Malrotation of the intestinal tract is a product of a well defined aberrant embryology. Because the consequences of malrotation associated with a midgut volvulus may be catastrophic, an understanding of the anatomy, diagnostic criteria, and appropriate therapy for this putative emergency illness is imperative. This report summarizes a recent 18-month experience with this diagnosis and contrasts this experience with that in the published literature. More than half (14/22) of the patients presented during the first month of life, and all had vomiting, which in most cases was bilious. The barium upper gastrointestinal series was the preferred diagnostic study, being both sensitive (18/19, 95%) and accurate (18/21, 86%). In this series two-thirds of the patients presented with volvulus (15/22, 68%) of whom five had ischemic intestine requiring resection. One of these children died of overwhelming sepsis. A Ladd procedure was the preferred treatment, which as defined by us includes evisceration and inspection of the mesenteric root, counterclockwise derotation of a midgut volvulus, lysis of Ladd's bands with straightening of the duodenum along the right abdominal gutter, inversion-ligation appendectomy, and placement of the cecum into the left lower quadrant. A high index of suspicion in the neonate with vomiting, rapid diagnosis, and appropriate operative therapy results in a predictable favorable outcome for children with intestinal malrotation.
Conjoined twins occur in approximately one in 50,000 or so births, and most do not survive. The authors report herein their experience with 13 conjoined twins over the last 30 years, involving those of the following forms: thoracopagus (4 cases), omphalopagus (1 case), ischiopagus (4 cases), pygopagus (1 case), craniopagus (1 case), and incomplete or parasitic varieties (2 cases). The various diagnostic and imaging studies used are described in detail for each form of twinning. Separation is best delayed until such infants are relatively mature (i.e., 6-12 months of age). Operative survival was 50% in those operated on in the neonatal period, but 90% in those over 4 months of age. Ten separations were attempted in 13 sets of twins, with 16 operative survivors. Significantly, up to 10 years after surgery, there were six late deaths due to serious associated congenital anomalies, predominantly cardiac. Improved recent survival is probably the result of the availability of more accurate imaging studies and better anesthetic and operative techniques, with great emphasis on performing immediate reconstruction whenever possible. Use of skin expanders and prosthetic mesh has facilitated wound closure. In the future, ex vivo cardiac reconstruction and autotransplantation may permit separation of twins with complicated conjoined hearts.
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