Journal of Pediatric Surgery Case Reports最新文献

{"title":"Repair of complex acquired tracheoesophageal fistula guided by 3D printing: a case report","authors":"Neal Mukherjee ,&nbsp;Michael Bunker ,&nbsp;Lia Jacobson ,&nbsp;Amar Nijagal ,&nbsp;Naveen Swami ,&nbsp;Shafkat Anwar","doi":"10.1016/j.epsc.2026.103196","DOIUrl":"10.1016/j.epsc.2026.103196","url":null,"abstract":"<div><h3>Introduction</h3><div>3D-printed (3DP) models have been used to for complex surgical simulation and planning in multiple surgical contexts, but never acquired tracheoesophageal fistulas (TEF).</div></div><div><h3>Case presentation</h3><div>A 10-month-old previously healthy female infant presented with 3 days of persistent fevers and worsening respiratory distress. A 2-cm button battery found in the esophagus on chest x-ray was urgently removed by rigid endoscopy. A remaining plaque-like tracheal lesion was later identified on rigid bronchoscopy as a 2 × 1.5cm TEF. Using the computer-assisted design software Mimics (Materialise v28.06, Leuven, BE) a 1:1 anatomical 3DP replica of the patient's anatomy was constructed using a computed tomography scan and printed using a polyjet resin printer (Stratasys J750, Minneapolis, MN). The model was used to plan the multi-disciplinary repair. The trachea and esophagus were mobilized through a median sternotomy while on cardiopulmonary bypass. The 270-degree esophageal defect was repaired with a partial circumference esophageal anastomosis. The tracheal defect required posterior reconstruction and end-to-end anastomosis. An endotracheal tube was positioned superior to the anastomosis via intraoperative bronchoscopy and a mediastinal chest tube was placed. She was monitored with daily bronchoscopies until extubation on post-operative day 7. A contrast esophagram on day 18 noted no strictures/leaks. The only postoperative complications were mediastinitis treated with antibiotics and left vocal chord hypomobility treated with injection laryngoplasty on post-operative day 23 before discharge home on day 36. She remained grossly asymptomatic through her first year of follow-up with unremarkable 1-year follow-up bronchoscopy.</div></div><div><h3>Conclusion</h3><div>3D printed models may be helpful in the preoperative planning of complex tracheoesophageal reconstruction.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"127 ","pages":"Article 103196"},"PeriodicalIF":0.2,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146057582","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Appendiceal inflammatory vascular congestion in a child: a case report","authors":"Hussein Ibrahim ,&nbsp;Ahmed Maher ,&nbsp;Ahmed zuhry Yassin Osman ,&nbsp;Mahmoud H. Abdelrazik","doi":"10.1016/j.epsc.2026.103206","DOIUrl":"10.1016/j.epsc.2026.103206","url":null,"abstract":"<div><h3>Introduction</h3><div>Appendiceal varices are an exceedingly rare entity in pediatric patients and are usually associated with portal hypertension. Severe inflammatory vascular congestion in acute appendicitis may closely mimic this condition intraoperatively.</div></div><div><h3>Case presentation</h3><div>An 8-year-old female child presented with a 24-h history of right lower quadrant abdominal pain associated with low-grade fever and vomiting. There was no history of gastrointestinal bleeding, liver disease, portal hypertension, or previous abdominal surgery. Physical examination revealed localized tenderness and guarding in the right iliac fossa. Laboratory investigations showed leukocytosis with neutrophil predominance. Abdominal ultrasonography demonstrated a non-compressible tubular structure in the right iliac fossa with surrounding inflammatory changes, consistent with acute appendicitis. Open appendectomy was performed through a Lanz incision. Intraoperatively, the appendix was markedly inflamed, edematous, and dusky in appearance with very large, prominent serpiginous vessels along the mesoappendix and appendiceal wall. These dilated veins were not detected preoperatively on imaging and raised suspicion of appendiceal varices. The dilated vessels were carefully controlled using standard ligation of the mesoappendix, and appendectomy was completed uneventfully without excessive bleeding. Gross examination showed a thickened inflamed appendix. Histopathological examination demonstrated transmural acute inflammation with marked vascular congestion but no venous ectasia or true varices. The postoperative course was uncomplicated, and the patient was discharged on the second postoperative day. At follow-up, the child remained asymptomatic with no clinical or radiological evidence of portal hypertension or recurrent symptoms.</div></div><div><h3>Conclusion</h3><div>Appendiceal varices in the context of acute appendicitis may be secondary to the inflammatory process and not a sign of portal hypertension.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"127 ","pages":"Article 103206"},"PeriodicalIF":0.2,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146081483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anorectal malformation, duodenal atresia, and intestinal malrotation: a case report","authors":"Camila Dardanelli,&nbsp;Georgina Falcioni,&nbsp;Luzia Toselli,&nbsp;Lucía Gutiérrez Gammino,&nbsp;Maximiliano Maricic","doi":"10.1016/j.epsc.2026.103207","DOIUrl":"10.1016/j.epsc.2026.103207","url":null,"abstract":"<div><h3>Introduction</h3><div>The combination of anorectal malformation, intestinal malrotation, and duodenal atresia is exceptionally rare. Such cases usually require staged surgical management.</div></div><div><h3>Case presentation</h3><div>A full-term female newborn (3.32 kg), prenatally diagnosed with duodenal atresia, Down syndrome, and imperforate anus without fistula, was admitted. Abdominal x-ray confirmed a double-bubble sign, and ultrasound revealed no other anomalies. A single minimally invasive surgery was planned on day of life three. First step: an exploratory laparoscopy was performed via a paraumbilical port and additional flank ports using 3-mm instruments and a 30-degree scope. Duodenal atresia and intestinal malrotation were identified, and Ladd bands were released. Second step: an intraoperative diagnostic contrast enema was done by a transabdominal puncture, followed by injection of water-soluble contrast. This demonstrated a 5-mm distance between the rectal pouch and the theoretical anal site, without evidence of a fistula. Third step: a side-to-end duodeno-duodenal anastomosis was performed. The anterior walls of both duodenal ends were opened, and a continuous running suture was done using 5-0 polydioxanone, starting on the posterior wall. Next, the patient was placed in prone position for a standard posterior sagittal anorectoplasty. The postoperative course was uneventful. Enteral feeding began on postoperative day six and anal dilatations on postoperative day nine. The patient was discharged home at one month of age having normal function. No reinterventions have been required at one year of follow-up.</div></div><div><h3>Conclusion</h3><div>The combination of anorectal malformation, duodenal atresia, Down syndrome, and intestinal malrotation is very rare. Repairing all defects in a single surgical stage seems safe and effective.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"127 ","pages":"Article 103207"},"PeriodicalIF":0.2,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146081482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary Ewing sarcoma of the breast in a male adolescent: A case report","authors":"Joie Sheen A. Bastian,&nbsp;Willie T. Hao,&nbsp;Lawrence Faith A. Lucañas-Yap","doi":"10.1016/j.epsc.2026.103205","DOIUrl":"10.1016/j.epsc.2026.103205","url":null,"abstract":"<div><h3>Introduction</h3><div>Ewing sarcoma predominantly affects children and adolescents. Breast Ewing sarcoma is exceptionally uncommon, with only 16 documented cases globally, just one being pediatric.</div></div><div><h3>Case presentation</h3><div>A 14-year-old male was referred by a pediatrician for evaluation of a rapidly growing right breast mass over the course of 2 months. Diagnosis was confirmed via tumor tissue biopsy, immunohistochemistry (CD99+, NKX2.2+), and fluorescent in situ hybridization, which showed an EWSR1 break-apart on chromosome 22q12. Initial contrast-enhanced computed tomography of the chest revealed a huge tumor on the right breast with 2nd to 6th anterior rib involvement measuring 16.8x18.1x9.5 cm, and prominent right axillary lymph nodes. No distant metastasis was seen on positron emission tomography during metastatic work-up. The patient received neoadjuvant chemotherapy using vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide over 12 weeks. The tumor size decreased significantly post-neoadjuvant chemotherapy, now measuring 3.9x12.2x6.5cm. After reassessment, the patient underwent wide excision of the right chest mass, en-bloc anterior 5th rib resection, right axillary lymph node dissection, with latissimus dorsi flap reconstruction with an uneventful perioperative course. He remained recurrence-free at the six-month follow-up.</div></div><div><h3>Conclusion</h3><div>Although rare, Ewing sarcoma must be included in the differential diagnosis of rapidly growing breast masses in male adolescents.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"127 ","pages":"Article 103205"},"PeriodicalIF":0.2,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146081484","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pancreatic Kaposiform haemangioendothelioma complicated by Kasabach-Merritt phenomenon and recurrent cholangitis in an infant: A case report","authors":"Hannah Triscott ,&nbsp;Halah Hassan ,&nbsp;Craig A. McBride ,&nbsp;Roy M. Kimble","doi":"10.1016/j.epsc.2026.103199","DOIUrl":"10.1016/j.epsc.2026.103199","url":null,"abstract":"<div><h3>Introduction</h3><div>Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive vascular tumour which primarily affects children. Pancreatic involvement is exceptionally uncommon, and its association with Kasabach-Merritt Phenomenon (KMP), a life-threatening consumptive coagulopathy, poses significant diagnostic and therapeutic challenges.</div></div><div><h3>Case presentation</h3><div>A 6-month-old female presented with a five-day history of bilious vomiting, lethargy, and poor oral intake. Initial investigations revealed severe anaemia (Hb 57 g/L), coagulopathy (INR 9.3) and thrombocytopenia (platelets 57 × 10⁹/L). Imaging identified bilateral subdural haemorrhages and a retroperitoneal pancreatic mass encasing vital structures causing obstructive hepatopathy, biliary dilation, and ascites. Biopsy confirmed KHE, with spindle cell proliferation and immunohistochemical features consistent with the diagnosis. The clinical course was complicated by KMP, recurrent cholangitis with Pseudomonas bacteraemia, and a pancreatic leak. Management included sirolimus, vincristine, corticosteroids, biliary drainage, and a feeding jejunostomy. Despite significant complications, including failure to thrive and prolonged jejunostomy site issues, the patient achieved resolution of coagulopathy, stabilisation of hepatic function, and stable disease on maintenance sirolimus therapy. Chemotherapy was discontinued after six months, with ongoing outpatient monitoring.</div></div><div><h3>Conclusion</h3><div>A combination of sirolimus, vincristine and supportive therapy including percutaneous biliary drainage seems to be a safe and effective management option for children with pancreatic KHE with KMP and cholestasis.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"127 ","pages":"Article 103199"},"PeriodicalIF":0.2,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146081605","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recurrent solid pseudopapillary neoplasm of the pancreas with hepatic and lymphatic metastasis in a teenager: A case report","authors":"Hoa Nguyen Viet ,&nbsp;Ngoc Nguyen Bich ,&nbsp;Phuong Tran Ha ,&nbsp;Trang Dang Thi Huyen ,&nbsp;Tuan Vu Hong ,&nbsp;Phuc Chu Minh","doi":"10.1016/j.epsc.2026.103179","DOIUrl":"10.1016/j.epsc.2026.103179","url":null,"abstract":"<div><h3>Introduction</h3><div>Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare neoplasm with low malignant potential, predominantly affecting young women. Despite its generally favorable prognosis following complete resection, recurrence or metastasis may occur years later.</div></div><div><h3>Case presentation</h3><div>A 15-year-old female who had undergone enucleation of a large pancreatic head SPN at the age of 6 years was admitted to the hospital due to persistent abdominal pain. She had been followed at a local hospital but the follow up was interrupted due to COVID-19 restrictions. She underwent a magnetic resonance that showed a recurrence of the SPN, with a hepatic. The diagnosis was confirmed with a core needle biopsy. We took her to the operating room and did a pancreaticoduodenectomy (Whipple procedure) with portal vein reconstruction, hepatic wedge resection, and extensive lymphadenectomy. Pathological analysis confirmed recurrent SPN with hepatic metastasis and found SPN infiltration in 3 out of 29 peripancreatic lymph nodes. The patient tolerated the procedure well. She developed a low-flow pancreatic fistula that was managed conservatively and resolved spontaneously. Oral feedings were started after the resolution of pancreatic fistula on postoperative day 7. She was discharged 13 days after the operation. At 4 years of follow up she remains asymptomatic and free of recurrence.</div></div><div><h3>Conclusion</h3><div>Simple enucleation may not be an acceptable approach for the management of large SPN of the pancreatic head, since as seen in our patient, it may be associated with recurrences.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"126 ","pages":"Article 103179"},"PeriodicalIF":0.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145940804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Currarino syndrome with presacral neuroblastoma: A case report","authors":"Rami Darawsheh ,&nbsp;Neal Mukherjee ,&nbsp;Ernesto J. Rojas ,&nbsp;Alberto Jarrin Lopez ,&nbsp;Olajire Idowu ,&nbsp;Sunghoon Kim","doi":"10.1016/j.epsc.2026.103192","DOIUrl":"10.1016/j.epsc.2026.103192","url":null,"abstract":"<div><h3>Introduction</h3><div>Currarino syndrome is a rare congenital disorder defined by the triad of sacral anomaly, presacral mass, and anorectal malformation. Presacral masses in Currarino syndrome are typically benign developmental lesions; however, the occurrence of a presacral neuroblastoma in a patient with the complete Currarino triad has not been reported.</div></div><div><h3>Case presentation</h3><div>A male newborn delivered at 37 weeks of gestation after a pregnancy complicated by maternal alcohol and marijuana use was found to have an imperforate anus, bilious emesis, and abdominal distension requiring orogastric decompression and diverting colostomy. Further evaluation revealed microcephaly, a tethered spinal cord, a left multicystic dysplastic kidney, severe hypospadias with a penoscrotal fistula draining meconium, and a sacral anomaly. At five months of age, a spinal MRI identified a 2 × 1.7 × 2 cm presacral mass. A screening ultrasound (US) obtained five months earlier during a VACTERL evaluation did not identify a presacral mass, likely reflecting US's limited sensitivity for presacral lesions. Surgical excision of the mass via sacral coccygectomy and anorectoplasty revealed a poorly differentiated neuroblastoma with favorable histopathologic features and negative margins. Staging studies showed no residual or metastatic disease. No oncologic therapy was initiated. Exome sequencing did not identify pathogenic MNX1 or other clinically significant germline variants. At three years of follow-up, the patient demonstrated normal growth without tumor recurrence.</div></div><div><h3>Conclusion</h3><div>This case demonstrates that presacral masses in Currarino syndrome, traditionally considered benign developmental lesions, can present with a neuroblastoma. Neuroblastoma should be included in the differential diagnosis for presacral lesions in Currarino syndrome.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"126 ","pages":"Article 103192"},"PeriodicalIF":0.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146038644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Magnetic functional undiversion in a patient with enteroatmospheric fistula and hostile abdomen: a case report","authors":"Cynthia Juliana Hernandez Yate ,&nbsp;Nidia Catalina León Rodríguez ,&nbsp;Germán Aquilino Mogollón Cruz ,&nbsp;Diana Alejandra Holguin Sanabria ,&nbsp;Pablo Eliecer Luengas Perez ,&nbsp;Isabel Gutierrez Soto","doi":"10.1016/j.epsc.2026.103171","DOIUrl":"10.1016/j.epsc.2026.103171","url":null,"abstract":"<div><h3>Introduction</h3><div>Enteroatmospheric fistula in the setting of a hostile abdomen represents a major surgical challenge. Conventional anastomotic techniques are often unfeasible due to friable tissue, extensive adhesions, and high operative risk. Magnetic compression anastomosis has emerged as a minimally invasive alternative for restoring intestinal continuity in complex surgical scenarios.</div></div><div><h3>Case presentation</h3><div>We report the case of a 14-year-old male with total colonic aganglionosis diagnosed in early childhood and initially managed with a diverting colostomy. Definitive reconstruction was delayed due to loss of follow-up after relocation to a remote region with limited access to specialized pediatric surgical care. At 14 years of age, the patient underwent intestinal reconstruction, which was complicated by an anastomotic leak, multiple laparotomies, and the development of a single enteroatmospheric fistula in the setting of a persistent hostile abdomen.</div><div>Given the high surgical risk, the patient was not considered a candidate for conventional reoperation. A custom-made magnetic compression device was therefore designed to adapt to the patient's altered intestinal anatomy and the absence of commercially available magnetic systems for pediatric functional undiversion in the region. The device consisted of two annular neodymium magnets coated with methyl methacrylate, one housed within a convex 3D-printed thermoplastic polyurethane cover, and was placed endoluminally under endoscopic guidance. Also radiographic flow up was made.</div><div>The magnets achieved proper alignment and were spontaneously expelled on postoperative day 7. By postoperative day 14, intestinal transit was restored and enteral feeding was resumed without evidence of leak or infection, achieving functional closure of the fistula. Due to severe loss of abdominal wall domain, abdominal wall closure was managed by secondary intention.</div></div><div><h3>Conclusion</h3><div>Magnetic compression anastomosis seems to be a safe and effective alternative for the management of single enteroatmospheric fistulas.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"126 ","pages":"Article 103171"},"PeriodicalIF":0.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145940805","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Robert's uterus with ipsilateral renal agenesis presenting as pelvic inflammatory disease in a teenager: A case report","authors":"Akkamahadevi C. Hiremath,&nbsp;Harshita Ramamurthy ,&nbsp;T.D. Nandeesha,&nbsp;R. Padmasri","doi":"10.1016/j.epsc.2026.103173","DOIUrl":"10.1016/j.epsc.2026.103173","url":null,"abstract":"<div><h3>Introduction</h3><div>Robert's uterus, with ipsilateral renal agenesis, is an exceptionally rare Mullerian duct anomaly (MDA). It usually presents with cyclical pain, but presentation as pelvic inflammatory disease in an adolescent is extremely uncommon.</div></div><div><h3>Case presentation</h3><div>We report a rare case of Robert's uterus with ipsilateral renal agenesis in a 13-year-old girl, eight months post-menarche, who presented with 15 days of abdominal pain, copious purulent vaginal discharge, and fever. Ultrasonography suggested a septate/bicornuate uterus with a large, irregular pelvic collection. MRI demonstrated an asymmetric uterine septum forming a non-communicating left hemi-cavity and left renal agenesis. As an emergency procedure under anesthesia, 100 ml of hematinic purulent material was drained from the pouch of Douglas. After clinical stabilization for one week, hystero-laparoscopy was performed. Hysteroscopy revealed a normal vagina, single cervix, and right hemi uterus with right tubal ostia, whereas laparoscopy revealed a broad fundus, a dilated left tube and flimsy adhesions, findings suggestive of Robert's uterus. Laparoscopic-guided hysteroscopic unification metroplasty was successfully done by incising the asymmetric septum using a bipolar Collins knife. A no. 8 Foley catheter was left in situ for one week to prevent adhesions. She recovered uneventfully and, at 9-month follow-up, experiences regular moderate menstrual cycles without dysmenorrhea or pain.</div></div><div><h3>Conclusion</h3><div>Robert's uterus is a rare Mullerian malformation that should be considered in the differential diagnosis of females presenting with features of pelvic inflammatory disease. Ipsilateral renal agenesis should be evaluated in patients diagnosed with Robert's uterus.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"126 ","pages":"Article 103173"},"PeriodicalIF":0.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145940806","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Wandering spleen in a Cornelia De Lange young woman: a case report","authors":"Francesca Nascimben ,&nbsp;Agnese Capalbo ,&nbsp;Valeria Nocera ,&nbsp;Maria Barbara Leone ,&nbsp;Rossella Angotti ,&nbsp;Francesco Molinaro","doi":"10.1016/j.epsc.2026.103194","DOIUrl":"10.1016/j.epsc.2026.103194","url":null,"abstract":"<div><h3>Introduction</h3><div><u>this is the first reported case of</u> splenic volvulus (SV) due to Wandering Spleen (WS) in a patient with Cornelia de Lange syndrome (CdLs). The aim of this paper is to share our experience regarding the diagnostic and therapeutic management of this rare condition.</div></div><div><h3>Case presentation</h3><div>an 18-year-old young woman affected by CdLS (NIPBL mutation), previously treated for ileal volvulus due to intestinal malrotation, was referred to our institution with vomiting, diarrhea and suspected abdominal pain. Urgent abdominal ultrasound and computed tomography (CT) revealed splenic volvulus. She underwent emergency laparotomy which confirmed splenic voluvlus associated with a wandering spleen; the splenic hilum was derotated and splenopexy was performed through the placement of a tailored polypropylene surgical mesh. The postoperative course was regular: analgesics were administered for 5 days and antibiotic therapy for 7 days; she was discharged on postoperative day 7 in good general condition after abdominal ultrasound examination. Follow up was uneventfull: postoperative ultrasounds performed 1 and 6 months after surgery showed a spleen of normal volume and morphology with preserved vascular pattern.</div></div><div><h3>Conclusion</h3><div>splenic volvulus should be always included in the differential diagnosis of abdominal pain in patients with CdLs in whom diagnosis and management may be complicated by neurological impairment, low compliance and non-specific symptoms.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"126 ","pages":"Article 103194"},"PeriodicalIF":0.2,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146078536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}