Third Time's the Charm? Indications and Outcomes for Redo-Redo IPAA.

BACKGROUND: Up to 20% to 40% cases of redo IPAA procedures will result in pouch failure. Whether to offer a second redo procedure to maintain intestinal continuity remains a controversial decision. OBJECTIVE: To report our institutional experience of second redo IPAA procedures. DESIGN: This was a retrospective review. Patient-reported outcomes were compared between patients undergoing second redo procedures and those undergoing first redo procedures using propensity score matching to balance the 2 cohorts. SETTINGS: Tertiary referral center. PATIENTS: Patients who underwent second redo IPAA procedures between 2004 and 2021 were included in this study. INTERVENTIONS: Second redo IPAA. MAIN OUTCOME MEASURES: Pouch survival and patient-reported outcomes were measured using the Cleveland Global Quality of Life survey. RESULTS: Twenty-three patients were included (65% women), 20 (87%) with an index diagnosis of ulcerative colitis and 3 (13%) with indeterminate colitis. The final diagnosis was changed to Crohn's disease in 8 (35%) cases. The indication for pouch salvage was the same for the first and second redo procedures in 21 (91%) cases: 20 (87%) patients had both redo IPAAs for septic complications. After a median follow-up of 39 months (interquartile range, 18.5-95.5 months), pouch failure occurred in 8 (30%) cases (7 cases due to sepsis, of whom 3 never had their stoma closed, and 1 case due to poor function); all patients who experienced pouch failure underwent the second redo procedure due to septic complications. Overall pouch survival at 3 years was 76%: 62.5% in patients with a final diagnosis of Crohn's disease versus 82.5% in patients with ulcerative/indeterminate colitis ( p = 0.09). Overall quality-of-life score (0-1) was 0.6 (0.5-0.8). Quality of life and functional outcomes were comparable between first and second redo procedures, except incontinence, which was higher in second redo procedures. LIMITATIONS: Single-center retrospective review. CONCLUSIONS: A second pouch salvage procedure may be offered with acceptable outcomes to selected patients with high motivation to keep intestinal continuity. See Video Abstract . LA TERCERA ES LA VENCIDA INDICACIONES Y RESULTADOS DE LA RERECONFECCION DE LA ANASTOMOSIS ANAL CON BOLSA ILEAL: ANTECEDENTES:Hasta un 20-40% de los casos de rehacer anastomosis anal con bolsa ileal (IPAA) resultarán en falla de la bolsa. La posibilidad de ofrecer un segundo procedimiento para mantener la continuidad intestinal sigue siendo una decisión controvertida.OBJETIVO:Reportar nuestra experiencia institucional de una segunda re-confección de la anastomosis anal con bolsa ileal.DISEÑO:Revisión retrospectiva; los resultados informados por los pacientes se compararon entre los pacientes que se sometieron a una segunda re-confeccion con los de los pacientes que se sometieron a una la primera re-confeccion utilizando el puntaje de propensión para equilibrar las dos cohortes.AJUSTES ENTORNO CLINICO:Centro de referencia terciario.PACIENTES:Pacientes que se sometieron a una segunda re-confeccion de de la anastomosis anal con bolsa ileal entre 2004 y 2021.INTERVENCIONES:Segunda re-confeccion de la anastomosis anal con bolsa ileal.PRINCIPALES MEDIDAS DE RESULTADO:Supervivencia de la bolsa, resultados informados por los pacientes medidos mediante la encuesta Cleveland Global Quality of Life.RESULTADOS:Se incluyeron veintitrés pacientes (65% mujeres), 20 (87%) con diagnóstico inicial de colitis ulcerosa y 3 (13%) con colitis indeterminada. El diagnóstico final se cambió a enfermedad de Crohn en ocho (35%) casos. La indicación para el rescate de la bolsa fue la misma para la primera y segunda re-confeccion en 21 (91%) casos: 20 (87%) pacientes tuvieron ambas re-confecciones de la anastomosis anal con bolsa ileal por complicaciones sépticas. Después de una mediana de seguimiento de 39 meses (RIC 18,5 - 95,5), se produjo falla de la bolsa en 8 (30%) casos (7 casos debido a sepsis, de los cuales 3 nunca cerraron el estoma y 1 caso debido a una mala función); todos los pacientes que experimentaron falla de la bolsa se sometieron a una segunda re-confeccion debido a complicaciones sépticas. La supervivencia global de la bolsa a los 3 años fue del 76%: 62,5% en pacientes con diagnóstico final de enfermedad de Crohn, versus 82,5% en colitis ulcerativa/indeterminada ( p = 0,09). La puntuación general de calidad de vida (0 -1) fue 0,6 (0,5 - 0,8). La calidad de vida y los resultados funcionales fueron comparables entre la primera y la segunda re-confeccion, excepto la incontinencia, que fue mayor en la segunda re-confeccion.LIMITACIONES:Revisión retrospectiva de un solo centro.CONCLUSIONES:Se puede ofrecer un segundo procedimiento de rescate de la bolsa con resultados aceptables a pacientes seleccionados con alta motivación para mantener la continuidad intestinal. (Traducción- Dr. Francisco M. Abarca-Rendon ).

Tofacitinib Is Associated With Increased Risk of Postoperative Venous Thromboembolism in Patients With Ulcerative Colitis.

BACKGROUND: In 2019, the Food and Drug Administration issued a black box warning for increased risk of venous thromboembolism in patients with rheumatoid arthritis exposed to tofacitinib. There are limited data regarding postoperative venous thromboembolism risk in patients with ulcerative colitis exposed to tofacitinib. OBJECTIVE: To assess whether preoperative exposure to tofacitinib is associated with increased odds of postoperative venous thromboembolism. DESIGN: Retrospective review. SETTINGS: Tertiary academic medical center. PATIENTS: Consecutive patients exposed to tofacitinib within 4 weeks before total abdominal colectomy or total proctocolectomy, with or without ileostomy, from 2014 to 2021, matched 1:2 for tofacitinib exposure or no exposure. INTERVENTION: Tofacitinib exposure versus no exposure. MAIN OUTCOME MEASURES: Ninety-day postoperative venous thromboembolism rate. RESULTS: Forty-two patients with tofacitinib exposure and 84 case-matched patients without tofacitinib exposure underwent surgery for medically refractory ulcerative colitis. Nine (22.0%) tofacitinib-exposed patients and 7 (8.5%) unexposed patients were diagnosed with venous thromboembolism within 90 days of surgery. In univariate logistic regression, patients exposed to tofacitinib had 3.01 times increased odds of developing venous thromboembolism within 90 days after surgery compared to unexposed patients ( p = 0.04; 95% CI, 1.03-8.79). Other venous thromboembolism risk factors were not significantly associated with venous thromboembolisms. Venous thromboembolisms in both groups were most commonly portomesenteric vein thromboses (66.7% in the tofacitinib-exposed group and 42.9% in the unexposed group) and were diagnosed at a mean of 23.2 days (range, 3-90 days) postoperatively in the tofacitinib-exposed group and 7.9 days (1-19 days) in the unexposed group. There were no statistically significant differences in location or timing between the 2 groups. LIMITATIONS: Retrospective nature of the study and associated biases. Reliance on clinically diagnosed venous thromboembolisms may underreport the true incidence rate. CONCLUSIONS: Tofacitinib exposure before surgery for medically refractory ulcerative colitis is associated with 3 times increased odds of venous thromboembolism compared with patients without tofacitinib exposure. See Video Abstract . TOFACITINIB SE ASOCIA CON UN MAYOR RIESGO DE TROMBOEMBOLISMO VENOSO POSTOPERATORIO EN PACIENTES CON COLITIS ULCEROSA: ANTECEDENTES:En 2019, la FDA emitió una advertencia de recuadro negro sobre un mayor riesgo de tromboembolismo venoso en pacientes con artritis reumatoide expuestos a tofacitinib. Hay datos limitados sobre el riesgo de tromboembolismo venoso postoperatorio en pacientes con colitis ulcerosa expuestos a tofacitinib.OBJETIVO:Evaluar si la exposición preoperatoria a tofacitinib se asocia con mayores probabilidades de tromboembolismo venoso postoperatorio.DISEÑO:Revisión retrospectiva.LUGARES:Centro médico académico terciario.PACIENTES:Pacientes consecutivos expuestos a tofacitinib dentro de las 4 semanas previas a la colectomía abdominal total o proctocolectomía total, con o sin ileostomía, entre 2014 y 2021, emparejados 1:2 para exposición a tofacitinib o ninguna exposición.INTERVENCIÓN(S):Exposición a tofacitinib versus ninguna exposición.PRINCIPALES MEDIDAS DE RESULTADO:Tasa de tromboembolismo venoso posoperatorio a los 90 días.RESULTADOS:Cuarenta y dos pacientes con exposición a tofacitinib y 84 pacientes de casos similares sin exposición a tofacitinib se sometieron a cirugía por colitis ulcerosa médicamente refractaria. Nueve (22,0%) pacientes expuestos a tofacitinib y 7 (8,5%) pacientes no expuestos fueron diagnosticados con tromboembolismo venoso dentro de los 90 días posteriores a la cirugía. En la regresión logística univariada, los pacientes expuestos a tofacitinib tuvieron 3,01 veces más probabilidades de desarrollar un tromboembolismo venoso dentro de los 90 días posteriores a la cirugía en comparación con los no expuestos ( p = 0,04, IC del 95 %: 1,03-8,79). Otros factores de riesgo de tromboembolismo venoso no se asociaron significativamente con el tromboembolismo venoso. Los tromboembolismos venosos en ambos grupos fueron más comúnmente trombosis de la vena portomesentérica (66,7% en los expuestos a tofacitinib y 42,9% en los no expuestos) y se diagnosticaron en una media de 23,2 días (rango, 3-90 días) después de la operación en los expuestos a tofacitinib y 7,9 días. (1-19 días) en los grupos no expuestos, respectivamente. No hubo diferencias estadísticamente significativas en la ubicación o el momento entre los dos grupos.LIMITACIONES:Carácter retrospectivo del estudio y sesgos asociados. La dependencia de tromboembolismos venosos diagnosticados clínicamente puede subestimar la tasa de incidencia real.CONCLUSIONES:La exposición a tofacitinib antes de la cirugía para la colitis ulcerosa médicamente refractaria se asocia con probabilidades 3 veces mayores de tromboembolismo venoso en comparación con los pacientes sin exposición a tofacitinib. (Traducción-Dr. Mauricio Santamaria ).

Segmental Colectomy in Ulcerative Colitis.

BACKGROUND: Segmental colectomy in ulcerative colitis is performed in select patients who may be at increased risk for postoperative morbidity. OBJECTIVE: To identify ulcerative colitis patients who underwent segmental colectomy and assess their postoperative and long-term outcomes. DESIGN: Retrospective case series. SETTING: A tertiary-care inflammatory bowel disease center. PATIENTS: Ulcerative colitis patients who underwent surgery between 1995 and 2022. INTERVENTION: Segmental colectomy. MAIN OUTCOME MEASURES: Postoperative complications, early and late colitis, metachronous cancer development, completion proctocolectomy-free survival rates and stoma at follow-up. RESULTS: Fifty-five patients were included [20 (36.4%) female; 67.8 (57.4-77.1) years of age at surgery; body mass index 27.7 (24.2-31.1) kg/m2; median follow-up 37.3 months]. ASA score was III in 32 (58.2%) patients, 48 (87.3%) had at least one comorbidity, 48 (87.3%) had Mayo endoscopic subscore of 0-1. Patients underwent right hemicolectomy (28, 50.9%), sigmoidectomy (17, 30.9%), left hemicolectomy (6, 10.9%), low anterior resection (2, 3.6%), or a non-anatomic resection (2, 3.6%) for; endoscopically unresectable polyps (21, 38.2%), colorectal cancer (15, 27.3%), symptomatic diverticular disease (13, 23.6%), and stricture (6, 10.9%). Postoperative complications occurred in 16 (29.1%) patients [7 (12.7%) Clavien-Dindo Class III-V]. Early and late postoperative colitis rates were 9.1% and 14.5%, respectively. Metachronous cancer developed in 1 patient. 4 (7.3%) patients underwent subsequent completion proctocolectomy with ileostomy. Six (10.9%) patients had stoma at the follow-up. Two and 5-year completion proctocolectomy-free survival rates were 91% and 88%, respectively. LIMITATIONS: Retrospective study, small sample size. CONCLUSIONS: Segmental colectomy in ulcerative colitis is associated with low postoperative complication rates, symptomatic early colitis and late colitis rates, metachronous cancer development and the need for subsequent completion proctocolectomy. Therefore, it can be safe to consider select patients, such as the elderly with quiescent colitis and other indications for colectomy. See Video Abstract.

American Board of Surgery In-Training Exam Performance Predicted by Question Bank Use While Unassociated With Other Learning Strategies.

INTRODUCTION: There is no consensus regarding optimal curricula to teach cognitive elements of general surgery. The American Board of Surgery In-Training Exam (ABSITE) aims to measure trainees' progress in attaining this knowledge. Resources like question banks (QBs), Surgical Council on Resident Education (SCORE) curriculum, and didactic conferences have mixed findings related to ABSITE performance and are often evaluated in isolation. This study characterized relationships between multiple learning methods and ABSITE performance to elucidate the relative educational value of learning strategies. METHODS: Use and score of QB, SCORE use, didactic conference attendance, and ABSITE percentile score were collected at an academic general surgery residency program from 2017 to 2022. QB data were available in the years 2017-2018 and 2021-2022 during institutional subscription to the same platform. Given differences in risk of qualifying exam failure, groups of ≤30th and >30th percentile were analyzed. Linear quantile mixed regressions and generalized linear mixed models determined factors associated with ABSITE performance. RESULTS: Linear quantile mixed regressions revealed a relationship between ABSITE performance and QB questions completed (1.5 percentile per 100 questions, P < 0.001) and QB score (1.2 percentile per 1% score, P < 0.001), but not with SCORE use and didactic attendance. Performers >30th percentile had a significantly higher QB score. CONCLUSIONS: Use and score of QB had a significant relationship with ABSITE performance, while SCORE use and didactic attendance did not. Performers >30th percentile completed a median 1094 QB questions annually with a score of 65%. Results emphasize success of QB use as an active learning strategy, while passive learning methods warrant further evaluation.

Understanding General Surgery Recruitment and Attrition During the COVID-Era: A 1-Year Follow-Up Study.

INTRODUCTION: The COVID-19 pandemic resulted in modifications to resident selection. The success of these new recruitment strategies as well as the impact on trainee attrition and competency is unknown. We previously evaluated how selection of general surgery applicants changed early in the pandemic. Here we supplement that work by reporting further modifications to the recruitment process and the perceived impact on resident attrition and competency. METHODS: An anonymous cross-sectional survey sent via the Association of Program Directors in Surgery listserv in June 2022 to programs directors (PDs) at Accreditation Council for Graduate Medical Education accredited general surgery programs. Surveys contained demographic questions, 5-point Likert scale questions evaluating factors related to recruitment and match process, and postgraduate year 1 performance. RESULTS: 60 PDs responded to the survey. PDs continue to value the same post-COVID factors related to determining a resident's commitment to surgery but began to shift back to nonvirtual based strategies to recruit applicants in this new interview cycle. PD commentary frequently noted desire to return to in-person interviewing. 5.4% of postgraduate year 1s comprising this first class of residents who underwent virtual-only interviews and rotations did not reach Accreditation Council for Graduate Medical Education level 1 milestones, similar to prior years. The attrition rate amongst this class increased from 1.3% to 2.7%. CONCLUSIONS: The attrition rate for postgraduate year 1 categorical general surgery residents has increased since the onset of the pandemic. The recruitment strategies adopted early in the pandemic have not maintained their initial perceived impact.

The mode of delivery does not influence the occurrence of post-partum perianal disease flares in patients with inflammatory bowel disease.

INTRODUCTION: Perianal disease occurs in up to 34% of inflammatory bowel disease (IBD) patients. An estimated 25% of women will become pregnant after the initial diagnosis, thus introducing the dilemma of whether mode of delivery affects perianal disease. The aim of our study was to analyze whether a cesarean section (C-section) or vaginal delivery influence perianal involvement. We hypothesized the delivery route would not alter post-partum perianal manifestations in the setting of previously healed perianal disease. METHODS: All consecutive eligible IBD female patients between 1997 and 2022 who delivered were included. Prior perianal involvement, perianal flare after delivery and delivery method were noted. RESULTS: We identified 190 patients with IBD who had a total of 322 deliveries; 169 (52%) were vaginal and 153 (48%) were by C-section. Nineteen women (10%) experienced 21/322 (6%) post-partum perianal flares. Independent predictors were previous abdominal surgery for IBD (OR, 2.7; 95% CI, 1-7.2; p = 0.042), ileocolonic involvement (OR, 3.3; 95% CI, 1.1-9.4; p = 0.030), previous perianal disease (OR, 22; 95% CI, 7-69; p < 0.001), active perianal disease (OR, 96; 95% CI, 21-446; p < 0.001) and biologic (OR, 4.4; 95% CI,1.4-13.6; p < 0.011) or antibiotic (OR, 19.6; 95% CI, 7-54; p < 0.001) treatment. Negative association was found for vaginal delivery (OR, 0.19; 95% CI, 0.06-0.61; p < 0.005). Number of post-partum flares was higher in the C-section group [17 (11%) vs. 4 (2%), p = 0.002]. CONCLUSIONS: Delivery by C-section section was not protective of ongoing perianal disease activity post-delivery, but should be recommended for women with active perianal involvement.

Outcome of incidental versus preoperatively diagnosed colorectal cancer during total proctocolectomy with ileal pouch-anal anastomosis for inflammatory bowel disease.

AIM: Total proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the treatment of choice for colorectal cancer (CRC) in inflammatory bowel disease. CRC may also be discovered incidentally at IPAA for other indications. We sought to determine whether incidentally found CRC at IPAA was associated with worse outcomes. METHODS: Our institutional pouch registry (1983-2021) was retrospectively reviewed. Patients with CRC at pathology after IPAA were divided into two groups: a preoperative diagnosis (PreD) group and an incidental diagnosis (InD) group. Their long-term outcomes (overall survival, disease-free survival and pouch survival) were compared. RESULTS: We included 164 patients: 53 (32%) InD and 111 (68%) PreD. There were no differences in cancer staging, differentiation and location. After a median follow-up of 11 (IQR 3-25) years for InD and 9 (IQR 3-20) years for the PreD group, deaths were 14 (26%) in the InD group and 18 (16%) in the PreD group. Pouch failures were five (9%) in the InD group and nine (8%) in the PreD group, of which two (5%) and four (4%) were cancer related. Ten-year overall survival was 94% for InD and 89% for PreD (P = 0.41), disease-free survival was 95% for InD and 90% for PreD (P = 0.685) and pouch survival was 89% for InD and 97% for PreD (P = 0.80). Pouch survival at 10 years was lower in rectal versus colon cancer (87% vs. 97%, P = 0.01). No difference was found in outcomes in handsewn versus stapled anastomoses. CONCLUSION: Inflammatory bowel disease patients with incidentally found CRC during IPAA appear to have similarly excellent oncological and pouch outcomes to patients with a preoperative cancer diagnosis.

Transanal ileal pouch-anal anastomosis for inflammatory bowel disease: a systematic review and meta-analysis of short-term outcomes.

AIM: Restorative proctocolectomy with transabdominal ileal pouch-anal anastomosis (abd-IPAA) has become the standard surgical treatment for medically refractory ulcerative colitis (UC). However, it requires a technically difficult distal anorectal dissection and anastomosis due to the bony confines of the deep pelvis. To address these challenges, the transanal IPAA approach (ta-IPAA) was developed. This novel approach may offer increased visibility and range of motion compared with abd-IPAA, although its postoperative benefits remain unclear. The aim of this work was to perform a systematic review and meta-analysis to compare and inform the frequency of postoperative outcomes between ta-IPAA and abd-IPAA for patients with UC. METHOD: Several databases were searched from inception until May 2022 for studies reporting postoperative outcomes of patients undergoing ta-IPAA. Reviewers, working independently and in duplicate, evaluated studies for inclusion and graded the risk of bias. Odds ratios (OR), mean differences (MD) and prevalence ratio (PR) and their corresponding 95% confidence intervals (CIs) were calculated using random-effects models. Sensitivity analysis was performed. RESULTS: Ten retrospective studies comprising 284 patients with ta-IPAA were included. Total mesorectal excision was performed in 61.8% of cases and close rectal dissection in 27.9%. There was no difference in the odds of Clavien-Dindo (CD) I-II complications, CD III-IV and anastomotic leak (OR 0.96, 95% CI 0.27-3.40; OR 1.18, 95% CI 0.65-2.16; OR 1.37, 95% CI 0.58-3.23; respectively) between ta-IPAA and abd-IPAA. The ta-IPAA pooled CD I-II complication rate was 18% (95% CI 5%-35%) and for CD III-IV 10% (95% CI 5%-17%), and the anastomotic leak rate was 6% (95% CI 2%-10%). There were no deaths reported. CONCLUSIONS: This meta-analysis compared the novel ta-IPAA procedure with abd-IPAA and found no difference in postoperative outcomes. While the need for randomized controlled trails and comparison of functional outcomes between both approaches remains, this evidence should assist colorectal surgeons to decide if ta-IPAA is a viable alternative.

Redo IPAA for long rectal cuff syndrome after ileoanal pouch for inflammatory bowel disease.

ABTRACT: BACKGROUND: When constructing an ileal pouch-anal anastomosis (IPAA), the rectal cuff should ideally be 1-2 cm long to avoid subsequent complications. METHODS: We identified patients from our IBD center who underwent redo IPAA for a long rectal cuff. Long rectal cuff syndrome (LRCS) was defined as a symptomatic rectal cuff ≥ 4 cm. RESULTS: Forty patients met the inclusion criteria: 42.5% female, median age at redo surgery 42.5 years. The presentation was ulcerative proctitis in 77.5% of the cases and outlet obstruction in 22.5%. The index pouch was laparoscopically performed in 18 patients (45%). The median rectal cuff length was 6 cm. The pouch was repaired in 16 (40%) cases, whereas 24 (60%) required the creation of a neo-pouch. At the final pathology, the rectal cuff showed chronic active colitis in 38 (90%) cases. After a median follow-up of 34.5 (IQR 12-109) months, pouch failure occurred in 9 (22.5%) cases. The pouch survival rate was 78% at 3 years. Data on the quality of life were available for 11 (27.5%) patients at a median of 75 months after redo surgery. The median QoL score (0-1) was 0.7 (0.4-0.9). CONCLUSION: LRCS, a potentially avoidable complication, presents uniformly with symptoms of ulcerative proctitis or stricture. Redo IPAA was restorative for the majority.

Effective Senior Surgical Residents as Defined by Their Peers: A Qualitative Content Analysis of Nontechnical Skills Development.

OBJECTIVE: This study aims to define an effective senior resident and understand the process of leadership and nontechnical skill development in the transition from junior to senior surgery resident. SUMMARY BACKGROUND: General surgery residents are responsible for patient care, technically demanding operations, and diverse care team management. However, leadership skill development for the transition from junior to senior resident roles is often overlooked. METHODS: We conducted 15 semi-structured focus groups with surgery residents from an urban, academic institution. Focus group transcripts were inductively coded. Using content analysis and constant comparative methodology, primary codes were refined into categories and organized into higher-level themes. RESULTS: Thirty-three general surgery residents completed fifteen focus groups. Six themes were identified. Three themes describe the process of becoming an effective senior resident: how to define a senior resident's scope of practice, the transition process, and the importance of personal investment. Three themes were identified regarding effective seniors: ideal traits, teachable skills, and the team and patient impact. CONCLUSIONS: Surgery residents define an effective senior resident as the team member with the highest level of experience who manages the big picture of patient care. The transition is improved by personal engagement and acknowledgement of the transition. Ideal traits of effective seniors, including emotional intelligence and inherent personality traits, allow a resident to more naturally assume this role; however, teachable skills, such as communication, expectation setting and competence, can be taught to improve one's effectiveness. The actions of a senior resident impact the team and patient care, underscoring the importance of understanding this role.

Long-term Outcomes of Perianal Fistulas in Pediatric Crohn's Disease.

BACKGROUND: Approximately 30% of Crohn's disease-related perianal fistulas heal in the adult population with conventional medical and surgical interventions. This healing rate remains unknown in pediatric patients. OBJECTIVE: This study aimed to determine the healing rate of pediatric perianal Crohn's fistulas and identify factors associated with healing. DESIGN: Retrospective case series. SETTING: A quaternary referral center. PATIENTS: Patients aged <18 years with a Crohn's perianal fistula, seen between January 1, 1991, and August 1, 2021, were included in the study. INTERVENTIONS: Multivariable logistic regression to identify factors independently associated with perianal fistula healing. MAIN OUTCOME MEASURES: Healing of Crohn's perianal fistula at the date of last clinical encounter, defined as the clinical note reporting a healed fistula or normal perianal examination. RESULTS: A total of 91 patients aged <18 years with a Crohn's disease-related perianal fistula were identified (59% female, 76% white). The mean (SD) age at Crohn's diagnosis was 12 (±4) years. The mean follow-up after Crohn's diagnosis was 10 (±7) years. Overall, 89% of patients had a perianal fistula, 2% had an anovaginal fistula, and 10% had an ileal pouch-associated fistula. Patients underwent a median (interquartile range) of 2 (1-5) operations. A seton was placed in 60% of patients, 47% underwent abscess drainage, and 44% underwent fistulotomy or fistulectomy. Fistula healing occurred in 71% of patients over a median of 1.3 (0.4-2.5) years. Seven patients (7%) underwent proctectomy, and 3 (3%) underwent ileal pouch excision. After multivariable adjustment, younger age at diagnosis of perianal fistula was associated with an increased likelihood of healing (OR 0.56 for each increased year; 95% CI, 0.34-0.92). LIMITATIONS: Retrospective, single institution. CONCLUSIONS: Over two-thirds of fistulas heal in pediatric Crohn's disease patients with conventional surgical and medical intervention. Younger age at fistula development is associated with an increased likelihood of healing. See Video Abstract at http://links.lww.com/DCR/C185 . RESULTADOS A LARGO PLAZO DE LAS FSTULAS PERIANALES EN LA ENFERMEDAD DE CROHN EN PACIENTES PEDITRICOS: ANTECEDENTES:Aproximadamente el 30% de las fístulas perianales relacionadas con la enfermedad de Crohn se curan en la población adulta con intervenciones médicas y quirúrgicas convencionales. Esta tasa de curación sigue siendo desconocida en pacientes pediátricos.OBJETIVO:Determinar la tasa de curación de las fístulas de Crohn perianales en población pediátrica e identificar los factores asociados con la curación.DISEÑO:Serie de casos retrospectiva.ESCENARIO:Un centro de referencia cuaternario.PACIENTES:Pacientes menores de 18 años con fístula(s) perianal(es) por enfermedad de Crohn, atendidos entre el 1 de enero de 1991 y el 1 de agosto de 2021.INTERVENCIONES:Regresión logística multivariable para identificar factores asociados de forma independiente con la cicatrización de la fístula perianal.PRINCIPALES MEDIDAS DE RESULTADO:Curación de la fístula perianal de Crohn en la fecha del último encuentro clínico, definida como la nota clínica que informa una fístula curada o un examen perianal normal.RESULTADOS:Se identificó un total de 91 pacientes <18 años de edad con una fístula perianal relacionada con la enfermedad de Crohn (59% mujeres, 76% blancos). La edad media (DE) al diagnóstico de Crohn fue de 12 (±4) años. El seguimiento medio tras el diagnóstico de Crohn fue de 10 (±7) años. En general, el 89 % de los pacientes tenía fístula perianal, el 2 % tenía fístula anovaginal y el 10 % de los pacientes tenía fístula asociada a reservorio ileal. Los pacientes fueron sometidos a una mediana (RIC) de 2 (1-5) operaciones. En el 60% de los pacientes se colocó sedal, en el 47% se drenó el absceso y en el 44% se realizó fistulotomía o fistulectomía. La curación de la fístula se produjo en el 71% de los pacientes durante una mediana de 1,3 (0,4-2,5) años. Siete pacientes (7%) se sometieron a proctectomía y 3 (3%) se sometieron a escisión del reservorio ileal. Después del ajuste multivariable, la edad más joven en el momento del diagnóstico de la fístula perianal se asoció con una mayor probabilidad de curación (OR 0,56 por cada año de aumento, IC del 95%, 0,34-0,92).LIMITACIONES:Retrospectivo, institución única.CONCLUSIONES:Más de dos tercios de las fístulas se curan en pacientes pediátricos con enfermedad de Crohn con intervención médica y quirúrgica convencional. Una edad más joven en el momento del desarrollo de la fístula se asocia con una mayor probabilidad de curación. Consulte Video Resumen en http://links.lww.com/DCR/C185 . (Traducción--Dr. Felipe Bellolio ).

Redo Continent Ileostomy in Patients With IBD: Valuable Lessons Learned Over 25 Years.

BACKGROUND: Patients with IBD with continent ileostomies may require revision surgeries. There remains a paucity of data regarding outcomes after redo continent ileostomy. OBJECTIVE: This study aimed to evaluate patient outcomes after redo continent ileostomy. DESIGN: Retrospective cohort study. SETTINGS: This study was conducted at a high-volume, specialized colorectal surgery department. PATIENTS: We identified patients who underwent redo continent ileostomy (defined as neo-pouch construction or major operations changing the pouch configuration) for IBD between 1994 and 2020. MAIN OUTCOME MEASURES: The main outcomes measured were patient demographics, short- and long-term outcomes, and quality of life. RESULTS: A total of 168 patients met inclusion criteria; 102 (61%) were female, the mean age was 51 years (±13.1), and the mean BMI was 24.4 (±3.9). The median time between primary and redo continent ileostomy was 16.8 years. One hundred twenty-two patients (73%) who underwent redo surgery had ulcerative colitis, 36 (21%) had Crohn's disease, and 10 (6%) had indeterminate colitis. Slipped nipple valve and valve stricture were the most common indications for redo continent ileostomy (86%). After a median follow-up of 4 years, 48 patients (29%) required a subsequent reoperation and 27 (16%) had pouch failure requiring pouch excision. The pouch survival rate was 89% at 3 years, 84% at 5 years, and 79% at 10 years. On univariate analysis, a shorter interval between the primary and redo continent ileostomy was associated with long-term pouch failure ( p = 0.003). Cox regression multivariate analysis confirmed that a shorter interval between surgeries was independently associated with pouch failure ( p = 0.014). The mean Cleveland Clinic Global Quality of Life score was 0.61 (± 0.23) among the 70 patients who responded to the questionnaire. LIMITATIONS: The main limitations were that this was a retrospective, single-center study and that it had a low response rate for the Global Quality of Life questionnaire. CONCLUSIONS: Redo continent ileostomy surgery is associated with a long-term pouch retention rate of 79% and satisfactory quality of life. Therefore, redo surgery should be offered to patients who are motivated to keep their continent ileostomy. See Video Abstract at http://links.lww.com/DCR/C87 . REHACER LA ILEOSTOMA CONTINENTE EN PACIENTES CON ENFERMEDAD INFLAMATORIA INTESTINAL VALIOSAS LECCIONES APRENDIDAS DURANTE AOS: ANTECEDENTES:Los pacientes con enfermedad inflamatoria intestinal con ileostomías continentes pueden requerir cirugías de revisión. Sigue habiendo escasez de datos con respecto a los resultados después de volver a realizar la ileostomía continente.OBJETIVO:Evaluar los resultados después de rehacer la ileostomía continente.DISEÑO:Estudio de cohorte retrospectivo.ESCENARIO:Departamento especializado en cirugía colorrectal de alto volumen.PACIENTES:Identificamos pacientes que se sometieron a una nueva ileostomía continente (definida como construcción de una nueva bolsa u operaciones mayores que cambian la configuración de la bolsa) por enfermedad inflamatoria intestinal entre 1994 y 2020.PRINCIPALES MEDIDAS DE RESULTADO:Datos demográficos de los pacientes, resultados a corto y largo plazo y calidad de vida.RESULTADOS:Un total de 168 pacientes cumplieron con los criterios de inclusión; 102 (61%) eran mujeres, la edad media fue de 51 años (±13,1) y el IMC medio fue de 24,4 (±3,9). La mediana de tiempo entre la ileostomía primaria y la nueva ileostomía continente fue de 16,8 años. Ciento veintidós pacientes (73%) que se sometieron a una nueva cirugía tenían colitis ulcerosa, 36 (21%) tenían enfermedad de Crohn y 10 (6%) tenían colitis indeterminada. El deslizamiento de la válvula del pezón y la estenosis de la válvula fueron las indicaciones más comunes para rehacer la ileostomía continente (86%). Después de una mediana de seguimiento de 4 años, 48 (29%) pacientes requirieron una reintervención posterior y 27 (16%) tuvieron falla de la bolsa que requirió la escisión de la bolsa. La tasa de supervivencia de la bolsa fue del 89 % a los 3 años, del 84% a los 5 años y del 79% a los 10 años. En el análisis univariable, un intervalo de tiempo más corto entre la ileostomía continente primaria y la nueva se asoció con falla de la bolsa a largo plazo (p = 0,003). El análisis multivariable de regresión de Cox confirmó que el intervalo más corto entre cirugías se asoció de forma independiente con el fracaso de la bolsa (p = 0,014). La puntuación media de la Calidad de Vida Global fue de 0,61 (± 0,23) entre los 70 pacientes que respondieron al cuestionario.LIMITACIONES:Estudio retrospectivo de un solo centro. Baja tasa de respuesta al cuestionario de Calidad de Vida.CONCLUSIÓN:La cirugía de ileostomía continente se asocia con una tasa de retención de la bolsa a largo plazo del 79% y una calidad de vida satisfactoria. Por lo tanto, se debe ofrecer una nueva cirugía a los pacientes que están motivados para mantener su ileostomía continente. Consulte Video Resumen en http://links.lww.com/DCR/C87 . (Traducción-Dr. Felipe Bellolio ).

Prolonged diversion after ileal pouch-anal anastomosis: Is it safe to wait?

AIM: Diverting loop ileostomy reversal (DLI-R) is routinely performed from 2-6 months following ileal pouch-anal anastomosis (IPAA). The safety of delayed reversal after IPAA is not well-defined. The aim of this study was to determine if prolonged diversion is associated with adverse outcomes compared to routine closure. METHODS: Adult patients undergoing primary IPAA with DLI from 2000 to 2021 were included in this retrospective cohort study from our institutional database. Patients were stratified into tertiles based on timing of reversal: Routine (56-116 days), Delayed (117-180 days), or Prolonged (>6 months). Univariate analysis compared categorical variables between groups. Patients reversed at <8 weeks were excluded. RESULTS: In total, 2615 patients underwent DLI-R following IPAA (3-stage 61%, 2-stage 39%; mean age 39.9 years). DLI-R was performed as Routine, Delayed and Prolonged in 1908 (72.9%), 426 (16.4%) and 281 (10.8%), respectively. Overall, DLI-R related complications occurred in 12.4% (n = 324). The complication rate in the Routine group was 11% (n = 210), in the Delayed group was 12.2% (n = 52) and in the Prolonged group was 22.1% (n = 62). Reasons for prolonged diversion in the Prolonged group were complications at the time of IPAA in 207 (73.9%) or patient preference/scheduling in 73 (26.1%). DLI-R >6 months after IPAA due to complications had higher rates of overall complications following ileostomy reversal (OR 2.6, 95% CI 1.85-3.72, p < 0.001) whereas when DLI-R was delayed due to preference/scheduling outcomes were not different than the Routine group (p = 0.28). CONCLUSION: Prolonged time to ileostomy reversal after IPAA is probably safe without increased risk of complications when due to patient preference.

Use of the Kono-S anastomosis in pediatric Crohn's disease: a single-institution experience.

OBJECTIVES: About 24% of children with Crohn's Disease (CD) require surgery. In 2003, Kono et al. described a novel anastomosis reported to decrease the rate of anastomotic CD recurrence. Subsequent studies have reproduced these outcomes, but none has demonstrated its effect in pediatric patients. This study evaluates short-term outcomes of pediatric patients following ileocolic resection and Kono-S anastomosis. METHODS: A retrospective review of patients < 18 years old who underwent ileocolic resection followed by Kono-S anastomosis compared with those who underwent a stapled anastomosis. RESULTS: Nine Kono-S patients were matched with nine patients preceding them who received traditional side-to-side and end-to-side anastomoses. All patients underwent minimally invasive surgery. Demographics, pre-operative medication usage, and symptom profiles were not significantly different. Traditional anastomosis (TA) patients had longer lengths of stay (4.6 vs 2.9 days; p = 0.03) but had no statistically significant differences in blood loss, procedure length, and pathologic findings. One Kono-S patient had a superficial surgical site infection, and one TA patient had an anastomotic leak requiring reoperation within 30 days. More TA patients experienced post-operative symptoms at both 30-day and 6-month follow-up (66.7% vs 33.3%; p = 0.16 and 77.8% vs 25%; p = 0.03). CONCLUSION: The Kono-S anastomosis appears to be safe in pediatric CD when compared to traditional stapled anastomoses.

Short- and Long-term Outcomes of Ileal Pouch Anal Anastomosis Construction in Obese Patients With Ulcerative Colitis.

BACKGROUND: Obese patients are traditionally considered difficult pouch candidates because of the potential for intraoperative technical difficulty and increased postoperative complications. OBJECTIVE: The purpose of this study was to compare the outcomes of obese versus nonobese patients with ulcerative colitis undergoing an IPAA. DESIGN: This is a retrospectively, propensity score-matched, prospectively collected cohort study. SETTING: This study was conducted at an IBD quaternary referral center. PATIENTS: Patients with ulcerative colitis undergoing IPAA (1990-2018) were included. Obesity was defined as a BMI ≥30 kg/m 2 . MAIN OUTCOME MEASURES: The primary measures included 30-day complications, long-term anastomotic leak, and pouch failure rate (excision, permanent diversion, revision). RESULTS: Of 3300 patients, 631 (19.1%) were obese (median BMI = 32.4 kg/m 2 ). On univariate analysis, obese patients were more likely to be >50 years old (32.5% versus 22.7%, p < 0.001), ASA class 3 (41.7% versus 27.7%, p < 0.001), have diabetes (8.1% versus 3.3%, p < 0.001), and have had surgery in the biologic era (72.4% versus 66.2%, p = 0.003); they were less likely to have received preoperative steroids (31.2% versus 37.4%, p = 0.004). After a median follow-up of 7 years, 66.7% had completed at least 1 quality-of-life survey. Pouch survival in the matched sample was 99.2% (99.8% nonobese versus 95.4% obese, p = 0.002). After matching and controlling for confounding variables, worse clinical outcomes associated with obesity included global quality of life (relative risk, -0.71; p = 0.002) and long-term pouch failure (HR, 4.24; p = 0.007). Obesity was also independently associated with an additional 27 minutes of operating time ( p < 0.001). There was no association of obesity with the likelihood of developing a postoperative complication, length of stay, or pouch leak. CONCLUSION: Restorative ileoanal pouch surgery in obese patients with ulcerative colitis is associated with a relatively decreased quality of life and increased risk of long-term pouch failure compared with nonobese patients. Obese patients may benefit from focused counseling about these risks before undergoing restorative pouch surgery. See Video Abstract at http://links.lww.com/DCR/B873 . RESULTADOS A CORTO Y LARGO PLAZO EN LA REALIZACIN DEL RESERVORIO ILEAL EN PACIENTES OBESOS CON COLITIS ULCEROSA: ANTECEDENTES:Habitualmente se considera a los obesos como pacientes difíciles para la realización de un reservorio ileal, debido a su alta probabilidad de presentar dificultades técnicas intraoperatoria y aumento de las complicaciones posoperatorias.OBJETIVO:El propósito de este estudio fue comparar los resultados de pacientes con colitis ulcerosa obesos versus no obesos sometidos a un reservorio ileal y anastomosis anal (IPAA).DISEÑO:Este es un estudio de cohorte recopilado prospectivamente, retrospectivo, emparejado por puntajes de propensión.AJUSTE:Este estudio se llevó a cabo en un centro de referencia de cuarto nivel para enfermedades inflamatorias del intestino.PACIENTES:Se incluyeron pacientes con colitis ulcerosa sometidos a un reservorio ileal y anastomosis anal (1990-2018). Obesidad definida como un IMC ≥ 30 kg/m2.PRINCIPALES RESULTADO MEDIDOS:Los principales resultados medidos incluyeron complicaciones a los 30 días, fuga anastomótica a largo plazo y tasa de falla del reservorio ileal (escisión, derivación permanente, revisión).RESULTADOS:De 3.300 pacientes, 631 (19,1%) eran obesos (mediana de IMC = 32,4 kg/m2). En el análisis univariado, los pacientes obesos tenían más probabilidades de ser > 50 años (32,5% frente a 22,7%, p < 0,001), clase ASA 3 (41,7% frente a 27,7%, p < 0,001), tener diabetes (8,1% frente a 3,3%, p < 0,001), haberse sometido a cirugía en la era biológica (72,4% frente a 66,2%, p = 0,003), y tenían menos probabilidades de haber recibido esteroides preoperatorios (31,2% frente a 37,4%, p = 0,004). Después de una mediana de seguimiento de 7 años, el 66,7% había completado al menos una encuesta de calidad de vida. La supervivencia de la bolsa en la muestra emparejada fue del 99,2% (99,8% no obesos versus 95,4% obesos, p = 0,002). Después de emparejar y controlar las variables de confusión, los peores resultados clínicos asociados con la obesidad incluyeron la calidad de vida global (RR = -0,71, p = 0,002) y el fracaso de la bolsa a largo plazo (HR = 4,24, p = 0,007). La obesidad también se asoció de forma independiente con 27 minutos adicionales de tiempo quirúrgico ( p < 0,001). No hubo asociación de la obesidad con la probabilidad de desarrollar una complicación posoperatoria, la duración de la estadía o la fuga de la bolsa.CONCLUSIÓNES:La cirugía restauradora del reservorio ileoanal en pacientes obesos con colitis ulcerosa se asocia a una disminución relativa de la calidad de vida y un mayor riesgo de falla del reservorio a largo plazo en comparación con los pacientes no obesos. Los pacientes obesos pueden beneficiarse de un asesoramiento centrado en estos riesgos antes de someterse a una cirugía restauradoracon reservorio ileal y anastomosis anal. Consulte Video Resumen en http://links.lww.com/DCR/B873 . (Traducción-Dr. Rodrigo Azolas ).

Mesenteric Excision and Exclusion for Ileocolic Crohn's Disease: Feasibility and Safety of an Innovative, Combined Surgical Approach With Extended Mesenteric Excision and Kono-S Anastomosis.

INTRODUCTION: Ileocolic resection for Crohn's disease traditionally does not include a high ligation of the ileocolic pedicle, and most commonly is performed with a stapled side-to-side ileocolic anastomosis. The mesentery has recently been implicated in the pathophysiology of Crohn's disease. Two techniques have been developed and are associated with reduced postoperative recurrence: the Kono-S anastomosis that excludes diseased mesentery and extended mesenteric excision that resects diseased mesentery. We aimed to assess the technical feasibility and safety of a novel combination of techniques: mesenteric excision and exclusion. TECHNIQUES: This initial report is a single-center descriptive study of consecutive adults who underwent mesenteric excision and exclusion for primary or recurrent ileocolic Crohn's disease from September 2020 to June 2021. Medication exposure and endoscopic balloon dilation before surgery were recorded. Phenotype was classified using the Montreal Classification. Thirty-day outcomes were reported. A video of the mesenteric excision and exclusion including the Kono-S anastomosis is presented. RESULTS: Twenty-two patients with ileocolic Crohn's disease underwent mesenteric excision and exclusion: 100% had strictures, 59% had fistulas, 81% were on biologics, and 27% had previous ileocolic resection(s). Seventy-two percent underwent laparoscopic procedures, a mesenteric defect was closed in 86%, omental flaps were fashioned in 77%, and 3 patients were diverted. Median operative time was 175 minutes. Median postoperative stay was 4 days. At 30 days, there were 2 readmissions for reintervention: 1 seton placement and 1 percutaneous drainage of a sterile collection. There were no cases of intra-abdominal sepsis or anastomotic leak. CONCLUSIONS: Mesenteric excision and exclusion represents an innovative, progressive, and promising approach that appears to be highly feasible and safe. Further study is warranted to determine if mesenteric excision and exclusion is associated with reduced postoperative recurrence of ileocolic Crohn's disease.

The Impact of Simulation Training on Operative Performance in General Surgery: Lessons Learned from a Prospective Randomized Trial.

BACKGROUND: Practice in the simulated environment can improve surgical skills. However, the transfer of open complex surgical skills to the operating room is unclear. This study evaluated the effect of resident operative performance following a simulation experience on a hand-sewn small bowel anastomosis and determined the impact of utilizing proficiency-based training. METHODS: Nine categorical interns performed a hand-sewn small bowel anastomosis in the operating room prior to (pre-test) and following (post-test) a 3-h simulation training session with an assessment at the end. Participants were randomly assigned to 1of 2 simulation training groups: proficiency-based or standard. Operative performance was videotaped. 2 independent, blinded faculty surgeons assessed performances by a global rating scale. Pre- and post-confidence levels were obtained on a 5-point Likert scale. RESULTS: Overall, pre-test and post-test operative performance was similar (3 [IQR, 2.5 -3.5] versus 3 [IQR, 3 -3], P = 0.59). Furthermore, no difference was observed in the post-test performance with proficiency-based or standard training (3 [IQR, 3 -3] versus 3 [IQR, 3 -3], P = 0.73). Self-reported confidence with the skills, however, significantly improved (median 1 versus 4, P = 0.007). CONCLUSIONS: In this prospective, randomized study, we did not observe an improvement in operative performance following simulation instruction and assessment, with both training groups. Overcoming barriers to skills transfer will be paramount in the future to optimize simulation training in general surgery. These findings highlight the importance of continued study for the ideal conditions and timing of technical skills training.

A Multicenter Analysis of the Early Impact of COVID-19 on Junior Resident Operative Case Volume.

INTRODUCTION: Institutions have reported decreases in operative volume due to COVID-19. Junior residents have fewer opportunities for operative experience and COVID-19 further jeopardizes their operative exposure. This study quantifies the impact of the COVID-19 pandemic on resident operative exposure using resident case logs focusing on junior residents and categorizes the response of surgical residency programs to the COVID-19 pandemic. MATERIALS AND METHODS: A retrospective multicenter cohort study was conducted; 276,481 case logs were collected from 407 general surgery residents of 18 participating institutions, spanning 2016-2020. Characteristics of each institution and program changes in response to COVID-19 were collected via surveys. RESULTS: Senior residents performed 117 more cases than junior residents each year (P < 0.001). Prior to the pandemic, senior resident case volume increased each year (38 per year, 95% confidence interval 2.9-74.9) while junior resident case volume remained stagnant (95% confidence interval 13.7-22.0). Early in the COVID-19 pandemic, junior residents reported on average 11% fewer cases when compared to the three prior academic years (P = 0.001). The largest decreases in cases were those with higher resident autonomy (Surgeon Jr, P = 0.03). The greatest impact of COVID-19 on junior resident case volume was in community-based medical centers (246 prepandemic versus 216 during pandemic, P = 0.009) and institutions which reached Stage 3 Program Pandemic Status (P = 0.01). CONCLUSIONS: Residents reported a significant decrease in operative volume during the 2019 academic year, disproportionately impacting junior residents. The long-term consequences of COVID-19 on junior surgical trainee competence and ability to reach cases requirements are yet unknown but are unlikely to be negligible.

The SAGES MASTERS program presents: the 10 seminal articles for the Laparoscopic Right Colectomy Pathway.

BACKGROUND: As one of the 12 clinical pathways of the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES) Masters Program, the Colorectal Pathway intends to deliver didactic content organized along 3 levels of performance (competency, proficiency and mastery) each represented by an anchoring procedure (laparoscopic right colectomy, laparoscopic left/sigmoid colectomy, and intracorporeal anastomosis during minimally invasive (MIS) ileocecal or right colon resection). In this article, the SAGES Colorectal Task Force presents focused summaries of the top 10 seminal articles selected for laparoscopic right colectomy which surgeons should be familiar with. METHODS: Using a systematic literature search of Web of Science, the most cited articles on laparoscopic right colectomy were identified, reviewed, and ranked by the SAGES Colorectal Task Force and invited subject experts. Additional articles not identified in the literature search were included if deemed impactful by expert consensus. The top 10 ranked articles were then summarized, with emphasis on relevance and impact in the field, findings, strengths and limitations, and conclusions. RESULTS: The top 10 seminal articles selected for the laparoscopic right colectomy anchoring procedure include articles on surgical techniques for benign and malignant disease, with anatomical and video illustrations, comparative outcomes of laparoscopic vs open colectomy, variations in technique with impact on clinical outcomes, and assessment of the learning curve. CONCLUSIONS: The top 10 seminal articles selected for laparoscopic right colectomy illustrate the diversity both in content and format of the educational curriculum of the SAGES Masters Program to support practicing surgeon progression to mastery within the Colorectal Pathway.

Approach to the Adult Colorectal Patient with a History of Pediatric Abdominal Surgery.

Children with colorectal diseases often undergo operative management in their youth. As these patients become adult, it is important for surgeons to understand their postoperative anatomy as well as the pathophysiology of their diseases. Here, we present a description of common colorectal diseases of childhood that may have significant impact on patients' presentations as adult. We also discuss the diagnosis and management of conditions that are usually seen early in life but may present during adulthood as well.