Refractory Chronic Constipation in an Adolescent Female Later Diagnosed With Internal Anal Sphincter Achalasia.

Internal anal sphincter achalasia (IASA) is a rare anorectal disorder that presents as chronic refractory constipation in pediatrics. With a poor response to conventional constipation-based therapy, it is often misdiagnosed as other conditions, such as ultra-short-segment Hirschsprung disease. This case report describes a rare case of IASA in an adolescent female, emphasizing the importance of ruling out other differentials, including Hirschsprung disease, via rectal biopsy and thus allowing for earlier targeted therapy to improve lifestyle conditions. A 20-year-old female with a history of IASA presents for semiannual botulism toxin injections. Despite initial relief, her constipation symptoms gradually returned after four to five months. She has had a history of ineffective conventional constipation treatments since childhood, which prompted a further workup. Biopsy results during her teenage years confirmed the presence of ganglionic cells, differentiating IASA from Hirschsprung disease. The management plan involved biannual perianal Botox injections, offering relief for approximately six months. IASA's physiological basis involves altered innervation, the absence of nitrergic nerves, and defective neuromuscular junctions in the internal anal sphincter. Diagnosis requires anorectal manometry and a rectal suction biopsy. Treatment options include botulism, toxin injections, and posterior internal anal sphincter myectomy. Botulism injections offer temporary relief, while myectomy provides long-term improvement.

Hemodynamic variables predict outcome of emergency thoracotomy in the pediatric trauma population.

BACKGROUND: Limited data exist regarding indications for resuscitative emergency thoracotomy (ETR) in the pediatric population. We attempt to define the presenting hemodynamic parameters that predict survival for pediatric patients undergoing ETR. METHODS: We reviewed all pediatric patients (age <18years), entered into the National Trauma Data Bank from 2007 to 2010, who underwent ETR within one hour of ED arrival. Mechanism of injury and hemodynamics were analyzed using Chi squared and Wilcoxon tests. RESULTS: 316 children (70 blunt, 240 penetrating) underwent ETR, 31% (98/316) survived to discharge. Less than 5% of patients survived when presenting SBP was ≤50mmHg or heart rate was ≤70bpm. For blunt injuries there were no survivors with a pulse ≤80bpm or SBP ≤60mmHg. When survivors were compared to nonsurvivors, blood pressure, pulse, and injury type were statistically significant when treated as independent variables and in a logistic regression model. CONCLUSIONS: When ETR was performed for SBP ≤50mmHg or for heart rate ≤70bpm less than 5% of patients survived. There were no survivors of blunt trauma when SBP was ≤60mmHg or pulse was ≤80bpm. This review suggests that ETR may have limited benefit in these patients.

Persistent gastrocutaneous fistula: factors affecting the need for closure.

PURPOSE: The occurrence of gastrocutaneous fistula (GCF) is a well-known complication after gastrostomy tube placement. We explore multiple factors to ascertain their impact on the rate of persistent GCF formation. METHODS: We retrospectively reviewed patient records for all gastrostomies (GT) constructed at our institution from 2007 to 2011. Association of GCF with method of placement, concomitant fundoplication, neurologic findings, duration of therapy, and demographics was evaluated using logistic regression. RESULTS: Nine hundred fifty patients had GTs placed, of which 148 patients had GTs removed and 47 (32%) of 148 required surgical closure secondary to persistent GCF. Laparoscopic and open procedures comprised 79 (53%) of 148 and 69 (47%) of 148, respectively. Seventeen (22%) patients in the laparoscopic group developed persistent GCF, compared to 30 (43%) in the open group (P=0.035, OR=2.52). Seventy-one patients had concomitant Nissen fundoplication. Thirty-one (44%) developed GCF, compared to 16 (21%) without a Nissen (P=0.002, OR=4.94). Patients with button in place for 303 days had persistent GCF incidence of 23%, compared to 45% at 540 days (P<0.001, OR=3.51) and 50% at 850 days (P=0.011, OR=4.51). Patients with device placed at 1.8 months of age were more likely to develop GCF compared to those with device placed at 8.9 months of age (P=0.017, OR=2.35). CONCLUSION: Open operations, concurrent Nissen and younger age at placement were all statistically significant factors causing persistent GCF.

An animal model of necrotizing enterocolitis (NEC) in preterm rabbits.

Creation of an animal model of necrotizing enterocolitis (NEC) allowing adjustment of severity and potential recoverability is needed to study effectiveness of prevention and treatment strategies. This study describes a novel model in preterm rabbits capable of adjusting severity of NEC-like histologic changes. Rabbit pups (n = 151) were delivered by cesarean section 2 days preterm. In the treatment groups, tissue adhesive was applied to anal openings to simulate the poor intestinal function and dysmotility of preterm neonates. Pups were placed into five groups: 3INT (3 day intermittent block), 4INT (4 day intermittent block), 3COM (3 day complete block), 4COM (4 day complete block), based on differences in type of anal blockage and day of life sacrificed. The fifth group, 4CON, was comprised of a control arm (n = 28) without anal block, with sacrifice of subjects on day 4. All pups were gavage fed with formula contaminated with Enterobacter cloacae, ranitidine, and indomethacin. Following sacrifice, the intestines were harvested for pathologic evidence of NEC. A blinded pathologist graded histologic changes consistent with NEC using a grading scale 0-4 with 4 being most severe. Fifty-seven pups (57/123) (46%) in the research arm survived to sacrifice, compared to 26/28 (93%) in the control arm of the investigation, p < 0.0001. The incidence and severity of NEC-like damage increased with the duration and completeness of the anal blockage. 44/57 (77%) of survivors revealed various degrees of NEC-like damage to large and small bowel, and 3/26 (12%) exhibited early NEC-like mucosal injury in the research and control arms, respectively. This animal model produces NEC-like pathologic changes in both small and large intestine in preterm rabbits. Because incidence and severity of damage increases with duration and completeness of intestinal dysmotility, this allows future effectiveness studies for nonsurgical treatment and prevention of NEC.

Restraint status improves the predictive value of motor vehicle crash criteria for pediatric trauma team activation.

BACKGROUND: Most trauma centers incorporate mechanistic criteria (MC) into their algorithm for trauma team activation (TTA). We hypothesized that characteristics of the crash are less reliable than restraint status in predicting significant injury and the need for TTA. METHODS: We identified 271 patients (age, <15 y) admitted with a diagnosis of motor vehicle crash. Mechanistic criteria and restraint status of each patient were recorded. Both MC and MC plus restraint status were evaluated as separate measures for appropriately predicting TTA based on treatment outcomes and injury scores. RESULTS: Improper restraint alone predicted a need for TTA with an odds ratios of 2.69 (P = .002). MC plus improper restraint predicted the need for TTA with an odds ratio of 2.52 (P = .002). In contrast, the odds ratio when using MC alone was 1.65 (P = .16). When the 5 MC were evaluated individually as predictive of TTA, ejection, death of occupant, and intrusion more than 18 inches were statistically significant. CONCLUSIONS: Improper restraint is an independent predictor of necessitating TTA in this single-institution study.

The effect of ACGME duty hour restrictions on operative continuity of care.

Resident work restrictions limit participation in operations that address problems created by a prior operation, because complications occur at any time. We compared resident and attending surgeon staffing of operative complications. We reviewed all complications that required a second operation reported at our Morbidity and Mortality Conference over 1 year, noting surgeons present, their postgraduate year level, and call shift. Comparisons were done using chi2. Of 142 cases, 39 involved a second operation. The same attending surgeon was present for both in 79 per cent of cases, whereas the same resident was present in only 44 per cent (P = 0.002). Postgraduate year 4 to 5 were less likely to be present for second operations than attendings (48% vs 87%, P = 0.011). Resident shift (day, night float, and weekend) was known in 32 cases. When the first operation occurred during day hours, attendings and residents were equally likely to be present at the second (55% and 45%, P = 0.16). When original operations took place during night float or weekend shifts, residents were less likely to be present (33%) than attendings (83%) at second operations (P = 0.036). Duty hour restrictions interfere with operative continuity of care. Reoperations should be exempted from duty hour restrictions.

Necrosis caused by intra-arterial injection of promethazine: case report.

Promethazine injections have led to necrosis and gangrene of the distal upper extremity when inadvertently injected into an artery. There have been few case reports of this alarming complication in the literature. We report on 2 cases of intra-arterial promethazine injection that led to amputation.

Industry support of graduate medical education in surgery.

The role of pharmaceutical and medical device companies ("industry") in graduate medical education (GME) is under debate. We surveyed program directors in general surgery and surgical specialties to determine industry activities in surgical GME. We used an internet-based questionnaire regarding industry marketing and educational activities in surgical programs, and their effects on surgical education. We received 65 responses to 377 requests (17%). Nearly two-thirds reported industry-sponsored meals. Industry-supported travel was infrequent ("never" and "seldom" in 56% of device workshops, 69% of lectures, and 74% of conferences). More than one-half reported support for academic events: paid lecturers and exhibition fees (both 58%), and unrestricted grants (62%). More than one-half (54%) reported industry-sponsored research. One-fourth believed their programs to be dependent on industry for their educational missions. Most disagreed that industry support posed a problem, either in general (55%) or for their program (71%). One-fourth of respondents (25%) advocated profession-wide restrictions of industry involvement with GME. Equal numbers agreed (39%) and disagreed (35%) with the view that pharmaceutical and medical device industries have motivations that are in conflict with those of doctors and their patients. Industry activities are widespread in surgical residencies, with approval of many program directors.

Cutaneous Rosai-Dorfman disease of the forearm: case report.

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare non-neoplastic pathologic condition that frequently pursues a prolonged clinical course marked by exacerbations and remissions. Cutaneous RDD is even less common than cases involving lymph nodes. We present the case of a patient with long-standing Crohn's disease who developed cutaneous RDD in the forearm.