Alejandra Vilanova Sánchez’s research while affiliated with Nationwide Children's Hospital and other places

Introduction Reconstructive techniques for cloaca, anorectal malformations (ARM), and Hirschsprung disease (HD) may require intestinal flaps on vascular pedicles for vaginal reconstruction and/or colonic pull-throughs. Visual assessment of tissue perfusion is typically the only modality used. We investigated the utility of intraoperative indocyanine green fluorescence angiography (ICG-FA) and hypothesized that it would be more accurate than the surgeon's eye. Materials and Methods Thirteen consecutive patients undergoing cloacal reconstruction (9), HD (3), and ARM repair (1) underwent ICG-FA laser SPY imaging to assess colonic, rectal, vaginal, and neovaginal tissue perfusion following intraoperative visual clinical assessment. Operative findings were correlated with healing at 6 weeks, 3 months, and 1 year postoperatively. Results ICG-FA resulted in a change in the operative plan in 4 of the 13 (31%) cases. In three cases, ICG-FA resulted in the distal bowel being transected at a level (>10 cm) higher than originally planned, and in one case the distal bowel was discarded, and the colostomy used for pull-through. Conclusion ICG-FA correctly identified patients who might have developed a complication from poor tissue perfusion. Employing this technology to assess rectal or neovaginal pull-throughs in cloacal reconstructions, complex HD, and ARM cases may be a valuable technology.

Introduction: Gastrointestinal (GI) operations represent a significant proportion of the surgical site infection (SSI) burden in pediatric patients, resulting in significant morbidity. We have previously demonstrated that a GI bundle decreases SSI rates, length of stay (LOS), and hospital charges. Following this success, we hypothesized that by targeting the preoperative antibiotics for stoma closures based on organisms found in infected wounds, we could further decrease SSI rates. Methods: As part of a broad quality improvement effort to reduce SSI rates, we reviewed the responsible pathogens and their sensitivities as well as the preoperative antibiotic used, and found that 15% of wound infections were caused by enterococcus. Based on this information, starting in April 2017, we changed the prior preoperative antibiotic cefoxitin to ampicillin-sulbactam, which more accurately targeted the prevalent pathogens from April 2017 to October 2018. Results: The baseline SSI rate for all stoma takedown patients was 21.4% (25 of 119). After bundle implementation, this decreased to 7.9% (17 of 221; p = 0.03) over a period of 2.5 years. Then, after changing the preoperative antibiotics, our rate of SSI decreased further to 2.2% (1 of 44; p = 0.039) over a period of 1.5 years. Conclusion: Significant reduction of SSI in GI surgery can be accomplished with several prevention strategies (our GI bundle). Then a change of the preoperative antibiotic choice, chosen based on causative wound infection organisms, may further decrease SSI rates. We recommend an institution specific analysis of wound infections and modification of preoperative antibiotics if the responsible organisms are resistant to the original antibiotic choice. Type of study: Retrospective cohort study. Level of evidence: Level III.

Introduction Total colonic Hirschsprung's disease (TCHD) presents a postoperative challenge due to multiple stools and perineal rash. We propose a protocol developed by pediatric surgeons and ostomy nurses to help prevent and treat hypermotility and severe perineal rash, especially in younger children who are not toilet trained. Materials and Methods We retrospectively reviewed our TCHD patients' charts from 2014 to 2017. All patients received a prescribed protocol for the treatment of hypermotility and perineal rash. We describe patients who underwent their pull through before and after the age of urine toilet training, and assessed the number of bowel movements, the perineal skin status, and growth. Results We treated 25 patients. Out of 25, 9 patients received a straight ileoanal pull through before the age of 18 months. Nine of 25 patients presented for a second opinion and had redo pull through. The remaining seven presented for bowel management after having a pull through at another institution. All these were treated following the hypermotility protocol. In total, 19 of 25 patients were not toilet trained. The mean number of bowel movements in all groups was 4 (3–5). All had a resolution of perineal rash and liquid stools after 3 months. Eleven of the 25 patients presented with failure to thrive. Two older patients experienced severe proctalgia requiring replacement of the ileostomy. Conclusion TCHD patients who underwent definitive pull through had nine high incidence of multiple stool, perineal rash, and low growth. With the implementation of bowel management care to slow the stools and a perineal skin protocol to treat the skin, we believe that these symptoms can be minimized even in patients who are not toilet trained. Since the implementation of this protocol, we have changed our practice to perform the pull through in such patients between the age of 6 and 18 months.

Introduction There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity. Methods Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for outcome variable: length-of-stay (LOS). Results Note that 21/93 (22.6%) patients with Hirschsprung's disease presented 50 HAEC episodes with a median of 2 (1–5) episodes during an 8.3-year (2–15.6) follow-up. The most common symptoms were foul-smelling (86% [43/50]) and explosive (60% [30/50]) diarrhea. Physical findings showed a distended abdomen (76% [38/50]) and fever (60% [30/50]) with dilated bowel (82% [41/50]) and rectosigmoid cut-off (80% [40/50]) identified on X-rays. Only 34% (17/50) showed abnormal laboratory findings. Patients were admitted with a median LOS of 7 days (1–28). A HAEC score of 9 (2–16) was found, and only 50% (25/50) of patients met the initial criteria (score of 10 points). However, the use of a 4-point cut-off would have allowed the diagnosis of 98% (49/50) of the patients. A positive linear correlation was found for LOS and HAEC score (r = 0.3, p = 0.014). Conclusion There is no standardized definition for HAEC. The initial HAEC score cut-off is restrictive and might fail to identify milder episodes. The positive correlation with LOS and thus HAEC severity might aid in patient information and anticipation of treatment.

Introduction Acquired skin-level strictures following posterior sagittal anorectoplasty (PSARP) and some rare cases of congenital anal stenosis can be managed using a Heineke–Mikulicz like anoplasty (HMA). We hypothesized that this procedure was an effective, safe, and durable outpatient procedure in select patients. Methods We retrospectively reviewed all patients who underwent HMA for skin level strictures following PSARP or for certain congenital anal stenoses from 2014 to 2017. Results Twenty-eight patients (19 males, 9 females) with a mean age of 5.8 years (range 0.5–24.4) underwent HMA. Twenty-six had a prior PSARP, of which 18 were redo, and 8 were primary procedures. Two patients had congenital skin level anal stenosis. The mean follow up was 1.0 years (range 0.4–2.9). The average preprocedure anal size was Hegar 8, which after HMA increased 8 Hegar sizes to 16 (95% CI 7–9, p < 0.001). There were no operative complications. One patient restenosed and required a secondary procedure. Conclusion HMA is a safe procedure for skin-level anal strictures following PSARP (primary and redo) and can also be used in some rare cases of congenital anal stenosis. Long-term follow up to determine the restricture rate is ongoing. A plan to do an HMA if a stricture develops may offer an alternative to routine anal dilations, particularly after a redo PSARP in an older child. Type of study Case series. Level of evidence Level IV.

Introduction Previous research in children with Hirschsprung's disease (HD) and Down's syndrome (DS) has focused on colorectal outcomes. We set out to review urinary outcomes in this patient group. Methods The medical records of all patients aged five years and older with HD were reviewed, and patients and caregivers filled out the Vancouver Symptom Score at intake, which is designed and validated to diagnose dysfunctional elimination syndrome. Results A total of 104 patients with HD were included in this study. Of these, 16 (15%) patients had DS. There were no significant differences in the prevalence of enterocolitis or colorectal symptoms between patients with or without DS. Five of 88 (6%) patients without DS and 7 of 16 (44%) (p = 0.00001) with DS reported having urinary accidents. Patients with HD and DS scored higher on the Vancouver score (9 vs. 17.5; p = 0.007), indicating more severe urinary symptoms. Patients who also reported fecal accidents scored significantly higher on the Vancouver (12 vs. 9; n = 61; p = 0.016), indicating more problems. Conclusion Patients with DS appear to be a unique subset of HD patients who have a higher prevalence of urinary symptoms after surgery. In the postoperative care of patients with HD and DS, a strong focus should be placed on postoperative urinary care in addition to their bowel care. This could significantly ease care and contribute to the quality of life of the parents and the patient.

Introduction and objectives Although standard surgical treatment of a testicular tumour is orchiectomy, use can be made of testis-sparing surgery in selected cases, based on tumour markers, tumour size, and histopathological findings. Our objective is to become acquainted with the indications of testis-sparing surgery as a treatment for the incidental finding of a palpable and non-palpable testicular mass. Material and methods A retrospective study was conducted on 22 patients younger than 18 years diagnosed with a testicular tumour between 2000 and 2014. An assessment was made of the condition, the history, ultrasound, histopathology, tumour markers (BHCG, AFP), therapeutic approach, and outcome. Results Of the 22 patients (10 prepubertal age) studied, 82% had palpable mass, and 18% were incidental findings. Two had cryptorchidism. The BHCG was increased in 27% and AFP in 45% of cases. There were 18 tumorectomies and 4 orchiectomies performed. The histopathology found 72% germ cell, 14 orchiectomy, and 2 tumorectomies (2 teratomas), with 27% non-germ cell tumours in 4 orchiectomies and 2 tumorectomies (2 cells of Leydig). Six patients received post-surgical chemotherapy (mixed tumours). The median tumour size was 1 (0.4–1.5) cm in tumorectomies, and 2.5 (0.5–14) cm in orchiectomies. The mean follow-up was 5 (1–15) years. One patient died due to metastatic disease. There was no local recurrence in the follow up of the tumorectomies. Conclusions A change in the trend of our therapeutic approach is demonstrated. We propose that testis-sparing surgery is indicated in prepubertal patients who meet the benignity criteria of the testicular mass (small size and negative tumour markers).

Introduction Repair of anorectal malformations (ARMs), primarily or with a reoperation, may be performed in certain circumstances without a diverting stoma. Postoperatively, the passage of bulky stool can cause wound dehiscence and anastomotic disruption. To avoid this, some surgeons keep patients NPO (nothing by mouth) for a prolonged period. Here, we report the results of a change to our routine from NPO for 7 days to clear fluids or breast milk. Methods After primary or redo ARM surgery, patients given clear liquids were compared to those who were kept strictly NPO. Age, indication for surgery, incision type, use of a peripherally inserted central catheter (PICC) line, and wound complications were recorded. Results There were 52 patients, including 15 primary and 37 redo cases. Group 1 comprised 11 female and 15 male patients. The mean age at surgery was 4.9 years (standard deviation [SD]: 2.3). There were 8 primary cases and 18 redo cases. Twelve (46.6%) received a PICC line. The average start of clear liquids was on day 5.3 (SD: 2.2) after examination of the wound, and the diet advanced as tolerated. The first stool passage was recorded on average on day 2.3 (SD: 1.3). Four minor wound complications and no major wound complications occurred. Group 2 comprised 14 females and 12 male patients. The mean age at surgery was 3.5 (SD: 2.4) years. There were 7 primary and 19 redo cases. One (3.8%) patient required a PICC line. A clear liquid diet was started within 24 hours after surgery. A regular diet was started on average on day 5.8 (SD: 1.3). The first stool passage was recorded on an average of day 1.6 (SD: 0.9). Three minor wound complications occurred; however, there was no significant difference between the two groups (SD: 0.71). One major wound complication occurred. However, there was no significant difference in major wound complications between the groups (SD: 0.33). Conclusion No increase in wound problems was noted in children receiving clear liquids or breast milk compared with the strict NPO group, and PICC line use was reduced. We believe this change in practice simplifies postoperative care without increasing the risk of wound complications.

Introduction and objectives: Although standard surgical treatment of a testicular tumour is orchiectomy, use can be made of testis-sparing surgery in selected cases, based on tumour markers, tumour size, and histopathological findings. Our objective is to become acquainted with the indications of testis-sparing surgery as a treatment for the incidental finding of a palpable and non-palpable testicular mass. Material and methods: A retrospective study was conducted on 22 patients younger than 18 years diagnosed with a testicular tumour between 2000 and 2014. An assessment was made of the condition, the history, ultrasound, histopathology, tumour markers (BHCG, AFP), therapeutic approach, and outcome. Results: Of the 22 patients (10 prepubertal age) studied, 82% had palpable mass, and 18% were incidental findings. Two had cryptorchidism. The BHCG was increased in 27% and AFP in 45% of cases. There were 18 tumorectomies and 4 orchiectomies performed. The histopathology found 72% germ cell, 14 orchiectomy, and 2 tumorectomies (2 teratomas), with 27% non-germ cell tumours in 4 orchiectomies and 2 tumorectomies (2 cells of Leydig). Six patients received post-surgical chemotherapy (mixed tumours). The median tumour size was 1 (0.4-1.5) cm in tumorectomies, and 2.5 (0.5-14) cm in orchiectomies. The mean follow-up was 5 (1-15) years. One patient died due to metastatic disease. There was no local recurrence in the follow up of the tumorectomies. Conclusions: A change in the trend of our therapeutic approach is demonstrated. We propose that testis-sparing surgery is indicated in prepubertal patients who meet the benignity criteria of the testicular mass (small size and negative tumour markers).