Congenital orbital teratomas are extremely rare, usually benign neoplasms, comprised of cells originating from all three germ cell layers. Clinically the tumor appears solid, most of the times is intraconal and presents as a rapidly growing mass leading to a massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision. To prevent these complications, tumor excision usually involves enucleation or even orbital exenteration.We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up.Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case. A teratoma is defined as a congenital neoplasm that contains elements deriving from all three germinal cell layers . Teratomas represent about 6–10% of pediatric tumors, most commonly located in the testes, ovaries or the retroperitoneum [A 1-day old female newborn presented with severe unilateral proptosis  , 6, 12.Although not many cases have been reported in literature, antenatal screening of congenital orbital teratomas is possible. The screening tool of choice is that of ultrasound which can detect a mixed sonographic image made up of cystic and solid complex masses with significant vascularization. If further study regarding the location, extent and nature of the tumor is needed, a fetal MRI can be considered .Fortunately, most of the congenital orbital teratomas are benign and therefore there is no need for post operative adjuvant radiotherapy and/or chemotherapy. Its prognosis is considered very good if treated early in the postnatal life, especially if complete surgical excision is achieved. Due to the fact that there have been a few cases of recurrence and, anectodically, of malignant transformation, there is the need of alertness and regular follow ups, ensuring that should a recurrance occurs, it will be addressed promptly .Treatment is by complete surgical excision, if possible. Due to the massive proptosis on presentation, the surgery is usually carried very early postpartum. The goal of early treatment is primarily to try and save the vision of the affected eye, if possible and, secondarily, to salvage the globe. Unfortunately, due to the massive proptosis and the propensity of the tumor to encase or adhere to the optic nerve, there is irreversible ischemic damage even at the earliest possible postpartum intervention. Our case had a nonreactive pupil and evidence of severe anterior segment ischemia manifesting as iris neovascularization when the child was examined at 24 h postpartum.Every effort should be made to salvage the globe. Even when the outlook seems dismal for globe preservation due to the dramatic clinical presentation, our case demonstrates that complete en bloc excision of the tumor was possible with a transconjunctival 360- degrees limbal peritomy approach without the need to rely on more aggressive orbital or craniofacial procedures. Disinserting one of the recti muscles provides enough space to dissect safely around the tissue plane of the tumor. Another learning aspect from this case is to avoid the urge to sacrifice the stretched, “redundant” upper and lower eyelid and conjunctival tissue that is often present after tumor removal. Placing full thickness forniceal sutures to tuck the conjunctiva and a reversible suture tarsorrhaphy and allowing the eyelid tissues to retract into their normal dimension and tone avoids further unnecessary iatrogenic trauma and may yield a better postoperative outcome.Review of the most recent literature post-1990) showed a tendency towards more aggressive surgery  than globe sparing surgery 90 showed–18.. TheGlobe sparing surgery encourages adequate orbito-facial development leading to better facial symmetry and improved cosmesis. The fitting of a custom-made scleral shell is easier than the handling and the aesthetics of the larger, heavier, and less physiological orbito-facial prostheses that are typically used after orbital exenteration especially in such young patients. Another disadvantage of orbital exenteration surgery is that, quite often, further reconstructive craniofacial surgery and rehabilitation depending on midfacial growth is required .An interesting point about our case is the significantly long postoperative follow up period that we provide. The patient’s facial development can be followed at different ages, providing valuable feedback on how the periorbital tissues develop over the years after tumor excision. There has been no recurrence, the postoperative period has been uneventful, and the overall aesthetic result is reasonable. The relative orbital volume loss and the contour asymmetry of the upper eyelid that was observed can be addressed with hyaluronic acid gels and/or upper eyelid surgery for further improvement in cosmesis in the future.The value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.Congenital orbital teratomas are rapidly growing masses leading to massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision.If treated early in the post-natal life, prognosis is considered good.Every effort to salvage the globe should be made to achieve the best possible orbito-facial development.Transconjunctival approaches with disinsertion of an extraocular muscle provide sufficient exposure to remove even massive tumorsIt is best to avoid removing “redundant” eyelid and conjunctival tissue after tumor removal and allow the tissues to retract into their normal dimensions over time.


A 29-year-old male developed acute onset severe shortness of breath and fevers and was found to have a 17 cm anterior mediastinal mass with immature teratoma and possible mixed germ cell tumor on biopsy. He remained hospitalized during neoadjuvant cisplatin-based chemotherapy due to compressive symptoms from his mass and neutropenic fevers. Despite 3 cycles of therapy, his tumor mildly increased in size. After multidisciplinary discussion, he underwent urgent en bloc resection with a right hemi-clamshell incision. His postoperative course was uncomplicated and he was discharged to home within a week. His final pathology demonstrated mixed germ cell tumor. Mediastinal germ cell tumors (GCT) are uncommon, and of these, immature teratomas are even more rare, comprising 1.8% of all mediastinal teratomas. These tumors are more prevalent in males and typically are diagnosed before the age of 40 . GCTs arWe report herein a case of chemotherapy-resistant immature teratoma in a patient who was unable to be discharged from the hospital due to compressive symptoms of his large mediastinal mass.A 29-year-old male with history of Kawasaki’s disease presented with 1 week of cough, dyspnea on exertion, weight loss and fevers. His workup revealed a WBC of 13 K/μl (ref. 3.7–11.1 K/μl) and a 17 × 16 × 17.3 cm heterogenous mass with calcifications in the right hemithorax causing compression of the right lung and bronchus and left-sided mediastinal shift . An echoPleural effusion analysis was negative for malignancy and cultures were negative. Ultrasound-guided biopsy of his mediastinal mass demonstrated immature teratoma with focal nonspecific AFP stain, and thus mixed GCT was unable to be ruled out. Scrotal ultrasound and metastatic workup were negative for other lesions. His admission serum markers were significant for AFP of 5756 ng/ml (ref 0.0–8.3 ng/ml) and LDH 343 U/l (ref <270 U/l). His beta HCG was within normal limits. He remained as an inpatient for his entire workup and eventual chemotherapy due to significant oxygen requirements and inability to mobilize or lay supine.He was started on a chemotherapy regimen of cisplatin, etoposide and ifosfamide (VIP) as an inpatient. His follow-up imaging demonstrated increase in mass size to 18 × 16 × 17 cm with increasing mass effect despite improvement of his serum tumor markers—AFP 243 ng/ml, LDH 347 U/l—at the end of 3 cycles. He continued to require inpatient telemetry admission throughout his neoadjuvant therapy due to neutropenic fevers, pancytopenia and compressive symptoms from his mass.After multidisciplinary tumor board discussion and consultants within national cancer centers, there were differing opinions regarding further chemotherapy versus surgical resection.Our facility, which is a major regional cancer center, was consulted and recommended surgical resection, since his symptoms persisted and the tumor increased in size despite chemotherapy. He was transferred to our facility and underwent urgent resection with cardiopulmonary bypass on standby. His operation consisted of median sternotomy and right hemi-clamshell incision for mass and right middle lobe en bloc resection. The mass was compressing, but not invading, the heart or pericardium. No other lesions were identified. The patient received 3 units of packed red blood cells and 3 units of fresh frozen plasma intraoperatively. Estimated blood loss was almost 2 l. Final specimen weight was 4.6 kg. His final pathology was mixed GCT with a predominant teratoma component. There was a positive margin at the staple line of the right middle lobe wedge resection .The patient was extubated in the operating room and transferred to the intensive care unit for close monitoring. He was transferred out of the unit on postoperative day 1. The remaining postoperative course was uncomplicated and he was discharged on postoperative day 6 with no oxygen requirements and good mobility.et al. concluded that complete resection after neoadjuvant therapy gave patients a favorable prognosis [et al. identified that patients, such as ours, who have increased tumor size despite decreasing tumor marker levels pose a challenge in determining whether systemic therapy is effective. This is possibly due to changing tumor characteristics and the rare somatic-type malignant transformation within the tumor during neoadjuvant therapy [This patient ultimately had a large mixed GCT not responsive to chemotherapy. Unlike mature mediastinal teratomas, immature teratomas behave aggressively, particularly in adult patients, and generally are not responsive to chemotherapy. Thus, complete surgical resection is crucial . Hiroshirognosis . The pedrognosis . Arai et therapy , 7. Tera therapy . This pa therapy .This patient’s tumor was much larger than the reported average mediastinal GCT of 10 cm and 415 g . We alsoMediastinal GCTs in adults are extremely rare. When these malignancies do not respond appropriately to systemic therapy, we recommend immediate radical surgical excision be performed at regional centers of excellence with adequate perioperative support.No conflicts of interest.


Intravascular lobular capillary haemangioma is a rare benign intravascular tumour, especially in large vessels. This is the report of a case and associated literature review.en bloc tumour excision was performed, followed by reconstruction of the SVC with an L shaped, ringed polytetrafluoroethylene (PTFE) prosthesis. Histopathology revealed the presence of an ILCH with free margins. A review of the literature identified 64 cases of ILCH to date, all of which underwent total resection. When reported, no recurrences were found during follow up.This is the report of the first case of an intravenous lobular capillary haemangioma (ILCH) of the superior vena cava (SVC). A 30 year old female presented with a collateral thoraco-abdominal venous circulation. Chest computed tomography angiography, thoracic magnetic resonance imaging, and positron emission tomography revealed an intraluminal SVC tumour extending from the left brachiocephalic venous trunk to the distal third of the SVC. No pre-operative biopsy was indicated. An In this case, the ePTFE reconstruction of the SVC must be checked regularly for any adverse events. Although ILCH is a benign tumour with no risk of recurrence, regular surveillance is advised. •Report of the first case of intraluminal capillary haemangioma involving the superior vena cava.•Literature review of intraluminal capillary haemangioma.•en bloc tumour excision followed by an L shaped prosthetic repair.Successful  The ISSVA Classification describes two main types of malignant vascular tumours: angiosarcoma,  and epithelioid haemangio-endothelioma. Lobular capillary haemangioma (LCH), also known as pyogenic granuloma, is one of the many benign vascular tumours. It occurs mainly in the skin and subcutaneous tissue of the upper limb, neck, and head. Intravascular lobular capillary haemangioma (ILCH) is a subtype of LCH and only few cases have been reported in the literature.en bloc resection of the tumour.This is the first case of intravenous lobular capillary haemangioma in the superior vena cava (SVC) reported in the literature. A technique is described for reconstruction of the SVC using an inverse L shaped synthetic graft, after an A 30 year old female, without significant medical history, presented with recent aggravation of a thoraco-abdominal venous circulation developed during her pregnancy, five years earlier. She had no other complaint or symptom. Clinical abdominal and cardiovascular examinations did not reveal any specific clinical signs of portal hypertension or SVC syndrome.Duplex ultrasound (DUS) showed dilatation of collateral veins, while the IVC was patent. Chest computed tomography angiography (CTA)  demonstrated an intraluminal heterogeneous mass in the SVC, extending from the left brachiocephalic venous trunk to the distal third of the SVC A. No othen bloc resection of the tumour through a median sternotomy. The SVC was resected from its junction with the right atrium without myocardial breach and taking both left and right brachiocephalic veins. The venous reconstruction consisted of a 16 mm diameter, ringed, expanded polytetrafluoroethylene (ePTFE) prosthesis  sutured distally to the right atrium junction and proximally, perpendicularly on an 8 mm diameter ringed ePTFE prosthesis, connecting both right and left venous confluences , the final diagnosis was an ILCH in the SVC, also known as pyogenic granuloma.Pathological examination showed a tumour measuring 55 × 34 × 25 mm, filling the SVC lumen A and B. A systematic search was performed of the Medline database from 1979 to 2020 by a combined search strategy of MeSH terms . All titles and abstracts collected from the search strategy were screened for relevance. The first 20 related items of all relevant articles were scanned for other potentially relevant studies. Full texts of all relevant articles were obtained and reviewed for relevance. The reference lists of each article were scanned for other potentially relevant studies. The systematic search identified 43 full text English studies, one full text German study, and one full text Korean study, including 39 single case reports, two case series, and four reviews, corresponding to a total of 64 cases.Ten cases (15.6%) were symptomatic, with symptoms such as abdominal pain, limb oedema, or pleural effusion, six (9.4%) were incidental findings after routine examinations, and 48 (75%) presented with nodules, pain free or not. The male/female ratio was 10:9 and the median age was 44 years old .30 in The treatment was not reported in two cases (3.1%). Total resection was the chosen treatment for 61 cases (98.3%), only one case (1.7%) had a biopsy and regressed spontaneously. Follow up was reported in 22 cases and all had no recurrence, from two months (see reference ,Primary SVC tumours are rare. Three cases of epithelioid haemangio-endothelioma of the SVC and 12 leiomyosarcomas have been reported in the literature.LCH was described for the first time in 1979.Despite being a benign tumour, surgical removal of ILCH is the first choice approach to make a histological diagnosis and to prevent tumour related complications, such as SVC occlusion or thrombosis, local compression and pulmonary embolisation. As SVC resection is not a frequent procedure, the replacement strategy remains controversial. The main risk is graft thrombosis, occurring between one and five months after implantation.In this case, an ePTFE vascular graft was chosen. As shown in Despite the excellent long term prognosis of the benign characteristics of the tumour, immediate post-operative anticoagulant treatment and close follow up with DUS of the graft will be mandatory to assess graft patency.en bloc resection of an SVC ILCH associated with SVC prosthetic reconstruction.This case reports successful management of


Giant dermatofibrosarcoma protuberans (DFSP) is a very rare dermal sarcoma whose diagnosis and management are important because of the high local recurrence but low metastatic potential. Complete surgical excision of giant DFSP in a single stage is difficult but has a high cure rate.A 47-year-old man presented with a gradually increasing large (18 x 15 x 7 cm) DFSP in the epigastrium. A 3 cm circumferential wide local excision (WLE) with microscopic tumor-free margin confirmed by frozen section was performed. Immediate single staged tension-free primary closure of resultant defect was done on the principle of abdominal wall reconstruction (AWR) in ventral hernia repair. This technique of anterior component separation and bridge meshplasty is functional, avoids multiple surgeries, is cost-effective, and can be done in a resource-limited setting in developing countries.A multidisciplinary and integrated surgical approach to treat giant DFSP over epigastrium, by three-dimensional WLE and immediate AWR with anterior component separation technique (CST) and bridging meshplasty, can be of immense help in managing such rare cases in developing countries. Dermatofibrosarcoma protuberans (DFSP) are rare, cutaneous mesenchymal neoplasm with an annual global incidence of 0.8-4.5 cases per million persons. Hoffman named this rare clinical entity DFSP which accounts for 1-6% of soft tissue sarcomas and 18% of cutaneous soft tissue sarcomas . Large-sA 47-year-old man presented with a slow-growing large tumor in the epigastrium for four years with occasional pain. Clinically, a 15 x 14 cm large, lobulated, ﬁrm, non-tender tumor covered by shiny telangiectatic skin was present over the anterior abdominal wall of the epigastrium Figure . Radio iAfter primary peritoneal closure, a preperitoneal polypropylene mesh was sutured to the defect margin of the posterior rectus sheath as a bridging mesh for a tension-free repair Figure . Then, bDFSP is a superficial fibroblastic dermal sarcoma of intermediate malignancy. The pathogenesis is still uncertain but 10-20% have a history of trauma and in 80-90% of cases, there is somatic mutation by a proto-oncogene COL1A1-PDGFB. This is formed by translocation of 17 and 22 chromosomes, which causes activation of the tyrosine-kinase pathway leading to PDGFβ overexpression .DFSP mostly presents as a slow-growing, painless, superficial, lobulated, and protuberant tumor over the trunk 40-50%) among the 20-50 years age group. The overlying skin is thin, shiny, telangiectatic, and adherent to the tumor ,5.0% among Distinctive histopathological findings of DFSP are spindle-shaped neoplastic cells arranged in a storiform or cartwheel pattern in the dermis, with neoplastic tentacles extending into the surrounding subcutaneous tissues . Hence, Very few studies are reported on the management of abdominal wall DFSP. Complete surgical excision with histologically negative margins is the standard treatment of DFSP . This caAfter WLE, the reconstruction of the resultant full-thickness defect aims to protect the abdominal viscera, restoring the functional integrity of the abdominal wall along with its aesthetic appearance and hernia prevention ,10. RecoAWR by CST is a recently developed technique of ventral hernioplasty with the principle of re-establishing a functional abdominal wall with autologous tissue repair. This technique can be modified and used in the reconstruction of post-oncologic full-thickness abdominal wall defects after abdominal DFSP excision ,10. So pIn the reported case, the resultant abdominal wall defect was repaired by AWR with anterior CST and bridge meshplasty for a single staged, tension-free primary functional reconstruction, customized as per the position of the defect. This technique is functional, cost-effective, avoids multiple surgeries, and can be done in a resource-limited setting even by experienced general surgeons. The aim was to simplify the surgical approach, decrease the operative time, cost, morbidity of multiple surgeries, and thus infection rate. So by the preoperative marking of tumor extent with MRI, WLE with 3 cm clear lateral and deep margins, preoperative frozen section histology along with extra shaved margins, aids in achieving oncological negative margins resection. A functional reconstruction can be achieved by AWR by anterior CST and meshplasty.In recurrent lesions or positive margins, re-excision followed by adjuvant radiotherapy or targeted therapy with tyrosine kinase inhibitors like imatinib is advocated . The proAwareness of DFSP, a rare surgically curable but locally aggressive dermal sarcoma, is important for a proper diagnosis and management to prevent recurrences. An integrated approach, involving surgery, histopathology, and immuno-radiotherapy, can act as an elixir for making DFSP a curative entity. Anterior CST with meshplasty is an accepted hernia repair technique that can be used for immediate AWR after oncological resection of DFSP with good results by general surgeons in resource-limited places. So to improve the functional and aesthetic outcome without compromising on oncological safety and hernia prevention, a modified three-dimensional excision of the tumor with deep fascia and 3 cm circumferential safe surgical margin, confirmed by frozen section with immediate reconstruction by CST with mesh reinforcement is recommended by us in full-thickness abdominal wall defects following DFSP resection.


Chiari malformations (CMs) are a group of disorders involving deformities of the posterior fossa and hindbrain. There are seven types of CMs: 0, I, 1.5, II, III, IV, and V. CMIII is a very infrequent disorder characterized by low occipital or superior cervical encephalocele and inferior displacement of the brainstem. Here we present a unique case of CMIII associated with cleft palate, abnormal midbrain, and abnormal corpus callosum. CMIII is a very rare condition, which needs to be reported. This study primarily aims to compare our case to other cases of CMIII. We did not find another case with cleft palate and CMIII. There are only a few cases reported of CMIII. That is why it is vital to report each of these cases. Among reported CMIII cases, our case is unique regarding the co-occurrence of cleft palate. There seems to be an embryological link between these two conditions. However, cleft palate is a relatively common congenital defect, which means that the co-occurrence could be just a coincidence as well. Further research is warranted to broaden the information of this extremely rare syndrome. The primitive brain has three main parts: the forebrain, the midbrain, and the hindbrain. The hindbrain later divides into the metencephalon and the myelencephalon. The metencephalon becomes the pons, and the myelencephalon becomes the cerebellum and medulla . Chiari Deformities of the posterior fossa and hindbrain define CMs. Depending on the type of CM, three is herniation of the brainstem and cerebellum through the foramen magnum and the cervical spinal canal . There aCM type III (CMIII) is defined either by a low occipital encephalocele and superior cervical encephalocele. There is also inferior brainstem displacement, but it also can be absent. Displacement of cerebellar tissue posteriorly and inferiorly is also seen . The natInitial presentationA 32-year-old Hispanic woman on her third pregnancy delivered a female child at 38 weeks by cesarean delivery. There were only three routine checkups during her pregnancy. The first control was at four months, and the mother found out she was pregnant approximately six weeks after conception. The mother consumed no prenatal vitamins, and folic acid was used after the first control.Prenatal monitoring was abnormal for encephalocele, cleft palate, and single umbilical artery, which were observed in the second-trimester sonogram performed at four months of pregnancy. The mother was obese throughout the pregnancy, and group B streptococcus was unknown. During delivery, meconium-stained amniotic fluid was noted. The Apgar score was 7/9 and 7/9 at 1 minute and 5 minutes, respectively.The neonate was then transferred to the neurology/neurosurgery neonatal intensive care unit for further management.Physical examinationThe head circumference was 32.5 cm above the encephalocele and 36 cm across the encephalocele. The eyes have small palpebral fissures bilaterally. The pupils were round and symmetric. Regular heart rhythm without heart murmurs was heard. A normal respiratory pattern was observed.The extremities appeared well perfused. However, contractures in the upper and lower extremities were noted. The skin did not show a stigma of neurological disease. The neurological examination showed that the infant was easily responsive to tactile stimuli and had some extremities movement. The eyes opened spontaneously, and there was not an apparent visual or auditory problem.Cranial nerves I-XII were intact. The arms and legs were held in a flexed position. There was an increased tone of the appendicular extremities, mainly in both legs. Regarding the sensory system, the infant responded easily to tactile stimuli.Diagnostic workupMagnetic resonance imaging (MRI) findings include low occipital and high cervical meningoencephalocele. There was hemosiderin deposition scattered throughout the herniated brain parenchyma, indicating prior hemorrhage. There was left parietal encephalocele, right periventricular areas of encephalomalacia, T1 hyperintensity in the median eminence, and cleft on the hard palate; only the genu of the corpus callosum was observed. The midbrain was abnormal. The third and fourth ventricles were not seen. Figure Figure Follow-upThe neonate was breathing normally on room air, but she started having apneustic episodes on day 11 of birth. She was put on nasal intermittent positive pressure ventilation and then intubated. The neurology team was consulted and suggested that the apneustic episodes could be seizure-related. Hence, she was administrated prophylactic medication (levetiracetam). The encephalocele did not allow the collocation of the electroencephalogram leads.The encephalocele was covered with a dressing that was changed daily. A ventricular reservoir was placed on the right side of the head for extraction of the cerebrospinal fluid (CSF).The neurosurgery team was consulted, which decided not to operate because the brainstem was embedded in the encephalocele.Impression and planThe patient was diagnosed with CMIII. In addition, abnormalities of the corpus callosum and midbrain, and clef palate, which could be related or unrelated, were noted. Based on the findings, the neurosurgery team continued with the decision of not operating. Given her significant, irreversible, and devastating diagnosis and multiple complex congenital anomalies, the prognosis is poor. The neurological damage is progressive and permanent to a degree of absence of voluntary action and purposeful cognitive ability to interact in a meaningful way with her environment.The neurology team will support the family’s decision to not resuscitate (DNR) status and withdraw life support if they choose to. Currently, the neonate is on mechanical ventilation, waiting for the family’s decision to withdraw life support and DNR status.In this section, we will discuss the classification, pathogenesis, clinical features, and management of CMIII, and associate these findings with our case.ClassificationTable Our patient had the features of CMIII, with low occipital/upper cervical encephalocele, cleft palate, an abnormal midbrain, and partial agenesis of the corpus callosum.Pathogenesis/embryologyThe etiology of CMIII is mainly unknown. However, there is a theory that an incorrect neurulation during the ventricle extension causes an imperfect formation of the occipital area. Later, there is a prolapse of the cerebellum and the brainstem . AnotherOur patient also had a cleft palate. Embryologically, the palate forms from mesoderm tissue and migration of neural crest cells. At week 4, the frontonasal prominence divides in the medial and lateral nasal processes. At week 6, the lateral nasal process fuses with the maxillary process bilaterally, forming the lower lip . A fusioBeyond sharing a mesoderm origin, it is difficult to establish an appropriate embryological link between CMIII and a cleft palate.Clinical presentationOnly the genu of the patient’s corpus callosum was formed, the midbrain was abnormal, and the venous system was thought to be abnormal, but in the final reading, there were no abnormalities. According to Castillo et al., partial agenesis was reported in 6/9 cases of CMIII, aberrant venous drainage was observed in 4/9 cases of CMIII, and abnormal midbrain was seen in 8/9 cases [According to Ivashchuk et al., only 57 CMIII cases have been reported . CMIII iThe patient’s brainstem was embedded in the encephalocele, discouraging the neurosurgery team from performing the surgery. The most common features of CMIII are highlighted in Table Cleft palate is a relatively common birth defect observed in ½,000 cases, and the co-occurrence of CMIII and cleft palate could only be a coincidence . BecausePrognosis and treatmentCMIII is not necessarily fatal. Some patients may have productive lives . HoweverCMIII is a very rare syndrome. It has a very low prevalence; therefore, it is essential to report it. The main feature of CMIII is the low occipital/upper cervical meningoencephalocele. However, our patient also presented with a cleft palate, abnormal midbrain, and partial agenesis of the corpus callosum.Most of the features of our patient have been reported except cleft palate. However, cleft palate is a relatively common congenital defect, which means that the co-occurrence of CMIII and cleft palate could be just a coincidence. The embryology of CMIII is not well established; therefore, it is difficult to find an embryological link with cleft palate.CMIII cases need to be reported. This is a rare condition, which limits physicians because of the lack of information on CMIII.


Echinococcus species cause hydatidosis, which is a verminosis. Echinococcus vogeli results in polycystic hydatid disease, and wild dogs are the definitive hosts. In fact, wild dogs and rats are the most common hosts. The transit of Echinococcus eggs through the liver and lungs is hypothesized to result in hydatid cyst formation in the subcutaneous tissue. In 1.5 percent of patients with hydatidosis, hydatid cysts of the subcutaneous tissue have been documented. They ranged from 0.6 percent to 2.6 percent. We here report a case of hydatid cyst of the abdominal wall which was surgically treated. A 30-year-old lady had been experiencing pain associated with lump in her right lower abdomen for three months. On clinical examination, an enlargement in the left side measuring 4 x 3 cm was discovered. Imaging was performed preoperatively to rule out other differential diagnoses. Ultrasound was performed, followed by computed tomography and magnetic resonance imaging, which revealed multilocular cystic mass measuring 9 x 8.5 x 4.7 cm along the right lower anterior abdominal wall muscles (with cysts inside cysts), which suggested hydatid cyst. Histopathology confirmed the diagnosis of hydatid cyst, after the mass was surgically removed. Treatment with albendazole was continued. Hydatid cyst of the subcutaneous tissue is uncommon, with only a few occurrences recorded in the literature. This study describes a case of hydatid cyst in an uncommon place. Imaging confirmed the diagnosis, and the tumour was surgically removed. It ruptured during surgery and was successfully treated with hypertonic saline and albendazole. Then it was adequately managed. Given that subcutaneous hydatid cyst is rare, it's vital to keep in mind that it can be a possible cause of abdominal wall mass. Specific imaging test is essential to correctly locate and remove it. It must be treated with anthelmintic before surgery, in order to reduce the risk of contamination due to cyst rupture during surgery. Subcutaneous hydatid cyst should be considered one of the differential diagnoses for soft tissue masses, in particular in patients living in endemic areas. To rule out alternative possibilities, proper imaging test is essential. The treatment of choice is complete excision. Echinococcus species produce hydatidosis, which is a verminosis. Echinococcus vogeli causes the polycystic kind, and the wild dog is the definitive host. Wild canines and rats are the most common hosts . The traPatient information: a 30-year-old woman with a known hydatid cyst. In Yamen, where she lived in 2000, she had a history of liver hydatid illness. She has no recollection of being treated or not. On July 10, 2021, she appeared to the surgery clinic. She had been suffering from abdominal pain and swelling around the umbilicus for the past seven years. She also has a lump in her right lower abdomen, which she says is related to pain that started three months ago. There have been no changes in bowel habits, nausea, or vomiting. There was no fever, no loss of appetite, no weight loss, and no night sweats. There has been no previous hospitalization. A history of CS from 5 years ago was mentioned in previous surgeries. A previous medical history included a diagnosis of hepatic hydatid disease in the year 2000, medical termination of missed miscarriage February 2021.Clinical findings: examination showed that vital signs were within normal range, the patient was alert, conscious, and oriented. The abdomen was soft and lax, no guarding, no rigidity. An infraumbilical hernia was discovered, as well as a 4 x 3 cm bulge on the left flank with neither erythema nor discomfort. Her laboratory results were normal , and serology was negative.Timeline: on July 10, 2021, the patient came to the surgery clinic. The patient´s medical history and examination were completed. The result of laboratory tests the next day were normal. The diagnosis of a multiloculated cystic mass along the right lower anterior abdominal wall muscles (with cysts inside cysts) and a liver calcified cyst measuring 9 x 8.5 x 4.7 cm was confirmed by ultrasound (US) and CT abdomen. An MRI abdomen was done on July 21, 2021, which yielded the same findings. The patient was taken to the hospital for surgical removal of a hydatid cyst in the abdomen wall on August 15,2021. The patient stayed in the hospital for three days before being discharged in a stable condition on anti-helminthic analgesia on August 19, 2021. She was followed in infectious disease and surgery clinic 1 week after discharge. Both visits were unremarkable.Diagnostic assessment: a CT abdomen was performed outside in July 2021 , metoclopramide 10 mg TID, omeprazole 40 mg once daily. Infectious disease team was consulted regarding hydatid cyst treatment. They started her on albendazole 400 mg PO BID and recommended to continue albendazole 400 mg BID PO for one month postoperatively.Follow-up and outcome of interventions: she was admitted to the hospital and stayed for three days. The hospital stay was uneventful. She reported mild pain in right lower quadrant, but no nausea, no vomiting. No constitutional symptoms. Tolerating orally. She was mobilizing and using the spirometer. Examination showed vitals were within normal range, alert, conscious, oriented. Abdomen was soft and lax She was followed up daily. J vac drain amount decreased daily and was at day 3 postoperatively at amount of 36 ml/day, serous in colour. The patient was sent home with the drain. She was discharged on albendazole 400mg BID PO for 1 month, analgesia , laxative (senna 45mg/ispagula husk 330 mg/ plantaginis 7800 mg once daily).One week after surgery, she was seen at an infectious diseases clinic. She was doing well, no complains. Tolerating albendazole 400 mg BID. The patient was advised to complete albendazole for one month .One week after her surgery, she was seen in the surgical clinic. She was in good health and had no complaints. Examination revealed that vital signs were within normal limits, that the patient was aware, conscious, and oriented, that the abdomen was soft and lax, and that the incision was dry and clean. The J vac drain was producing 15-20 mL/day and was therefore removed. One week after surgery, her laboratory workup  was within normal range. In addition, the patient was examined at the surgical clinic two weeks after surgery. She was in good health and had no complaints. The wound was found to be clean, and the staplers were removed.Histopathological examination confirmed the diagnosis of umbilical hernia sac (biopsy 1) and right abdominal wall hydatid cyst (biopsy 2). (Biopsy1) gross description showed single irregular piece of yellowish-brown fatty with rubbery tissue measures 7.1*4.5*1.3 cm. Serial cut sections showed grossly unremarkable tissue surface. (Biopsy 2) gross description showed single irregular piece of greyish dark brown rubbery to firm corrugated tissue measured 10*7.2*2.1 cm. Serial cut sections showed greyish dark brown haemorrhagic tissue surface.Patient perspective: when the patient was examined in the clinic after surgery, she had no complaints, examination was unremarkable. She expressed her sincere gratitude to the surgical team.Informed consent: consent was obtained from the patient for publishing the article.In the Mediterranean, the Middle and Far East, and South America, hydatid disease is considered as an endemic disease. Direct contact with a dog or eating of foods contaminated by dog excrement are the most common ways for humans to become infected . Single Complete excision of hydatid cyst in the subcutaneous tissue can be an efficient surgical option, where all cysts are radically removed ,10. The The study´s strength is that the hydatid cyst was found in an uncommon place. Imaging confirmed the diagnosis, and the cyst was surgically removed. It ruptured during an operation room excision and was successfully treated with hypertonic saline and albendazole. The study's fault is that a hydatid cyst ruptured in the operating room but was handled adequately. Because subcutaneous hydatid cyst is a rare diagnosis, it's vital to keep it in mind as a possible cause of abdominal wall mass. It also necessitates precise imaging in order to correctly locate and extract it. It should be treated with anti-helminthic medications before surgery to reduce the risk of contamination during the procedure if ruptured.The fact that a hydatid cyst in subcutaneous tissue is a rare diagnosis is one of our limitations. There had been few cases of its management that had been reported. In addition, there are just a few published studies on the management of ruptured hydatid cysts in the operating room and their outcomes.To summarize, a subcutaneous hydatid cyst should be considered as one of the differential diagnoses for soft tissue masses, particularly in patients who have lived in endemic areas. To rule out alternative possibilities, proper imaging is essential. The treatment of choice is complete excision.


Gastroduodenal artery aneurysms have a low incidence of less than 1.5% of all splanchnic aneurysms. Rupture is frequent and life-threatening. The diagnosis is made by CT scan and by coeliac angiography, which also plays a therapeutic role in stable or stabilized patients. Surgery remains the treatment of choice in case of hemodynamic instability. We reviewed the case of a ruptured aneurysm of the gastroduodenal artery mimicking a tumor of the pancreas’ head in a patient who died following a hemorrhagic shock. Gastroduodenal artery aneurysms are rare, corresponding to less than 1.5% of splanchnic artery aneurysms Patient data: 51-year-old patient, active smoker (8 packs per year), ethylic, non-HTA, non-diabetic, received on account of cholestasis jaundice.Clinical findings: oedemato-ascitic syndrome, generalized weakness and hemodynamically stable.Diagnostic approach: Hematology and biochemistry findings show microcytic hypochromic anemia with a hemoglobin level of 8.4 g/dl, high neutrophils, leukocytosis with a positive C-RP of 24 mg/dl, a biological cholestasis syndrome with total and conjugated hyper-bilirubinemia, increased PAL and GGT and hepatic transaminases.Abdominal ultrasound showed dilatation of the intra- and extra-hepatic bile ducts without visualized obstruction. An abdominal CT scan had shown the presence of a spontaneously dense, heterogeneous, poorly bounded formation appearing to grow partially over the head of the pancreas A. It is Gastroduodenal artery aneurysms are mostly asymptomatic, discovered incidentally on imaging, or present with a vague symptomatology of atypical abdominal pain or a pulsatile mass on clinical examination According to Green et al True aneurysms are very rare and the etiological factors that have been suggested are atherosclerosis, periarteritis nodosa, and fibromuscular dysplasia Other factors may be associated such as trauma, stenosis of the celiac trunk, alcohol abuse, previous cholecystectomy, Marfan's syndrome or hepatic cirrhosis A few atheromatous calcified plaques were also described during the CT scan. Other etiological factors were not reported.The work of Moore et al Compression of the bile ducts was due to a perianeurysmal hematoma leading to dilatation of the latter. The main pancreatic duct of Wirsung was also dilated. It was therefore a mass located at the level of the pancreatic cephalic pole responsible for a bichannel dilatation. It is thanks to the injection at the arterial phase that it allowed us to unmask an aneurysm by specifying its shape, size and location . The defect of the mass enhancement around the aneurysm confirms its nature (hematoma). Coeliomy arteriography proposed in stable or stabilized patients goes further beyond its diagnostic role, it allows treatment by embolization The patient could not benefit from an emergency embolization because it was not available in the “Thies region” located about 70 km from Dakar.Surgical management consists of ligation of the gastroduodenal artery after complete resection of the aneurysm This observation shows a rare mode of expression of a ruptured gastroduodenal artery aneurysm as a pseudotumor of the pancreatic head. It raises the interest of an early diagnosis for a rapid management by endovascular interventional techniques. Although there is an increasing trend towards their use, surgery remains the treatment of choice in cases of hemodynamic instability.


Cecal bascule is a rare type of cecal volvulus occurring in the elderly population with a male predominance. It is a condition where the cecum folds upward upon itself.There are only a handful of case reports published about cecal bascule. This is the first case being reported from Pakistan.A 68 year old female, known hypertensive and osteoporosis presented in the ER with complaints of vomiting, abdominal distension and absolute constipation. Radiology was non-diagnostic. She was treated with cecectomy and anastomosis. Post operatively she developed ileus for which was managed. She was subsequently discharged at 8th day post op.The management of cecal bascule is always surgery with predilection towards cecopexy or cecetomy to prevent recurrence.This manuscript is as per the SCARE case report checklist . It is c2A 68 year old female, known case of hypertension and osteoporosis presented to the emergency department with complaints of absolute constipation for five days, abdominal distension for three days and vomiting for one day. Abdominal distention was gradual in onset and generalized. Vomitus was feculent and large in quantity. Apart from an uneventful caesarian section 35 years prior, she had no other past surgical history. On examination, the abdomen was tense, tympanatic and mildly tender on the right side on deep palpation. DRE revealed collapsed ano-rectal canal with no fecal staining. Her Xray abdomen supine shown below  demonstrCT scan with IV contrast demonstrated marked diffuse dilatation of small bowel loop and the cecum with abrupt point of transition at the junction of cecum and the ascending colon. Mild ascites was seen in the peri-hepatic region, in the pericecal region and the pelvis , Fig. 4.Based on radiology, no proper cause of intestinal obstruction could be identified. The patient was offered colonoscopy before laparotomy but she chose to get operated. Surgery was performed by consultant surgeon and senior registrar. Patient was counselled for right hemicolectomy, diversion and stoma formation.On laparotomy, around 200 ml reactionary fluid was aspirated. The cecum and small bowel were distended but healthy looking. Cecum was found rotated anteriorly upon itself . Distal Post operatively, the patient was kept in the general ward, and she developed ileus . She wasPatient was called for follow-up after two weeks in which she reported uneventful post discharge recovery.3Cecal bascule is a rare form of cecal volvulus, where the cecum folds anteriorly instead of twisting upon its axis . This reThe term embryogenic mobile cecum is used to define congenital mal-fixation of cecum to the right iliac fossa. This is due to a long mesentery which is fixed to the retroperitoneum by a narrow base of origin or the inability of right colonic mesentery to fuse to the peritoneum .Cecal bascule is difficult to diagnose and maybe misdiagnosed as recurrent appendicitis until a severe attack ensues . Unlike The treatment of choice for ceacal bascule is surgery. Simple reduction can lead to recurrence, and hence cecopexy or resection and anastomosis should be the preferred approach .4Cecal bascule is a rarity in surgical patients. Due to its non-specific radiology, it may be undiagnosed. A CT scan may or may not prove to be a useful modality. In patients who have undergone surgery before, this diagnosis should be placed in the differential. Surgical aim should be to prevent recurrence. Cecopexy or resection should be preferred.The authors report no declarations of interest.No funding.Study exempt from ethical approval.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Dr. S. Areesha Shakeel: Data curation, Writing - Original draft preparation.Dr. Junaid Zaman: Supervision.Dr. Hamza Haroon: Writing - Reviewing and Editing.Not applicable.Dr. Syeda Areesha Shakeel.Not commissioned, externally peer-reviewed.S. Areesha Shakeel: Data curation, Writing - original draft. Junaid Zaman: Supervision. Hamza Haroon: Writing - review & editing.


Bronchial artery aneurysm and pseudoaneurysm is a rare but life-threatening diagnosis due to catastrophic complications from rupture. Prompt detection and management is key to prevent complications. CT angiogram and digital subtraction angiography are preferred diagnostic imaging modalities. Being very uncommon, these entities can be misdiagnosed as a nonspecific mediastinal soft tissue mass, which can lead to delay in diagnosis and inappropriate or delayed management. We present a case of 72-year-old woman with incidentally detected large bronchial artery pseudoaneurysm, incorrectly classified as mediastinal malignancy at outside facility, receiving follow-up exams for 2 years, before correct diagnosis and management. Bronchial artery aneurysm is a rare finding, with less than 1% reported in patients undergoing selective bronchial arteriograms CT Angiography and digital subtraction angiography are diagnostic, with sensitivity of 67% and 100% respectively. These entities could be intrapulmonary, mediastinal or both. Bronchial artery pseudoaneurysm may be missed diagnostic on unenhanced CT as mediastinal lymphadenopathy and/or malignancy, delaying the diagnosis, as in our case . RecogniSince bronchial artery pseudoaneurysm is life-threatening, prompt diagnosis and endovascular treatment regardless of presentation is advocated to present catastrophic complications from rupture. Management options include transcatheter embolization, covered stent placement, and surgical excision .Here we present a case of a 72-year-old woman with incidentally detected large bronchial artery pseudoaneurysm, misdiagnosed as mediastinal lymphadenopathy on serial unenhanced cross-sectional imaging including CT and PET at outside facility.A 72-year-old female with no relevant past medical history presents to the clinic with chronic dyspnea. On arrival, she was afebrile and had stable vitals. CT chest without intravenous contrast was performed, which showed a middle mediastinal, smooth, round mass measuring 4.2 x 5.5 x 6.7 cm, with internal areas of increased attenuation . FindingSubsequently, unenhanced FDG PET/CT was performed. Increased metabolic activity as indicated by increased FDG uptake was noted in the periphery of the aforementioned mass, with central areas of no FDG uptake. Findings at outside facility were reported as compatible with necrotizing mediastinal mass concerning for malignancy .Fig. 2SuPatient refused biopsy and hence received serial follow-up unenhanced FDG PET/CT every 6-month , which wPatient was subsequently treated with endovascular coil embolization .Fig. 7ReEtiology of bronchial artery aneurysm is not well understood. It can be congenital, related to pulmonary sequestration or pulmonary agenesis, or it can be acquired from atherosclerosis, inflammatory lung disease, bronchiectasis, infection or trauma Clinical presentation depends on the location of the lesion. Intrapulmonary bronchial artery aneurysm presents most commonly with hemoptysis. Whereas mediastinal bronchial artery aneurysm has more varied presentations including hemoptysis, dysphagia, dysphonia, hematemesis or hemoptysis if it ruptures into esophagus or airway Mediastinal bronchial artery aneurysm and pseudoaneurysm can be misdiagnosed as mediastinal soft tissue mass on imaging. There are several cases reported in the literature . MediastSome important imaging features on unenhanced CT may help in prompt detection and correct characterization as aneurysm or hematoma, needing further assessment with contrast enhanced studies. These include: (1) Low-attenuation rounded structure adjacent to the vessels, and (2) Areas of intermediate or high attenuation (reflecting hemorrhage) adjacent to the above mentioned finding (pseudoaneurysm) indicating pseudoaneurysm rupture. The attenuation will vary depending on the chronicity and over time the internal characteristics will change In conclusion, a rare case of asymptomatic large bronchial artery pseudoaneurysm is presented. It was misdiagnosed as a nonspecific mediastinal soft tissue mass on multiple consecutive unenhanced exams for two-years. Although contrast enhanced CT and digital subtraction angiography are preferred imaging modalities, we discuss characteristic findings of pseudoaneurysm on unenhanced CT which could raise suspicion and prompt additional evaluation with appropriate imaging. Bronchial artery aneurysm/pseudoaneurysm can lead to catastrophic bleeding if ruptured or biopsied in error and hence require prompt detection and management.Informed consent was obtained from the patient. No identifiable information is shared in current case report.


The familial occurrence of childhood cancers has been proven for a long time. Wilms’ tumors often do not have a clear germline genetic cause. However, approximately 2% of all nephroblastoma cases are familial. Descriptions of twins with the same cancer are extremely rare, so our aim was to present the background of the available literature of the occurrence of Wilms’ tumor in a pair of monozygotic twin girls with detailed clinical, histological, and molecular analysis. Two twins were born of unrelated Caucasian parents. Family history revealed no known chronic diseases or malformations. At the age of 3.5 years, the first twin was admitted to the emergency department due to hematuria and abdominal pain. Ultrasound examination revealed an enlarged right kidney, 12.8 cm, with a mass in the upper pole measuring 56 × 69 × 78 mm. The second girl was referred for an abdominal ultrasound, which revealed a right kidney measuring 8.6 cm with a central mass measuring 54 × 45 × 41 mm. Both children underwent surgical resection, and the histopathological result showed a mixed form of nephroblastoma, predominantly epithelioid with residual blastemal compartment. Detailed clinical, histological, cytogenetic, and molecular analyses were performed on both sisters. It was also decided to identify environmental factors. Information was obtained that the girls’ parents run a farm and regularly use pesticides and chemical rodenticides. Based on our observations and the available literature, Wilms tumor in monozygotic twins may be present. Both genetic and environmental factors may be involved in the development of tumors. After excluding methylation abnormalities and mutations in the genes studied, we questioned whether the onset of Wilms tumor in both sisters could be the result of exposure of the twins’ parents to pesticides. The familial occurrence of childhood cancers has been proven for a long time . One of Afshar and Golden described a case of monozygotic twins with neuroblastoma . Wilms’ Although it has been described that the risk of WT is reduced in multiples , a few cDescriptions of twins with the same cancer are extremely rare, so our aim was to present the background of the available literature of the occurrence of WT in a pair of monozygotic twin girls with detailed clinical, histological, and molecular analysis.Two twins were born of unrelated Caucasian parents. Family history revealed no known chronic diseases or malformations. The twins were from the mother’s first pregnancy and were delivered by cesarean section at 36 weeks gestation. A single placenta was found.The first twin had Apgar scores of 8-10-10 and a birth weight of 1940 g. At birth, the neonate appeared normal, without any disease or malformation. On day 5 of life, supraventricular tachycardia was diagnosed, which required pharmacological treatment. At 12 months of age, the child required no further cardiac care or treatment. Her psychomotor development was within normal limits. At the age of 3.5 years, the patient was admitted to the emergency room due to hematuria and abdominal pain. Ultrasound examination revealed an enlarged right kidney with a mass in the upper pole measuring 10 × 9 × 12 cm . Chest XThe second twin at birth had an Apgar score of 8-9-9  with a body weight of 1760 g. The child’s development was normal. Due to the Wilms tumor found in her sister, 3 days after the sister’s diagnosis, the girl was referred for an abdominal ultrasound, which revealed a right kidney with a central mass measuring 7 × 6 × 8 cm . CT scan2 every week, three doses of actinomycin 45 ug/kg every 2 weeks, and two doses of doxorubicin 50 mg/m2 at week 1 and 5. Tumor’s size on CT examination decreased before and after 6 weeks of chemotherapy, respectively, in the first twin from 10 × 9.3 × 12.6 cm to 5.2 × 4.5 × 6.7 cm, and in the second twin from 7 × 6.1 × 7.7 cm to 3 × 4 × 2.8 cm  and NGS testing as well as examination for imprinting loci. NGS using Agilent’s SureSelect XT Target Panel, which includes a set of probes for the analysis of 220 genes with documented clinical relevance to oncologic disorders, was performed on an Illumina NexSeq 550 instrument. Analysis conducted using Variant Studio v.3.0, and IGV v.2.3 software did not show the presence of pathogenic single nucleotide changes. Analysis of imprinted DNA methylation was performed at the following loci: DIRAS3 (1p31); PLAGL1 (6q24); GRB10 (7p12); PEG1/MEST (7q32); KCNQ1OT1/H19/IGF2 DMRO (11p15); DLK1 (14q32); SNRPN (15q11), PEG3 (19q32); and NESPAS/GNAS (20q13). DNA from both sisters showed no deviation from normal methylation levels at any of these loci.Familial occurrence of Wilms tumor is very rare and usually affects siblings and first cousins. This fact was first described by Gaulin in 1951. A pair of monozygotic twins were operated on at 15 months of age . SubsequThe occurrence of the tumor in both twin sisters, the early age of onset, and the identical histology of the tumors may suggest the involvement of genetic factors, such as the WT1 gene, in the etiology of the disease in our girls. Several studies have suggested an association between bilateral WT, early diagnosis, and germline mutations in the WT1 gene ,16,17,18Although the accurate reason for the origins of the same tumors at a similar time in multiples is not well known yet, there are two hypotheses explaining that phenomenon. The first theory suggests that tumors in twins arise from transplacental spread ,21. In aThe second assumption indicates that the twins share the same genetic abnormality that causes the tumor, nephroblastoma . In the Detailed analysis of NGS and methylation abnormalities performed in our patients did not clearly confirm either of the two theories mentioned above. Therefore, an environmental factor began to be suspected. Information was obtained that the girls’ parents run a farm and regularly use pesticides and chemical rodenticides. Based on their study, Rios et al. suggested an association between Wilms tumor incidence and maternal exposure to household pesticides during pregnancy . These cIn conclusion, based on our observations and the available literature, Wilms tumor in monozygotic twins may be present. Both genetic and environmental factors may be involved in the development of tumors, including WT. Wilms tumor may be caused by increased exposure to pesticides and rodenticides in the parents of the affected child.


Intra-abdominal and intramural hematomas are well-known complications of pseudoaneurysms. We present a case of small bowel obstruction as a result of external mechanical compression from hematoma. Bleeding was localized to the pseudoaneurysm of the gastroduodenal artery and inferior pancreaticoduodenal artery. Angiography was used to control the bleeding with coil embolization. This rare clinical manifestation represents just one of the symptoms associated with pseudoaneurysms of the gastrointestinal tract. Therapeutic options are discussed along with a review of the literature. Visceral arterial aneurysms of the splenic, renal, hepatic, or mesenteric arteries are associated with a wide variety of clinical presentations. A pseudoaneurysm represents arterial dilatation with disruption of the vessel wall due to injury from trauma, inflammation, infection, or connective tissue disease, whereas true aneurysms are the result of vessel wall malformations . PerivasA 62-year-old woman with a past medical history of degenerative joint disease, cholecystectomy, total abdominal hysterectomy, and multiple back surgeries, presented to a peripheral hospital with a sudden onset of acute epigastric abdominal pain associated with nausea and vomiting. Further investigation revealed intra-visceral hemorrhage from the GDA. She was transferred to our hospital where the patient underwent elective airway protective intubation and further diagnostic CT-guided angiography.The patient underwent embolization of the GDA and IPDA for bleeding pseudoaneurysms. Interventional radiology findings included pseudoaneurysms from the GDA and IPDA axis. The patient was also noted to have celiac artery stenosis and a 1.6-cm splenic artery aneurysm. The patient required a second angiography by Interventional radiology for an acute drop in hemoglobin and hypotension. Further embolization of the residual vessels in the peri duodenal area was performed. The patient was subsequently extubated, was able to tolerate diet, and was discharged home.Three weeks later the patient presented to the emergency room with abdominal pain, nausea, and vomiting. CT of the abdomen-pelvis revealed dilatation of the second and third portions of the duodenum. After an extensive discussion among the gastroenterology, surgical, and interventional radiology services, nasogastric tube decompression with bowel rest and medical management was pursued. An abdominal x-ray was performed to demonstrate the extent of the stenosis, which revealed narrowing of the second and third portions of the duodenum, secondary to known extrinsic compression from a hematoma, with free flow of contrast through the segmental narrowing into the distal duodenum.As the patient’s nausea and abdominal pain improved, the nasogastric tube was taken out and the patient underwent an esophagogastroduodenoscopy (EGD). EGD revealed a moderate stenosis in the second portion of the duodenum from compression from the adjacent hematoma. However, the stenosis was able to be traversed into the distal portions of the duodenum.The patient remained stable and was able to tolerate a mechanical soft diet prior to discharge. Figures Figure  Figure The pathogenesis for the development of true aneurysms consists of trauma, hypertension, and atherosclerotic disease . For exaIn a similar fashion, the development of a pancreaticoduodenal artery aneurysm may arise from celiac artery stenosis. Anatomically, the pancreaticoduodenal artery is the main collateral vessel between the celiac axis and the superior mesenteric artery . TherefoThe most common cause of pseudoaneurysm is related to pancreatitis resulting in vessel wall deterioration . PseudoaIntramural hematoma of the duodenum causing obstruction has been reported with pancreatitis, pancreatic carcinoma, blood dyscrasia, mesenteric cyst, and ruptured aneurysms Figure  17]. Th. Th17]. Intra-abdominal hematoma as a result of trauma usually presents as small bowel obstruction with a delay of symptomatic onset. For the physical exam, a palpable mass is an uncommon finding. As the blood products of hemoglobin undergo protein disintegration, the osmolality of the hematoma increases causing a fluid influx. Over time, the residual intra-abdominal hematoma may cause an increase in extrinsic pressure and result in multiple postoperative complications . This miIn terms of clinical signs and symptoms, patients typically present with abdominal pain, nausea, vomiting, and hematochezia. Many of these symptoms overlap with a broad differential diagnosis including small bowel obstruction, gastritis, colitis, or even gastric outlet obstruction. Imaging plays a vital role in narrowing the differential diagnosis. While abdominal x-rays may help to rule out any obstruction, CT scan of the abdomen with contrast provides a more detailed examination of the abdominal etiology.Spontaneous intramural small-bowel hematomas are usually located within the submucosal layer, and typically cause symptoms after several delays. This delayed onset can be attributed to slow bleeds from small vessels, also resulting in intraluminal, intramesenteric, and retroperitoneal hemorrhages ,19. HemoLiterature reviewA review of the literature spanning from 1956 to 2011 related 74 incidents of GDA aneurysms, which described hemorrhage as the most common clinical presentation seen in 52% of all cases, followed by abdominal pain seen in 46% of cases . . 2]. TheVascular reconstruction after the resection of the GDA aneurysm is not always necessary because adequate collateral circulation is usually present from visceral arteries. Unless celiac artery occlusion is present, the GDA and superior mesenteric arteries provide adequate blood supply to the stomach. Ligation of the GDA may cause gangrene of the gallbladder and stomach, and even splenic necrosis -37.Typically, endovascular options of treatment may be performed under local anesthesia, provide a good therapeutic approach for patients with multiple comorbidities. Transcatheter embolization remains the most popular endovascular intervention.In summary, intramural hematomas are a relatively rare complication associated with visceral abdominal pseudoaneurysms. Bleeding can be life-threatening requiring emergent intervention. The development of extrinsic compression by the hematoma can manifest in a similar way associated with small bowel obstruction. However, unlike the standard management of small bowel obstruction, the therapeutic approach may require image investigation and prompt endovascular intervention.


In recent years, conservation laryngeal surgeries, including partial pharyngectomy, have been introduced as an alternative procedure for selected cases of hypopharyngeal squamous cell carcinoma (HSCC). Reconstruction of these defects presents a considerable challenge for the surgeon after partial pharyngectomy due to its circumferential nature. In this case report, we represent the innovative “End to side” technique to reconstruct hypopharyngeal defect using the rolled supraclavicular flap after laryngeal-preserving partial pharyngectomy.  A 70-year-old female presented with a history of progressive dysphagia and odynophagia. The evaluations revealed a T3N0M0 SCC of pyriform sinus. The mass was successfully resected through partial pharyngectomy, and the hypopharyngeal defect reconstruction was achieved using the rolled supraclavicular flap via the “End to side” technique. The patient was discharged after decannulation on day 10. The 3-week barium swallow was performed with no evidence of anastomotic leakage, and the oral feeding was started after NG tube removal. At week 5, complete movement of the true vocal cord on the one side and good phonation and deglutition was observed. There was no evidence of recurrence after 1 year.  Laryngeal-preserving partial pharyngectomy and hypopharyngeal reconstruction with the rolled supraclavicular flap via the “End to side” technique could lead to good oncological and functional outcomes in selected cases of pyriform sinus. Hypopharyngeal squamous cell carcinoma (HSCC) is an aggressive lesion, presenting the worst prognosis among head and neck cancers with a 5-year survival of approximately 30% –4. PatieIn recent years, conservative surgeries with laryngeal preservation protocols have been introduced as an alternative procedure for selected cases of invasive HSCC , 14. ParReconstruction of the hypopharyngeal defect represents a considerable challenge for the surgeon after partial pharyngectomy due to its circumferential nature . The ideUnfortunately, few articles focus on the larynx-preserving pharyngectomy approach as a primary treatment of HPSCC, and most of them performed total pharyngectomy or included cases with early tumors –27. In oA 70-year-old female was admitted to our otorhinolaryngology clinic with a history of progressive dysphagia and odynophagia with neither hoarseness nor respiratory symptoms. The patient underwent direct laryngoscopy as a retropharyngeal mass was revealed involving the apex and lateral wall of the left pyriform sinus accompanied by circumferential involvement of the cervical esophagus, sparing the postcricoid area. A biopsy was taken from the lesion, and the histopathologic result was suggestive of SCC lesion. The preoperative staging was T3N1M0 based on the American Joint Committee on Cancer (AJCC) TNM system .We obtained informed consent from the patient for all diagnostic and therapeutic procedures. The patient underwent partial pharyngectomy, cervical esophagectomy, total thyroidectomy, and bilateral neck dissection of levels 2–4.Using the apron incision, we approached the hypopharynx laterally; preserving the larynx, we resected the retropharyngeal wall to the level of the hyoid bone, left lateral pharyngeal wall, and pyriform sinus in a circumferential manner to the inlet of the esophagus; the left recurrent laryngeal nerve was sacrificed due to tumoral invasion. The mass was successfully excised with a lateral margin of 2 cm, an inferior margin of 1.5 cm, and a superior margin up to the epiglottis tip that left us with right piriform sinus, right lateral pharyngeal wall, a strip of retropharyngeal wall in the posterior, and esophageal lumen in the distal part.After harvesting the supraclavicular fasciocutaneous flap, we rolled it into an 8 cm tube to reconstruct the circumferential surgical defect Figures  and 3.We linked the distal part of the rolled flap to the proximal part of the cervical esophagus using modified Gambee suture and then attached the other end of the tube to the lateral side of oropharyngeal and postcricoid mucosa from the left, applying the “End to side” technique, with Vicryl 4-0.During the abovementioned surgery, the larynx was preserved. Flap viability was evaluated using the flap pedicle which was visible and accessible from the anterior aspect of the neck . The posTwo key points should be considered when using this technique. First, deepithelialization was not performed on the base of the supraclavicular flap and it remained on the skin surface to monitor its postoperative viability. Second, the skin to skin suturing technique should be used for rolling various flaps, and deep sutures are to be avoided as they can lead to microcirculation dysfunction in distal parts.Although organ-preserving CRT is the treatment of choice for early stage HSCC , primary surgery with reconstruction remains the favored therapeutic choice for patients with an advanced lesion due to CRT limitations, such as the long-term effects on laryngopharynx function and shorter survival in the event of salvage surgery , 29, 30.The PLE provides adequate oncologic control, with a considerable negative impact on patients' quality of life. Thus, conservation laryngeal procedures were developed to address the poor functional outcomes . PartialIn our previous study , we descIn the present case report, we performed the “End to side” technique to reconstruct the hypopharyngeal defect with a rolled supraclavicular flap in a patient with an advanced SCC of the pyriform sinus (T3N1M0). In this particular patient, the larynx, medial wall of sinus pyriform, and postcricoid were not involved; thus, total laryngectomy could be avoided. This procedure enabled us to preserve the larynx and laryngeal orifice when either the lateral or medial wall of the pyriform sinus was involved. We rolled the easy harvested supraclavicular flap, placed it on the circumferential pharyngeal defect, and sutured it to the preserved larynx using the “End to side” technique to attach the rolled supraclavicular flap to proximal larynx similar to the technique applied in the previous study to attach the esophagus to the larynx. The main complications following conservative surgeries are aspiration and pharyngocutaneous fistula , which wAn important aspect of this method is that the laryngeal orifice should be reconstructed using oropharynx and postcricoid mucosa in such a way to create a defined lumen with the larynx and does not lead to laryngeal closure.Few authors investigated the role of conservative surgeries in advanced HSCC. Jonnalagadda  used theChung et al.  performeIn Yoo et al.' study , 7 patieLim et al.  evaluateAnother larynx-preserving technique in patients with advanced pyriform sinus is the supracricoid hemilaryngopharyngectomy . AccordiCompared to similar articles, our suggested technique not only could preserve the esophagus and larynx with good postoperative speech and swallowing function but also was associated with acceptable oncologic results and no recurrence after 1 year.Reconstruction of the hypopharyngeal defect is a challenging issue for the surgeons . The ideRecently, the supraclavicular fasciocutaneous flap has been popularized as the reconstructive method for a variety of head and neck defects –42. The This technique has several advantages over free tissue transfer. As it is adjacent to the donor site, it matches the recipient site, properly . A supraLaryngeal-preserving partial pharyngectomy and hypopharyngeal reconstruction using the rolled supraclavicular flap via the “End to side” technique could lead to good oncological and functional outcomes in selected cases of pyriform sinus SCC.


A 51‐year‐old man had hematochezia, anemia, and an intraabdominal mass. Gastroscopy and colonoscopy showed no significant lesions with intraluminal bleeding, while radiological examinations showed bulky swelling of the lymph nodes around the abdominal aorta and a tumor in the left ischial bone. Small intestine endoscopy detected a dark purpuric protruding tumor of the jejunum and its biopsy specimen brought a definitive diagnosis of primary jejunal epithelioid angiosarcoma from positive staining of AE1/AE3, CD31, and erythroblast transformation specific related gene in immunohistochemical studies. The patient underwent surgical resection with adjuvant chemotherapy but died of progression of metastases 7 months after the diagnosis. Epithelioid angiosarcoma of the gastrointestinal tract is an extremely rare malignancy with poor prognosis and it is challenging to distinguish from undifferentiated carcinoma or melanoma. Immunohistochemistry is necessary for a definitive diagnosis. Sufficient biopsy specimen may aid a prompt diagnosis of this disease of the small intestine, which may present as obscure gastrointestinal bleeding. We present a case of successful diagnosis of small intestinal angiosarcoma settled by endoscopic biopsy of the jejunum. Angiosarcoma is a rare malignant entity, accounting for just 1–2% of all types of sarcomas; it generally occurs in the skin and the subcutaneous tissue.A 51‐year‐old man visited a clinic with tarry stool, loss of body weight, and severe anemia for a month. The patient was a government official and not an occupationally exposed person. Ultrasound scan showed bulky swelling of the lymph nodes around the superior mesenteric artery (SMA). He was presented to our hospital with a temporal diagnosis of malignant lymphoma for a detailed treatment. He was febrile and had fatigue, and laboratory tests showed anemia  and hypoalbuminemia . Other significant laboratory data were serum iron level 10  and serum soluble interleukin 2 receptor (sIL2R) level 775 . Computed tomography (CT) showed enlarged lymph nodes around the SMA to the abdominal aorta and segmental wall thickness of the jejunum Figure . F‐18 flWe performed laparoscopic partial resection of the jejunum as the patient's recovery from anemia was difficult, despite repeated blood transfusion. A protruding tumor of 10 × 12 mm in size was found in resected tissue of the jejunum Figure  and it hPostoperatively, he received weekly administration of paclitaxel as a first line chemotherapy. His therapeutic regimen was then changed to doxorubicin because of allergic reaction. Rapid disease progression with cancer pain and cancerous cachexia resulted in his death 7 months after the diagnosis.Angiosarcoma is a rare malignancy of endothelial origin, which ranges from well‐differentiated tumor to high‐grade spindle cell type and a unique morphologic subtype of angiosarcoma that shows a predominantly epithelioid appearance with sheet‐like growth. This atypical epithelioid subtype has been defined as epithelioid angiosarcoma,The etiology of angiosarcoma is still unclear, although several risk factors  relate to its pathogenesis.Definitive diagnosis of epithelioid angiosarcoma of gastrointestinal tract is quite difficult because of its various symptoms, insufficient specimen sampling and histopathological similarity of poorly differentiated carcinoma.Treatment modalities for gastrointestinal epithelioid angiosarcoma include surgical resection, radiation therapy, and chemotherapy, which can be performed alone or in combination depending on the progression of each case.In conclusion, our patient with jejunal epithelioid angiosarcoma may be the first case to be successfully diagnosed by biopsy specimen from small intestine endoscopy. Gastroenterologists should consider this rare disease as a differential diagnosis for obscure gastrointestinal bleeding. We recommend immunohistochemical staining of CD34 and ERG positively and S‐100 and EMA negatively.The authors declare that there is no conflict of interest.The authors report the details of the patient's case in accordance with the ethical standards of the Declaration of Helsinki.None.


Introduction. Nontraumatic splenic rupture is a rare event. On the other hand, splenic metastasis is also rare and usually found in the context of disseminated disease. Spontaneous splenic rupture caused by a metastatic deposit has been reported only as case reports. To the best of our knowledge, pancreatic cancer being the primary site has been reported in only a handful of cases. Case Presentation. A case of spontaneous splenic rupture in a 68-year-old male presenting with acute onset left shoulder pain, caused by metastatic pancreatic cancer to the spleen, is presented herein. During the investigation, the patient developed hypovolemic shock due to intra-abdominal hemorrhage, necessitating emergency splenectomy. The patient recovered well and was discharged from the hospital on the 8th postoperative day. Discussion. This study underlines the fact that the spleen is a rare site of metastasis, remaining mostly asymptomatic. However, it can nevertheless prove to be a life-threatening condition. The exact pathophysiological mechanism of splenic rupture due to metastasis still remains unknown, requiring further research. Emergency splenectomy remains the standard of care, and this clinical condition should be considered in the differential diagnosis of cases with acute abdomen and malignant neoplasm history. Spontaneous splenic rupture (SSR) due to metastatic deposit rupture is a rare condition. Among such cases, the primary tumor site being the pancreas is even rarer –3. It isA 68-year-old man presented to the emergency department with acute onset pain located in the back of his left shoulder. The patient had a medical history of percutaneous coronary intervention (PCI) with stent insertion in his right coronary artery and Type II diabetes mellitus. Physical examination revealed no specific findings, and his abdomen initially was soft and mildly tender in the left upper quadrant.Laboratory tests showed a hemoglobin level of 8.2 g/dl with the rest of the blood tests being unremarkable. While awaiting imaging investigations, the patient became restless with profound abdominal pain. He underwent computed tomography (CT) scan which revealed splenic rupture and bleeding located in the left upper quadrant . ShortlyOn exploratory laparotomy, an amount of 600 ml of blood was found mostly in the left upper abdominal quadrant and the spleen was found to be ruptured. An emergency splenectomy was performed. No other macroscopic findings were noted intraoperatively.Histopathological examination of the specimen showed a capsular disruption on the inferior-posterior surface of the spleen. In the bed of that capsular rupture, a tumor mass measuring 2.4 × 2.3 × 2 cm was found. Microscopically, its morphological characteristics were indicative of metastatic adenocarcinoma moderately and focally poorly differentiated, with necrosis present . Immunohth postoperative day. He underwent chest and abdominal CT scans for staging which demonstrate the primary site in the pancreatic head, with concomitant lymph nodal disease locally and in the mediastinum. The multidisciplinary team decided for palliative chemotherapy.The patient recovered well and was discharged on the 8The spleen is a rare site of metastasis regardless of the primary malignant neoplasm origin. Choriocarcinoma (13.3%) and melanoma (10%) are the most commonly reported primary sites with gastric carcinoma being third (6.7%) for splenic metastasis . AlthougThe presence of splenic metastasis is mostly asymptomatic  with theMetastasis to the spleen leading to splenic rupture is extremely rare. However, when patients with known splenic metastasis present with acute abdomen, it should always be considered part of the differential diagnosis . Given tSSR is usually treated with emergency splenectomy regardless of the cause.There are several theories regarding pathophysiological mechanisms of metastasis. Those theories focus on either anatomical, mechanical, or immunological factors and explain the rarity of solid tumor metastasis to the spleen. In summary, the spleen is rarely affected because of the constant blood flow to it, the rhythmical splenic sinusoid contractions, the sharp angle of the splenic artery with the celiac axis, the lack of afferent lymphatic vessels to the spleen, and the hypothesis that the splenic capsule acts as a protecting factor from intraparenchymal metastasis. Furthermore, splenic microenvironment  and high concentration of some factors such as angiostatin make the spleen an unfavorable site for metastasis growth due to increased immune surveillance in its lymphoid cells. Considering the above, it is speculated that tumor emboli can hardly reach and implant the spleen , 8.The exact mechanism of rupture of the metastasized spleen is currently unknown. Neoplastic invasion and disruption of the capsule, tumor necrosis, and subsequent bleeding as well as neoplastic infarctions leading to subcapsular rupture are the most accepted theories .Considering the above, our case confirmed the fact that splenic metastases usually go unnoticed. Our patient presented with SSR, which was successfully treated with emergency splenectomy, and the fact that the spleen was metastasized was not realized until the final histopathology examination. Moreover, the primary site was suggested to be the pancreas or biliary tract, while both of them rarely metastasize to the spleen.In conclusion, no matter how rare splenic rupture due to the presence of metastasis is, it must be considered in the differential diagnosis of an acute abdomen in patients with known cancer. More data should be collected about the pathophysiology, risk factors, and incidence of this troublesome complication.


Atraumatic splenic rupture is a rare but dangerous complication of chronic pancreatitis, vastly ignored in emergency literature. The anatomical relationship between the spleen and the tail of the pancreas contributes to the pathophysiology when an inflammatory process is in progress, although the mechanisms are not fully understood. The authors report the case of a 41-year-old male, previously undiagnosed with chronic pancreatitis, presenting with atraumatic splenic rupture. Due to worsening abdominal pain and hemodynamic instability, he underwent total splenectomy. The final diagnosis was obtained through contrast-enhanced abdominal computed tomography scans, intraoperative findings and histopathological examination of the surgical specimen, as frequently reported in previous cases. Total splenectomy is the treatment of choice, as the failure rate of the conservative approach is high. Few of these cases are described and a deeper understanding of the subject is needed. As this condition can worsen in a short time, a prompt diagnosis followed by adequate treatment can impact the morbidity and mortality associated with splenic rupture. High clinical suspicion is essential and increased knowledge about the pathophysiology and presentation of splenic complications in pancreatitis may alert emergency physicians to these fatal complications. Atraumatic splenic rupture (ASR) is rare, although being a well-documented injury in the setting of blunt abdominal trauma. This diagnosis is often overlooked in a patient without a history of trauma but it is of utmost importance to be done in due time since it is a life-threatening condition . Acute oWe present the case of an ASR in a male patient not previously diagnosed with chronic pancreatitis. Due to its rarity, the understanding of these particular cases is sparse, making it fundamental for emergency surgeons to be aware of this entity.This article is a development of a previously presented poster at the XLI Congresso Nacional de Cirurgia on June 18, 2021.A 41-year-old man, with a history of alcohol-induced acute pancreatitis eight months before, presented to the emergency department of Hospital Vila Franca de Xira. He complained of nausea, vomiting and severe abdominal pain in the left upper quadrant radiating to his left shoulder for two days. He also referred to anorexia, unquantified weight loss and night sweats in the three previous weeks. There was not any history of significant abdominal trauma.In his previous hospitalization, he had elevated serum amylase and lipase levels. Imaging revealed a normal homogeneous liver with the absence of gallstones, a normally sized pancreas with regular contours and a normal-sized spleen with homogeneous echotexture. He was treated conservatively, abandoned alcohol consumption and recovered uneventfully, maintaining sporadic abdominal pain in the left upper quadrant since then.On this admission, the patient was hemodynamically stable, afebrile, oxygen saturation at 97% on room air and the abdominal examination revealed moderate upper abdominal pain but no rebound tenderness. The laboratory test results were remarkable for white blood cell count (19.5×103/μL) and CRP (4.95 mg/dL). The serum hemoglobin level was normal (14.5 g/dL), as well as the platelet count, prothrombin time and serum bilirubin, amylase, lipase, lactate dehydrogenase and haptoglobin levels. There was no evidence of red blood cell destruction.An abdominal ultrasound scan revealed a heterogeneous spleen with imprecise borders, with a liquid collection of undetermined etiology in subphrenic topography. The liver had normal dimensions, regular contours and homogeneous echotexture Figure . The ultOver the following 24 hours, although being treated with fluid resuscitation and antibiotic administration, the patient became tachycardic, with fever and signs of peritonism. The hemoglobin level dropped to 12.8 g/dL and the inflammatory parameters worsened. Since his serial serum amylase levels remained within the normal range, the diagnosis of acute pancreatitis became less likely. A new CECT scan was performed, which revealed worsening of the free heterogeneous fluid, not clearly hematic, in perigastric and perisplenic location, involving the tail of the pancreas and in the pelvic cavity Figure .The patient was transfused with a unit of packed red blood cells and proposed an exploratory laparotomy to which he consented. Intra-operatively we found an infected hemoperitoneum, splenomegaly with multiple subcapsular hematomas and disintegration of the parenchyma in the inferior pole, due to the rupture of one of the subcapsular hematomas. A total splenectomy was performed.Until the pathology report came, we also excluded infectious mononucleosis, infective endocarditis, sarcoidosis, symptomatic human immunodeficiency virus infection, systemic lupus erythematosus or myeloproliferative disorders. The patient presented a normal erythrocyte sedimentation rate, a normal peripheral blood smear and a normal bone marrow biopsy.Histopathological examination of the spleen specimen was compatible with the first diagnosis and identified fragments of pancreatic parenchyma with fibrosis, acinar atrophy and irregular ducts Figure . These pOn the seventh day of post-op, the patient developed anorexia and pain in the right lower quadrant and an abdominal computed tomography (CT) scan revealed an acute appendicitis. He was submitted to a relaparotomy and an appendectomy was performed. The patient fully recovered from the second surgery and was discharged, free of complications, five days later.The spleen remains the most frequently affected organ in blunt trauma, accounting for up to 50% of all abdominal solid organ injuries  as well The pathophysiological mechanisms of splenic rupture in chronic pancreatitis are not fully understood . The disClinical findings of ASR are nonspecific, challenging the diagnosis that relies mostly on imaging studies. The commonest symptom is abdominal pain in the left upper quadrant, radiating to the left shoulder (Kehr’s sign) in 50% of the cases, followed by nausea or vomiting and pain in the left chest due to pleural effusion. A decrease in serum hemoglobin level can also be verified as well as normal serum amylase, lipase and troponin levels ,11,15,16Splenic vein thrombosis and probable segmental portal hypertension present an increased risk of splenic rupture and nonsurgical management can be deleterious ,12. The Whenever the hemodynamic status is stable and the etiological factors are evident, the conservative approach, with percutaneous drainage and/or splenic artery embolization, can be weighted, provided that there is strict monitoring with serial ultrasound or CT scans ,15,17,18Hemodynamically unstable patients will need an emergency laparotomy to perform a total splenectomy or distal pancreatosplenectomy, which may lessen the risk of pancreatic fistula or leak ,10,12. TIn this case report, the patient's clinical worsening led to the need for urgent surgery. A laparotomy was performed due to hemodynamic instability and the possibility of finding severe adhesions that could complicate a laparoscopic procedure. According to the literature, more than 90% of the ruptured cases were managed with laparotomy and splenectomy ,3. RenzuASR has greater surgical difficulty and a poorer outcome than TSR . ReporteASR is a rare and dangerous complication of chronic pancreatitis that can potentially lead to a lethal outcome. Splenic parenchyma complications are increasingly being documented in pancreatitis and should be considered in patients with inflammation or necrosis of the tail of the pancreas. Diagnosis is challenging and requires a high index of suspicion. Contrast-enhanced computed tomography scan is the method of choice and intraoperative examination is helpful in discovering underlying abnormalities. Exploratory laparotomy with splenectomy remains the first-line treatment in most cases.Knowledge about this condition is insufficient. Clinical reports such as the one presented are important to remind emergency physicians that a ruptured spleen can occur in the absence of major trauma or previous splenic disease. Prompt recognition and intervention are essential to change the associated high morbidity and mortality.


Most congenital soft tissue masses are benign. Ewing's sarcoma (ES) is a highly malignant tumor that commonly occurs in children and adolescents and rarely occurs during the fetal period. Cases of congenital soft tissue ES with magnetic resonance imaging (MRI) findings are scarce. To the best of our knowledge, no previous reports have described the pre- and postnatal MRI findings of ES.We present a case of congenital soft tissue ES arising in the body wall, which was examined using MRI during the prenatal and neonatal periods.Malignancy was suspected by diffusion-weighted imaging, which demonstrated restricted diffusion within the mass even during the fetal period. ES was confirmed via histopathological examination after birth.The patient initially underwent conservative treatment for suspected hemangioma. Tumorrectomy was undergone after three weeks based on previously dissatisfied therapeutic effects.The patient died of multiple distant metastases despite undergoing postoperative chemotherapy and metastasectomies.Fetal or neonatal soft tissue ES may be clinically misdiagnosed as a hemangioma. It is important to suspect this through an imaging approach such as diffusion-weighted imaging. Most congenital solid masses such as hemangiomas and teratomas are benign. However, although uncommon, malignant soft tissue masses that develop during the prenatal and neonatal periods have poorer outcomes.Ewing sarcoma (ES) is a highly malignant tumor that commonly occurs in children and adolescents. However, this rarely occurs during the fetal period. Congenital extraskeletal ES tumors are typically located in the extremities, pelvis, or soft tissues of the trunk. Herein, we report a congenital soft tissue ES on the body wall, which was documented using pre- and postnatal magnetic resonance imaging (MRI).2A 29-year-old woman  was referred and admitted to the hospital at 37 weeks of gestation for ultrasonographic detection of a fetal body wall mass. Her medical history was unremarkable and she was not taking any medications during her pregnancy. Antenatal screening revealed a low risk of trisomies 13, 18, and 21. The maternal viral serological test results were negative. Prenatal ultrasound showed a mixed echogenic mass on the lateral side of the left lower abdominal wall of the fetus, with abundant blood flow signals inside the mass and along its margins. No other abnormalities were observed in the patient. Subsequent prenatal MRI revealed a large subcutaneous solid mass located in the left lower lumbar region  is rare, and few cases of soft tissue ES have been reported. Among the 34 cases of congenital ES and PNET documented by Haas et al 18 occurred in the extraskeletal tissues, including the head and neck, body wall, upper limb, abdomen, and retroperitoneum. To date, approximately 20 congenital ES/PNET cases have been reported in soft tissues previously.–5 However, few of these cases have documented MRI results. To the best of our knowledge, this is the first reported case of congenital ES with MRI findings from both the pre- and postnatal  periods.ES belongs to the Ewing sarcoma family of tumors, which include primitive neuroectodermal tumors (PNETs) and Askin tumors. reported a case of fetal ES that presented as a cervical mass containing cystic and solid areas on T2-weighted images. Case reports of postnatal ES with MR images obtained during the neonatal period are scarce.–8 In these few reports, soft-tissue ES masses were characterized by heterogeneous and hyperintense enhancement. Several of the masses had necrotic or cystic areas and involved the adjacent tissues. However, DWI was not included in any previous pre- or postnatal ES reports. The DWI technique is a special MRI sequence developed to detect water diffusion in vivo. Currently, it is the only non-invasive method that provides crucial diagnostic information regarding the presence or absence of restricted diffusion. The restricted diffusion is due to the high density of malignant cells and scant cytoplasm, both of which strongly indicate malignancy. Malignant tumors visualized with DWI exhibit highly restricted diffusion as opposed to the free diffusion observed in benign tumors (such as hemangiomas). In the current case, we suspected malignancy based on DWI findings.Yildiz et alThe main differential diagnoses include hemangioma, infantile fibrosarcoma, and teratomas. Fetal hemangiomas in the body wall are more common than those in subcutaneous ES. Hemangiomas have low-intermediate or predominantly high signals on T2-weighted images. Masses containing more vessels and a reticulated mixture exhibited a mixed-signal intensity with decreased intensity dots and increased intensity voids. A hemangioma usually exhibits isointense or slightly hypointense signals on DWI owing to unrestricted water diffusion, which is a differential point with ES and other malignant tumors. Congenital fibrosarcoma and extraskeletal ES are similar, in terms of the sites of predilection and imaging findings. Both of these pathologies present with high signal intensity on DWI, and differentiating between the two via prenatal MRI evaluation is difficult. However, fibrosarcoma has a better prognosis than ES, and rarely metastasizes. Fetal subcutaneous teratomas primarily occur in the sacrococcygeal, oral, and maxillofacial regions. Teratomas exhibit infiltrative spread and calcified cystic areas on MRI, which are not typically observed in subcutaneous ES. Based on the currently unfavorable prognosis of fetal soft tissue ES, it is crucial to investigate the possibility of prenatally diagnosing this disease. We suggest that MRI, including DWI, should be performed when prenatal ultrasound reveals a solid soft tissue tumor. If an apparent high signal intensity is observed in a fetal mass using DWI, clinicians should suggest a potential malignancy so that appropriate prenatal counseling can be offered to parents.Currently, the treatment of choice for congenital ES includes radical surgical resection, chemotherapy, and radiotherapy. Despite these treatments, the disease has a poor prognosis, and most diagnosed newborns do not survive for more than two years.4Subcutaneous soft tissue ES during the fetal or neonatal period may be misdiagnosed as a hemangioma. MRI techniques, particularly DWI, can play an important role in the differential diagnosis of benign and malignant masses.Resources: Wenjun Chen, Penghui Han.Writing – original draft: Chaoxiang Yang.Writing – review & editing: Chaoxiang Yang, Wenjun Chen.