Abdalla E. Zarroug

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.

Impact of Childhood Inguinal Hernia Repair in Adulthood: 50 Years of Follow-Up

BACKGROUND Short-term follow-up of pediatric inguinal herniorrhaphies has yielded low morbidity and recurrence rates. Nonetheless, the impact of childhood inguinal herniorrhaphy on the adulthood risk for repeat groin operation, chronic groin pain, and infertility has not been established. STUDY DESIGN A survey was mailed to all patients who underwent a primary inguinal hernia repair as a child at our institution from 1956 to 1960. The survey inquired about repeat groin operation, chronic groin pain, and fertility status. Demographic and operative information from respondents was obtained from medical record review. RESULTS Of 332 eligible patients, 213 (66%) responded to the survey, accounting for 252 inguinal herniorrhaphies (174 unilateral, 33 bilateral, and 6 sequential contralateral). All hernias were indirect, more common on the right (right, 49%; left, 25%; bilateral, 26%), with males predominating (91%). History of premature birth was present in 5 (2%) patients. High ligation of the hernia sac was performed in 90% of the repairs. Hydrocelectomy was performed in 25 (13%) and orchidopexy in 37 (19%) patients. With a median follow-up of 49 years (range 47.2 to 52.1 years), a total of 21 (8.4%) repeat and 13 (6%) contralateral groin operations were observed. Chronic groin pain and a medical diagnosis of infertility were reported by 7 (3%) and 10 (5%) patients, respectively. CONCLUSIONS Childhood inguinal hernia repairs do not appear to decrease or increase the risk of inguinal hernia development in adulthood. Parents and children undergoing a childhood inguinal hernia repair should be informed that although the risk for a true indirect hernia recurrence is low, the risk of repeat groin operation (8.4%) and chronic groin pain (3%) remain present in adulthood.

Fifty-three–year experience with pediatric umbilical hernia repairs

PURPOSE The aim of this study was to evaluate the long-term surgical and patient-reported outcomes of pediatric umbilical hernia (UH) repairs. METHODS A retrospective review of all children (<18 years old) who underwent UH repair at Mayo Clinic-Rochester in the last half century was done. Follow-up was obtained by mailed survey. RESULTS From 1956 to 2009, 489 children (boys, 251; girls, 238) underwent a primary UH repair. The mean age was 3.9 years (range, 0.01-17.8 years). Complicated UHs that required emergent repair (n = 34, or 7%) included recurrent incarceration (22), enteric fistula (7), strangulation (4), and evisceration (1). Mean UH size was 1.3 cm (range, 0.2-7.0 cm), varying by operative indication (1.0 cm emergent vs 1.5 cm elective repairs, P = .008) and decade of repair (2.2 cm, 1950s-60s vs 1.3 cm, 1990s-2000s; P = .001). Postoperative morbidity (2%) consisted of superficial wound infection (7), hematoma (3), and seroma (1). With a 66% survey response rate and mean follow-up of 13.0 years (range, 0-53.8 years), 8 (2%) patients experienced a recurrence. Most patients reported satisfaction (90%) with the cosmetic appearance of their umbilicus and are pain free (96%). CONCLUSION Pediatric UH repairs have low morbidity and recurrence rates. Most patients are satisfied and pain free. Importantly, complicated UHs were more likely to be associated with smaller defects; therefore, parental counseling for signs of incarceration is recommended even in small defects.

Pediatric chronic ulcerative colitis: does infliximab increase post–ileal pouch anal anastomosis complications?

BACKGROUND Total proctocolectomy with ileal pouch anal anastomosis (IPAA) is a common surgical approach to chronic ulcerative colitis (CUC). Preoperative use of Infliximab (IFX) has raised concern of increased postoperative complications. We sought to compare outcomes of pediatric patients (≤ 18 years) who were treated with IFX before IPAA to those who did not. METHODS Patients (≤ 18 years of age) who underwent IPAA from 2003 to 2008 for CUC were included, and their records were retrospectively reviewed for preoperative medications, operative technique, and 1-year postoperative complications (leak, wound infection, small bowel obstruction, pouchitis). Subjects were divided into 2 groups--those who received IFX preoperatively and those who did not. RESULTS Eleven patients received IFX preoperatively, and 27 children did not. All complications following IPAA were more frequent in the IFX group compared to controls (55% vs 26%). Small bowel obstruction was significantly higher in the IFX group (55% vs 7%). Long-term complications occurred in 64% of the IFX group and 61% of the controls. CONCLUSION Children that were treated with IFX prior to IPAA suffered twice as many postoperative complications. Long-term outcomes are similar. Currently, we recommend colectomy with end ileostomy for patients that receive IFX within 8 weeks of colectomy for CUC.

Nitrergic mechanisms mediating inhibitory control of longitudinal smooth muscle contraction in mouse small intestine

Studies using genetic manipulation to investigate mechanisms of control of physiologic function often necessitate mouse models. However, baseline functional analysis of murine small intestinal motility has not been well defined. Our aim was to define nitrergic mechanisms regulating mouse small intestinal longitudinal muscle. Endogenous nitric oxide (NO) is an important neuroregulatory substance mediating inhibition of contractile activity in murine small bowel. Full-thickness muscle strips of jejunum and ileum from C57BL/6 mice (n ≥6 mice) cut in the direction of longitudinal muscle were studied. Numerous conditions of electrical field stimulation (EFS) and effects of exogenous NO and NO donors were studied in the absence or presence of inhibitors of nitric oxide synthase (NOS) and 1H-[1,2,4]-oxadiazaolo-[4,3-a]-quinoxalin-1-one (ODQ), a downstream inhibitor of guanylyl cyclase. EFS induced a frequencydependent inhibition of contractile activity in both jejunum and ileum (P < 0.05). As the voltage of EFS was increased, inhibition turned to excitation in the jejunum; in contrast, the ileum demonstrated a voltage-dependent increasing inhibition (P < 0.05 each). EFS-induced inhibition was blocked by NOS inhibitors and ODQ. NO donors inhibited spontaneous contractile activity abolished by ODQ. NO appears to be an endogenous inhibitory neurotransmitter in murine longitudinal small bowel muscle. Nitrergic mechanisms mediate inhibitory control of murine longitudinal small intestinal muscle. Differences exist in neuroregulatory control between jejunum and ileum that may be related to their known difference in motor patterns.

Benchmarks for splenectomy in pediatric trauma: How are we doing?☆☆☆★★★

BACKGROUND/PURPOSE Following publication of American Pediatric Surgical Association (APSA) hospital benchmarks for the operative management of blunt splenic trauma in specialized centers, it was found that most hospitals exceeded these benchmarks. We sought to determine if benchmarks were being met a decade later and to identify factors associated with splenectomy in injured children. METHODS Rates of splenic procedures were calculated for children≤19 with a blunt splenic injury (ICD-9 865) using the 2010-2011 National Trauma Data Bank. Multivariable analysis was performed to determine independent predictors of splenectomy. RESULTS Of 8597 children, 24.3% received care at pediatric trauma centers (PTC), 34.6% at adult trauma centers (ATC), and the remaining 41.2% at other centers (OTC). The overall operative rate was 9.2% (3.9% if age≤14, 6.7% if ≤17). Operative rates were higher in children treated at ATC and OTC when compared to PTC. On multivariable analysis, age>14, coexisting injuries, severity of splenic injury, and care at ATC or OTC were predictive of undergoing operative treatment. CONCLUSIONS Operative rates for splenic injuries meet APSA benchmarks at PTC yet remain high at other centers. Care at an ATC or OTC is associated with greater odds of operative management after adjustment for age and injury severity.

Accidental Burials in Sand: A Potentially Fatal Summertime Hazard

Accidental burial in sand is a tragically unrecognized risk associated with a popular childhood recreational activity. We describe 4 boys, aged 10 to 13 years, who were accidentally buried by sand. One boy died after his self-made tunnel in a sandbox collapsed. In a separate incident at a construction site, 1 boy died, and 2 were injured after a 30-foot sandpile collapsed as they ran down the embankment; all 3 were buried by the sand. In both incidents, play was unsupervised, and burial was sudden and complete. The calculated weight of the sand exceeded the expected maximal muscle effort of the chest, leading to traumatic asphyxiation secondary to restrictive compression of the chest. Only 15 accidental burials have been reported in the literature. To our knowledge, this is the first report describing children who died of respiratory asphyxia due to overwhelming thoracic compression after sand burial. Greater awareness by public health and safety officials at beaches, sandboxes, sandpiles, and natural play areas may prevent potentially lethal accidents.

Long-term outcomes of ileal pouch-anal anastomosis for pediatric chronic ulcerative colitis.

BACKGROUND Ileal pouch-anal anastomosis (IPAA) is the surgical treatment of choice for patients with chronic ulcerative colitis (CUC). In the pediatric population, short-term outcomes of IPAA are excellent but long-term data limited. The purpose of this study is to report long-term functional and quality of life outcomes of IPAA in pediatric patients. METHODS Functional outcomes and quality of life (QoL) following IPAA in patients ≤ 18 years of age were prospectively assessed by survey over a 30 year period. Preoperative information, chronic pouchitis and pouch loss were retrospectively reviewed. RESULTS Over 30 years, 202 children with CUC underwent IPAA. Questionnaires were returned by 87% and median (range) survey follow-up was 181.5 (7.8-378.5) months. Postoperative day and night-time stool frequency did not increase over time though incontinence increased slightly. Quality of life (QoL) was generally excellent and stable over time. Crohns disease (CD) was diagnosed in 33 (16%) patients during the follow-up period. Chronic pouchitis occurred in 22 patients and pouch failure in 13 patients. Kaplan Meier estimates of pouch survival at 20 years were 61% for patients with CD and 92% for CUC. CONCLUSIONS Ileal pouch-anal anastomosis has long-term durability as a cure for pediatric chronic ulcerative colitis, with most patients reporting stable bowel function and QoL. Chronic pouchitis and pouch failure affect a minority of patients and require further study.

A simple algorithm reduces computed tomography use in the diagnosis of appendicitis in children

BACKGROUND A diagnostic algorithm for appendicitis in children was created to reduce computed tomography (CT) use owing to the risk of cancer from radiation exposure and cost of CT. This study evaluates the impact of the algorithm on CT use and diagnostic accuracy of appendicitis. METHODS Patients ≤18 years who underwent appendectomy for suspected appendicitis after presenting to the emergency department for 2 years before and 3 years after algorithm implementation were identified. Clinical characteristics and outcomes, including use of CT and negative appendectomy rate, were compared between the pre- and post-implementation periods. Multivariable analysis was used to determine the impact of CT on negative appendectomy. RESULTS We identified 331 patients-41% in the pre- and 59% in the post-implementation period. CT utilization decreased from 39% to 18% (P < .001) after implementation. The negative appendectomy rate increased from 9% to 11% (P = .59). Use of CT did not impact the risk of negative appendectomy (P = .64). CONCLUSION Utilization of CT was significantly reduced after implementation of a diagnostic algorithm for appendicitis without impacting diagnostic accuracy. Given the concern for increased risk of cancer after CT, these results support use of an algorithm in children with suspected appendicitis.