Penny Stavlo

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.

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.

Outcome of adrenocortical tumors in children

PURPOSE This study reviews adrenocortical tumors in children to determine factors that significantly affect outcome. METHODS An institutional review board-approved retrospective review from 1976 to 2005 identified 23 patients younger than 19 years old with histologic confirmation of adrenocortical carcinoma (ACC) and adenomas. RESULTS The mean age of the 23 children was 9.0 +/- 1.6 years; girls predominated (female-to-male ratio = 1.9:1) as did cancers (ACC 16, adenoma 7); tumor hormone production (74%); and advanced stage for disease (66%). All malignancies were more than 2.5 cm. Adrenalectomy, including en bloc resection of adjacent structures (35%) achieved grossly negative margins in 70% of patients. Three patients received chemotherapy or chemoradiation as primary treatment without surgery. There was no perioperative mortality; morbidity was 10% (pneumothorax, acute renal failure, chylous ascites, and thrombocytosis). Surgical cure without adjuvant therapy was achieved for all adenomas and ACC stages I and II. For ACC stage III and IV, median survival was 21 months, 5-year survival was 0%. All advanced-staged ACC received adjuvant therapy. Surgically negative margins conferred a survival advantage. CONCLUSIONS Children, especially females with ACC present with large advanced-staged tumors. Surgically negative margins with or without en bloc resection improves survival. The high percentage of children with functioning tumors suggests earlier detection is possible.

Minimally Invasive Repair for Pectus Excavatum in Adults

BACKGROUND The purpose of this study is to review the minimally invasive pectus excavatum repair in adults to determine the safety and effectiveness. METHODS An Institutional Review Board approved chart review identified patients 17 years or older who underwent minimally invasive pectus excavatum repair (MIPER) between January 1999 and January 2004. RESULTS Nineteen patients underwent MIPER. Indications for surgery were reduced exercise tolerance (13), dyspnea on exertion (17), improve self-perception (10), and chest pain (6). There were no intraoperative complications or conversions to open repair. Twelve patients (63%) required one strut and seven patients (37%) required two struts. Postoperative complications included self-resolving asymptomatic pneumothorax in six patients and pneumonia in one. Pain at six weeks postoperatively was mild to none in most patients and all had no pain at three months postoperatively except one patient with strut displacement. Two patients required removal of one of two struts due to displacement. The mean postoperative pectus index was significantly lower than preoperative value: 2.5 versus 4.6, p = 0.002. Among six patients with strut removal at two years postoperatively, two patients had mild recurrence of their deformity. CONCLUSIONS Minimally invasive pectus excavatum repair can be performed safely in adults. This approach is technically more challenging in adults with one-third of the patients requiring two struts for optimal repair. The risk of strut displacement is higher than in the pediatric population. The long-term effectiveness and durability of this procedure in adults is still unknown.

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.

Snowmobile Injuries in Children and Adolescents

OBJECTIVE To characterize the risk factors and patterns of injury for children involved in snowmobile incidents. PATIENTS AND METHODS We reviewed the medical records of patients younger than 18 years who required hospital admission for snowmobile-related incidents from 1992 to 2001. Information obtained from these records and from the trauma database included patient demographics, mechanism of injury, injury patterns, medical care, and outcomes. RESULTS Forty-three patients were admitted to our hospital for snowmobile-related incidents. Snowmobile incidents occurred most commonly in male adolescents. The 2 most common mechanisms of injury were ejection and striking a stationary object. Twenty-seven (63%) of the patients drove the snowmobile. Only 23 patients (53%) wore a helmet. At presentation, the mean +/- SEM Injury Severity Score (ISS) was 12.1 +/- 1.4. Orthopedic injuries predominated (n = 42); however, abdominal (n = 12) and head (n = 8) injuries were also common. Four patients were intubated, and 15 required intensive care unit admission. Twenty-nine patients (67%) required surgical intervention. The mean +/- SEM length of hospitalization was 6.7 +/- 1.4 days. No deaths occurred; however, 7 patients (16%) had long-term disabilities. A significant improvement occurred in both Glasgow Coma Scale (GCS) score and ISS for patients using a helmet. In addition, helmet use increased with age (P = .01). Days in the intensive care unit were proportional to both GCS score (r(s) = -0.47; P = .002) and ISS (r(s) = 0.6; P < .001). Length of hospitalization also correlated with both GCS score (r(s) = -0.03; P = .008) and ISS (r(s) = 0.54; P = .02). CONCLUSION Snowmobiles are a significant source of multitrauma for children. Orthopedic injuries predominate, especially in older children, and can lead to long-term disabilities. Helmet use significantly reduces injuries; however, vulnerable younger patients do not frequently wear helmets.