Is routine pathological evaluation of pediatric hernia sacs justified?

BACKGROUND/PURPOSE: Herniorrhaphy is the most common general surgical procedure performed on children, and hernia sac material is one of the most common tissue specimens microscopically examined in the authors' surgical pathology laboratory. The risk of accidental vas deferens ligation has prompted the recommendation that all hernia sacs be examined pathologically. The authors hypothesized that the incidence of unrecognized vas deferens or epididymis ligation is actually very low and may not warrant routine pathological examination of all pediatric hernia sacs. METHODS: Over a 3-year period (1994 to 1996), pathology reports from all hernia repairs at the authors' institution were reviewed. A total of 1,494 inguinal hernia sacs were pathologically evaluated from 1,077 pediatric patients (417 were bilateral). Pathological diagnoses not affecting clinical management (ie, chronic inflammation, irritated hernia sacs, embryonal remnants, adrenal cortical rests) were classified as incidental findings. Identification of true vas deferens was classified as a positive finding. RESULTS: The study population had a mean age of 3.9 +/- 0.1 years and 963 (89%) were boys. The incidence of vas deferens injury from herniorrhaphy was found to be 0.13% (2 of 1,494), and these were recognized by the pediatric surgeon in the operating room. CONCLUSIONS: When vas deferens injury is suspected, the sample should always be sent to the pathology department for confirmation. However, no occult carcinoma or other pathology was identified, and the remainder of the histological findings did not change the clinical treatment of any child. Given a fixed cost of pathological analysis, elimination of routine hernia sac examination may result in substantial annual savings. Therefore, in the current era of cost containment, recommendations for routine pathological examination of excised pediatric hernia sacs should be reevaluated.

Vitamin E reduces oxidant injury to mitochondria and the hepatotoxicity of taurochenodeoxycholic acid in the rat.

BACKGROUND & AIMS: Hydrophobic bile acids have been implicated in the pathogenesis of cholestatic liver injury. The hypothesis that hydrophobic bile acid toxicity is mediated by oxidant stress in an in vivo rat model was tested in this study. METHODS: A dose-response study of bolus intravenous (i.v.) taurochenodeoxycholic acid (TCDC) in rats was conducted. Rats were then pretreated with parenteral alpha-tocopherol, and its effect on i.v. TCDC toxicity was evaluated by liver blood tests and by assessing mitochondrial lipid peroxidation. RESULTS: Four hours after an i.v. bolus of TCDC (10 mumol/100 g weight), serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels peaked, hepatic mitochondria showed evidence of increased lipid peroxidation, and serum bile acid analysis was consistent with a cholestatic injury. Liver histology at 4 hours showed hepatocellular necrosis and swelling and mild portal tract inflammation. Treatment with parenteral alpha-tocopherol was associated with a 60%-70% reduction in AST and ALT levels, improved histology, and a 60% reduction in mitochondrial lipid peroxidation in rats receiving TCDC. CONCLUSIONS: These data show that hepatocyte injury and oxidant damage to mitochondria caused by i.v. TCDC can be significantly reduced by pretreatment with the antioxidant vitamin E. These in vivo findings support the role for oxidant stress in the pathogenesis of bile acid hepatic toxicity.

Combined therapy with inhaled nitric oxide and intravenous prostacyclin in an infant with alveolar-capillary dysplasia.

Severe persistent pulmonary hypertension of the newborn (PPHN) remains a significant cause of neonatal morbidity and mortality with limited effective treatment options. We present the first case of a neonate with PPHN treated concurrently with inhaled nitric oxide (iNO) and intravenous prostacyclin (PGI2). He ultimately was diagnosed with alveolar-capillary dysplasia, a rare and fatal cause of pulmonary hypertension. However, his partial response to treatment demonstrates a possible role for combined therapy with iNO and PGI2 in infants with severe PPHN.

Cavernous lymphangioma presenting as a vaginal discharge in a six-year-old female: a case report.

STUDY OBJECTIVE: This case report is written to describe a new and unusual presentation of retroperitoneal cavernous lymphangioma. INTERVENTIONS: A large cystic abdominal mass was discovered during an examination under anesthesia to evaluate a vaginal discharge in a 6-year-old girl. The mass was diagnosed by computed tomography (CT) and removed via laparotomy with argon beam coagulation of smaller cystic lesions. RESULTS: Six months postoperatively, the patient had a negligible amount of vaginal discharge. CONCLUSIONS: Cavernous lymphangioma should be considered as a rare cause of vaginal discharge in young females.

Infrequent mutation of the WT1 gene in 77 Wilms' Tumors.

Homozygous deletions in Wilms' tumor DNA have been a key step in the identification and isolation of the WT1 gene. Several additional loci are also postulated to contribute to Wilms' tumor formation. To assess the frequency of WT1 alterations we have analyzed the WT1 locus in a panel of 77 Wilms' tumors. Eight tumors showed evidence for large deletions of several hundred or thousand kilobasepairs of DNA, some of which were also cytogenetically detected. Additional intragenic mutations were detected using more sensitive SSCP analyses to scan all 10 WT1 exons. Most of these result in premature stop codons or missense mutations that inactivate the remaining WT1 allele. The overall frequency of WT1 alterations detected with these methods is less than 15%. While some mutations may not be detectable with the methods employed, our results suggest that direct alterations of the WT1 gene are present in only a small fraction of Wilms' tumors. Thus, mutations at other Wilms' tumor loci or disturbance of interactions between these genes likely play an important role in Wilms' tumor development.

Osteogenic sarcoma arising from bony regenerate following Ilizarov femoral lengthening through fibrous dysplasia.

Sarcomatous degeneration of fibrous dysplasia has been previously documented in rare cases, often in association with prior radiation therapy. This case report describes the occurrence of osteosarcoma arising in the distal femur of a 15-year-old male with previous biopsy-proven fibrous dysplasia, who had undergone limb lengthening through a distal femoral corticotomy using the technique described by Ilizarov. In addition, distraction osteogenesis through an area of fibrous dysplasia in the proximal tibia produced a new area of fibrous dysplasia in the regenerate tissue as shown by histopathologic examination. This case raises two questions: (a) whether the biologic stimulus of distraction osteogenesis through abnormal metaplastic bone increases the risk of malignant neoplasia, and (b) whether distraction osteogenesis through dysplastic bone is contraindicated, since the regenerate tissue does not form normal bone.

Neonatal lupus liver disease.

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by complete congenital heart block and/or transient skin lesions of subacute cutaneous lupus erythematosus. We report that in approximately 10% of cases of NLE with heart block or skin disease, liver disease also occurs (4 of 35 cases in our series). Cholestasis was the major feature in our cases. Although the cholestasis may be severe, the disease process appears to be transient and surviving babies have been healthy on follow-up. In one liver examined for antibody deposition, IgG antibody deposits, presumably of maternal origin, were present. Three maternal sera were examined for autoantibodies, including liver-specific autoantibodies. No liver-specific autoantibodies were found. Rather, the maternal autoantibodies too were the ubiquitous Ro/SSA-associated autoantigens. The autoantibodies bound the 60 kDa SSA/Ro ribonuclear protein (three of three sera), the 52 kDa SSA/Ro protein (two of three sera) and the SSB/La ribonuclear protein (two of three sera).