Comprehensive developmental mechanisms in gastroschisis.

INTRODUCTION: The development of gastroschisis remains an area of controversy. Various theories have been proposed, but none has ever been supported by a thorough embryological study. MATERIAL AND METHODS: We herein report anatomical and microscopic observations of the developing abdominal wall and cord of embryos and fetuses, along with clinical features of gastroschisis. RESULTS: It appears that the developing cord normally has two parts, a firm left-sided part formed by the vessels and urachus, and a thin right-sided pouch covering the intestinal loops (the 'physiological umbilical hernia'), which could rupture, giving the basis of gastroschisis. DISCUSSION: Gastroschisis could be the result of amniotic damage, possibly from some as yet unidentified toxin. Further bowel damage can be explained by the subsequent mesenteric injury.

Preventive antireflux surgery in patients with congenital diaphragmatic hernia.

BACKGROUND: Gastroesophageal reflux (GER) is common in children with congenital diaphragmatic hernia (CDH), and the optimal management in these patients is not well established. The aim of this study was to assess whether a preventive fundoplication performed during the first surgery will improve GER and nutritional morbidity in patients with a left CDH. METHODS: Thus we studied one group of 17 patients treated with a one-step antireflux procedure (group A) and a group of 19 patients with only initial diaphragmatic repair (group B) between January 1994 and December 2004. The patients were classified in three stages, mild intermediate and severe form, based on anatomic criteria (liver position and patch requirement). Most of the patients with the severe form of disease were in group A. Clinical charts were compared between the two groups with a mean follow-up of 3 years. RESULTS: For patients with intra-abdominal liver and direct diaphragmatic closure, the duration of hospitalization was significantly longer in group A than in group B (p = 0.08). The incidence of GER was significantly lower in group A (17.6%) than in group B (52.6%) at 1-year follow-up (p < 0.5). In the last group, 30% required a fundoplication before 6 months. Seven patients (19%) required intermittent nutritional supplementation via nasogastric tube feeding; for these patients there was no difference between the two groups. A gastrostomy was performed in only one patient. Twelve percent of patients had growth retardation during most of their first year, again with no difference between the two groups. CONCLUSIONS: Based on the published data, recommendations can be made regarding the efficacy of adding an antireflux surgery procedure to left CDH repair in only patients with intrathoracic liver and/or patch requirement.

[Medium and long-term evaluation of spongioplasty in hypospadias repair]. / Spongioplastie dans la réparation de l'hypospadias: évaluation à moyen et long terme.

INTRODUCTION: Spongioplasty for the treatment of hypospadias consists of tubulization urethroplasty combined with dissection and midline approximation of the two divergent hemicorpora spongiosa. The objective of this study was to evaluate the medium-term and long-term cosmetic and functional results, and satisfaction of patients operated by this technique. MATERIAL AND METHODS: Fifty spongiopasties were performed by the same surgeon between 1990 and 1996. Foreskin reconstruction was performed in 40 patients. 48 patients were reviewed between 2002 and 2003. RESULTS: The immediate results revealed fistula in 8% of cases, meatal stricture in 4% of cases, and foreskin complications in 14% of cases. The long-term results showed persistence of an unsightly operative scar in 8% of cases, residual chordee in 6% of cases, meatal retraction in 8% of cases, and persistent partial stricture in 4% of cases. 92% of patients declared that they were satisfied with the results of surgery. CONCLUSION: The immediate results of spongioplasty are similar to those obtained by other techniques and long-term follow-up shows maintenance of the cosmetic and functional results.

[Spongioplasty in the treatment of hypospadias]. / La spongioplastie dans le traitement des hypospades.

OBJECTIVE: Hypospadias is a frequent anomaly, for which many repair techniques have been proposed. In 2000, we described a method of one-stage anatomical correction, called spongioplasty. The present study evaluated the functional and cosmetic results of this technique. MATERIAL AND METHODS: 154 patients treated by this technique over a 10-year period were reviewed. All complications reported were compared according to the anatomical form, age at the time of surgery, and year of operation. RESULTS: We demonstrate a learning curve lasting about 6 years, after which the complication rate of this technique becomes lower than that associated with other methods, particularly for distal forms. In our experience, this complication rate is lower for an operative age between 5 and 8 months. CONCLUSION: In the light of these results, this type of surgery can be proposed at an earlier age than other techniques.

In utero allotransplantation of fetal hepatocytes in primates.

OBJECTIVE: Because intrauterine transplantation of fetal hepatocytes could become an effective approach for treating severe genetic disorders of the liver, the objective of this study was to demonstrate the feasibility of in utero allotransplantation of fetal hepatocytes in a nonhuman primate model using direct intraparenchymal administration of donor cells. METHODS: Fetal primary hepatocytes were isolated from 3 fetal primates (MACACA MULATTA) at 89-120 days of gestation, and cryopreserved. When a recipient was available, the cells were thawed and transduced by a beta-galactosidase-expressing retrovirus (3 cases) or labelled with a fluorescent dye (4 cases). Hepatocytes were infused directly into the fetal liver under surgical visual control. Engraftment was assessed by surgical liver biopsies taken 8-60 days following transplantation. RESULTS: Six recipients survived until liver biopsy, and 1 died during the surgical procedure. There was no evidence of engraftment in the 3 fetuses that received genetically marked hepatocytes. All 3 monkeys who received 20-25 x 10(6) hepatocytes from an 89-day-old donor labelled with fluorescent dye had positive liver biopsies 8-11 days following intrauterine transplantation. CONCLUSIONS: In utero allotransplantation of fetal hepatocytes is feasible in the nonhuman primate, and direct intraparenchymal administration enables short-term detection of persisting donor hepatocytes.