Antenatally diagnosed cloacal exstrophy variant with intravesical phallus in a twin pregnancy.

We report a rare case of covered cloacal exstrophy variant with a hemiphallus trapped within partially closed bladder halves. The persistence of the cloacal membrane until at least 18 weeks' gestation, confirmed by antenatal ultrasound scanning, is discordant with existing theories of embryogenesis of cloacal exstrophy. The clinical presentation highlights the need for careful assessment, before and during surgery, to obtain a complete understanding of the anatomic defect before gender assignment and appropriate reconstructive efforts. A multispecialty approach and antenatal counseling are important, especially when only one fetus of twins has major birth defects.

Integrin alpha1beta1 and transforming growth factor-beta1 play distinct roles in alport glomerular pathogenesis and serve as dual targets for metabolic therapy.

Alport syndrome is a genetic disorder resulting from mutations in type IV collagen genes. The defect results in pathological changes in kidney glomerular and inner-ear basement membranes. In the kidney, progressive glomerulonephritis culminates in tubulointerstitial fibrosis and death. Using gene knockout-mouse models, we demonstrate that two different pathways, one mediated by transforming growth factor (TGF)-beta1 and the other by integrin alpha1beta1, affect Alport glomerular pathogenesis in distinct ways. In Alport mice that are also null for integrin alpha1 expression, expansion of the mesangial matrix and podocyte foot process effacement are attenuated. The novel observation of nonnative laminin isoforms (laminin-2 and/or laminin-4) accumulating in the glomerular basement membrane of Alport mice is markedly reduced in the double knockouts. The second pathway, mediated by TGF-beta1, was blocked using a soluble fusion protein comprising the extracellular domain of the TGF-beta1 type II receptor. This inhibitor prevents focal thickening of the glomerular basement membrane, but does not prevent effacement of the podocyte foot processes. If both integrin alpha1beta1 and TGF-beta1 pathways are functionally inhibited, glomerular foot process and glomerular basement membrane morphology are primarily restored and renal function is markedly improved. These data suggest that integrin alpha1beta1 and TGF-beta1 may provide useful targets for a dual therapy aimed at slowing disease progression in Alport glomerulonephritis.

Variability of the obturator vessels.

The obturator artery and vein are usually described as branches or tributaries of the internal iliac vessels although variations with connections to the external iliac or inferior epigastric vessels have been reported. Because these anomalous vessels are at risk in groin or pelvic surgeries that require dissection or suturing along the pelvic rim, we measured the frequency of these variations in 105 pelvic walls (45 in the United States and 60 in China). Our data show that 70-82% of pelvic halves and 83-90% of whole pelves had an artery, vein, or both in the variant position. Arteries were most often found in the normal position only but normal and anomalous veins were most frequently found together. These data show that it is far more common to find a vessel coursing over the pelvic rim at this site than not and have implications for both pelvic surgeons and anatomists.

The clinical anatomy of laparoscopic inguinal hernia repair.

Laparoscopic approaches for abdominal surgery are being used with increasing frequency. Their advantages are sometimes negated by the disturbing incidence of postoperative sequelae. In the case of inguinal hernia repair, these are often the result of failing to understand that the anatomy of the anterior approach to the abdominal wall cannot necessarily be directly applied to laparoscopy. The inguinal ligament, easily identified in an anterior approach, is only seen laparoscopically after removal of the iliopubic tract, a key structure which lies in the plane of the original defect of most groin hernias. Thus, an understanding of the incompletely trilaminar anterior abdominal wall, including the iliopubic tract, is the foundation for effective inguinal hernia repair using any approach (anterior or posterior) or technique (sutures, mesh or staples). Laparoscopic inguinal hernia repair has produced an increase in the frequency of debilitating neuropathies, most notably of the lateral femoral cutaneous nerve (LFCN). This is directly related to the variable intrapelvic course of this nerve or its branches. In more than 13% of the 114 pelves we examined, the LFCN was within 0.5 cm of the iliopubic tract or in the vertical plane of the anterior superior iliac spine, key lateral landmarks and anchoring sites for mesh in laparoscopic hernia repairs. Medial landmarks also have variable features. These data indicate that the identity of anatomical landmarks and the variability of other structures will continue to be important in the successful development of new laparoscopic techniques.

Variations in the inferior pelvic pathway of the lateral femoral cutaneous nerve: implications for laparoscopic hernia repair.

Laparoscopic repair of inguinal hernias is gaining acceptance in the repertoire of the general surgeon. However, nerve entrapment sequelae have been reported and appear to be higher with the laparoscopic approach. Contributing factors include pelvic variations in nerve pathways and the use of staples. We examined the pelvic relations of the lateral femoral cutaneous nerve (LFCN) to the anterior superior iliac spine (ASIS) and the iliopubic tract (IPT) because of the high morbidity of entrapment of this nerve, despite its low incidence. The LFCN, ASIS, and IPT were identified and their relationships measured in 48 male and 24 female cadavers ranging in age from 61 to 96 yr. The LFCN was located 1.7 (+/- 1.2) cm medial to the ASIS along the IPT and 1.4 (+/- 0.7) cm posterior (deep) to the IPT at this point, with no significant sex differences. The intrapelvic pathway of the LFCN, including its branches, varied widely so that in 18% of these specimens the LFCN was in either the vertical plane of the ASIS (13%) or in the plane of the IPT (5%). In 11% this nerve was within 1 cm of the ASIS. These data indicate that exclusive use of the ASIS as a guide for staple placement may result in entrapment of this nerve or its branches.