INTRODUCTION:Urethral duplication associated with anorectal malformation (ARM) is rare and can make both diagnosis and surgical reconstruction difficult. CASE PRESENTATION:A male infant was born at 31 weeks' gestation, weighing 1362 g, with multiple congenital anomalies, including duodenal atresia, right renal agenesis, a tethered cord with a terminal filar lipoma, and a ventricular septal defect. He developed neonatal urinary retention; urethral drainage was possible only with a fine intravenous catheter, and when this became obstructed, a percutaneous cystostomy was placed. Contrast studies demonstrated intermediate-type ARM with a rectobulbar urethral fistula, an enlarged prostatic utricle, and complete sagittal urethral duplication (Effmann type IIA2). Distal to the bulbar urethra, the urethra divided into a severely stenotic dorsal channel and a ventral channel that carried voiding but was also stenotic. At 8 months of age, laparoscopic-assisted anorectoplasty (LAARP) was performed with excision of the prostatic utricle; the rectourethral fistula was divided on the rectal side to avoid urethral injury, and the retained remnant formed an acquired posterior urethral diverticulum (PUD). The residual stenosis and the PUD were then managed endoscopically by balloon dilation of the ventral channel and holmium:yttrium-aluminum-garnet laser ablation of the PUD. The dilation restored spontaneous voiding and allowed cystostomy removal at 21 months of age without open urethral reconstruction. At the most recent follow-up (age 28 months), the patient maintains spontaneous voiding with preserved renal function. CONCLUSIONS:We encountered ARM associated with urethral duplication in which both urethral channels were stenotic and caused neonatal urinary retention. Early urinary diversion allowed accurate anatomic assessment, and the functional ventral channel was preserved by keeping the fistula dissection on the rectal side during LAARP. Staged endoscopic management of the residual stenosis and the acquired PUD achieved stable spontaneous voiding without extensive open urethral reconstruction.
INTRODUCTION:The double-flap technique (DFT) is an established anti-reflux reconstruction method after proximal gastrectomy (PG) and may be advantageous for mediastinal reconstruction because of its low incidence of anastomotic leakage. However, thoracoscopic DFT reconstruction remains technically demanding because flap-related procedures increase procedural complexity. The non-flap tunnel technique (NFTT) has recently been introduced as a simplified alternative to DFT, but its application in thoracoscopic mediastinal reconstruction has not been reported. CASE PRESENTATION:A 56-year-old man with Siewert type II esophagogastric junction cancer required a high intramediastinal anastomosis because a proximal submucosal tumor necessitated extended esophageal resection. After laparoscopic PG and lower esophagectomy, a seromuscular tunnel was created extracorporeally on the remnant stomach. The remnant stomach was elevated into the mediastinum, and thoracoscopic reconstruction was performed in the prone position. Following fixation of the remnant stomach to the esophagus and rotation of the esophagogastric axis, esophagogastrostomy was completed using hand-sewn sutures, followed by seromuscular roof closure over the anastomosis. Reconstruction was completed in 60 minutes. A contrast study on POD 5 demonstrated no anastomotic leakage or reflux. The patient resumed oral intake on POD 6 and was discharged on POD 11 without complications. CONCLUSIONS:Thoracoscopic reconstruction using the NFTT is feasible and may represent a practical and technically simplified alternative to thoracoscopic DFT in selected patients requiring high intramediastinal anastomosis.
INTRODUCTION:Chronic expanding hematoma (CEH) is a slowly enlarging hematoma that persists for more than 1 month after the initial hemorrhagic event. Intrathoracic CEH is rare and often requires technically demanding surgery because of dense adhesions and the risk of massive intraoperative bleeding. We report a case of giant intrathoracic CEH managed using a surgical strategy that included preoperative selective transcatheter arterial embolization (TAE). CASE PRESENTATION:A 49-year-old man with a history of tuberculous pleuritis approximately 10 years earlier was referred to our hospital because of a gradually enlarging asymptomatic right intrathoracic mass. Contrast-enhanced CT revealed a 95-mm heterogeneous mass with peripheral enhancement, consistent with CEH. Selective embolization of the right 8th to 11th intercostal arteries was performed on the day before surgery. Through a posterolateral thoracotomy with resection of the 7th and 8th ribs, the hematoma was completely resected with partial resection of the right lower lobe. Additional procedures, including decortication, adhesiolysis, and chest wall plasty, were performed to facilitate lung re-expansion and reduce the residual thoracic cavity. Although prolonged postoperative air leakage required pleurodesis on POD 10, the patient was discharged on POD 12 without major complications. No recurrence has been observed during follow-up. CONCLUSIONS:Preoperative selective TAE, combined with an appropriate surgical strategy, may reduce intraoperative bleeding and enable safe and controlled radical resection of intrathoracic CEH. Careful preoperative planning, including bleeding control and optimization of the surgical approach, is essential for successful treatment.
INTRODUCTION:Colonic lipomas are benign mesenchymal tumors composed of mature adipocytes and are most commonly located in the submucosal layer. They are usually asymptomatic and are often managed conservatively. Although adenomas arising overlying colonic lipomas have been reported, the coexistence of adenocarcinoma directly overlying a lipoma is extremely rare. The pathogenic relationship between submucosal tumors and epithelial malignancies remains unclear. CASE PRESENTATION:A 76-year-old man was diagnosed with a lipoma in the descending colon (diameter, approximately 30 mm) and was managed with surveillance. After 26 months of follow-up, colonoscopy revealed a newly developed small type 2 ulcerative lesion located directly over the lipoma. A biopsy confirmed adenocarcinoma. Endoscopic ultrasonography demonstrated a homogeneous hyperechoic lesion in the 3rd layer consistent with a lipoma, along with focal thickening of the 2nd layer, without evidence of deep invasion. CT and MRI showed imaging features characteristic of a lipoma, with a peripheral component suspicious for malignancy. The patient was diagnosed with descending colon cancer (cT2N0M0, Stage I) and underwent laparoscopic left hemicolectomy with lymph node dissection. A histopathological examination revealed a lipoma extending from the submucosa into the muscularis propria, while the carcinoma was confined to the mucosa and muscularis propria overlying the lipoma, without invasion into the lipomatous tissue. No histological findings indicative of chronic inflammation were observed. CONCLUSIONS:We report a rare case of adenocarcinoma developing directly over a colonic lipoma during surveillance. Although chronic inflammation has been proposed as a possible mechanism linking submucosal tumors to epithelial malignancies, no such features were identified in this case, and a causal relationship could not be established. While colonic lipomas are generally managed conservatively, careful endoscopic surveillance may be considered in selected patients with relatively large lipomas requiring long-term follow-up.
INTRODUCTION:Pancreatoduodenectomy (PD) for pancreatic head malignancies can be complicated by coexisting vascular anomalies. Median arcuate ligament syndrome (MALS), occurring in 2.1%-7.6% of PD cases, causes celiac artery (CA) stenosis and poses a significant risk of ischemic complications. While division of the median arcuate ligament (MAL) may restore blood flow in some cases, arterial reconstruction is occasionally necessary. We report a challenging case of pancreatic head adenocarcinoma with MALS requiring emergent intraoperative arterial reconstruction. CASE PRESENTATION:A 69-year-old man presented with a 31-mm pancreatic head tumor in contact with the superior mesenteric artery (SMA) and complete CA occlusion due to MALS. Preoperative imaging revealed well-developed collateral circulation via the dorsal pancreatic artery (DPA). After 6 cycles of neoadjuvant chemotherapy with gemcitabine plus nab-paclitaxel, the tumor decreased to 18 mm. Anticipating the potential need for arterial reconstruction during PD, preoperative simulations were conducted with the vascular surgery team. Intraoperatively, MAL division was performed, and a gastroduodenal artery (GDA) clamp test confirmed adequate hepatic arterial flow via the DPA. However, hepatic arterial flow ceased during subsequent surgical manipulation, requiring emergent arterial reconstruction. Initial middle colic artery-GDA anastomosis was complicated by repeated thrombosis; definitive reconstruction using a lesser saphenous vein graft successfully restored hepatic arterial flow. Pathological examination revealed a complete pathological response. The patient was discharged on POD day 18 without major complications and remains recurrence-free at 22 months. CONCLUSIONS:This case demonstrates that in PD for patients with concomitant MALS, collateral circulation may be disrupted by intraoperative manipulation, potentially necessitating emergent arterial reconstruction. Therefore, meticulous preoperative imaging evaluation, vigilant intraoperative monitoring of hepatic arterial flow, and thorough preparation for vascular reconstruction are essential for the safe management of these complex cases.
INTRODUCTION:Anti-Yo antibody-associated paraneoplastic neurological syndrome (PNS) is a rare immune-mediated disorder that often presents with subacute cerebellar symptoms and is frequently associated with breast and gynecological malignancies. CASE PRESENTATION:A woman in her 50s developed subacute progressive neurological symptoms, including dizziness, diplopia, dysphagia, gait disturbance, ataxia, and nystagmus. Brain MRI showed no obvious abnormalities. Although Miller-Fisher syndrome was initially suspected, intravenous immunoglobulin therapy had limited efficacy, and serum anti-Yo antibodies were subsequently detected. Further investigation revealed human epidermal growth factor receptor 2-positive breast cancer with axillary lymph node metastasis. Because her neurological symptoms rapidly progressed and her performance status deteriorated, neoadjuvant chemotherapy was considered difficult. Left mastectomy with axillary lymph node dissection was therefore performed as prompt tumor-directed treatment. The final pathological stage was pT1aN1aM0, stage IIA. Postoperatively, additional immunomodulatory treatments and rehabilitation were provided for the neurological deficits. CONCLUSIONS:This case highlights the importance of considering anti-Yo antibody-associated PNS in patients with unexplained subacute progressive neurological symptoms, even when brain imaging shows no clear abnormalities. Early recognition of PNS and prompt screening for an underlying malignancy, including breast cancer, are essential to allow timely tumor-directed treatment before severe neurological deficits become established.
INTRODUCTION:Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that most commonly arises in the pleura but can occur at virtually any anatomical site, including the abdominal cavity. Although rupture of SFT has occasionally been reported, most such cases involve intrathoracic tumors presenting with hemorrhage, and peritonitis caused by rupture of an intra-abdominal SFT is exceedingly rare. CASE PRESENTATION:A man in his 70s was referred to Hokkaido University Department of Gastroenterological Surgery II after contrast-enhanced CT revealed a 12 × 9-cm heterogeneous pelvic mass with poor enhancement accompanied by ascites. Endoscopic US-guided fine-needle aspiration had been performed at the previous hospital but failed to yield a definitive diagnosis, and elective tumor resection was planned. One week after the initial visit, the patient was brought to our hospital with abdominal pain. Emergency CT showed slight enlargement of the tumor, intratumoral fluid collection, and increased fat stranding around the tumor. Conservative management with intravenous antibiotics was initiated because there were no overt signs of generalized peritonitis. However, inflammatory markers worsened and fever developed, prompting emergency surgery on hospital day 2. Laparotomy revealed partial rupture of the tumor wall with leakage of contaminated intratumoral fluid into the peritoneal cavity. The tumor pedicle was identified at the base of the sigmoid mesocolon, and complete resection was achieved. The operative time was 124 min, and the estimated blood loss was 1100 mL. Histopathological examination revealed a patternless proliferation of spindle cells within a fibrous stroma with staghorn-like vasculature. Immunohistochemistry showed nuclear positivity for signal transducer and activator of transcription 6 and positivity for CD34, confirming the diagnosis of SFT. According to the modified Demicco model, the tumor was classified as intermediate risk. The patient was discharged on POD 31. CONCLUSIONS:When managing a large intra-abdominal tumor whose growth rate cannot be reliably assessed, the possibility of rupture should be kept in mind, and emergency surgery should not be delayed when abdominal findings worsen. Although the prognostic impact of tumor rupture in SFT remains unclear, this event may justify closer postoperative surveillance than that indicated by existing risk classification schemes.
INTRODUCTION:Obturator hernia is a rare cause of small-bowel obstruction and is frequently associated with strangulation. Even after successful reduction, bowel ischemia-reperfusion injury may occur and result in life-threatening metabolic complications. CASE PRESENTATION:An 87-year-old woman presented with a 3-week history of nausea and vomiting. CT revealed a right obturator hernia with incarceration of a small-bowel loop. US-guided manual reduction was successfully performed. Contrast-enhanced CT obtained immediately after reduction demonstrated preserved bowel-wall enhancement with circumferential mural thickening. The serum potassium level at presentation was 4.6 mmol/L. Despite apparently preserved bowel perfusion on CT, she continued to experience abdominal pain, tachypnea, and progressively deteriorating consciousness. Approximately 5 h after reduction, she developed sudden bradycardia followed by cardiac arrest. Continuous electrocardiographic monitoring showed progression from sinus rhythm to loss of P waves, QRS widening, and finally a sine-wave pattern. Laboratory testing during resuscitation revealed severe hyperkalemia (8.5 mmol/L). Despite intensive resuscitative efforts, the patient died. CONCLUSIONS:Preserved bowel-wall enhancement after reduction of a strangulated obturator hernia does not necessarily indicate bowel viability. A prolonged clinical course, persistent symptoms or systemic abnormalities, and residual CT abnormalities such as bowel-wall thickening and ascites should be considered together when assessing bowel viability after reduction. Such findings should prompt careful reassessment, close monitoring, and consideration of early surgical exploration despite preserved bowel-wall enhancement.
INTRODUCTION:An extrahepatic biliary neuroendocrine tumor (EB-NET) is an extremely rare malignancy with a poor prognosis and no established standard of care. CASE PRESENTATION:We herein report the case of a 35-year-old Japanese woman with no significant past medical or family history who presented with epigastric and back pain and was diagnosed with an EB-NET after pylorus-preserving pancreaticoduodenectomy. Despite initial resection, she developed recurrent liver metastases that required repeated hepatic resections and multiple systemic therapies, including chemotherapy, somatostatin analogs, everolimus, and peptide receptor radionuclide therapy (PRRT). Eventually, owing to progressive liver metastasis, she underwent living-donor liver transplantation (LDLT) from her brother 6 years after the initial surgery. Post-transplantation, the patient has remained disease-free for >3 years while on maintenance immunosuppression. CONCLUSIONS:Multimodal treatment, including resection of metastases, PRRT, and LDLT, significantly prolonged survival. This case highlights the potential role of LDLT as a curative strategy for patients with EB-NETs and controlled extrahepatic disease following multimodal treatment. Our case suggests that liver transplantation may provide long-term survival even in cases of a rare primary EB-NET, prompting the consideration of broader transplant indications in selected patients.
INTRODUCTION:Entero-appendiceal fistula is an extremely rare entity, with very few cases reported in the literature. Most previously described cases presented with abdominal pain or were diagnosed incidentally. To our knowledge, there have been no reports of such a fistula presenting with overt gastrointestinal bleeding. CASE PRESENTATION:A 56-year-old man with a medical history of human immunodeficiency virus infection, alcoholic cirrhosis, and paroxysmal atrial fibrillation, who was under regular follow-up at our hospital, presented with a 4-day history of hematochezia that was occurring 3-4 times per day. He was found to have severe anemia with a hemoglobin level of 6.6 g/dL and was emergently hospitalized on the same day. Contrast-enhanced CT did not reveal active extravasation. Colonoscopy showed multiple diverticula but no clear bleeding source. Recurrent hematochezia prompted repeat imaging, and subsequent CT showed extravasation in the ileum. Colonoscopy again revealed blood originating from a segment proximal to the reach of the colonoscope, suggesting bleeding from a more proximal segment. Double-balloon enteroscopy revealed a hemorrhagic submucosal tumor-like lesion located approximately 100 cm proximal to the ileocecal valve. Biopsy was inconclusive, but surgical resection was indicated because of the ongoing bleeding and the need to exclude malignancy. Laparoscopic resection revealed that the tip of the appendix had formed a fistula into the ileal lumen. Histopathological examination confirmed a full-thickness fistula between the appendix and ileum, with no evidence of malignancy. CONCLUSIONS:This is the first reported case of an entero-appendiceal fistula presenting with overt gastrointestinal bleeding and endoscopic features mimicking a submucosal tumor. Although extremely rare, this condition should be considered in the differential diagnosis of small intestinal tumors, particularly in patients with a history of complicated appendicitis or immunosuppression.
INTRODUCTION:Secondary thoracoscopic posterior tracheopexy (PT) for severe tracheomalacia (TM) following esophageal atresia (EA) repair is challenging because of postoperative adhesions and scarring. Meticulous dissection is required to separate the esophagus from the trachea, increasing the risk of iatrogenic injury. We report the first use of a biologically transparent (BT) illumination guide, Tumguide (Otsuka Pharmaceutical Factory, Tokushima, Japan), to assist in identifying the course of the esophagus during secondary thoracoscopic PT. CASE PRESENTATION:A 5-month-old infant with a history of thoracoscopic EA repair complicated by an anastomotic leak and stricture developed severe TM requiring surgical intervention. Because dense adhesions were anticipated, the Tumguide was activated in blinking mode and inserted after induction of general anesthesia to facilitate identification of the esophagus. Intraoperatively, the normal anatomical planes within the mediastinum were completely obliterated by severe fibrosis. However, the blinking BT red light was clearly visible through the thick scar tissue, helping clarify the overall orientation of the esophagus; a bronchoscope was also used as appropriate to transilluminate the tracheal wall from within, helping delineate the tracheal course. This combined visual guidance facilitated safe adhesiolysis and craniocaudal dissection, enabling successful fixation of the posterior membranous trachea to the anterior longitudinal ligament. The patient's respiratory status improved markedly, and the patient remained stable in room air without symptoms 6 months postoperatively. CONCLUSIONS:The use of a BT illumination guide may serve as a useful and simple adjunct during complex redo surgeries such as secondary thoracoscopic PT. By helping delineate the course of the esophagus, it may improve spatial awareness and facilitate safe dissection even when normal anatomical planes are obliterated by dense adhesions.
INTRODUCTION:Migration of cervical hardware into the esophagus after anterior cervical discectomy and fusion (ACDF) is a rare but serious complication. Most esophageal injuries present within days of surgery, and delayed diagnosis may lead to perforation and deep-neck or mediastinal abscess. Safe, definitive treatment is only possible with implant removal to prevent mediastinitis, aspiration pneumonia, and sepsis. Herein, we present a case of an uncommon late-onset esophageal complication due to metallic plate migration 20 years after ACDF, which was managed using direct suturing and sternocleidomastoid muscle (SCM) flap reinforcement. CASE PRESENTATION:A 63-year-old man who had undergone ACDF 20 years previously was admitted to a local hospital with fever, dysphagia, and neck pain. Based on the CT findings, the patient was diagnosed with esophagitis secondary to a foreign body in the cervical esophageal diverticulum. An endoscopic study showed that a metallic plate for the ACDF was exposed in the posterior wall of the cervical esophageal diverticulum. The patient was then transferred to our hospital for surgical treatment. During surgery, the metallic plate was found to be completely exposed to the lumen of the diverticulum of the cervical esophagus. After opening the posterior wall of the esophagus, the metallic plate was successfully removed and the diverticulum was resected. The defect in the cervical esophagus was closed and reinforced using an SCM flap. The patient recovered uneventfully and was discharged after radiologic confirmation of unhindered passage of contrast medium and no leakage. Histological examination of the resected diverticulum wall revealed the mucosa and submucosa, but not the muscular layer. CONCLUSIONS:Metallic plate migration into a pseudodiverticulum of the cervical esophagus occurring 20 years after ACDF is a rare but serious complication. The metallic plate was removed and the esophageal diverticulum was closed using direct suturing, followed by reinforcement with an SCM flap.
