Biomedical subjects
Publications and source records attributed to Stephen E Dolgin.
Retrograde intussusception is a rare postoperative complication, most commonly after gastric resection or gastrojejunostomy. This report describes a case of retrograde jejunojejunal intussusception after duodenal atresia repair.
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BACKGROUND/PURPOSE: Complete androgen insensitivity syndrome (CAIS) is a rare disorder; however, surgeons have noted a higher rate in girls with inguinal hernias. A few retrospective studies have estimated the incidence of CAIS to be 0.8% to 2.4% in girls with inguinal hernias. An inexpensive, quick screening method for this population has not been established. Because CAIS is associated with a short vagina, measuring vaginal length could serve this purpose if normal standards were known. The authors endeavored to (1) prospectively confirm the incidence of CAIS, (2) identify normal standards of vaginal length, and (3) assess the usefulness of measuring vaginal length to screen for CAIS in girls with inguinal hernias. METHODS: Vaginal lengths were measured in 270 girls with inguinal hernias at a university hospital from 1991 to 2003. A fallopian tube was identified to exclude CAIS. If CAIS was suspected, gonadal tissue was sampled and karyotyping was performed. Linear regression analysis was performed, and 95% confidence intervals were calculated for individual values. RESULTS: Normal vaginal length for age was established. Three patients were found to have significantly short vaginas: 2 were confirmed to have CAIS, 1 did not (false-positive). One other infant was proved to have CAIS despite having a normal vaginal length (false-negative). The incidence of CAIS in our study was 1.1% (3/270). CONCLUSIONS: This is the largest prospective study of the incidence of CAIS in girls with hernias. The authors found that 1.1% of premenstrual girls with inguinal hernias have this syndrome. The authors also provide standards for normal vaginal length in the pediatric population. Vaginal length increases predictably with age, and some patients with CAIS have significantly shorter vaginas. Vaginal length is not a perfect predictor of this disorder, but can be a useful adjunctive screening tool.
Almost a half-century since Kasai described the portoentersotomy for extrahepatic biliary atresia (EHBA), some questions about the management of this condition have been resolved and many are unanswered. The most useful diagnostic steps to aid in the diagnosis are debated. Sonography can be helpful but its sensitivity and selectivity are arguable with strong advocates for its effectiveness. Likewise, the magnetic resonance imaging has forceful advocates but also has not been universalized. The liver biopsy, done commonly before an operation for cholestasis, is often not discriminating. The radionuclide scan hepatobiliary iminodiacetic acid (HIDA) scan after phenobarbital stimulation is helpful if negative, but false positive results are common. It is agreed to proceed expeditiously to the operation in the cholestatic infant after a prompt investigation. The proposal to avoid this step and provide liver transplantation as initial management for EHBA has been suppressed by several clinical findings. The Kasai procedure has not worsened the outcome of eventual liver transplantation. The Kasai, even if it eventually fails, will often buy time and allow the child to grow before transplantation is needed. Multiple reoperations prior to the transplant are discouraged. Revisions to improve bile flow have not gained wide popularity. Use of a stoma to divert the bile has been largely abandoned. The need for frozen section examination of the liver at the site chosen for the portoenterostomy is no longer demanded. The preferred type of intestinal conduit is argued. Unanswered questions about the post-operative management include the role of steroids and of prophylactic antibiotics. The Biliary Atresia Research Consortium, a multi-institutional National Institutes of Health (NIH)-supported project, will address many of the unanswered issues.
A teenage boy presented in the early stage of pyomyositis. He had neck pain, tenderness, and fever. A computed tomography scan showed inflammation in the sternocleidomastoid muscle with no fluid collection. This progressed to a pus-filled drainable mass caused by Stapylococcus aureus. The authors describe this case to highlight the predictable stages and increase the index of suspicion to enhance its early recognition.
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