Christopher R. Moir

Surgical management of Meckel's diverticulum: An epidemiologic, population-based study

ObjectiveThe authors determined whether Meckels diverticulum, discovered incidentally at operation, should be removed. Summary Background DataIt is not clear from the medical literature whether the risk of an incidental Meckels diverticulectomy is greater than the risk of leaving the diverticulum in place. MethodsThe authors used the medical experience of Olmsted County, Minnesota residents for the period 1950 to 1992 to answer the question. ResultsDuring the period, 58 residents developed Meckels complications that required diverticulectomies. The incidence of complications was 87 per 100.000 person-years, and the lifetime risk (to 80 years of age) of developing them was 6.4%. The risks were similar throughout the period and at all ages of life, but were greater among men (124 per 100,000 person-years) than women (50 per 100,000 person-years, p < 0.05). Diverticulectomies for complications carried an operative mortality and morbidity of 2% and 12% and a cumulative risk of long-term postoperative complications of 7%, whereas incidental diverticulectomies done in 87 residents during the period carried corresponding rates of only 1%, 2%, and 2%, respectively. ConclusionsMeckels diverticula discovered incidentally at operation should be removed for most patients, regardless of age.

Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery

BACKGROUND Long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are two of the most common, potentially lethal, cardiac channelopathies. Treatment strategies for the primary and secondary prevention of life-threatening polymorphic ventricular tachycardia/fibrillation include pharmacotherapy with beta-blockers, implantable cardioverter defibrillators, and left cardiac sympathetic denervation (LCSD). OBJECTIVES This study sought to report our institutional experience with LCSD using video-assisted thoracic surgery (VATS). METHODS From November 2005 through November 2008, 20 patients (8 female, average age at surgery 9.1 +/- 9.7 years, range 2 months to 42 years) underwent LCSD via either a traditional approach (N = 2) or VATS (N = 18). A total of 12 patients had genotype-positive LQTS (7 LQT1, 2 LQT2, 1 LQT3, 2 LQT1/LQT2), 2 had JLNS, 4 had genotype-negative LQTS, and 2 had CPVT1. Electronic medical records were reviewed for patient selection, perioperative complications, and short-term outcomes. RESULTS LCSD was performed as a secondary prevention strategy in 11 patients (8 LQTS patients, average QTc 549 ms) and as primary prevention in 9 patients (average QTc 480 ms). There were no perioperative complications, including no intraoperative ectopy, no uncontrolled hemorrhage, and no VATS cases requiring conversion to a traditional approach. The average length of available follow-up was 16.6 +/- 9.5 months (range 4 to 40 months). Among the 18 patients who underwent VATS-LCSD, the average time from operation to dismissal was 2.6 days (range 1 day to 15 days), the majority being next-day dismissals. Among those receiving LCSD as secondary prevention, there has been a marked reduction in cardiac events. CONCLUSIONS We present a series of 20 patients with LQTS and CPVT who underwent LCSD, 18 using VATS. The minimally invasive VATS surgical approach was associated with minimal perioperative complications, including no intraoperative ectopy and excellent immediate and short-term outcomes. Videoscopic denervation surgery, in addition to traditional LCSD, offers a safe and effective treatment option for the personalized medicine required for patients with LQTS/CPVT.

Primary Hyperparathyroidism in Pediatric Patients

Objective. Primary hyperparathyroidism (HPT) is unusual in children. We reviewed our experience with HPT to better characterize these children. Methods. The retrospective review of patients <19 years old who underwent parathyroid resection for primary HPT from 1970 to 2000 was performed at a single institution.. Results. Fifty-two patients were identified. Median age was 16.8 years (range: 4–18.9) with a female-to-male ratio of 3:2. Eighty-five percent had an elevated parathyroid hormone (PTH) level, and 15% had an inappropriately normal PTH level during hypercalcemia. Serum calcium was elevated in all patients except for 2 with multiple endocrine neoplasma (MEN)-IIA and 1 with familial non-MEN HPT, but both had elevated PTH levels. Alkaline phosphatase levels were significantly higher in children with documented bone involvement. At presentation 41 patients (79%) were symptomatic and end-organ damage (nephrocalcinosis, nephrolithiasis, acute pancreatitis, or bone involvement) occurred in 23 patients (44%). Thirty-four patients (65%) had a single adenoma; hyperplasia was identified in 16 patients (27%), and of these cases, 57% occurred in patients diagnosed with MEN-I. Short-term complications included transient hypocalcemia in 29 patients (56%) and transient vocal cord paralysis in 2 patients (4%). Long-term complications were significant for permanent hypocalcemia in 2 patients (4%) and no recurrent laryngeal nerve injuries. No parathyroid abnormalities were identified during exploration in 4 (8%) children. Long-term follow-up was achieved in 98% of patients for a mean and median of 13 years. Resolution of hypercalcemia was achieved in 94% of cases. Conclusion. The diagnosis of primary HPT in pediatric patients is frequently delayed, is commonly symptomatic, and has significant morbidity. For children in whom HPT is suspected, evaluation of serum calcium and PTH levels is diagnostic in 100% of children. Parathyroid resection is effective at restoring normal serum calcium, has few complications, and is the treatment of choice for children with primary hyperparathyroidism.

Pheochromocytoma and Paraganglioma in Children: A Review of Medical and Surgical Management at a Tertiary Care Center

OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children. METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients <18 years of age with histologically confirmed pheochromocytoma or paraganglioma. RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 ± 32 months. CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia The Role of Left Cardiac Sympathetic Denervation

Background— Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. &bgr;-&Bgr;lockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results— We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P<0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year (P<0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P<0.01) than those with a complete LCSD. Conclusions— LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks.Background— Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results— We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% ( P <0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year ( P <0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P <0.01) than those with a complete LCSD. Conclusions— LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks. # CLINICAL PERSPECTIVE {#article-title-34}

Predictive factors of the outcome of traumatic cervical spine fracture in children

The uncommon traumatic cervical spine fractures and dislocations were studied in 227 consecutively treated children (1 to 17 years of age). Lower cervical spine injuries (C-3 to C-7) affected 73% of our patients. However, among the 38 patients younger than 8 years of age, 87% had an injury at the C-3 level or higher (P < .0001). There were 19 fatalities (8.4%), all of which were associated with injuries at the C-4 level or higher. Of the 11 patients with atlanto-axial fracture or dislocation, all died soon after the injury. All had an unstable fracture and cord transection that resulted in cardiorespiratory collapse. Injuries at the vertebral levels C-1, C-2, C-3, and C-4 were associated with fatality rates of 17%, 9%, 4.3%, and 3.7%, respectively. No fatalities were encountered among patients with lesions lower than C-4. The authors conclude that the younger the age, the higher the cervical spine injury, and that the fatality rate correlates directly with the level of cervical spine fracture.

Pediatric inflammatory myofibroblastic tumor: Anaplastic lymphoma kinase (ALK) expression and prognosis

Pediatric inflammatory myofibroblastic tumor (IMT) is rare, with unpredictable clinical behavior. Recently, it has been associated with anaplastic lymphoma kinase (ALK) expression.

Surgical management of intestinal malrotation in adults: comparative results for open and laparoscopic Ladd procedures

BackgroundThis study aimed to characterize the clinical; features of intestinal malrotation in adults, and to compare the results for the open and laparoscopic Ladd procedures.MethodsBetween 1984 and 2003, 21 adult patients with a mean age of 36 years (range, 14–89 years) were surgically treated for intestinal malrotation. The clinical data collected included age, gender, presenting symptoms, diagnostic tests, type of operation, operative time, narcotic requirement, time to oral intake, length of hospital stay, and outcome. The groups (open vs laparoscopic) were comparatively analyzed using two-sample t-tests and Wilcoxon rank sum tests.ResultsThe two groups were similar in terms of age, clinical presentation, and diagnostic tests performed. The most common presenting symptoms were chronic abdominal pain, nausea, and repeated vomiting. Upper gastrointestinal barium studies (UGI/SBFT) were diagnostic for all patients with malrotation as compared with computed tomography (CT) scanning, which was falsely negative in 25% of patients. A total of 21 patients underwent the Ladd procedure, either open (n = 10) or laparoscopic (n = 11). Three laparoscopic procedures were converted to open. Overall, the laparoscopic group resumed oral intake earlier than the open group (1.8 vs 2.7 days; p = 0.092), had a shorter hospital stay (4.0 vs. 6.1 days; p = 0.050), and required less intravenous narcotics on postoperative day 1 (4.9 vs 48.5 mg; p = 0.002). The laparoscopic group underwent a longer operation (194 vs 143 min; p = 0.053). Sixteen of eighteen patients available for follow-up reported complete resolution of symptoms, 2 felt greatly improved. No patient required a second operation related to volvulus or recurrent symptoms.ConclusionsThe laparoscopic Ladd procedure is feasible, safe, and as effective as the standard open Ladd procedure for the treatment of adults who have intestinal malrotation without midgut volvulus. Patients also benefit from this minimally invasive approach, as manifested by an earlier oral intake, a decreased need for intravenous narcotics, and an earlier discharge from the hospital.

Videoscopic left cardiac sympathetic denervation for patients with recurrent ventricular fibrillation/malignant ventricular arrhythmia syndromes besides congenital long-QT syndrome.

Background— Treatment options for patients with recurrent ventricular arrhythmias refractory to pharmacotherapy and ablation are minimal. Although left cardiac sympathetic denervation (LCSD) is well established in long-QT syndrome, its role in non–long-QT syndrome arrhythmogenic channelopathies and cardiomyopathies is less clear. Here, we report our single-center experience in performing LCSD in this setting. Methods and Results— In this institutional review board–approved study, we retrospectively reviewed the electronic medical records of all patients (N=91) who had videoscopic LCSD at our institution from 2005 to 2011. Data were analyzed for the subset (n=27) who were denervated for an underlying diagnosis other than autosomal dominant or sporadic long-QT syndrome. The spectrum of arrhythmogenic disease included catecholaminergic polymorphic ventricular tachycardia (n=13), Jervell and Lange-Nielsen syndrome (n=5), idiopathic ventricular fibrillation (n=4), left ventricular noncompaction (n=2), hypertrophic cardiomyopathy (n=1), ischemic cardiomyopathy (n=1), and arrhythmogenic right ventricular cardiomyopathy (n=1). Five patients had LCSD because of high-risk assessment and &bgr;-blocker intolerance, none of whom had a sentinel breakthrough cardiac event at early follow-up. Among the remaining 22 previously symptomatic patients who had LCSD as secondary prevention, all had an attenuation in cardiac events, with 18 having no breakthrough cardiac events so far and 4 having experienced ≥1 post-LCSD breakthrough cardiac event. Conclusions— LCSD may represent a substrate-independent antifibrillatory treatment option for patients with life-threatening ventricular arrhythmia syndromes other than long-QT syndrome. The early follow-up seems promising, with a marked reduction in the frequency of cardiac events postdenervation.

Pediatric ileal pouch-anal anastomosis: functional outcomes and quality of life.

BACKGROUND/PURPOSE The aim of this study was to assess and correlate functional outcomes and surgical results with health-related quality of life after ileal pouch-anal anastomosis (IPAA) in pediatric patients. METHODS Functional outcome was determined by questionnaire and telephone interview. Surgical results were determined by retrospective chart review. RESULTS Data were gathered from 26 patients (mean age at IPAA, 12 years; mean follow-up, 3.7 years). Diagnoses were ulcerative colitis in 18, indeterminate colitis in 4, and familial polyposis in 4. Indications for IPAA included intractability, medication toxicity, growth delay, and cancer prophylaxis. Short-term complications (5 patients; 19%) included partial small bowel obstruction, stomal revision, pouch abscess, and negative exploration. Long-term complications (8 patients; 31%) were chronic pouchitis and anal stricture. The average number of stools per 24 hours was 3.9. No incontinence was reported; dietary restrictions were negligible. Although there were minimal differences from population norms, parental anxiety remained high. Chronic pouchitis correlated negatively with physical summary score. Nocturnal stooling negatively affected psychosocial quality of life. CONCLUSIONS Pediatric IPAA resulted in excellent bowel health. Quality of life, physical function, mental health, and self-esteem were equivalent to those of healthy children. These data may help families and physicians make informed surgical decisions.