel syndrome

BACKGROUND: Glucagon-like peptide-2(GLP-2) induces small intestine mucosal epithelial cell (EC) proliferation; and may have benefit for patients suffering from short bowel syndrome (SBS). However, GLP-2 is rapidly inactivated in vivo by dipeptidyl peptidase IV (DPPIV). Therefore, we hypothesized that selectively inhibiting DPPIV would prolong the circulating life of GLP-2 and lead to increased intestinal adaptation after development of SBS. METHODS: 8-week old C57BL/6J mice underwent a 50% proximal small bowel resection and were treated with either sitagliptin, a DPPIV-inhibitor (DPPIV-I), starting 1 day before surgery versus placebo. DPPIV-I efficacy was assessed 3 days after resection, including intestinal morphology, EC apoptosis and EC proliferation. Adaptive mechanisms were assessed with quantitative real-time PCR, and plasma bioactive GLP-2 was measured by radioimmunoassay. RESULT: Body weight loss and peripheral blood glucose levels did not change compared to SBS controls. DPPIV-I treatment led to significant increases in villus height and crypt depth. DPPIV-I treatment did not significantly change EC apoptosis rates, but significantly increased crypt EC proliferation versus placebo-SBS controls. DPPIV-I treatment markedly increased mRNA expression of β-catenin and c-myc in ileal mucosa. Plasma GLP-2 levels significantly increased(~40.9%) in DPPIV-I-SBS mice. CONCLUSIONS: DPPIV- I treatment increased SBS adaptation, and may potentially be useful for SBS patients

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

Undifferentiated sarcoma developing 14 years after colocystoplasty: Our experience and literature review

A boy with myelomeningocele who had sigmoidocolocystoplasty and ureteric reimplantation when 2-years old and normal annual cystoscopies, developed hematuria and abdominal pain with liver dysfunction 14 years postoperatively. Computed tomography showed a tumor on the left side of the augmented bladder, a large lymph node, and large multiple probable metastases in the liver. Cystoscopy 3 months earlier had been normal, but when repeated showed a tumor originating from the augmented sigmoid colon. Biopsy showed undifferentiated sarcoma. Despite chemotherapy, he died 3 months later. The diagnosis at autopsy was undifferentiated sarcoma originating from the sigmoid colon. We report the first case of undifferentiated sarcoma developing 14 years after sigmoidocolocystoplasty for meningomyelocele, and also review the 55 cases of post-bladder augmentation malignancy in the literature

Thoracoscopic resection of an unusually hypervascular extra-lobar pulmonary sequestration that resembled an arteriovenous malformation in a 2-year-old boy

There is a risk for high-output congestive heart failure to be associated with an extra-lobar pulmonary sequestration (ELS) when there is left-to-left shunting caused by a large systemic arterial supply to a sequestration and venous drainage via the pulmonary veins into the left atrium. We present a 2-year-old boy who underwent thoracoscopic resection of an unusually hypervascular right ELS with a high output left-to-left shunt between the aorta and the left atrium via a pulmonary vein. This case is of particular interest because computed tomographic angiography identified a hypervascular nidus with indistinct borders between arterial and venous vessels suggestive of an arteriovenous malformation