Doctor of Medicine, Duke University (2008)
BA, Harvard College, Physics (2003)
Mark Krasnow, Postdoctoral Faculty Sponsor
Abstract Background: Femoral hernias in young children are relatively rare and can be difficult to diagnose as they are often mistaken for inguinal hernias. Although a few reports have described laparoscopic techniques, most traditional repair methods still focus on an open approach using either an inguinal or crural incision. Here we describe a laparoscopic-assisted technique that is buttressed by a cigarette of mesh for the repair of this uncommon pediatric entity. Subjects and Methods: We report three consecutive cases of children with femoral hernias repaired with only two small incisions: a 5-mm umbilical incision for a 30° camera and a 1-cm groin incision for dissection and ligation of the hernia sac. After sac ligation, the repair was buttressed with a small mesh cigarette. Results: Using this approach, right femoral hernias were repaired without complication in three children, between 8 and 9 years of age. Two patients had ipsilateral indirect inguinal hernias. No contralateral groin hernias were identified in any of the patients. Operative time averaged 40 minutes, recovery time was quick, and follow-up at 6 months revealed good cosmesis. Conclusions: This laparoscopic-assisted approach to pediatric femoral hernia repair with a small mesh plug is a safe, effective, and efficient technique. Because only two incisions are required, postoperative pain is minimal, and cosmesis is excellent. Nonetheless, more patients and longer follow-up will be required to accurately judge the long-term implications of this novel technique.
View details for DOI 10.1089/lap.2013.0199
View details for PubMedID 24015871
Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder associated with ciliary defects and situs inversus totalis, the complete mirror image reversal of internal organ situs (positioning). A variable incidence of heterotaxy, or irregular organ situs, also has been reported in PCD patients, but it is not known whether this is elicited by the PCD-causing genetic lesion. We studied a mouse model of PCD with a recessive mutation in Dnahc5, a dynein gene commonly mutated in PCD. Analysis of homozygous mutant embryos from 18 litters yielded 25% with normal organ situs, 35% with situs inversus totalis, and 40% with heterotaxy. Embryos with heterotaxy had complex structural heart defects that included discordant atrioventricular and ventricular outflow situs and atrial/pulmonary isomerisms. Variable combinations of a distinct set of cardiovascular anomalies were observed, including superior-inferior ventricles, great artery alignment defects, and interrupted inferior vena cava with azygos continuation. The surprisingly high incidence of heterotaxy led us to evaluate the diagnosis of PCD. PCD was confirmed by EM, which revealed missing outer dynein arms in the respiratory cilia. Ciliary dyskinesia was observed by videomicroscopy. These findings show that Dnahc5 is required for the specification of left-right asymmetry and suggest that the PCD-causing Dnahc5 mutation may also be associated with heterotaxy.
View details for DOI 10.1172/JCI33284
View details for Web of Science ID 000251396600023
View details for PubMedID 18037990