Primary yolk sac tumor of the endometrium combined with situs inversus totalis: a case report and literature review.

BACKGROUND: Yolk sac tumor (YST) is a highly malignant germ cell tumor, a majority of which originate from the gonads and are extremely rare from endometrium. CASE PRESENTATION: Here we present a case of a 42-year-old woman suffered from primary pure yolk sac tumor of the endometrium complicated with situs inversus totalis. The patient presented at our hospital with irregular vaginal bleeding. Imageological examination showed a space-occupying lesion in the cervix and the serum Alpha-fetoprotein (AFP) level was significantly high (more than 1210ng/ml). Then she underwent total hysterectomy, bilateral salpingo-oophorectomy and pelvic lymph node dissection. The subsequent postoperative pathological diagnosis was yolk sac tumor arising from the endometrium. Next, the patient was treated with 6 cycles of chemotherapy with Pingyangmycin, etoposide and cisplatin regimen and was alive without evidence of recurrence or distant metastases for 13 months. CONCLUSIONS: This rare disease needs to be differentiated from endometrial epithelial neoplasia and the significant increase in AFP is helpful for diagnosis. Combined with previous literature reports, comprehensive staging laparotomy or maximum cytoreductive surgery complemented by standard chemotherapy can usually achieve a good efficacy.

[Right-sided acute appendicitis in situs inversus totalis. Case report]. / Apendicitis aguda del lado derecho en situs inversus totalis. Reporte de caso.

Background: Acute appendicitis is widely recognized as the prevailing abdominal surgical emergency globally, exhibiting an annual incidence ranging from 96.5 to 100 cases per 100,000 adults. Conversely, situs inversus totalis is a rare anatomical anomaly characterized by the complete reversal of the chest and abdominal organs, occurring in approximately 1 out of every 10,000 to 50,000 individuals. Clinical case: 53-year-old female patient who presented to the emergency department with a complaint of diffuse abdominal pain of colic nature that had persisted for 5 days. The patient referred systemic hypertension, pre-diabetes mellitus type 2, and situs inversus as relevant medical history. A laparotomy procedure was conducted, revealing a perforated appendix located in the right hypochondrium. Additionally, the patient exhibited situs inversus totalis, with the colon positioned in its normal anatomical location. Conclusions: Due to the low incidence of situs inversus totalis, in cases similar to the one presented, the utilization of image studies and laboratory studies is imperative for accurate diagnosis. In the face of diagnostic suspicions and inconclusive paraclinical studies, the most effective approach is to pursue surgical examination and intervention, preferably utilizing laparoscopic techniques.

Introducción: la apendicitis aguda es la urgencia quirúrgica abdominal más frecuente en el mundo, con una incidencia anual de 96.5 a 100 casos por 100,000 adultos. Por otra parte, el situs inversus totalis es un trastorno posicional inverso de los órganos torácicos y abdominales con una incidencia de 1 en 10,000 a 50,000 personas. Caso clínico: paciente del sexo femenino de 53 años que se presentó en sala de urgencias refiriendo dolor abdominal difuso tipo cólico de 5 días de evolución. La paciente refirió hipertensión arterial sistémica esencial, prediabetes mellitus tipo 2 y situs inversus. Se realizó intervención quirúrgica (laparotomía) en la que se encontró el apéndice perforado en hipocondrio derecho y situs inversus totalis con colon en posición anatómica normal. Conclusiones: debido a la baja incidencia del situs inversus totalis, el diagnóstico en casos como el presentado representa un reto en el que el uso de estudios de imagen y laboratorio resulta indispensable. Ante la sospecha diagnóstica y estudios paraclínicos con resultados no concluyentes, la mejor estrategia consiste en la exploración y el manejo quirúrgicos, preferentemente laparoscópicos.

Aphasia due to right hemisphere infarction in a patient with situs inversus after carotid artery stenting: a case report.

BACKGROUND: Situs inversus (SI) is a rare congenital anomaly in which systemic organs and vessels are positioned in a mirror image of their normal positions. An interesting issue regarding individuals with such a condition is whether they also have reversed brain asymmetries. Most of studies on this issue indicate that, similarly to many people with normal visceral alignment, patients with SI have a left hemispheric dominance for language functions. CASE PRESENTATION: We report a rare occurrence of anomalous cerebral dominance for language in a patient with complete situs inversus. The right-handed patient developed aphasia after carotid stenting, and brain magnetic resonance imaging showed cerebral infarction in the right parietal lobe. CONCLUSION: Anomalous cerebral dominance for language and visceral situs inversus in our patient both may result from a single, genetically coded atypicality of developmental gradient.

Anaesthetic implications in situs inversus totalis: A case report.

Situs inversus totalis (SIT) is a rare congenital condition which is characterised by the reversal of orientation of abdominal and thoracic organs where heart is on the right side of the thoracic cavity and liver on the left side, whereas stomach and spleen are on the right side in the abdomen. The reported prevalence of this anomaly is one in 5,000- 20,000 live births. This case reports the anaesthetic management of situs inversus totalis in a 38-year-old male patient, with a history of poorly differentiated adenocarcinoma of the colon, who underwent laparoscopic intervention converted to open nodular excision with incisional hernia repair. The report analyses the anaesthetic implications and challenges associated with situs inversus totalis during surgery, including preoperative evaluation, monitoring techniques and potential complications.

Liver and pancreas transplantation in adult donor and recipients with situs inversus totalis: a case series and review of the literature.

BACKGROUND: Situs inversus totalis is a rare congenital anomaly characterized by a mirror-image orientation of abdominal, and in some cases, thoracic organs. Here, we report our situs inversus totalis transplantation experience and further review liver transplantations in adult recipients and donors with situs inversus totalis. CASE PRESENTATION: We describe three cases with situs inversus totalis. The first case was liver transplantation in a recipient (a 61-year-old Iranian man) with situs inversus totalis, the second was a liver transplantation from a donor (a 52-year-old Iranian woman) with situs inversus totalis, and finally, for the first time, a simultaneous pancreas and kidney transplantation in a recipient (a 26-year-old Iranian man) with situs inversus totalis. In patient one, hepatectomy could be performed according to the standard method and on the basis of preoperative studies. Hepatic vein and arterial anastomosis were performed as in every other patient without situs inversus totalis. To prevent biliary complications, a Roux-en-Y hepaticojejunostomy was performed. In patient two, implantation time, suprahepatic vein, portal vein, arterial, and biliary reconstruction could be done as in any other case without situs inversus totalis. Plication of the right-sided diaphragm and fixation of the falciform ligament was done for our patient. In patient three, systemic drainage was preferred to portal flow for establishing the outflow drainage of the pancreas compared with otherwise normal patients. CONCLUSION: Although situs inversus totalis is a rare condition, our reported techniques are suitable, considering advantages such as easier accessibility, more acceptable placement of the implanted organs regarding vascular variations, and the appropriate location of the allograft in the proximity of other organs.

[Emergent Off-pump Coronary Artery Bypass Grafting in a Patient with Situs Inversus Totalis: Report of a Case].

Dextrocardia associated with situs inversus totalis is a rare congenital anomaly. We herein report a patient with this condition and ischemic coronay artery disease who underwent emergency surgical myocardial revascularization. A 76-year-old man was admitted to our hospital with a diagnosis of unstable angina pectoris. He had incessant ventricular fibrillation attack on the second day of hospitalization. Emergent coronary angiography revealed total obstruction of the anatomically left anterior descending and right coronary arteries, and severe stenosis in the anatomically left circumflex artery. Emergent off-pump coronary artery bypass using saphenous vein grafts was successfully performed. Intraoperatively, the main surgeon continuouly stood on the patient's right side except during anastosiso of a vein graft to the left circumflex artery. Postoperative course was almost uneventful aside from transient renal replacement and prolonged ventilation.

Laparoscopic cholecystectomy and laparoscopic common bile duct exploration for cholecystolithiasis and choledocholithiasis in a patient with situs inversus totalis: A case report.

Situs inversus complicates diagnosis and treatment due to the mirrored organ placement in relation to normal anatomy. This report describes a 78-year-old female patient with situs inversus totalis who underwent laparoscopic cholecystectomy and laparoscopic common bile duct exploration for cholecystolithiasis and choledocholithiasis. Utilizing the "French mirror technique" for port placement, the surgeon adeptly mirrored standard maneuvers with a 2-mm needle forceps in the left hand and a 5-mm forceps in the right in a reversed anatomical setting. This technique maintained familiar hand movements, despite the patient's unique anatomy. The surgeon applied transcystic ductal bile duct exploration, using choledochoscopy for duct exploration and a basket catheter for stone removal. Laparoscopic cholecystectomy and common bile duct exploration through the transcystic ductal route are viable and effective for patients with situs inversus.

Minimally invasive left axillary thoracotomy for the total repair of fallot, dextrocardia, situs inversus and anomalous coronary artery origins.

We present a rare case of tetralogy of Fallot accompanied with dextrocardia, situs inversus and anomalous origin of the right coronary artery. Total repair was accomplished successfully using a minimally invasive left axillary thoracotomy.

A novel homozygous splice site variant in ARL2BP causes a syndromic autosomal recessive rod-cone dystrophy with situs inversus, asthenozoospermia, unilateral renal agenesis and microcysts.

BACKGROUND: This report presents a clinical case of syndromic rod-cone dystrophy due to a splice site variant in the ARL2BP gene causing situs inversus, asthenozoospermia, unilateral renal agenesis and microcysts. The presence of renal agenesis and cryptorchidism expands the clinical manifestations due to ARL2BP variants. The detailed, long-term follow-up contributes valuable insights into disease progression, aiding clinical diagnosis and patient management. CASE PRESENTATION: The male patient complained of photophobia as the first symptom when he was 20 years old followed by nyctalopia, loss of central visual acuity and peripheral visual field ten years later. Genetic analysis identified a likely pathogenic homozygous variant (c.294-1G > C) involving the splicing acceptor site of intron 4. Reported symptoms together with full-field stimulus threshold testing, electroretinogram and advanced multimodal imaging allowed us to recognize the typical characteristics of a mixed retinal dystrophy. Despite the end-stage retinal disease, this patient still retained a useful residual vision at 63 years and had a slow disease progression during the last 5 years of evaluation. DISCUSSION AND CONCLUSIONS: Our findings underscore the variable clinical presentation of ARL2BP variants, emphasizing the importance of a nuanced approach in diagnosing and managing patients. The presence of renal cysts warrants consideration of a differential diagnosis, particularly with Senior-Loken (SLS), Bardet-Biedl (BBS) and Joubert syndromes (JS) but also with Short Rib Thoracic Dysplasia 9, highlighting the need for careful phenotypic evaluation in these cases.

A case report of perihilar cholangiocarcinoma in a patient with situs inversus totalis.

Situs inversus totalis is a rare congenital malformation in which organs are positioned in a mirror-image relationship to normal conditions. It often presents with vascular and biliary malformations. Only a few reports have pointed out the surgical difficulties in patients with situs inversus totalis, especially in those with perihilar cholangiocarcinoma. This report describes a 66-year-old male patient who underwent left hemihepatectomy (S5, 6, 7, and 8) with combined resection of the caudate lobe (S1), extrahepatic bile duct, and regional lymph nodes for perihilar cholangiocarcinoma with situs inversus totalis. Cholangiocarcinoma was mainly located in the perihilar area and progressed extensively into the bile duct. Surgery was performed after careful evaluation of the unusual anatomy. Although several vascular anomalies required delicate manipulation, the procedures were performed without major intraoperative complications. Postoperatively, bile leakage occurred, but the patient recovered with drainage treatment. The patient was discharged on the 29th postoperative day. Adjuvant chemotherapy with S-1 was administered for approximately 6 months. There was no recurrence 15 months postoperatively. Appropriate imaging studies and an understanding of unusual anatomy make surgery safe and provide suitable treatment for patients with situs inversus totalis.

Coronary Artery Bypass Grafting in a Patient With Multivessel Disease and Dextrocardia With Situs Inversus Totalis.

Dextrocardia with situs inversus totalis is a rare hereditary condition characterized by reversed orientation of the major thoracic and abdominal organs. Though dextrocardia itself is not believed to increase the risk of coronary artery disease, the workup and surgical management of patients with this condition may be technically challenging to heart team clinicians. This report describes the case management of a high-risk 56-year-old man with dextrocardia who presented with multivessel coronary artery disease.

BILE DUCT INJURY REPAIR IN A PATIENT WITH SITUS INVERSUS TOTALIS.

BACKGROUND: Bile duct injury (BDI) causes significant sequelae for the patient in terms of morbidity, mortality, and long-term quality of life, and should be managed in centers with expertise. Anatomical variants may contribute to a higher risk of BDI during cholecystectomy. AIMS: To report a case of bile duct injury in a patient with situs inversus totalis. METHODS: A 42-year-old female patient with a previous history of situs inversus totalis and a BDI was initially operated on simultaneously to the lesion ten years ago by a non-specialized surgeon. She was referred to a specialized center due to recurrent episodes of cholangitis and a cholestatic laboratory pattern. Cholangioresonance revealed a severe anastomotic stricture. Due to her young age and recurrent cholangitis, she was submitted to a redo hepaticojejunostomy with the Hepp-Couinaud technique. To the best of our knowledge, this is the first report of BDI repair in a patient with situs inversus totalis. RESULTS: The previous hepaticojejunostomy was undone and remade with the Hepp-Couinaud technique high in the hilar plate with a wide opening in the hepatic confluence of the bile ducts towards the left hepatic duct. The previous Roux limb was maintained. Postoperative recovery was uneventful, the drain was removed on the seventh post-operative day, and the patient is now asymptomatic, with normal bilirubin and canalicular enzymes, and no further episodes of cholestasis or cholangitis. CONCLUSIONS: Anatomical variants may increase the difficulty of both cholecystectomy and BDI repair. BDI repair should be performed in a specialized center by formal hepato-pancreato-biliary surgeons to assure a safe perioperative management and a good long-term outcome.

Laparoscopic retroperitoneal lymphadenectomy for ovarian mixed germ cell tumor in a patient with situs inversus totalis; reporting the first case worldwide with literature review and in silico analysis.

BACKGROUND: Situs inversus totalis (SIT) is a rare autosomal recessive inheritance at which the abdomino-thoracic organs are mirror-image transposed. Germ cell tumors originate from the primitive germ cell of the ovary and testis. CASE REPORT PRESENTATION: A rare association between malignant ovarian mixed germ cell tumor and SIT was presented in a 32-years-old Egyptian female, successfully treated with laparoscopic total abdominal hysterectomy, right salpingo-oophorectomy, and retroperitoneal lymphadenectomy (laparoscopic retroperitoneal lymphadenectomy) of both sides. This case is considered the first of its kind worldwide. CONCLUSION: SIT may be associated with malignant ovarian germ cell tumors. Surgical intervention could be done laparoscopically.

Successful Endoscopic Treatment of a Pancreatic Pseudocyst in a Patient with Situs Inversus Totalis and Upper GI Duplication.

BACKGROUND Duplication of the gastrointestinal tract is a rare congenital malformation that can develop in any part of the digestive tract. These duplications may be asymptomatic into adult age. Situs inversus totalis is a rare congenital anomaly characterized by a mirror transposition of thoracic and abdominal organs. We present a case of a pancreatic pseudocyst in a patient with a combination of situs inversus totalis and doubling of the esophagus, stomach, and first part of the duodenum. CASE REPORT A 64-year-old woman presented with epigastric pain. Abdominal computed tomography revealed a pancreatic pseudocyst and a previously identified duplication of the esophagus, stomach, and duodenum with situs inversus totalis. The patient underwent esophagogastroduodenoscopy (EGD) with endoscopic ultrasonography for pseudocyst drainage. During EGD, a bifurcation of the esophagus was found. Duplication of the esophagus, stomach, and first part of the duodenum was evident on further advancement. A week later, there was repeated filling of the pseudocyst with a liquid component, and the patient underwent cystogastrostomy with stenting. Five months after discharge, the stent was removed without complications. CONCLUSIONS Duplication of the gastrointestinal tract and situs inversus totalis are very rare congenital malformations that require early diagnosis. While situs inversus totalis does not represent any medical disadvantage, physicians should be aware of abnormal anatomy before procedures to prepare specialists for this in case of the need for special techniques. Endoscopic treatment of pancreatic pseudocysts is safe and effective even in such rare cases. The use of endoscopic methods also minimizes intervention and decreases the length of the patients' stays in the hospital.

Congenital hemivertebrae combined with situs inversus totalis: A rare case report.

RATIONALE: Situs inversus totalis is a rare malposition of organs that typically involves lesions in the respiratory, circulatory, or urinary systems. Cases of congenital hemivertebrae combined with situs inversus totalis are extremely rare and have limited reports. PATIENT CONCERNS: We report a 2.5 years old girl with 2 congenital hemipyramids and complete visceral inversion who ultimately underwent hemilaminectomy. DIAGNOSIS: Congenital hemivertebrae combined with situs inversus totalis. INTERVENTION: The patient underwent hemilaminectomy. OUTCOMES: The spinal deformity was corrected. LESSONS: For patient with spinal deformities combined with situs inversus totalis, surgery can be an effective treatment method. But we also need to be vigilant about the dysfunction of various systems.

Association between types of abdominopelvic cancer in patients with situs inversus total: Systematic review.

BACKGROUND: Situs inversus is a rare congenital anatomical variant that involves a group of anomalies regarding the arrangement of intrathoracic and intraabdominal organs. Being able to find in the abdominal region the liver, gallbladder, inferior vena cava, and head of the pancreas and ascending colon on the left side of the abdomen, while on the right side there is the spleen, the stomach, the body of the pancreas, the ligament of Treitz, descending colon among others. In this same way, the thoracic organs, lungs and heart, are changed in their position in a mirror translocation. METHODS: We systematically searched MEDLINE, Web of Science, Google Scholar, CINAHL, Scopus, and LILACS; the search strategy included a combination of the following terms: "Situs inversus," "Situs inversus totalis," "Cancer," "Neoplasm," "Abdominopelvic regions," and "clinical anatomy." RESULTS: Within the 41 included studies, 46 patients with situs inversus who had cancer, in addition to being found in this organ and in these regions, we also found as a result that the majority of the studies in the research were in stage II; finally, no one study could assert the direct relationship between the situs inversus totalis and the cancer. CONCLUSION: If our hallmarks could make us think that more exhaustive follow-up of the stomach and other organs should be carried out in these patients, there could also be other predisposing factors for cancer, which is why more studies are suggested to give future diagnostic and treatment guidelines treatment.