[Show abstract][Hide abstract] ABSTRACT: Little information exists regarding the optimal surgical treatment of pediatric primary hyperparathyroidism. We hypothesized that primary hyperparathyroidism in children, in the absence of a family history, is caused by single-gland disease and is amenable to minimally invasive parathyroidectomy (MIP).
We reviewed the records of individuals younger than 25 years who underwent parathyroidectomy in a prospectively collected database at a single tertiary hospital from 2003 to 2009.
Twenty-five patients were identified, with a mean (SD) age of 19 (3.7) years. Sixty percent had single-gland disease (n = 15). Familial disease was present in 6 patients. All of the children younger than 18 years without a family history of disease (9/9) were found to have a single-gland disease (P < .001). Seventy-eight percent of patients without a family history were successfully treated without a bilateral exploration. Average length of stay was less than 1 day with no complications or recurrences.
Primary hyperparathyroidism in patients younger than 18 years without a family history was uniformly caused by single-gland disease. Minimally invasive parathyroidectomy was successful in these patients and avoided the morbidity of bilateral exploration. We recommend MIP be used in pediatric patients at large referral centers with prior successful institutional experience with the technique.
Full-text · Article · Jun 2010 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: In adults, mesenteric venous thrombosis with extension into the portal system is a known complication of total proctocolectomy with pouch ileoanal anastomosis. Although frequently reported in adults, this complication is rare in pediatric patients undergoing this operation. We report 2 cases of adolescent patients with ulcerative colitis who experienced portal vein thrombosis after this procedure. Both were treated with systemic anticoagulation therapy with complete resolution of their clots. We recommend that mesenteric/portal venous thrombosis be considered in the differential diagnosis in any child presenting with fever, abdominal pain, and leukocytosis after restorative proctocolectomy with ileal pouch anastomosis and that imaging obtained to evaluate abdominal complaints in this population be directed toward ruling out this complication.
Full-text · Article · May 2010 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: Intestinal malrotation in adulthood may present with a variety of chronic symptoms. Surgical intervention frequently leads to other complications in these patients. We hypothesized that the chronic nature of malrotation in adults could cause a delay in diagnosis and increased perioperative complications.
All patients diagnosed with intestinal malrotation from July 2002 through July 2006 were included. IRB approval was obtained. Outcomes in patients less than 16 years of age were compared with outcomes from those older than 16. Presenting symptoms, initial diagnosis, results of imaging data, and time to diagnosis were evaluated. Surgical management, resulting complications, and rate of reoperation were analyzed.
Twenty-four patients with intestinal malrotation were identified (age range, 10 days to 89 years old; 10 adults, 14 children). Seventy percent of adults experienced chronic symptoms for 6 months or more before the diagnosis of malrotation was made (children, 14%, p = 0.017). No patients in the adult group were initially diagnosed with malrotation, although 57% of children were correctly diagnosed at the time of presentation of symptoms (p=0.006). Postoperative complications occurred in 60% of adults, but in only 29% of children, though this did not reach significance (p=0.211). Forty percent of adult patients required reoperation (p=0.020).
Intestinal malrotation in adults is often associated with a delay in diagnosis and increased morbidity. Enhanced awareness of this entity in adults may enhance patient counseling and improve therapeutic outcomes in these patients.
No preview · Article · May 2008 · Journal of the American College of Surgeons
[Show abstract][Hide abstract] ABSTRACT: A large intra-abdominal mass was discovered in a 6-month-old boy during a routine well-child examination. Imaging studies revealed a solid mass which appeared to arise from the left lobe of the liver, extending caudally and filling the entire pelvis. At the time of surgical excision, the mass was found to be unassociated with the liver, but was instead localized to the omentum. Histologic examination revealed adipocytes of varying stages of maturation arranged in a lobular architecture, consistent with a lipoblastoma. This unusual tumor is only the eighth reported omental lipoblastoma [J. Hicks, A. Dilley, D. Patel, J. Barrish, S. Zhu, M. Brandt, Lipoblastoma and lipoblastomatosis in infancy and childhood: histologic, ultrastructural, and cytogenetic features. Ultrastruct. Pathol. 25 (2001) 321-333; J. Harrer, G. Hammon, T. Wagner, M. Bolkenius, Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature. Eur. J. Pediatr. Surg. 11 (2001) 342-349; S. Weiss and J. Goldblum, Enzinger and Weiss's Soft Tissue Tumors, fourth ed., Mosby, St. Louis, MO, 2001, pp. 601-605, 670-686; S. Soin, S. Andronikou, R. Lisle, K. Platt, K. Lakhoo, Omental lipoblastoma in a child; diagnosis based in CT density measurements. J. Pediatr. Hematol. Oncol. 28(1) (2006) 57-58; A. Prando, S. Wallace, J.L. Marins, R.M. Pereira, E.R. de Oliveira, M. Alvarenga, Sonographic features of benign intraperitoneal lipomatous tumors in children-report of 4 cases. Pediatr. Radiol. 20(8) (1990) 571-574; C. Blank, E. Schoenmakers, P. Rogalla, E. Huys, A. Van Rijk, N. Drieschner, J. Bullerdiek, Intragenic breakpoint within RAD51L1 in a t(6;14)(p21.3;q24) of a pulmonary chondroid hamartoma. Cytogenet. Cell Genet. 95 (2001) 17-19; S. Ingraham, R. Lynch, S. Kathiresan, A. Buckler, A. Menon, hREC2, a RAD51-like gene, is disrupted by t(12;14)(q15;q24.1) in a uterine leiomyoma. Cancer Genet. Cytogenet. 115 (1999) 56-61]. Cytogenetics revealed a karyotype of 46,XY,t(8;14)(q13;q24). While lipoblastomas characteristically involve 8q, only one prior case has been reported with 14q24 as its fusion partner [M. He, K. Das, M. Blacksin, J. Benevenia, M. Hameed, A translocation involving the placental growth factor gene is identified in an epithelioid hemangioendothelioma. Cancer Genet. Cytogenet. 168 (2006) 150-154]. We report this unique case of an omental lipoblastoma with a focus on its unusual karyotype, as well as its differentiation from myxoid liposarcoma.
No preview · Article · Feb 2008 · Pathology - Research and Practice
[Show abstract][Hide abstract] ABSTRACT: Academic medical centers, which have traditionally been relatively inefficient, have increasing difficulty in meeting the missions of patient care, teaching, and research in a progressively competitive medical marketplace. One strategy for improved efficiency in patient care while keeping quality high is utilization of a product line matrix. This study addresses the outcome of utilizing a product line strategy consisting of 3 service lines during the past 5 years at the University of Wisconsin Hospital and Clinics (UWHC).
Service lines in heart and vascular surgery, oncology, and pediatrics have been organized since 2001, and report directly to hospital leadership as a product line. Service line leadership consists of a combination of medical leaders plus representatives of hospital administration, and service lines are allowed direct access to resources for program development, marketing, and resource allocation. Measurements of patient numbers, market share, length of stay, net margin, and patient satisfaction have been gathered and compared with the preproduct line era.
In the 3 service lines, UWHC has seen variable but steady growth in patient numbers, enhanced market share, positive net margins, and improved patient satisfaction during the period of measurement. During this same period, the insurance milieu has resulted in consistent downward pressure on reimbursement, which has been offset by improved patient care efficiency as measured by length of stay, enhanced preferred provider status, and gains in market share. Scorecard measures of quality are also being developed and show enhanced teaching and research opportunities for students and trainees as well as improved Press Ganey patient satisfaction scores.
At UWHC, the development of a product line matrix consisting of 3 service lines has resulted in more patient care efficiency, enhanced patient satisfaction, improved margin for the hospital, and enlargement of teaching and research opportunities. The key to successful implementation of the product line concept is a close working relationship between the hospital administration and service line medical leadership.
No preview · Article · Nov 2007 · Annals of Surgery
[Show abstract][Hide abstract] ABSTRACT: The increase in longevity among patients with cystic fibrosis has brought to light comorbidities associated with the disease that are less acutely fatal than its pulmonary manifestations. Modern retrospective analysis has demonstrated an increased risk of gastrointestinal malignancy in patients with cystic fibrosis, with a marked elevation in malignancy rates among patients in their 20s and 30s. Here we report a case of colonic adenocarcinoma presenting as pneumaturia in a 13-year-old patient with cystic fibrosis.
No preview · Article · Nov 2007 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: A 28-year-old man with a history of total colonic Hirschsprung's disease treated with a Duhamel-Martin procedure in infancy, presented with signs and symptoms of acute colonic diverticulitis. Flexible sigmoidoscopy demonstrated a 20 cm jejunocolonic anastomosis, consistent with his previous operation in childhood, with a large diverticulum at the proximal end of the anastomosis containing fecal concretions. The patient returned one month later after a course of antibiotics for definitive resection of the diverticulum. Intra-operative colonoscopy localized three large diverticula and resection of the involved segment was performed. The case is presented as an adult complication of total colonic Hirschsprung's disease treated with childhood resection and reconstruction.
No preview · Article · Apr 2007 · Pediatric Surgery International
[Show abstract][Hide abstract] ABSTRACT: Children, just as adults, have a variety of common anorectal problems that can be quite bothersome. The presentation of these problems may be age-specific. Abscesses, fistulas, and fissures appear more commonly in infants and young children, whereas hemorrhoids and pilonidal disease are more common in teens and young adults. Fissures often can be treated medically but may require surgical treatment with lateral internal sphincterotomy. Abscesses and fistulas are common in infant males, especially robust infants who are breastfed. They may resolve with medical therapy but anal fistulotomy is not infrequently required. Hemorrhoids are rare in young children but may be an issue for teenagers. Acute symptomatic lesions may require excision if local measures cannot control the symptoms. Finally, pilonidal disease is a difficult problem for the patient and the surgeon. Persistently symptomatic lesions demand some type of surgical treatment but wound healing is poor in the intergluteal cleft region. More extensive procedures requiring the transfer of fasciocutaneous flaps may be necessary to provide definitive relief. Anorectal problems in infants and children are frequent and bothersome. Although most are not associated with tremendous morbidity, they can lead to much patient and parent anxiety as well as frequent medical consultation until the problem is successfully treated or resolves.
No preview · Article · Mar 2007 · Seminars in Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: We report the largest clinical experience to date of surgically treated patients with blue rubber bleb nevus syndrome (BRBNS).
BRBNS is a rare congenital disorder presenting with multifocal venous malformations of the skin, soft tissues, and gastrointestinal (GI) tract. Patients with BRBNS develop anemia from chronic GI bleeding, and require lifelong treatment with iron and blood transfusions. An aggressive surgical approach to treat the GI venous malformations of BRBNS has been considered unlikely to be successful because of the large number of lesions, their position throughout the GI tract, and the likelihood of recurrence. Based on our belief that eradicated lesions would not recur, we undertook the removal of all GI tract lesions in an effort to eliminate bleeding.
Ten patients with BRBNS were treated from 1993 to 2002. Lesions were identified using complete GI endoscopy. The multiple venous malformations were removed by a combination of wedge resection, polypectomy, suture-ligation, segmental bowel resection, and band ligation.
Patient ages ranged from 2 to 36 years, and patients received an average of 53 prior blood transfusions. A mean of 137 focal GI venous malformations per patient were resected at operation (range 4-557), with a mean operative duration of 14 hours (range 7-23 hours). Only 1 patient who had a less extensive procedure developed recurrent GI bleeding. The mean follow-up period was 5.0 years (range 2.9-10.3 years).
We believe that an aggressive excisional approach is indicated for the venous anomalies that cause GI bleeding in BRBNS.
Full-text · Article · Apr 2005 · Annals of Surgery
[Show abstract][Hide abstract] ABSTRACT: Glutamine (Gln) is a non-essential amino acid that plays an important role in energy metabolism for gastrointestinal epithelia and other cells with rapid turnover. We evaluated the effects of enteral supplementation with Gln in infants undergoing surgery for congenital or acquired gastrointestinal disease.
This was a randomized, double-masked, controlled clinical trial.
Twenty infants were randomly assigned to receive Gln (n = 9) or placebo amino acid (n = 11), with a goal of supplemental amino acid intake of 0.4 g.kg(-1).d(-1). Infants were weaned from parenteral nutrition, and enteral feeds were started according to a standardized feeding protocol. Median (interquartile range) durations of parenteral nutrition were 39 d (12 to 99) in the Gln group and 21 d (6 to 59) in the control group (P = 0.201). Median (interquartile range) durations needed to reach 80% of the US recommended dietary allowance for energy with enteral nutrition were 24 d (8 to 55) in the Gln group and 12.5 d (5 to 32) in the control group (P = 0.313). There were no differences in the occurrence of infections between groups. Among all infants enrolled, significant correlations were found between duration of parenteral nutrition and residual small bowel length, peak concentrations of direct bilirubin, and alanine aminotransferase. Peak direct bilirubin was associated with longer duration of parenteral nutrition, shorter gestation, older age before feeds were started, shorter bowel length, and larger amounts of parenteral energy and protein intake.
In this pilot trial, enteral Gln supplementation was well tolerated among infants with surgical gastrointestinal disease. There was no effect observed on the duration of parenteral nutrition, tolerance of enteral feeds, or intestinal absorptive or barrier function. Larger, multicenter trials in infants with surgical gastrointestinal disease are needed due to the variability in important outcome measurements.
[Show abstract][Hide abstract] ABSTRACT: A premature neonate had pneumoperitoneum 5 days after discontinuation of extracorporeal membrane oxygenation therapy. A perforated appendix was found at exploratory laparotomy. Pathologic examination of the appendix found mucormycosis.
Full-text · Article · Aug 2004 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: Total colonic aganglionosis (TCA) occurs in 3% to 12% of Hirschsprung's disease patients. Although numerous surgical techniques have been utilized for the treatment of these patients, little information is available regarding optimal surgical management of their frequent complications or failured procedures. The ileoanal S pouch (IASP) technique has been utilized in the treatment of children with familial adenomatous polyposis and ulcerative colitis. The authors present the results of salvage IASP in 3 TCA patients who had poor results after total colectomy and Soave pull-through.
Full-text · Article · Aug 2004 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: Several studies in animal models demonstrate that peel formation in gastroschisis is due to the accumulation and activation of intestinal waste products (IWP) in the amniotic fluid. We reviewed our recent experience with gastroschisis and asked the following questions: First, does staining of the bowel and amniotic fluid with IWP correlate with intestinal peel formation? Second, what prenatal ultrasound findings indicate that peel formation is occurring in utero? Over two years, 16 neonates were treated for gastroschisis; twelve had been diagnosed by prenatal ultrasound and followed closely. Patients were grouped based on the presence of IWP in the amniotic fluid at the time of delivery (staining or no staining), and outcomes were reviewed. All neonates in the staining group ( n =7) had a fibrinous peel present at the time of birth whereas a peel was absent in all neonates in the no-staining group ( n =9). Matting of the bowel was seen by prenatal ultrasound in four patients in the staining group (0/8 in the no-staining group) and correlated with peel formation (Fisher’s exact test p
=0.007). Primary closure was done in 14 of the infants, and two required silo closure. In neonates with gastroschisis, staining of the amniotic fluid and bowel serosa with IWP correlated with intestinal peel formation. The ultrasound findings of matting correlated with both peel formation and staining with IWP. These results suggest that spillage of IWP into the amniotic fluid is one of the factors in peel formation in gastroschisis. Identification of matting of the bowel by prenatal ultrasound indicates formation of a peel.
Full-text · Article · Apr 2004 · Pediatric Surgery International
[Show abstract][Hide abstract] ABSTRACT: The purpose of this report is to detail the nutritional sequelae seen in survivors of congenital diaphragmatic hernia (CDH) followed in a multidisciplinary clinic.
Data on 121 surviving CDH patients seen between 1990 and 2000 were collected. Regression analysis was used to determine the impact of factors such as Apgar score, birth weight, extracorporeal membrane oxygenation (ECMO), and patch repair on outcomes associated with nutritional morbidity.
There were 100 left and 21 right CDH defects. Mean birth weight and 5-minute Apgar score were 3.1 kg (+/-0.8) and 6.8(+/-2), respectively. Extracorporeal membrane oxygenation was required in 43 (36%) patients and patch repair in 39 (32%). A gastrostomy was required in 39 (32%) patients and a fundoplication in 23 (19%) patients. The side of the defect did not affect the frequency of these procedures. Fifty-six percent of patients were below the 25th percentile for weight during most of their first year. Regression analysis found that duration of ventilation (P <.001) and the presence of a patch repair (P =.03) were independent variables predictive of failure to thrive thereby requiring a gastrostomy tube. Patch repair also was predictive of need for subsequent fundoplication caused by gastroesophageal reflux (P <.001). Twenty-nine patients (24%) had severe oral aversion. Risk factors were prolonged ventilation (P =.001) and oxygen requirement at discharge (P =.015). Two thirds of these patients subsequently improved.
Nutritional problems continue to be a source of morbidity for survivors of CDH, particularly in the first year of life. Not surprisingly, patients who had prolonged intubation and prosthetic material at the gastroesophageal junction fared worse. Despite aggressive nutritional management, 56% of the population remained below the twenty-fifth percentile for weight. These data show the need for careful nutritional assessment in all CDH patients, especially those at high risk for malnutrition.
No preview · Article · Sep 2001 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: To determine correlates of clinical outcomes in patients with short bowel syndrome (SBS).
Retrospective medical record review of neonates treated between 1986 and 1998 who met our criteria for SBS: dependence on parenteral nutrition (PN) for at least 90 days after surgical therapy for congenital or acquired intestinal diseases.
Thirty subjects with complete data were identified; 13 (43%) had necrotizing enterocolitis, and 17 (57%)had intestinal malformations. Mean (SD) residual small bowel length was 83 (67) cm. Enteral feeding with breastmilk (r = -0.821) or an amino acid-based formula (r = -0.793) was associated with a shorter duration of PN, as were longer residual small bowel length (r = -0.475) and percentage of calories received enterally at 6 weeks after surgery(r = -0.527). Shorter time without diverting ileostomy or colostomy (r = 0.400), enteral feeding with a protein hydrolysate formula (r = -0.476), and percentage of calories received enterally at 6 weeks after surgery (r = -0.504) were associated with a lower peak direct bilirubin concentration. Presence of an intact ileocecal valve and frequency of catheter-related infections were not significantly correlated with duration of PN. In multivariate analysis, only residual small bowel length was a significant independent predictor of duration of PN, and only less time with a diverting ostomy was an independent predictor of peak direct bilirubin concentration.
Although residual small bowel length remains an important predictor of duration of PN use in infants with SBS, other factors, such as use of breast milk or amino acid-based formula, may also play a role in intestinal adaptation. In addition, prompt restoration of intestinal continuity is associated with lowered risk of cholestatic liver disease. Early enteral feeding after surgery is associated both with reduced duration of PN and less cholestasis.
No preview · Article · Aug 2001 · Journal of Pediatrics
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to evaluate the outcomes of reconstruction of all cloacal exstrophy cases seen by the authors from 1974 to 1999.
A retrospective chart review and personal follow-up was conducted on all 50 patients, who ranged in age from newborn to 35 years. Thirty-eight were secondary cases, 12 were primary (no previous surgery). Twenty-eight (56%) were genetic boys; 6 were raised as boys and 22 as girls. Forty patients underwent extensive reconstructive surgery; 6 await reconstruction, and 4 were seen only in consultation. For urinary continence, 21 had bladder neck narrowing, 7 received a bowel nipple, and 12 had a Mitrofanoff catheterizable conduit on the abdominal wall, using tapered intestine in 8, ureter in 3 and the appendix in 1. Four patients had a urostomy. Bladder augmentation was performed in 35 patients (18 with stomach, 11 small bowel, and 6 with both). Twenty-five patients had pull-through of their colon to the perineum. Thirty-two have had a vagina constructed. Forty-seven of the 50 patients had spinal cord tethering, and most underwent neurosurgical release.
Overall survival rate was 98%; 1 patient died preoperatively at another institution. Acceptable bowel continence was achieved with enema washouts in 19 of 25 pullthroughs; 4 failed and were reversed. One case is too recent to judge. Of 40 reconstructed cases, 31 were dry, 3 had slight leakage, and 1 is too early to evaluate. Five had enough leakage to require further surgery.
The once hopeless anomaly of cloacal exstrophy is treatable with extensive reconstructive surgery. Continence of urine (mainly by catheterization) and stool (mainly by enema washouts) is achievable in most. The hindgut, including the cecum and the usually rudimentary distal colon, should be saved and used as colon, not for urinary or vaginal reconstruction. In genetic boys, the authors continue to believe that gender assignment should depend on the likelihood for reconstructing an adequate phallus.
No preview · Article · Feb 2001 · Journal of Pediatric Surgery