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Background The rapid development of video surgery and minimally invasive surgical techniques prompted many studies on the methods of teaching these techniques to young surgeons in training. However, the characteristics of a short-term course that is both easily accessible and efficient for this group of surgeons remain controversial. To investigate this issue, a short-term training method was proposed for first year surgery residents, using inexpensive handmade wooden simulation boxes with the students smartphones as cameras. Its effectiveness was evaluated, as well as possible factors that could influence student performance, such as gender and previous experience with video games. Methods Thirty-six first-year General Surgery residents, entering in 2019 and 2020, participated in the study: 21 were males and 15 were females with ages between 22 and 29 years old, (mean 25.47 years). All participants performed a pre-established exercise (placing two simple stitches using a laparoscopic simulator), which was timed and scored. They then participated in a short theoretical-practical course, consisting of an initial lecture followed by 4 exercises on handcrafted wooden laparoscopic video surgery simulators. Afterwards, they were asked to repeat the same exercise from the first step. Finally, they answered a questionnaire that included questions on previous videogame experience. The data were tabulated and submitted to statistical analysis. Results In the pre-training exercise, 15 (41.66%) participants were able to perform the two simple stitches in the simulator box within the maximum time limit of 5 minutes. After the short course, 22 (61.11%) of participants were able to perform the complete exercise. Improvement in the time to complete the practical exercise was statistically significant (p = 0.0296) after participating in the theoretical-practical course. A better pre- and post-training performance was demonstrated by the 17 participants with experience with video games (p = 0.0116), and a better post-training performance was demonstrated by female participants (p = 0.0405). Conclusion This short-term inexpensive theoretical-practical course in laparoscopic training for surgeons in training was effective in reducing the execution time of a laparoscopic stitch in a simulation box. Previous experience with video games and/or female gender appear to be associated with improved performance.

Background: Ovotesticular disorder of sex development (OT-DSD) (true hermaphroditism) is an anatomopathological diagnosis based on the findings of testicular and ovarian tissues in the same subject, in the same gonad (ovotestis), or in separate gonads. OT-DSD is a rare cause of sex ambiguity, and the most common karyotype is 46,XX; mosaics and chimeras are found only in 10%–20%. Aim: To report a case of an OT-DSD patient with a rare karyotype constitution. Case report: A 2-month-old child with male sex assignment was referred to our clinic for investigation of sex ambiguity. He was the second child of healthy unrelated parents; pregnancy and labor were uneventful. On physical examination, he had a 2.3-cm phallus and perineal hypospadias (Prader grade III); the right gonad was in the labioscrotal fold and the left was found in the inguinal channel. Karyotype was 46,XX/47,XXY/48,XXYY. Anatomopathological examination of gonads revealed right testis and left ovotestis. The male sex assignment was maintained; the child underwent left gonadectomy, removal of Mullerian structures and urethroplasty. Conclusion: A thorough revision of literature revealed a single case of OT-DSD with the same chromosome constitution. Gonadal biopsy is necessary to establish diagnosis in cases of sex chromosome mosaicism.

We report the case of a 46,XX infant referred at 3 months of age for evaluation of ambiguous genitalia with no palpable gonads. The phallus was replaced by a homogeneous peduncular structure covered by skin, and a second peduncular structure covered by mucosa was located between the labia minora above the urethral meatus and the vaginal opening. There were no associated anomalies, and she had normal growth and neuromotor development. During surgery to remove these structures, a bifid clitoris was detected and cystoscopy and vaginoscopy revealed normal female urethra and vagina. The structure located on the phallic region was a superficial lipomatous nevus, and the other mass was a congenital urethral caruncle with ectopic migration/differentiation of colonic epithelium into ventral and anterior part of the urogenital sinus. Both genital lipomas and congenital caruncles of the female urethra are very rare and their etiology is unknown. There is no previous report of coincidence of these two rare findings in an otherwise healthy girl.

Correction of gastroschisis may be accomplished by either primary or staged closure or even delayed primary closure after the use of a preformed silo. However, there is neither a consensus on the best approach nor established criteria to favor one method over the other. The aim of this paper was to investigate the role of intravesical pressure (IVP) as a tool to prevent abdominal compartment syndrome in newborns undergoing correction of abdominal wall defects.We retrospectively analyzed 45 newborns with gastroschisis in whom trans-operative intravesical pressure was used to choose between primary or staged closure. A threshold of 20 cm H(2)O was used and the outcomes between the two methods were compared.In 24 children delayed primary closure was achieved while the remaining 21 underwent staged reduction and closure. There was no difference in the frequency of complications, time to begin oral feeding, length of parenteral nutrition or length of hospital stay between the children of the two groups. The incidence of temporary oliguria or anuria, averaged 33% and it was similar in both groups of children.The data here presented suggests that monitoring intraoperative IVP during correction of gastroschisis may help to select children in whom staged closure is necessary, keeping their complication rate and overall outcome similar to that of children undergoing delayed primary closure. Further prospective studies should investigate more deeply the correlation between type of closure and the development of a compartment syndrome.

To report a series of children with pelviureteric junction obstruction (PUJO) due to lower polar crossing vessels who underwent laparoscopic vascular transposition. In order to confirm the relief of the obstruction and avoid unnecessary additional procedures, we suggest performing an intraoperative measure of the ureteral opening pressure.From January 2007 and January 2014, 11 children underwent laparoscopy to treat well-documented PUJO by polar vessels. In the first 7 cases, children underwent a careful dissection of the polar vessels that were transposed cranially in the pelvis. In the last 4 cases, a percutaneous needle was inserted into the renal pelvis, and the ureteral opening pressure was obtained intraoperatively, before and after the vascular hitch procedure, in 3 cases. No vascular relocation was necessary except in 1 case with a polar vessel unrelated to the obstruction.The laparoscopic procedure was feasible in all cases. Median operative time was 90 minutes without intraoperative complications. In the last 3 cases, a decrease in the renal pelvic pressure was demonstrated just after releasing the ureter from the polar vessels, confirming the extrinsic obstruction. In 1 case, the intraoperative pelvic pressure measurement showed that there was no vascular compression but that obstruction was due to renal rotation. During follow-up (range, 12-96 months) all patients reported resolution of their symptoms, nine children showed a decrease in the hydronephrosis grade, and all but one with poor function had improved drainage on diuretic renography.Intraoperative measurement of ureteral opening pressure may help to confirm that the vascular hitch procedure has relieved the pelvic obstruction, precluding the need for dismembered procedures. We believe that in some doubtful cases, with the addition of intraoperative pelvic pressure measurement, vascular hitch may be considered a safe procedure to treat selected cases of PUJO in children.

Background/Purpose Stenosis of the vaginal introitus is the most frequent complication after genital reconstruction for ambiguous genitalia associated with congenital adrenal hyperplasia (CAH). With the aim of enlarging the vaginal introitus, the authors present a technical modification of the introitoplasty that uses a bilateral cutaneous island flap based on the perineal superficial branches of the internal pudendal artery. Methods Eleven girls with CAH and Prader III to V genital ambiguity were included. Feminizing genitoplasty was performed in 1 stage. Bilateral cutaneous labioescrotal island flaps, based on the posterior labial artery, were included in the introitoplasty. The cosmetic results of the genitoplasty were evaluated by photographic analysis of the external genitalia. Results Integrity of the vaginal introitus as well as excellent integration of the flap and absence of additional scars in the donor area were assessed in all girls. Conclusions This modified island flap is technically feasible and reproducible producing no additional sequels in the donor area. It uses perineal skin that is usually excised in other techniques avoiding the use of harvesting skin from adjacent areas. Thus, it can be a useful additional procedure in the introitoplasty in association with the currently used techniques.

Background Congenital perineal lipoma is extremely rare and may lead to a misdiagnosis of ambiguous genitalia. Case reports We report on two girls referred to our service for ambiguous genitalia. Patient 1 (17 days old) and patient 2 (2 months old) had unremarkable gestational and perinatal histories. Both had normal female external genitalia and a 46,XX karyotype. Patient 1 had a polypoid, protruding 3.0 x 2.0 x 1.5-cm phallic-like mass arising at the inferior border of the left labium majora, and patient 2 had a similar mass of 1.5 x 1.5 x 1.0 cm at the same site and an imperforate anus. In both cases the mass was removed and found to be a lipoma. Discussion To our knowledge, perineal lipoma has been reported only in eleven girls, nine of them with associated anorectal malformation. Migration and fusion of the labioscrotal folds and formation of the urorectal septum are simultaneous developmental events occurring in the same region, which may explain the association of perineal lipoma and anorectal malformations.

OBJECTIVE: Report the results of laparoscopic upper-pole nephroureterectomy in infants. MATERIALS AND METHODS: Six consecutive infants underwent 7 laparoscopic upper-pole nephroureterectomy. Pre and postoperative evaluation included renal sonography, voiding cystourethrogram and renal scintigraphy. All infants showed upper-pole exclusion. Surgery was performed through a transperitoneal approach with full flank position in all infants. Three or 4 ports were used according to the necessity of retracting the liver. The distal ureter was ligated close to the bladder whenever reflux was present and the dysplastic upper-pole was divided with the help of an electrocautery. Data regarding operative time, postoperative use of analgesics, time to resume oral feeding, hospital stay and tubular function were collected and analyzed. RESULTS: All procedures were concluded as planned. Mean operative time was 135 min. One patient underwent staged bilateral upper-pole nephrectomy. There were no complications and the postoperative hospital stay was 48 hours in 5 procedures and 24 hours in 2 procedures. Pain medication was required only in the first day. Renal tubular function showed improvement in half of the cases. CONCLUSION: Laparoscopic partial nephrectomy is a safe and feasible procedure in infants. Due to the magnification provided by the lenses, a better vision of the structures is achieved, facilitating selective dissection of vascular upper-pole, renal parenchyma and distal ureter. This approach is less damaging to the lower pole, and is associated to low morbidity and a short hospital stay.

To evaluate the anatomical and cosmetic results of feminizing genitoplasty in girls with ambiguous genitalia due to Congenital Adrenal Hyperplasia (CAH).Twenty-seven females with genital ambiguity (Prader III-V) due to CAH underwent an one-stage feminizing genitoplasty and were followed for a mean time of 4 years. In twelve girls, two cutaneous labioscrotal island-flaps were associated with the conventional genitoplasty, to decrease the excess of labia majora and to enlarge the vaginal introitus. Urinary tract infections were observed in sixteen girls in the preoperative and in only seven in the postoperative period. The cosmetic results were obtained by the systematic anatomical evaluation of the different structures that compose the genitalia.The results of clitoroplasty were considered good in 63% of the cases; however, 4 girls developed clitoral atrophy. An excess of labia majora was present in 25% and persistence of the urogenital sinus occurred in 11% (three children without labioscrotal island flap). The labioscrotal island flap was considered technically feasible and reproducible. Five girls had to be re-operated. The cosmetic results were considered excellent or good in 63% of the cases, satisfactory in 18.5% and unsatisfactory or bad in 18.5%.The anatomical and cosmetic results were good in majority of the cases; however, some technical aspects should be adjusted, to avoid irreversible sequels and re-operations.

Prenatal diagnosis of myelomeningocele (MM) allows planning its management and, recently, a possible in utero repair.To describe the perinatal outcome of fetuses with MM, in a Fetal Medicine Unit, identifying possible candidates for the in utero surgical repair.Retrospective and descriptive study of 58 cases of prenatally diagnosed MM, at CAISM-UNICAMP, from January 1997 to December 2001, identifying possible fetal candidates for in utero repair.the diagnosis mean gestational age was 29 weeks (17-39); level of lesions was above sacral region in 85%, association with hydrocephaly in 86%. Surgical complications were present in 39% of the neonates. During follow-up, 98% presented neurogenic bladder and 60% neurological/mental handicap. Twenty eight fetuses (42%) could have indication of in utero repair.MM is associated with severe and frequent poor results. Almost one third of our cases could had fetal repair as a treatment choice.

Deficiency of the enzyme P450 oxidoreductase is a rare form of congenital adrenal hyperplasia with characteristics of combined and partial impairments in steroidogenic enzyme activities, as P450 oxidoreductase transfers electrons to CYP21A2, CYP17A1, and CYP19A1. It results in disorders of sex development and skeletal malformations similar to Antley-Bixley syndrome. We report the case of a 9-year-old girl who was born with virilized genitalia (Prader stage V), absence of palpable gonads, 46,XX karyotype, and hypergonadotropic hypogonadism. During the first year of life, ovarian cyst, partial adrenal insufficiency, and osteoarticular changes, such as mild craniosynostosis, carpal and tarsal synostosis, and limited forearm pronosupination were observed. Her mother presented severe virilization during pregnancy. The molecular analysis of P450 oxidoreductase gene revealed compound heterozygosis for the nonsense p.Arg223*, and the novel missense p.Met408Lys, inherited from the father and the mother, respectively. Arq Bras Endocrinol Metab. 2012;56(8):578-85 A deficiência da enzima P450 oxidorredutase é uma forma rara de hiperplasia congênita da adrenal com características de inibição combinada e parcial de enzimas esteroidogênicas, pois a enzima P450 oxidorredutase participa da transferência de elétrons para as enzimas CYP21A2, CYP17A1 e CYP19A1. Essa deficiência causa um distúrbio do desenvolvimento do sexo e alterações esqueléticas semelhantes às da síndrome de Antley-Bixley. Relatamos o caso de uma menina, atualmente com 9 anos de idade, que apresentava ao nascimento genitais virilizados (Prader 5) sem gônadas palpáveis, com cariótipo 46,XX e hipogonadismo hipergonadotrófico. No primeiro ano de vida, foram observados cisto ovariano, insuficiência adrenal parcial e alterações osteoarticulares como leve craniossinostose, sinostose carpal e tarsal e limitação de pronossupinação dos membros superiores. Sua mãe apresentou intensa virilização durante a gestação. O estudo molecular do gene P450 oxidorredutase revelou a heterozigose composta das mutações nonsense p.Arg223* e da missense nova p.Met408Lys, herdadas do pai e da mãe, respectivamente. Arq Bras Endocrinol Metab. 2012;56(8):578-85