Rectal Biopsy for Hirschsprung's Disease: A Multicentre Study Involving Biopsy Technique, Pathology and Complications.

BACKGROUND: The heterogeneity of rectal biopsy techniques has encouraged us to search for a surgical and pathological standardisation of this diagnostic technique to exclude Hirschsprung's disease. The different amounts of information on the anatomopathology report prompted us to compile a template for the anatomopathology report for diagnostic rectal biopsies for surgical colleagues and pathologists working on Hirschsprung's disease. METHODS: We gathered the anonymous biopsy information and its pathology information from five hospitals for all patients in which rectal biopsies were taken to diagnose Hirschsprung's disease over two years (2020-2021). RESULTS: Of the 82 biopsies, 20 suction (24.4%), 31 punch (37.8%) and 31 open biopsies (37.8%) were taken. Of all biopsies, 69 were conclusive (84.2%), 13 were not (15.8%). In the suction biopsy group, 60% were conclusive and 40% were not; for punch biopsy, the values were 87% and 13%, respectively and for open biopsy, 97% and 3%. Inconclusive results were due to insufficient submucosa in 6/8 suction biopsies, 4/4 punch biopsies and 0/1 open biopsies. An insufficient amount of submucosa was the reason for an inconclusive result in 6/20 cases (30%) after suction biopsy, 4/31 (12.9%) cases after punch biopsy and 0 cases (0%) after open biopsy. We had one case with major postoperative bleeding post suction biopsy; there were no further adverse effects after biopsy. CONCLUSIONS: Diagnostic rectal biopsies in children are safe. Non-surgical biopsies are more likely to give inconclusive results due to smaller amounts of submucosa present in the specimen. Open biopsies are especially useful when previous non-surgical biopsies are inconclusive. An experienced pathologist is a key factor for the result. The anatomopathology report should specify the different layers present in the specimen, the presence of ganglion cells and hypertrophic nerve fibres, their description and a conclusion.

Pediatric necrotizing soft tissue infection after elective surgery: A case report and literature review.

INTRODUCTION: Necrotizing soft tissue infections (NSTIs) are rare but often lethal. Early diagnosis and aggressive surgical debridement are essential to achieve the best possible outcome. CASE PRESENTATION: A 12-year old boy was diagnosed with a necrotizing soft tissue infection following elective revision surgery for functional impairment resulting from scar tissue of the neck. Fever and inflammation of the surgical wound manifested 24-36 hours postoperatively. Antibiotic therapy with amoxicillin-clavulanic acid was initiated, but the patient was unresponsive. Ultrasonography, a wound culture and surgical exploration confirmed the diagnosis. The culture was positive for a Streptococcus pyogenes infection and antibiotic treatment was switched to penicillin and clindamycin. Following the diagnosis, surgical debridement was performed subcutaneously, and only necrotic tissue was removed to preserve as much skin tissue as possible. After eradication of the infection, vacuum-assisted closure of the wound was used to close the subcutaneous space. The patient was discharged after 40 days. CONCLUSION: In this patient, we treated a necrotizing soft tissue infection with antibiotics, skin sparing surgeries and negative pressure wound therapy (NPWT). We used ultrasonography as imaging technique to help with the diagnosis. The extensiveness of surgical debridement was rather limited. We focused on opening all affected fascial layers. The surgical debridement was subcutaneous, and only necrotic tissue was removed. Because of the location in the neck, we tried to avoid an aggressive skin debridement to preserve as much skin tissue as possible. Negative pressure wound therapy is not frequently used in this context but it contributed to an enhanced wound healing. Ultrasonography for diagnosing NSTIs is useful, but the clinical findings and an explorative surgery will remain most important.

Preoperative risk score for prediction of long-term outcomes after hepatectomy for intrahepatic cholangiocarcinoma: Report of a collaborative, international-based, external validation study.

PURPOSE: A preoperative risk score (PRS) to predict outcome of patients with intrahepatic cholangiocarcinoma treated by liver surgery could be clinically relevant.To assess accuracy for broadly adoption, external validation of predictive models on independent datasets is crucial. The objective of this study was to externally validate the score for prediction of long-term outcomes after liver surgery for intrahepatic cholangiocarcinoma proposed by Sasaki et al. and based on preoperative albumin, neutrophil-to-lymphocytes-ratio, CA19-9 and tumor size. METHODS: Patients treated by liver surgery for intrahepatic cholangiocarcinoma at 11 international HPB centers from 2001 to 2018 were included in the external validation cohort. Harrell's c-index and Hosmer-Lemeshow analyses were used to test PRS discrimination and calibration. Kaplan-Meier curve for risk groups as described in the original study were displayed. RESULTS: A total of 355 patients with 174 deaths during the follow-up period (median = 41.7 months, IQR 32.8-50.6) were included. The median PRS value was 14.7 (IQR 10.7-20.6), with normal distribution across the cohort. A Cox regression on PRS covariates found coefficients similar to those of the derivation cohort, except for tumor size. Measures of discrimination estimated by Harrell's c-index was 0.61(95%CI:0.56-0.67) and Hosmer-Lemeshow p = 0.175. The Kaplan-Meyer estimation showed reasonable discrimination across risk groups, with 5years survival rate ranging from 20.1% to 0%. CONCLUSION: In this external validation cohort, the PRS had mild discrimination and poor calibration performance, similarly to the original publication. Nevertheless, its ability to identify different classes of risk is clinically useful, for a better tailoring of a therapeutic strategy.

Tailgut cyst: report of three cases and review of the literature.

INTRODUCTION: Tailgut cysts or retrorectal cystic hamartomas are congenital developmental lesions which are often misdiagnosed due to their rare incidence, anatomical position and variable clinical presentation. CASE REPORT: We report three clinical cases: one of a 67-year old woman with high fever and anal bulging; the second case was a 50-year old woman with diffuse abdominal pain and the third case was a 52-year old woman with high fever and no abdominal or rectal pain. The rectal examination and MRI indicated the presence of a tailgut cyst. In all the cases a complete resection through a posterior perineal route was performed. Histopathological examination confirmed the diagnosis of a tailgut cyst, with a malignant component identified in the third case. DISCUSSION: The discussion presents a brief review of the relevant information described in the literature to highlight the cornerstones for appropriate diagnosis and treatment of a tailgut cyst. Tailgut cysts are to be considered in the differential diagnosis of retrorectal or presacral masses as malignant transformation can occur.

[Torsion of wandering spleen: a case report in the Kara University Hospital (Togo)]. / Torsion d'une rate ectopique: a propos d'un cas au CHU Kara (Togo).

Wandering spleen is a rare pathology. Spleen infarction which results from the torsion of the pedicle is its main complication. We report a case of torsion of a wandering spleen diagnosed by computed tomography. The therapeutic sanction was a splenectomy because of the infarction. Nowadays, splenopexy is the best treatment if the diagnosis is done early.

La rate ectopique ou baladeuse est une entité pathologique rare. Sa complication majeure est la torsion de son pédicule pouvant conduire à un infarctus splénique. Nous rapportons un cas de torsion de rate ectopique dont le scanner avait permis de poser le diagnostic. La splénectomie a été la sanction thérapeutique à cause de son infarcissement. Actuellement, la splénopexie est proposée quand le diagnostic est fait précocement.

Thrombolysis for Severe Intestinal Ischemia due to Midgut Volvulus in a Neonate.

Intestinal malrotation complicated by the development of midgut volvulus presents a difficult management dilemma because of the risk of short bowel syndrome. Here, we present our experience with a case of severe intestinal ischemia after derotation in a newborn successfully managed using systemic tissue plasminogen activator. The present report supports the usefulness of thrombolysis as a therapeutic option for reperfusion of ischemic small bowel due to midgut volvulus in neonates.

Nonpalpable testes: is there a relationship between ultrasonographic and operative findings?

BACKGROUND: Ultrasonography (US) as a diagnostic tool in the work-up of boys with nonpalpable testes (NPT) is still controversial. OBJECTIVE: To evaluate the relation between US and operative findings in boys with NPT. MATERIALS AND METHODS: During a 7-year period we saw 135 boys with 152 NPT. All were examined by the referring physician or a paediatric surgeon, underwent US examination, and were then re-examined afterwards by a specialist. Finally, all boys were surgically explored for testicular position. RESULTS: US located 103 NPT (68%), 16 within the abdomen and 87 in the inguinal canal. With knowledge of the US result, 37 testes were palpable on re-examination. The sensitivity of US was 97% for inguinal and 48% for abdominal viable testes. Of the 49 testes (32%) missed by US, 16 were viable in either the abdominal (n=14) or the inguinal (n=2) position. CONCLUSION: All boys with presumed NPT should be referred to a specialist. US is useful to determine localization of NPT, which facilitates planning the surgical procedure. An inguinal exploration is called for when US identifies the testis in the inguinal canal. Because the sensitivity of US for viable abdominal testes is only 48%, we now always perform laparoscopic exploration when US is negative.