We report a 43-year-old female who presented with recurrent non-massive hemoptysis for four months. Chest radiograph was normal. Thoracic computed tomography (CT) scan revealed a 0.8-centimeter intraluminal polypoid mass abutting the middle part of trachea. A fiber-optic bronchoscopy demonstrated a lobulated reddish mass at dorsolateral aspect of the right side of tracheal wall. Cryotherapy to remove the mass and argon plasma coagulation to stop bleeding at the stump was performed. The histological evaluation conﬁrmed the diagnosis of a tracheal lobular capillary hemangioma. There was no recurrence hemoptysis nor the recurrence of tumor during a one-year follow-up. Primary tumors of the trachea are uncommon and they are mostly malignant in nature . TracheaA 43-year-old non-smoking female, previously healthy, came to the hospital with history of chronic cough and blood-streaked sputum for four months. She had neither symptoms of wheezing nor dyspnea. Four weeks before hospitalization, she developed difficulty breathing during inspiration. Chest auscultation demonstrated inspiratory stridor over the upper chest. Her chest radiograph was normal, however the thoracic computed tomography (CT) scan for initial evaluation of hemoptysis revealed a 0.8-cm intraluminal polypoid mass with tumor contrast enhancement of 43.1 HU abutting the right side of the middle part of trachea Figure , 1B. FibAdult tracheal LCH is very rare, with fewer than 20 cases reported in the literature so far ,3. TrachThe most common clinical presentations of tracheal LCH are recurrent cough and hemoptysis due to hypervascularization of the tumor . HemoptyChest X-ray findings in tracheal LCH, like our patient, are generally normal. Thoracic CT is more sensitive than standard chest radiography for the diagnosis of endotracheal lesion. Although CT findings are usually non-specific, a densely homogeneous contrast enhancement with an average density of more than 100 HU is very suggestive of tracheal LCH . TracheaBronchoscopic appearance is typically glittering sessile or polypoid cherry-like lesion . This clThe benefit of bronchoscopy is not for only diagnosis, but also definite treatment. Interventional bronchoscopy has been reported to successfully treat tracheal LCH. Various methods, including Nd-YAG laser therapy, electrocautery, excision by biopsy forceps, brachytherapy or cryotherapy through either rigid or fiberoptic bronchoscope, have been used for treatment of this tumor ,3. In ouAlthough tracheal CH is a rare cause of radiologically-negative hemoptysis, it should be kept in mind for differential diagnosis. Thoracic CT is very sensitive for identifying tracheal tumor and diagnosis of tracheal LCH should be suspected in case of densely homogeneous contrast-enhancement of the lesion. Bronchoscopy is beneficial for both definite diagnosis and treatment. Interventional bronchoscopy using combined cryotherapy and argon plasma coagulation demonstrates excellent outcomes without any significant acute and long-term complications.


Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease. CT plays an important role in the evaluation of metastatic disease. Given the wide range of imaging findings, correlation with clinical findings, specific risk factors and patterns of metastatic disease can help narrow the differential diagnosis. The final diagnosis should be confirmed with histopathology and immunohistochemistry in combination with clinical and imaging findings in a multidisciplinary setting with specialist sarcoma expertise. The purpose of this review is to describe the clinical and imaging features of primary sites and metastatic patterns of angiosarcomas utilising CT and MRI. Angiosarcomas originate from the endothelial cells of lymphatic or vascular origin.Imaging presentation is variable and relies on correlation with history and demographics.MRI is preferred for the assessment of localised disease pre- and post-treatment.CT is utilised for the detection and response assessment of metastatic disease.Cystic lung nodules with surrounding ground-glass changes are characteristic of metastatic angiosarcoma.Angiosarcomas are rare, aggressive malignant tumours originating from the lymphatic or vascular endothelial cells, making up less than 2% of all soft tissue sarcomas , 2. A laThe aetiology is unknown in most cases, but there are several risk factors that are associated with angiosarcomas . PreviouExcessive ultraviolet (UV) radiation from prolonged sun exposure has also been implicated as a risk factor for cutaneous angiosarcomas, given that they occur more commonly in the face and scalp of elderly Caucasian men , 12.The relation between angiosarcoma and chronic lymphoedema  has been historically established in post-mastectomy patients with lymphoedema in the upper limb but in can be of any origin . Any lonHistologically, features of angiosarcoma are highly variable, and distinguishing benign and malignant vascular tumours can be challenging on light microscopy. The spectrum of findings can range from malignant endothelial cells forming vascular sinusoids which can dissect through collagen bundles, to solid sheets of spindle or epithelioid cells without definite vasoformation , 2, 8. WAccording to Young et al. , angiosaThe aim of this review is to present the clinical and imaging findings of angiosarcoma of different anatomical sites and to demonstrate the metastatic patterns of angiosarcoma utilising CT and MRI. The cases presented in this review are drawn from 399 cases of histologically confirmed angiosarcomas referred to the Sarcoma Unit at The Royal Marsden Hospital over a 10-year period from 1 January 2011 to 31 December 2020.MRI is the preferred modality of choice for the assessment of localised soft tissue sarcomas . It provCT with intravenous contrast is most useful for staging and response assessment of metastatic disease. It is easily accessible and cheaper than MRI or PET-CT. CT can be useful for assessing for bony involvement and detecting the presence of calcifications. The disadvantages of using CT include exposure to ionising radiation and poorer soft tissue contrast compared to MRI.In the breast, mammography and ultrasound are not particularly helpful due to their non-specific findings, and the diagnosis should be confirmed with a punch or percutaneous biopsy, depending on whether the lesion is superficial or deep. Ultrasound can be useful in some cases, for example in the initial assessment of suspected aggressive deeper masses in the extremities. In this setting, ultrasound can also provide information on tumour vascularity and suitability of a percutaneous biopsy.In the UK, the use of positron emission tomography-computed tomography (PET-CT) is currently not part of routine investigation in soft tissue sarcoma although it may be considered in some cases such as prior to radical surgery .The head and neck is the commonest primary anatomic site, accounting for 27% of all angiosarcomas . Head anCutaneous thickening and infiltration of the subcutaneous fat can be seen on CT , 23. CT Differentiating between benign and malignant lesions in the head and neck can be very challenging . The imaPooled data from 534 patients from several studies show that 15.3% of all angiosarcomas occur in the extremities . The tumOn CT, multiple cutaneous nodules with increased density of the subcutaneous adipose tissues and exuberant skin thickening have been reported of lymphoedema-associated angiosarcoma . On MRI,Given that a majority of angiosarcomas in the extremities are superficial in nature, the diagnosis is made clinically and confirmed with a punch biopsy before imaging is undertaken. Imaging may be performed following histological confirmation if assessment of local disease extent is required for treatment planning, or for surveillance in a post-treatment setting.The breast is one of the most common sites for angiosarcomas to develop and can be categorised into primary and secondary angiosarcomas .Primary breast angiosarcomas account for 0.04% of all breast tumours and 8% of breast sarcomas . They arSecondary or radiation-associated angiosarcomas (RAS) of the breast are more frequently seen in older women who have undergone treatment for breast cancer, with a median age of 70 . IncidenPrimary cardiac malignancies are rare with cardiac metastases being 20–40 times more common, but angiosarcoma is the most common primary malignancy of the heart and pericardium . CardiacTwo common patterns of growth have been described in the literature. The more common pattern is a large, well-defined mural mass replacing the atrial wall which protrudes into the cardiac chamber . On CT, Given the non-specific appearances on imaging, there is a wide range of differential diagnosis which may demonstrate similar appearances such as metastases, thrombus, lymphoma, myocarditis and rhabdomyosarcoma , 46. It Although primary hepatic angiosarcoma only accounts for 2% of primary hepatic tumours, it is the most common mesenchymal malignancy in the liver . It is oHepatic angiosarcomas can present in several different growth patterns—single dominant large mass, multiple nodules, a combination of a dominant mass with nodules, and diffuse infiltrative nodules , 52. A mThere is a variable pattern demonstrated on CT and MRI, as summarised in Table Progressive enhancement of angiosarcomas, however, can be mistaken for other lesions such as epithelioid haemangioendotheliomas (EHE) and haemangiomas. A retrospective study by Kim et. al.  comparinPrimary splenic angiosarcoma is exceptionally rare, with an annual incidence of 0.14–0.25 cases per million , 57. TheOn CT, there are a number of features which may indicate the diagnosis of splenic angiosarcoma. The commonest findings are splenomegaly with a spleen size measuring over 12 cm and diffuse infiltration by a large heterogeneous complex mass (or masses), but multiple discrete masses, a solitary mass or a poorly defined mass associated with intraperitoneal haemorrhage may also be seen . On non-On MRI, angiosarcomas can show nodular hypointense signal on T1 and T2-weighted images. Large masses can also show high T1 and T2 signal intensity representing subacute haemorrhage or necrosis. Areas of low T1 and T2 signal intensity may also be seen if there is haemosiderin deposition from chronic haemorrhage , 59. On A retrospective study by Thompson et al. indicates that a diagnosis of a primary malignant splenic tumour can be diagnosed in 10 of 12 patients 83%) in their series . The ima3% in theAngiosarcomas of the bone are very rare, accounting for less than 1% of primary malignant bone tumours and approximately 6% of angiosarcomas occur in the bones . The peaThe histological and biological behaviour of malignant vascular bone lesions is variable with a myriad of names used to describe each entity, which consists of haemangioendothelioma, angiosarcoma and epithelioid haemangioendothelioma . It has On plain radiography and CT, the appearances of malignant vascular bone tumours are variable, but the lesions are usually lytic with ill-defined margins Fig.  63]. Ot. Ot63]. On MRI, malignant vascular bone tumours show low to intermediate T1 signal intensity and high T2 and STIR signal intensities, with heterogeneous contrast enhancement Fig.  64]. Ar. Ar64]. Angiosarcomas can occur in the kidney, although these are very rare with less than 40 cases documented in the literature . The peaThe imaging characteristics of renal angiosarcoma can be difficult to distinguish from renal cell carcinoma. The commonest pattern is a large mass (over 5 cm) arising from the kidney associated with peripheral rim enhancement with a non-enhancing centre suggestive of necrosis . MRI mayOther uncommon sites of angiosarcoma reported in the literature include the gastrointestinal tract, urinary bladder, testes and penis .Angiosarcomas spread haematogenously, most frequently to the lungs, followed by liver and bones, but spread to the brain, lymph nodes and spleen have also been reported , 5–7.The most common CT findings for lung metastasis are multiple pulmonary nodules (63–85%), followed by multiple cysts which can either be thick or thin-walled (21–58%) Fig.  67, 68], 6867, 6Other common CT findings include the presence of ground glass change surrounding the nodules or cysts  corresponding to alveolar haemorrhage, which were demonstrated in 25–58% of patients in previous studies Fig.  67, 68], 6867, 6CT appearances of liver metastasis in angiosarcoma are typically multiple hypoattenuating lesions with foci of enhancement peripherally or centrally Fig.  70]. Mu. Mu70]. There is very little literature available describing the radiological findings of bone metastases from angiosarcoma. In our experience, bone metastases from angiosarcoma can be occult on CT, and MRI is often needed for the evaluation of bone metastases.The diagnosis and management of angiosarcoma require a multidisciplinary approach in a specialist soft tissue sarcoma setting given the complexity of the diagnosis and aggressive behaviour of the tumour. The spectrum of imaging and clinical presentation is wide given the heterogeneity of the disease. Imaging alone is not reliable in differentiating soft tissue sarcomas from other benign and malignant lesions , althougRadical surgery is often the primary treatment of choice, ensuring wide margins are achieved, but positive margins are common due to the invasive and multifocal nature of the disease –7. For aCytotoxic chemotherapy is the primary treatment option for metastatic angiosarcoma with a number of agents used including taxanes, anthracyclines, gemcitabine and ifosfamide . ParticiAngiosarcomas are rare soft tissue sarcomas associated with a poor prognosis which can affect any age and any part of the body. The most common presentation is purplish cutaneous masses or nodules involving the scalp and face in elderly men. Imaging features are variable and non-specific reflecting the heterogeneous behaviour of the disease, and patients often present with advanced or metastatic disease. Angiosarcoma should be considered in the differential diagnosis if imaging shows metastatic disease in the lungs with nodules and cysts surrounded with ground-glass opacification and hydropneumothorax. Given the rarity and often dismal outlook for these complex tumours, radiology has a crucial role in diagnosis, intervention and staging. A background knowledge of this interesting and challenging disease with its diverse manifestations is essential for diagnosticians in the specialist oncology setting. Correlation with the clinical history, particularly of any specific risk factors such as previous radiotherapy, chronic lymphoedema or specific familial syndromes, is also vital to narrow the differential diagnosis, but the final diagnosis should always be confirmed with histopathology and immunohistochemistry. The management of this complex disease would require a multidisciplinary approach at a specialist sarcoma unit.


Small jejunal diverticulitis is very rare, presenting in 0.06% to 1.3% of the population. Many patients remain asymptomatic or have nonspecific symptoms such as malabsorption and abdominal pain, making diagnosis complicated. Up to 6% of patients present with acute perforation. Here, we present such a case involving a 69-year-old female who presented with altered mental status due to sepsis and generalized peritonitis from a perforated jejunal diverticulum that was successfully managed with definitive surgery. We highlight the importance of maintaining a broad differential, early resuscitation, and prompt surgical management in complicated jejunal diverticulitis. Although adjunctive studies such as computed tomography may be helpful in stable patients, definitive surgery was both diagnostic and therapeutic in this case. Jejunal diverticulosis is characterized by multiple herniations of the mucosa and submucosa through the muscular layer of the bowel. These diverticula are thought to occur through weaknesses in the muscular layer at sites where the vasa recta enter the muscularis propria . As descSmall intestinal diverticula were first described by Sommering and Baille in 1794 and are very rare, with an incidence of 0.06% to 1.3%. The incidence is higher in the sixth to eighth decades of life and in men . Many caSmall intestinal diverticula are typically diagnosed incidentally on imaging or intraoperatively because symptoms are usually vague and can mimic many other disease processes. These include abdominal pain, pseudo-obstruction, and malabsorptive complications .Overall, 10-30% of patients present with acute complications including perforation, obstruction, adhesions, fistulae, peritonitis, and lower gastrointestinal bleeding . PerforaA 69-year-old woman presented to our institution complaining of a six-day history of abdominal pain and nausea. The patient had an extensive surgical history including diverticulitis, a Hartmann procedure, subsequent colostomy reversal, cholecystectomy, hysterectomy, and tubal ligation.At the time of presentation, the patient described associated dysphagia and poor tolerance of her diet, along with a progressive worsening of the pain. She also described fevers up to 101°F and more than 10 watery bowel movements per day for the two days prior to her presentation.In the emergency department, she was hemodynamically unstable with a blood pressure of 75/36 mmHg, heart rate of 94 beats per minute, temperature of 98.1°F, and oxygen saturation of 95% on room air. The patient was in clear discomfort but remained alert and oriented. Abdominal examination was remarkable for diffuse peritonitis with slightly more tenderness on her left side.Intravenous fluid resuscitation was initiated with lactated Ringer’s solution administered as two 1 L boluses considering her hypotension and recent diarrhea. Laboratory studies showed an elevated white blood cell count of 26,000 cells/µL with a left-shift, a hemoglobin of 8.8 g/dL, hematocrit of 26.9%, sodium of 125 mmol/L, blood urea nitrogen of 77 mg/dL, creatinine of 6.3 mg/dL, and lactate of 1.1 mmol/L. Based on these results, her fluid resuscitation was transitioned to 0.9% normal saline which was administered as multiple additional boluses. Output data revealed oliguria, which did respond to fluid resuscitation.The patient’s hypotension also transiently responded to fluid resuscitation, and therefore, a computed tomography (CT) scan of the abdomen was performed with intravenous contrast. CT demonstrated moderate pneumoperitoneum and nonspecific inflammatory fat stranding in the left upper quadrant consistent with bowel perforation. No small bowel dilation, evidence of obstruction, or sigmoid diverticulitis or diverticulosis were appreciated Figure .Based on the CT findings, the surgical team suspected an upper gastrointestinal source of perforation. However, the patient’s blood pressure fell once again to 80/30 mmHg despite ongoing resuscitation with normal saline. Therefore, operative intervention was indicated and further diagnostic testing was not pursued. The patient’s clinical situation and the risks and benefits of surgery were discussed with the patient and her family and informed consent was obtained for surgery. A right internal jugular central venous catheter and left radial arterial line were placed and norepinephrine infusion was initiated. A Foley catheter was also inserted.Diagnostic laparoscopy revealed dense intra-abdominal adhesions. The procedure was quickly transitioned to an open approach due to ongoing hemodynamic instability. After extensive lysis of adhesions attributed to her previous surgeries, a perforated jejunal diverticulum was discovered with a 3 cm perijejunal abscess which was draining pus Figure . This waOverall, 18 cm of jejunum was resected beginning at approximately 10-20 cm distal to the ligament of Treitz. A stapled anastomosis was performed. The procedure was completed without any complications, but the patient remained on norepinephrine infusion postoperatively.She had a long but relatively uncomplicated postoperative course. She was extubated on postoperative day (POD) one. Her hypotension resolved and pressors were weaned by POD two. She was also managed expectantly for mild demand cardiac ischemia and pre-renal acute kidney injury. She experienced Intensive Care Unit (ICU) delirium and underwent a neurology evaluation as well as CT and magnetic resonance imaging of the head which revealed no focal deficits, evidence of ischemia, hematoma, or mass effect. She was started on total parenteral nutrition and was gradually transitioned to oral feeds. She was discharged from the ICU on POD four and was discharged home on POD 14.The final pathology of the small bowel showed segments of diverticular disease with perforated diverticulum. There was significant inflammation in the bowel wall with extensive acute peritonitis, focal abscess formation, granulation tissue, and serosal adhesions.Given the nonspecific symptoms of jejunal diverticulitis, the diagnosis is often unclear on initial presentation; therefore, it is important to maintain a broad differential diagnosis. If perforation has occurred and the patient is hemodynamically unstable, prompt resuscitation and surgical management are indicated. In our case, with an unclear etiology, ongoing resuscitation, and fluid responsiveness, imaging was obtained for further clarity.Evaluation of such cases begins with a thorough history and physical examination. History should include risk factors for perforation such as recent endoscopy, surgery, history of cancer, inflammatory bowel or diverticular disease, and foreign body ingestion or insertion. The physical examination is crucial for identifying localized upper abdominal tenderness or, in our case, diffuse peritonitis.Diagnostic CT scan is the gold standard for identification of diverticulitis, classically described as out-pouching lesions with thickened bowel walls . In the Operative management was clearly indicated in this case involving a patient with peritonitis who only transiently responded to fluid resuscitation. When summarized as such, the preoperative decision-making is obvious, highlighting the continued importance of basic surgical principles in the modern era of ubiquitous CT scans. Once in surgery, such principles continue to guide the surgeon; diagnostic laparoscopy did not easily identify the source of perforation and was quickly transitioned to exploratory laparotomy. In retrospect, proceeding directly to exploratory laparotomy would have been preferable for this quasi-stable patient. A thorough examination of all of the bowel was also performed and is crucial in any case where the diagnosis or site of perforation is unclear. This allows one to identify any concurrent, otherwise potentially missed, disease .Unlike this case, uncomplicated colonic and small intestinal diverticulitis can be managed nonoperatively with intravenous fluids, bowel rest, and antibiotics ,11. PerfAlthough jejunal diverticulitis is rare, it is important to include it in the differential diagnosis of patients with abdominal pain and no colonic diverticulitis. Early diagnosis is key to prompt treatment to improve patient outcomes.Small bowel diverticulitis is an uncommon diagnosis with a variable presentation ranging from asymptomatic to nonspecific abdominal symptoms to perforation with frank peritonitis. Given its low incidence, jejunal diverticulitis can easily be overlooked in the working differential diagnosis; however, it is critical to maintain a high degree of clinical suspicion. In cases of complicated jejunal diverticulitis, early recognition and prompt surgical intervention are critical to improving patient outcomes.


Pulmonary artery sarcoma is a rare, but complex pathology requiring multidisciplinary preoperative and postoperative planning, including challenging intraoperative techniques for successful outcomes.317.See Article page Pupovac and colleaguesThe clinical presentation of PAS and the radiologic findings are often difficult to interpret, as they can be similar to pulmonary thromboembolism, as in the current case. PAS often presents with symptoms of thromboembolic disease, heart failure, and pulmonary hypertension. Early diagnosis is crucial to perform a curative complete resection. Yeung and colleaguesCurative surgical resection of the tumor is not always possible due to advanced disease. Debulking of the obstructive tumor may be chosen to restore hemodynamics, but incomplete resection can lead to early relapse. The primary goal of surgery in PAS is restore the blood flow, decompress the right ventricle, and optimally achieve R0 resection margins. Curative surgical procedures for treating the PAS include tumor endarterectomy, graft reconstruction of the pulmonary artery, and pneumonectomy. In addition, as demonstrated in this report, radical resection of the entire pulmonic root, incorporating pulmonic valve replacement and extensive reconstruction of the right ventricular outflow tract, including combination with multimodality treatment, leads to better survival.Blackmon and colleaguesHigh-volume centers of excellence report similar outcomes, as Wong and colleagues


Acute cholecystitis is a very common acute abdominal disease that mostly indicates abdominal surgery. Appendiceal adhesion to the gallbladder is a very rare condition detected during laparoscopic surgery. A 54-year-old female patient, with a known case of diabetes and hypertension, presented with right upper quadrant abdominal pain of four months’ duration. The pain was increasing in severity and associated with fatty meals. She was diagnosed with acute cholecystitis and was sent to the operating room for laparoscopic cholecystectomy. Appendiceal adhesion to the gallbladder was found, and laparoscopic cholecystectomy and laparoscopic appendectomy were performed. Adhesion of the appendix to the gallbladder should be considered by general surgeons. Acute calculous cholecystitis (ACC) is the third most common surgical emergency worldwide . ACC is A 54-year-old female patient, with a known case of diabetes and hypertension, visited a general surgery clinic complaining of abdominal pain of four months’ duration. The abdominal pain was described as a sharp pain localized to the right upper quadrant area. The pain started gradually and was increasing in severity. It was intermittent, did not radiate anywhere, and was associated with fatty food. There were no nausea or vomiting, no history of jaundice, and no diarrhea or constipation. She is neither a smoker nor an alcoholic. The patient does not have any allergies. She has a history of lipoma on the back that was surgically removed one year ago. On physical examination, the patient was conscious and oriented with a normal temperature, pulse rate of 70 beats/minute, respiratory rate of 20 breaths/minute, blood pressure of 138/71 mmHg, and oxygen saturation of 100%. General examination showed normal respiration with bilateral air entry and normal heart sounds. On focused abdominal examination, the abdomen was soft and lax with right upper quadrant tenderness. Murphy’s sign was negative. The patient was admitted as a case of ACC. An ultrasound of her abdomen revealed thickened-wall, inflamed, distended gallbladder (more than 5 mm), small multiple stones, and pericholecystic fluid. Her laboratory results were all normal, including blood count, liver function test, and kidney function test. The patient was diagnosed with ACC and has been sent to the operating room for laparoscopic cholecystectomy. Surgery was performed under general anesthesia with the patient in the supine position. Two adhesions were found during surgery. The first one is adhesion between the gallbladder and the omentum. The second one is adhesion between the appendix and the gallbladder (Figure Firstly, the omentum adhesion has been removed from the gallbladder. Then, the appendix has been separated from the gallbladder using a ligature. After completion of cholecystectomy Figure , adhesioThis report illustrates the possibility of appendiceal adhesion to unusual organs and unexpected locations. Older and comorbid patients may present with a nontypical presentation where the diagnosis may be reached only during surgery ,8. AppenAdhesion of the appendix could occur in many ways. However, adhesion of the appendix to the gallbladder is rarely seen. Thus, even in the absence of abnormal laboratory results, surgeons should be aware of these types of variations to avoid unfamiliar complications. As we reported in this case, appendiceal adhesion was found during laparoscopic cholecystectomy.


Hemangiomas are commonly encountered benign vascular tumors in clinical practice. They are easily diagnosed clinically, but it is essential to know atypical and rare varieties of these tumors to avoid confusing them with vascular malformations. The traditional approach in managing hemangiomas has been a “wait and watch” policy as most of these lesions undergo spontaneous regression with time. There are multiple treatment modalities in managing these lesions, but with specific indications for each of them. We report a case of Nicolau syndrome following injection sclerotherapy for a residual ear hemangioma, which lead to necrosis and total loss of skin and cartilage. However, the full-thickness defect in the ear was restored with a composite temporalis fascial flap, conchal cartilage graft, and split skin graft reconstruction. A detailed literature review of the presentation and management of this vascular tumor is discussed with a special emphasis on avoiding complications and maximizing patient outcomes. Hemangiomas are the most common benign vascular tumors of infancy . They arA detailed history and clinical examination are the keys to accurate diagnosis and differentiation between hemangiomas and vascular formations. The timing of appearance of the lesion after birth and rapid initial growth followed by gradual involution with residual scars point towards the diagnosis of hemangioma. However, there may be atypical clinical presentations of hemangiomas leading to confusion and a wrong diagnosis of vascular malformation (VM) due to similar clinical features and imaging findings. The author presents a rare case of Nicolau syndrome in an ear hemangioma following injection sclerotherapy leading to necrosis and full-thickness loss of skin and cartilage. This may be the first report of such a complication occurring in the ear, which was successfully reconstructed with a temporoparietal fascial (TPF) flap and conchal cartilage graft. A detailed literature review about the management of hemangiomas is discussed, emphasizing accurate clinical diagnosis and optimal treatment strategy.A 30-year-old male patient presented to the clinic with blackish discoloration of his left ear of one-month duration. The dermatologist in our hospital referred him. However, he had a history of vascular growth of the left ear, for which he underwent biopsy and injection sclerotherapy one month ago at another hospital. After a few days, he reported blackish discoloration with infection of the surrounding tissue of the affected ear Figures , 1B. On examination, there was necrosis over the superior and middle one-third of the ear involving the scaphal cartilage, antihelical fold, and conchal cartilage sparing the helical rim and the meatus Figures , 2B.The previous biopsy record showed the lesion to be a hemangioma. As the necrosis was irreversible and well-demarcated, it was decided to proceed with excision of the necrotic tissue and reconstruct the defect with conchal cartilage from the contralateral ear for support, TPF flap for cover, and split skin graft for the outer lining. The outline of TPF flap was marked, the superficial temporal vessels dopplered and the flap harvested using a zig-zag incision Figures -3C.The conchal cartilage graft was harvested from the conchal bowl of the opposite ear Figure .The TPF flap was turned over itself to cover the cartilage graft and the flap inset over the defect and final outer lining was provided by a split skin graft harvested from the thigh Figures , 5B.The postoperative period was uneventful, and the biopsy confirmed the lesion to be a hemangioma. After surgery, he came for regular follow-up, and the outcome after six months is shown Figures , 6B.He was satisfied with the postoperative result. However, there was postoperative notching, probably due to scar contracture at the helical margin. Therefore, a secondary scar revision (Z-plasty with/without full-thickness skin graft) was planned; unfortunately, he was lost to further follow-up after returning to his home country.Most hemangiomas arise as a single, sporadic postnatal lesion with gradual onset of regression at 18 months. However, approximately half of these regressing hemangiomas leave behind residual lesions with permanent anatomical distortion, prominent scars, and altered skin pigmentation and texture. Unfortunately, atypical hemangiomas are mistaken for VMs because of the similarities in presentation and imaging properties. Two variants of atypical  hemangiomas rarely encountered include the rapidly involuting congenital hemangioma (RICH) and the non-involuting congenital hemangioma (NICH). These present as mature lesions at birth and may be mistaken for VMs. The NICH presents as a solitary, well-circumscribed, plaque-like lesion, does not undergo involution, instead grows with the child similar to a VM; diagnosis of this rare lesion will require histological studies and/or imaging studies .Residual lesions were found in 69% of untreated cases in a 2011 study by Bauland et al. The main predictor for residual lesion was epidermal involvement, with no correlation being reported between growth pattern of hemangioma and risk of the residual lesion. Other studies reported a 50% incidence of residual lesions ,4.Hemangiomas are diagnosed clinically, but it is essential to do imaging studies to know the extent of soft tissue involvement and its flow characteristics when there is uncertainty. Therefore, MRI is the best imaging method for the diagnosis of hemangiomas. The tumor is isointense on T1, hyperintense on T2, and enhances during the proliferating phase. Ultrasound with duplex imaging is the initial modality of choice to assess hemangiomas as they are cheap, easily available, and non-invasive. They appear as a soft-tissue mass with the fast flow, decreased arterial resistance, and increased venous drainage. The only indication of angiography is if the patient is to undergo therapeutic embolization ,6.The management of hemangiomas with a conservative vis-à-vis surgical approach is a matter of debate. However, the natural history of hemangiomas, where most of these tumors undergo gradual regression followed by resolution, supports the traditional “wait and watch” policy. The guiding principles in the treatment of these tumors are to minimize complications that may lead to morbidity and mortality, improve physical appearance and psychosocial well being of the patient and family, and avoid overly aggressive procedures, which may lead to complications in hemangiomas with a high probability for excellent prognosis without intervention .The options available for medical management of hemangiomas are varied and include corticosteroids, propranolol, interferon, lasers, and embolization.Corticosteroids can be used either locally or systemically for the treatment of hemangiomas. The response to corticosteroid therapy is quite dramatic, as evidenced by growth arrest or rapid involution within the first week of initiation of therapy. Therefore, they are mainly indicated for use in small, isolated lesions in sensitive areas or large, complicated, life-threatening hemangiomas. However, in a study by Enjolras et al., 30% of study participants displayed accelerated involution following corticosteroid therapy, while 30% showed no response . NeverthThe serendipitous discovery of propranolol by Léauté-Labrèze et al. has brought about a paradigm shift in the management of proliferative hemangiomas. However, propranolol does not affect NICH and non-proliferative infantile hemangiomas . It is nRecombinant interferon alpha-2a and alpha-2b is a second-line option in the medical management of hemangiomas. They are mainly indicated in large, disfiguring hemangiomas in sensitive areas and complicated, life-threatening tumors. However, interferon therapy must be initiated early and maintained for at least 15 months for maximum beneficial effect and to prevent rebound growth following abrupt cessation of therapy. It is essential to note that spastic diplegia may occur as a complication following interferon therapy ,10.Lasers cause photodisruption of tissues, which may help to arrest or regress the growth of hemangiomas. The pulsed dye laser (585 nm) can target vascular tissue and speed up ulcerated lesions' healing process. However, the penetration of pulsed dye laser is only 1 mm; hence it cannot be used in deep hemangiomas. The neodymium:yttrium-aluminum-garnet laser (Nd: YAG) can penetrate to 8 mm; however, its extensive effect on surrounding tissues can cause skin color, texture changes, and extensive scarring. The use of lasers in managing hemangiomas is debatable. Still, there are specific scenarios where it may be beneficial, such as an early therapeutic option during infancy, large hemangiomas located in unfavorable anatomical regions, and the treatment of residual lesions after involution ,12.There have been only isolated case series and reports about the use of sclerosants to treat hemangiomas. Therefore, it is not considered a first-line option because of the high risk of complications. However, it has been traditionally used in VMs, especially - low flow VMs, venous and lymphatic malformations. In the case of hemangiomas, sclerosants are mainly used as part of embolization therapy for large, life-threatening hemangiomas, usually multifocal hepatic lesions, that can cause high-output congestive heart failure. However, sclerotherapy for hemangiomas can lead to more scarring in the long term ,14.A rare and dreaded complication associated with injection sclerotherapy is Nicolau syndrome, leading to variable degrees of necrosis of skin and underlying soft tissue. This manifests initially as skin color changes, severe pain, numbness, and paresthesia. If missed, frank skin ulceration and necrosis can progress, leading to irreversible tissue damage and permanent scarring ,16. NeveIn our case report, the patient had ear necrosis following injection sclerotherapy for a residual ear hemangioma leading to Nicolau syndrome and consequently full-thickness loss of skin and cartilage of the upper and middle thirds of the ear. This is the first report of Nicolau syndrome following sclerotherapy for an ear hemangioma to the best of our knowledge. This complication has also been reported after sclerotherapy in a pyogenic granuloma .The complications seen following injection sclerotherapy are mainly due to accidental extravasation into the surrounding tissue. The collateral damage following extravasation of sclerosant can be minimized by ligating the stalk of pedunculated lesions, pressure along the lesion's periphery, tourniquet use in extremities, cyanoacrylate, or balloon embolization before sclerotherapy. In addition, certain medications, if initiated early, can reverse the side effects of sclerosant extravasation, and these include anticlotting drugs (heparin), vasodilator drugs , systemic corticosteroids, and non-steroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation and local wound care. Injection sclerotherapy is better avoided in vascular lesions on fingers and toes, ear, nose tip, penile tip (end arteries), and children below 18 years ,19.Finally, the traditional approach of “masterly inactivity” in the management of hemangiomas is still relevant today because of the tendency of hemangiomas to involute with time. Surgery during the proliferative phase is not recommended due to the possibility of excessive bleeding and poor visualization. Instead, surgery is mainly indicated for the removal of residual fibrofatty tissue and scar. However, when large, complicated, and life-threatening hemangiomas in sensitive areas cause obstructive symptoms , early surgical intervention is warranted. Surgical intervention is also sometimes indicated for large, prominent lesions without functional compromise, but for aesthetic reasons to minimize the psychological issues faced by the growing child and parents. Late surgery is done only after completing the involution phase and is mainly indicated to correct abnormal contour and anatomical distortion caused by residual scarring and fibrofatty tissue .This article highlights the importance of proper clinical diagnosis of hemangiomas and discusses common treatment strategies to optimize patient outcomes with particular emphasis on minimizing complications. There should be an initial wait and watch approach in managing hemangiomas with a low threshold for medical therapy and a high threshold for surgery. Surgery should be reserved for involuted hemangiomas and those associated with severe life-threatening complications.The role of injection sclerotherapy in managing hemangiomas is debatable, and further studies are warranted before definitive conclusions can be made regarding the precise indications for this modality. Furthermore, judicious patient selection with informed written consent and counseling are advised to avoid complications and medico-legal issues.


Ectopic pancreas (EP) is defined as the presence of pancreatic tissue outside the pancreas with no anatomical connection to the pancreas. It is also known as pancreatic heterotopia, accessory pancreas, aberrant pancreas, or pancreatic rest. The first case of EP was described in 1727 when pancreatic tissue was identified in the ileal diverticulum. Abdominal pain and lower gastrointestinal bleeding are the most common symptoms. On histopathological examination, EP can be classified into four subtypes. Once identified and diagnosed, the treatment is surgical resection and the post-operative course is asymptomatic in most cases. We describe a rare case of EP, which was discovered on the CT scan of the abdomen as a jejunal mass and successfully treated with surgical resection. We have also described the possible role of chromogranin A for diagnosing EP cases and for post-operative follow-up. The first case of ectopic pancreas (EP) or pancreatic heterotopia was described in 1727 when it was identified in the ileal diverticulum . It is mA 48-year-old male with a past medical history of hypertension and diabetes presented to the emergency room (ER) with left lower quadrant stabbing pain which was intermittent, moderate to severe with no worsening or relieving factors. He denied any fever, chest pain, shortness of breath, nausea, vomiting, dysuria, hematuria, diarrhea, or constipation. His last bowel movement was on the day of the admission. The patient was a nonalcoholic and a nonsmoker. There was no family history of any pancreatic or gastrointestinal cancers. The pain was present for one year before the admission and had worsened two days before the ER visit. Vital signs on admissions were: temperature 97.9°F, heart rate 84 beats/min, blood pressure 170/92 mmHg, respiratory rate 14/min, and oxygen saturation 99% on room air. On physical examination, the abdomen was soft, nontender, and nondistended without any guarding or rigidity. There was no evidence of swelling or mass. Digital rectal examination was negative for any signs of blood. Laboratory studies including lipase, amylase, and lactate were normal. CT scan of the abdomen with IV contrast showed a 1.5 cm mass in the proximal loop of the jejunum with contrast enhancement Figures . He undeThe true incidence of EP is not known as most patients are asymptomatic. In the majority of cases, EP is identified incidentally during the surgery performed for other indications . When syPre-operative diagnosis of EP is extremely difficult though high clinical suspicion can help. It appears as an endoluminal enhancing lesion on the CT scan. On endoscopy, it appears as a round to oval submucosal lesion with central ulceration or umbilication . DefinitEctopic pancreas should be considered in the differential diagnosis of small bowel tumors whether identified on the imaging studies or incidentally discovered during surgery performed for other reasons. Pre-operative presumptive diagnosis requires a high degree of clinical suspicion. Survival after resection is good and the post-operative course is generally asymptomatic, highlighting the importance of the early diagnosis of this rare entity. It is important that radiologists, gastroenterologists, surgeons, and oncologists are aware of this rare entity to prevent the delay in care and appropriate management.


Lipomas are benign lesions of adipose tissue, which commonly affect the soft tissues but are rarely found in the musculoskeletal system. Intraosseous lipomas are rare benign tumors and even rarer in calcaneum, only to be found incidentally in the majority of cases. We report a case of a 45-year-old male patient who presented to the outpatient department with complaints of bilateral heel pain, which was initially treated conservatively as the presentation was similar to plantar fasciitis. On further follow-up, a plain radiograph of the ankles was taken, which showed a lytic lesion of the calcaneum with mild sclerotic margins on the right side with normal left foot radiographs. On magnetic resonance imaging, the lytic lesion demonstrated hyperintense signals on T1-weighted sagittal images, characteristic of fatty tissue, which helped us in arriving at the diagnosis of an intraosseous lipoma. The patient was treated by conservative means with physiotherapy, which relieved the pain, and on serial follow-ups, the lesion was found non-progressive on successive radiological evaluation. The differential diagnosis of such an entity includes plantar fasciitis, tumors such as an aneurysmal bone cyst, bone infarct, etc. With the increasing use of magnetic resonance imaging and computed tomography scans, physicians should be aware of the possibility of an intraosseous lipoma of the calcaneum, which should be ruled out during evaluation. Although possible, malignant pathology or aggressive transformation of such lesions is very rare; however, the lesion should be evaluated adequately and managed by surgical means in cases non-responsive to various conservative modalities. Lipomas are benign lesions of adipose tissue and affect soft tissues, including the musculoskeletal system . HoweverA 45-year-old male patient, a laborer by occupation, presented to the outpatient department with bilateral heel pain for the past three months, which was aggravated on weight-bearing. There was no history of significant trauma or similar kind of illness in the past. He was an occasional alcoholic and smoker for the past 10 years. On examination, there was no swelling, deformity, or any inflammatory/trophic changes. Tenderness was present over bilateral heels, more on the right side, and clinically plantar fasciitis was suspected. No palpable mass or bony swelling was appreciated on palpation. Plantar flexion and dorsiflexion movements were normal, while inversion and eversion movements were terminally painful on the right foot. Previously, the patient was diagnosed and treated for plantar fasciitis with analgesics, hot fomentation, and shoe modification, where he got relieved symptomatically in the left foot. All the routine hematological and systemic investigations were normal. A plain radiograph of both ankles' lateral view revealed a lytic lesion in the body of the right calcaneum measuring around 3 x 2.5 cm with mild sclerotic margins, while the left side was found to be normal , with calcaneum accounting for around 32% of all such cases ,7. The i1%, with There are a number of modalities used for symptomatic relief, including analgesics, anti-inflammatory medications, sitz bath, soft sole or shoes, and temporary immobilization with silicon casts ,10. SurgThis report describes a rare entity of an intraosseous lipoma of the calcaneum, which was initially treated as plantar fasciitis. Although intraosseous lipoma is a rare benign tumor, malignancy has been reported, so it needs to be evaluated properly. With the increasing use of MRI and CT scans, it is important to be aware of various differentials of chronic heel pain, including an intraosseous lipoma of the calcaneum.


These tumors are initially small and can be easily missed if not looked for carefully. As they grow, these tumors are more demanding to treat and hence the importance of detecting them early and treating them. Herein, we describe and review the optical coherence tomography angiography (OCTA) of the early- stage lesion, which suggested the involvement of superficial and a deeper retinal capillary plexus. In addition, to helping us detect these lesions earlier, OCTA may also help to understand the  Von-Hippel-Lindau (VHL) syndrome is an exceedingly rare, multi-organ disease with a prevalence of one in 230,000, characterized by the development of focal benign or malignant vasoproliferative tumors (hemangioblastoma). The syndrome occurs because of the mutation in the VHL gene (chromosome 3) and consequent increased expression of vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), erythropoietin (EPO), and transforming growth factor (TGF). Systemic manifestations of VHL include hemangioblastomas in the brain and spinal cord, renal and pancreatic cysts, pheochromocytoma, islet cell tumors, epididymal cystadenomas, endolymphatic sac tumor of the inner ear, and adnexal papillary cystadenoma of probable mesonephric origin (AMPO) of the broad ligament. All the systemic features are manifested in only a few cases with VHL, whereas only 50% of patients display only one systemic feature. Many of these manifestations may occur years after the initial presentation and evolves over a long period (complete penetrance of VHL is seen by 65 years of age) .Retinal capillary hemangioblastoma (RCH) is a clinically visible pathology that develops in 44%–66% of patients, usually in the third decade. These tumors develop denovo from the retinal vascular plexus and progress from an occult angioma to huge angiomas with subsequent complex retinal detachments . New lesLaser photocoagulation is the most common modality used because of its ease of use, fewer side effects, and results. The different photocoagulation techniques are direct photocoagulation of angioma, treatment of the feeder’s vessel, or a combination of both. Smaller lesions are more amenable to regress with treatment, whereas larger lesions require numerous treatment sessions. However, it is effective in lesions up to 4.5 mm size only and is used for peripheral and juxtapapillary lesions. Apart from laser therapy, trans-scleral cryotherapy is effective for the destruction of these lesions, even in the presence of simultaneous exudation, hemorrhage, or fibrosis. Thus, if we detect these lesions early, they are more amenable to treatment. Therefore, periodic screening is recommended for all patients with VHL .There are various imaging modalities for the diagnosis of retinal angioma. Fluorescein angiography is the most pivotal because of the tumors rich vascularity. On the arterial phase of fluorescein angiography, there is a prominent dilated feeder arteriole along with an adjacent homogenously hyperfluorescent tumor because of a fine capillary network. The venous phase demonstrates an enlarged draining venule. Throughout the late phase of the angiogram, dye leaks into the surrounding tissues . While tOn histopathology, early-stage angiomas show foci of the nodular proliferation of the endothelial cells, abnormal capillary vasculature with later development of reactive gliosis , 6. ReceA 33-year-old male diagnosed as VHL presented with decreased visual acuity in both eyes and no other systemic manifestations. Written informed consent was obtained from the patient for investigation, management, and publication purpose. He had a history of surgery in the past for spinal hemangioblastoma. Ocular examination detected multiple angiomas in zone 1 of the right eye and total retinal detachment in the left eye. Left eye vitreoretinal surgery was undertaken while the angiomas in the right eye were treated with direct retinal photocoagulation. The angiomas in the right eye remained stable, and some regressed following laser therapy. The patient underwent routine follow-up examination tri-monthly for early detection of any new angiomas. On a recent follow-up, no diminution of vision was noticed. Fundus examination showed lasered areas, multiple RCH, and localized retinal detachment. One of these lesions was a barely visible early-stage 1a lesion .A B-scan OCT lesion image revealed that the angioma was entirely intra-retinal, well defined, and vertically oval. The lower end had a basal hypo-reflectivity with the absence of an ellipsoid zone and an external limiting membrane. The upper end exhibited a focal hump with an intact overlying inner retinal layers .On the en face image of superficial retinal layers -E, a foc


The incidence of iatrogenic foreign bodies rise recently due to increasing number of surgical procedure. According to the literature, fewer than 2% of all bladder calculi occur in female subjects and, thus, their presence should provoke careful assessment of the etiology. There have been a number of reports on bladder calculi in women with a history of gynecologic procedures. We report a case of bladder calculus after hysterectomy that was treated successfully by open surgeryA 54 year old lady with dysuria, had a history of urinary stone for one last year. She was complaining hematuria. She had radical hysterectomy 20 years ago and cystoscopy Lithotripsy because of bladder stone a year ago. Physical examination was unremarkable. Abdominal CT scan with contrast had revealed multiple vesicolithiasis and irregular calcifications attached to the superior aspect of vesical urinary.Cystoscopic evaluation was performed and confirmed presence of calculi forming around several surgical sutures fixed to the bladder wall. The intravesical calculus had developed from non-absorbable sutures and hung on the dome of the urinary bladder. The stone and residuum of the suture were retrieved by performing an open surgery.The presence of an intravesical stone should be suspected in patients with a history of hysterectomy who have symptoms in the lower urinary tract. A hanging stone on the dome of the urinary bladder implies that non absorbable suture materials intrusion into the urinary bladder. The complication can be prevented by the routine use of absorbable material and doublechecking with cystoscopy. •The incidence of iatrogenic foreign bodies rise recently due to increasing number of surgical procedure.•According to the literature, fewer than 2% of all bladder calculi occur in female subjects•The presence of an intravesical stone should be suspected in patients with a history of hysterectomy•A hanging stone on the dome of the urinary bladder implies that non absorbable suture materials intrusion in bladder•There have been a number of reports on bladder calculi in women with a history of gynecologic procedures Hence the occurrence of bladder stones in careful assessment of the disease etiology Identification of bladder stone is usually done through serial kidney-ureter-urinary bladder (KUB) X-ray films for radiopaque stones and ultrasonography for radiolucent stones. Bladder stones usually move when the patient is repositioned. In some cases, however, bladder stones are immovable. Most of these stones are found during reconstructive procedures for urinary bladder 2This study is based on a case from our urology clinic which is inspected with relevant literatures. Consent from the patient has been obtained for this study. This case has been reported in accordance to the SCARE Guideline 3A 54 years old woman came to our urology clinic with a chief complaint of dysuria for more than a year prior to admission. She also complained hematuria, urgency and increase of urinary frequency. The patient had a history of radical hysterectomy for myoma uterine 20 years earlier with benign pathological result. Radiotheraphy and chemotheraphy was denied.There were history of spontaneous stone passage, sandy urine, and cloudy urine. The patient was fully conscious with stable hemodynamic status, conjunctiva were not anemic and sclera no jaundice. Laboratory studies revealed Hb of 10.9 g/dl, white blood counts of 11.560/ul, blood urea nitrogen of 21.0 g/dl, blood creatinine of 0.6 mg/dl. Urinalysis showed >50 white blood cells per high power field, and >50 blood cells per high power field. Non-contrast abdominal pelvic computed tomography was conducted with confirmed multiple vesicolithiasis  and irregular calcifications attached to the superior aspect of vesical urinary size ±11 × 18 × 15 mm, 677–855 HU .Fig. 1NoCystoscopy showed a hanging stone on the dome of the urinary bladder . Our fir4Bladder calculi account for 5% of all urinary calculi and usually occur due to bladder neck outlet obstruction, indwelling foreign bodies, or urinary tract infection. Bladder calculi occur more frequently in males, across nations, races, and age groups. Females account for only 2% of all patients. The frequency of occurrence increases yearly after the age of 50 years Bladder calculi are uncommon in women and any history of previous pelvic surgery must, therefore, raise the suspicion of an iatrogenic origin Endoscopy is an effective and safe method for removing bladder stones. However, a bladder stone resulting from a foreign body that has become fixed on the bladder wall may require an open surgery for its removal A hanging intravesical stone on the dome of urinary bladder is rare and usually associated with procedures on the organs surrounding the urinary bladder, for example, hysterectomy or the placement of intrauterine devices Abdominal radiography may reveal most intravesical stones. However, radiolucent stones would be missed by a plain X-ray film. Ultrasonography is useful for primary evaluation of intravesical stone formation 5A hanging intravesical stone on the dome of the urinary bladder is rare. It usually hints that the bladder stone was derived from encrustation of suture material. The complication can be prevented by the routine use of absorbable material in sutures outside the urinary bladder, no use of any suture through the urinary bladder and double-checking with cystoscopy.None declared.None declared.N/a.Not commissioned, externally peer-reviewed.dr. Ahmad Agil, Sp.U(K)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 requestAndrey Satria Julian: Conceptualization, Methodology, Software, Validation, Formal analysis, Investigation, Resources, Data curation, Writing – original draft, Writing – review & editing, Visualization, Supervision. Ahmad Agil: Conceptualization, Methodology, Software, Validation, Formal analysis, Investigation, Resources, Data curation, Writing – original draft, Writing – review & editing, Visualization, Supervision.None declared.


Mesenteric cyst is a rare entity with a very low incidence. The majority of the cases are incidental. Despite several theories, its etiology remains unknown. Some cases present with non-specific symptoms such as pain abdomen, swelling, and abdominal mass. It may rarely get complicated due to hemorrhage, torsion, or rupture of the cyst. Large mesenteric cysts are quite uncommon. However, these cysts seldom grow to produce clinical symptoms arising from compression of adjoining structures, such as vomiting, constipation due to intestinal obstruction, or dyspnoea due to compression of the diaphragm. Despite several theories, its etiology remains unknown. Diagnosis can be achieved with the help of radiological examinations such as ultrasonography (USG), contrast-enhanced computed tomography (CECT), magnetic resonance imaging (MRI), and confirmed by histopathological examination. We report a case of a giant mesenteric cyst in a five-year-old girl. Mesenteric cysts are rare abdominal tumors with a reported incidence varying in the literature between one in 100,000 and 250,000 . DiagnosTreatment requires complete surgical removal either by open or laparoscopy method. Complete surgical excision is important to avoid a risk of recurrence . We presA five-year-old female child arrived at the emergency department with a hugely distended abdomen. She had a history of abdominal distension for the last two years. The patient was not able to squat because of her massively distended abdomen. She complained of respiratory distress and repeated vomiting on taking meals. On examination, the abdominal swelling was soft in consistency with palpable fluid wave. However, there was no shifting dullness appreciated. Her routine investigations were insignificant except for a slightly raised leucocyte count. Contrast-enhanced computed tomography (CECT) of the whole abdomen showed a large cyst occupying the entire abdominal cavity. The small gut was pushed to the left upper quadrant of the abdomen Figures , 1B.Because of the large size, a laparoscopic approach to remove the cyst was deferred. The abdomen was explored by a midline incision under general anesthesia. The cyst bulged out of the incision with the adjoining involved gut depicting the highly increased intra-abdominal pressure Figure . The cysThe first report of a mesenteric cyst was an incidental autopsy finding dates to 1507 found by Benevenni, Italy. Rokitansky described the features of a mesenteric cyst which was published in a handbook of pathology . The mesThe most accepted classification was given by Beahrs et al. in 1955, in which cysts were grouped into four types such as developmental, traumatic, infectious, and neoplastic. They can range in size from a few millimeters to a few centimeters in diameter. But in some cases, a massively enlarged cyst may mimic ascites, as in our case . Though Assessment requires complete medical history and clinical examination and confirmation by appropriate radio-pathology evaluation. Ultrasound and CT scan easily detect the size, location, thickness of the wall, fluid or solid component, and septation in the cyst. CECT is very much helpful as it was in our case. Treatment of choice is complete surgical excision of the cyst . The surA mesenteric cyst should be included in the differential diagnosis of any abdominal mass or distension besides common differential diagnoses. The larger cysts require a high degree of clinical suspicion, as they may fail to produce the relevant clinical signs. Assessment should involve a proper medical history, clinical examination coupled with complete radiological and pathological evaluation. The surgical approach depends upon the size, symptoms, and present complications. A judicious selection of the surgical approach is valuable for a better therapeutic outcome.


An intestinal stoma is an opening of the intestinal tract onto the anterior abdominal wall. It is a commonly performed surgical procedure done for various benign and malignant pathologies. The construction of the stoma is temporary or permanent. Loop stoma is usually performed to divert the faecal stream for protection of the downstream anastomosis. They are usually reverted once the purpose of their creation is served. Spontaneous closure is a rare event that could result from a gradual stomal retraction. However, a normal bowel with no distal obstruction would be a prerequisite for it to be asymptomatic. Here, we report a case of spontaneous closure of a diversion loop sigmoid colostomy which had a delayed presentation. This is the second case of spontaneous closure of a sigmoid loop colostomy and the first report on the management of ventral hernias following spontaneously closed stoma in the English literature to the best of our knowledge. Spontaneous closure of a stoma is a rare occurrence . It is tA 42-year-old male with a past medical history of Hirschsprung's disease presented to our outpatient department with worsening constipation over the last ten years. He was submitted to a Duhamel procedure with a diverting sigmoid loop colostomy at the age of 15 years. Following this, he had an unusual history of spontaneous closure of the colostomy at eight weeks post-surgery with normal passage of stool per rectum. After 15 years, he developed worsening constipation, which was managed with an increasing dose of laxatives. Evaluation with colonoscopy showed a sigmoid stricture and a pseudo-diverticulum formation proximal to the stricture. There was no gross mucosal abnormality at the site of narrowing and biopsy revealed chronic granulation tissue. The biopsies distal and proximal to this site showed ganglion cells in the submucosa. A contrast-enhanced CT of the abdomen revealed a 4cm x 3cm ventral hernia at the previous colostomy site, with the sigmoid colon reaching the level of the skin Figure , 1B. SysThe procedure was completed laparoscopically. A closed pneumoperitoneum was established with Veress needle through Palmer’s point and a 5mm port was inserted. Another 5mm port was inserted in the left anterior axillary line. After adhesiolysis in the epigastric region, a third 5mm port was inserted in the epigastrium. Omental and small bowel adhesions to the parietal wall were lysed by sharp dissection with scissors. Subsequently, a 10mm port was inserted to the right of the umbilicus in the mid-clavicular line. A fifth port (5mm) placed in the right iliac fossa served as the right-hand working port for the surgeon Figure , 2B.After the initial entry to the peritoneal cavity, adhesiolysis were performed with the surgeon on the left of the patient and the camera assistant to his left. However, the sigmoid colon proximal and distal to the herniated site was mobilized from the right side Figure . An elliThe post-operative period was uneventful. The patient was started on an oral diet on the first post-operative day and discharged by the third post-operative day. On follow-up at six months, there was no evidence of a hernia.Spontaneous closure of a loop colostomy is a rare occurrence, underlying mechanisms of which are poorly understood . The repSpontaneous closure of a colostomy stoma is a serendipitous event for the patient in whom there is no pathology of the distal bowel, thus avoiding an elective surgery for stoma closure . FurtherThe reported incidence of incisional hernia following stoma closure is around 30% . This riThere are no reports in the literature on the management of ventral hernias following spontaneously closed stoma to the best of our knowledge. The concern specific to this problem is the bowel just below the skin, which can be breached during hernia repair. This would convert the wound into a clean-contaminated wound, increasing the risk of infection to a mesh used for hernia repair. This led us to offer repair as a two-stage procedure for our patient.In our patient, there was a mechanical obstruction because of the kink of the bowel at the site at which it had adhered to the skin, seen as stricture with pseudo-diverticulum on colonoscopy. This was resected using a stapler. Although contamination would be limited by the use of a stapling device, the wound would still qualify as a clean-contaminated wound. Therefore, an anatomical repair was undertaken. The use of biological mesh to prevent an incisional hernia at a stoma closure site in a retro rectus position has been reported by Sepehr Lalezari et al., albeit in a few patients .In summary, though a spontaneous closure of a loop colostomy is rare and may be seen as a fortuitous outcome in the short term, the inevitable consequence of a ventral hernia at the site would mandate an elective or emergency repair in the long term. Patients with a spontaneous closure should thus be counselled accordingly.Spontaneous closure of the stoma is a rare occurrence. The underlying mechanisms are poorly understood. Even though it can be a serendipitous event preventing a surgery in a few, a ventral hernia is inevitable. This can present in a delayed fashion with mechanical obstruction secondary to bowel adhesions. These patients need to be followed in the long-term for a ventral hernia at the site of previous stoma and are best served by an elective repair.


Neurosarcoidosis involving the spine is uncommon. Sarcoidosis of the spine usually presents as an intramedullary lesion and rarely an epidural lesion. To have recurrence of neurosarcoidosis is an even rarer presentation. Here, we present a 37-year-old man with poorly controlled sarcoidosis who initially presented to our medical center in 2015 with thoracic myelopathy from epidural spinal sarcoidosis treated with thoracic decompression and fusion. He presented to the hospital 5 years later with a month history of progressive upper extremity weakness. MRI revealed recurrent stenosis and spinal cord compression in the cervicothoracic junction. Urgent surgical intervention along with medical management resulted in symptomatic and functional improvement. Surgical intervention and compliance with postoperative corticosteroid therapy seem to yield a favorable prognosis for patients with epidural spinal sarcoidosis and to avoid recurrence. Several case reports have documented the presentation of sarcoidosis in the vertebral bodies.23456789101112 However, cases of intraspinal sarcoidosis have been reported but are almost exclusively that of intradural spinal cord disease.13141516171819 There are eight reported cases of intraspinal epidural sarcoidosis published.2021222324252627 Most cases present in the thoracic spine23242526 with one reported case in the lumbar spine27 and one in the cervical spine.24 To the best of our knowledge, the reporting of recurrent intraspinal epidural sarcoidosis in the spine has only been reported once in a pediatric patient. Thus, we report a unique case of intraspinal epidural sarcoidosis in an adult patient presenting with recurring sarcoidosis-associated myelopathy in both the thoracic spine and the cervical spine on two separate presentations 5 years apart.Sarcoidosis is a systemic disease of unknown etiology characterized by granulomatous inflammation. It most commonly affects the lungs, skin, and eyes but can also manifest in other organs.A 37-year-old African American man presented to our emergency department with 1-month history of progressive bilateral upper extremity weakness, with his right side worse than his left and gait instability. He did not report urinary or bowel incontinence. He has a medical history of poorly controlled sarcoidosis, with a known history of uveitis, pulmonary nodules, mediastinal adenopathy, and lytic hand lesions. He had stopped his medications approximately 1 year before his presentation.Approximately 5 years before the current presentation, he presented to our emergency department with back pain and progressive bilateral lower extremity weakness, paresthesia, and gait disturbance. He had a normal motor examination except for 4/5 grade strength with left hip flexion with diffuse paresthesia over the entire left leg. Perianal sensation and rectal tone were intact with negative Hoffman sign, Babinski, and clonus. MRI revealed a thoracic epidural mass compressing the spinal cord at T4-T6 Figure . A CT-guAt his present visit, he had an ataxic gait, manual muscle testing showed 5/5 bilaterally in the deltoid and biceps, 4/5 bilaterally with wrist extension, 4/5 in the triceps on the left and 3/5 in the triceps on the right, 4/5 bilaterally with finger flexion, and 3/5 in the interosseous muscles of hands bilaterally. Inspection of the upper extremities revealed intrinsic wasting of his hands and atrophy of his forearms, with right being greater than left. He also had pain and swelling of both hands over his interphalangeal joints. In addition, soft-tissue nodules were present on the right side of the posterior aspect of his neck and over his previous surgical spine incision. Upper motor neuron signs were not present in his bilateral upper and lower extremities. His perianal sensation was intact, and he had normal rectal tone. MRI of the entire spine with and without contrast showed enhancing soft tissue extending along the right lateral and posterior epidural space from C4 to T1, causing severe stenosis and mass effect and leftward deviation of the spinal cord. The epidural tissue extends into the right C4-5, C5-6, C6-7, and C7-T1 neural foramina. There are T2-hyperintense signal changes within the cord at the level of C5-6 and C6-7, suggestive of myelomalacia (Figure He was initially treated with intravenous Decadron 10 mg every 8 hours for 24 hours, for which he reports improvement in hand paresthesia. Given the patient's symptoms of progressive upper extremity weakness with imaging findings of cervical stenosis and spinal cord compression from likely epidural sarcoidosis, he was recommended urgent surgical intervention which he agreed to after discussing the risks, benefits, and expectations. Before surgery, pulmonology, rheumatology, and medicine service were consulted for medical optimization because patient is at risk of cardiopulmonary sarcoidosis as a former smoker with a known history of pulmonary sarcoidosis. He underwent chest CT that showed mediastinal adenopathy and multiple small pulmonary nodules in bilateral upper lobes without parenchymal disease.The patient was surgically treated with C3-C7 laminectomy with extension of his previous fusion from C2 to T6. The posterior spine was exposed from C2 to the previous fusion at T3-T6. Exposure of the previous surgical site revealed a solid fusion mass from T3 to T6 bilaterally with adequate fixation of all previously placed pedicle screws. Instrumentation was placed from the previous fusion to C2 to adequately stabilize the cervicothoracic spine for the necessary decompression Figure . At his 1 A large number of case reports on neurosarcoidosis are available in the literature. Approximately 5% of all sarcoidosis involve the central nervous system, with most being intracranial.26 Only 10% of neurosarcoidosis involve the spine.26 Sarcoidosis has been described to manifest both in the vertebral bodies and within the spinal canal.234567891011121314151617181920212223242526 Those with intraspinal presentation are almost exclusively intradural sarcoidosis.131415161718192021 To the best of our knowledge, there are nine reported cases of intraspinal epidural sarcoidosis in the literature, including ours (Table Neurosarcoidosis of the spine is a rare presentation of this systemic disease characterized by granulomatous inflammation.Our case is unique in that our patient developed recurrence of intraspinal epidural spinal sarcoidosis-associated myelopathy. Approximately 5 years before his recent presentation, he came to medical attention for epidural spinal sarcoidosis-associated thoracic myelopathy. He endorsed progressive weakness in his legs and was treated with a thoracic decompression and instrumented fusion in addition to steroids. His neurologic symptoms improved after surgery, but unfortunately, he did not comply with the recommended systemic treatment, and it was likely the cause of his recurrent epidural spinal sarcoidosis. MRI from 5 years ago did show anterior epidural soft tissue at the C5-6 level with marrow replacement involving the C6, thoracic, and lumbar vertebra; however, there was no evidence of cord compression at that time. With the absence of systemic treatment, this allowed the epidural tissue to expand and grow.On presentation to us, our patient endorsed progressive upper extremity weakness for approximately 1 month. Given his medical history of poorly controlled sarcoidosis and surgical treatment of epidural spinal sarcoidosis-associated thoracic myelopathy, the presence of a compressive cervical epidural mass on imaging with MRI increased our suspicion for a diagnosis of recurrence of epidural spinal sarcoidosis. Given the presence of a progressive neurologic deficit and the successful outcome of the previous surgical intervention for his epidural disease, the patient was recommended surgical intervention again in addition to preoperative and postoperative corticosteroids as a treatment for which he consented.20-24252627 After surgical decompression, each patient underwent medical management with steroid taper20-24252627 and two patients had additional methotrexate therapy.24 Longo et al.23 reported a patient with multiple epidural lesions at T4-T6, T8, and L2-L3 who only received medical management, initially with 10 mg bolus of dexamethasone followed by 4 mg dexamethasone every 6 hours. She was then discharged with oral prednisone taper over a 4-month period. At 1 week after discharge, her gait had improved markedly, and at 18 months after initial admission, she was ambulating without difficulty, neurologically intact, and employed.23 Similarly, Nardon et al.25 also reported a case of an 80-year-old woman with cervical neurosarcoidosis who was also managed with 2 months of steroids with improved neurologic function and MRI demonstrating a resolution of epidural mass.As with our case, seven of the eight cases of intraspinal epidural sarcoidosis presented with neurologic deficits, and surgical treatments were undertaken in each with improvement in their neurologic symptoms.21 reported one case of neurosarcoidosis in a pediatric patient who developed recurrent neurosarcoidosis due to noncompliance with medical treatment similar to our patient. They presented a case of a 9-year-old girl with rapid onset thoracic myelopathy who was experiencing progressive lower extremity weakness with falls, urinary symptoms, and bilateral thigh pain over the course of 2 weeks. MRI noted extensive circumferential enhancing epidural mass from T3-6 with severe spinal cord compression and cord signal change, and an emergent open biopsy was done using a left T3-4 hemilaminotomy and debulking of the epidural mass. Corticosteroids were started in the immediate postoperative period for a short postoperative course. Within a week, her neurologic deficit had improved. She would go on to have a couple of recurrences within months, the first recurrence treated with additional thoracic laminectomies and mass debulking and the second recurrence managed medically, with the first recurrence noted after only a short course of corticosteroids was given and the second recurrence noted from noncompliance with medical therapy. One year after the initial presentation, she was noted to have normal function.Galgano et al.17In the eight cases of epidural spinal sarcoidosis, including ours, all of whom presented with a neurologic deficit, a combination of surgical intervention and medical therapy ultimately resulted in an improvement of their neurologic deficit and thus yielded a favorable outcome. In addition, our case and the case reported by Galgano et al. shed light on the importance of postoperative medical therapy because our patient had demonstrated that noncompliance with medical therapy resulted in the recurrence of neurosarcoidosis. However, the case reports of epidural spinal sarcoidosis are not consistent with those of intradural diseases regarding steroid treatment and recurrence. Some cases used postoperative steroid treatment, but others did not, and regardless of whether steroids were given, no recurrence was observed.In conclusion, epidural spinal sarcoidosis is rare. From the cases reported in the literature, including our patient, they all presented with a neurologic deficit that improved with a combination of surgical intervention and medical therapy, consisting mainly of corticosteroids. Two of the cases stress the importance of postoperative systemic therapy to prevent recurrence of the epidural disease. Surgical intervention and compliance with postoperative corticosteroid therapy seem to yield a favorable prognosis for patients with epidural spinal sarcoidosis.


Nasolabial cyst or klestadt’s cyst is a rare nonodontogenic lesion occurring in the maxillofacial region. It is commonly seen in the nasoalveolar area, lateral to ala of nose.It is usually asymptomatic and is often ignored by the patient unless it enlarges in size resulting in cosmetic deformities. It is often challenging not only to maxillofacial surgeonsbut also to other head and neck surgeons. It is often misdiagnosed as other common dental related or odontogenic lesions and mistreated. Here we are describing such a surgically managed caseas a case report and also discussing the etiopathogenesis and management. Nasolabial cyst is a benign slow growing extra osseous lesion usually seen in the nasal alar region which was first reported by Emil Zukerkandl  . KlestaA 48-year-old female reported to the Department of Oral and Maxillofacial surgery (OMFS) with a painless swelling on the left side of the nose, which was noticed 8 months back.No history of trauma was elicited. Swelling was persistent and was enlarging in size, as per history.On extraoral examination, a swelling sized 2×2 cm with ill-defined borders was elicited, lateral to the ala of the nose. The surface was smooth, and no punctum was seen.The swelling was soft and cystic with mild tenderness on palpation. The swelling was palpable intra-orally into the buccal vestibule of the upper left canine region . It was nonmobile and cystic in nature. Infective foci of dental origin were ruled out, with thermal and electrical vitality testing of teeth, Hence the possibility of a radicular cystor dentoalveolar abscess was ruled out.A computerized tomographic (CT) scan revealed a well-defined round soft tissue lesion in the lateral pyriform apertureFigures  and 3. Based on the findings, a provisional diagnosis of a non-odontogenic/ soft tissue cyst or tumor was made, which included nasolabial cyst, lipoma, dermoid cyst and epidermoid cyst.An excision biopsy was planned under local anesthesia.Left infraorbital nerve block and local infiltration in vestibule and palatally infiltration was performed for the patient. Then, intraorally vestibular incision at level of nasal flooronly through the mucosa, and careful blunt dissection done to deepen to the lesion  was perfOnce the lesion was exposed, blunt dissection was proceeded around to free it from the floor of nose .IntraopNasolabial cyst is purely a soft tissue nonodontogenic cyst though it has been classified under jaw cysts. It is also termed as the mucoid cyst of nose, nasal cyst and sometimeseven as nasoalveolar cyst. Most of the reported cases have been unilateral in nature, but bilateral cases have also been reported in the literatures  - 7. ThNasolabial cyst exhibits wide variety of clinical symptoms and usually varies from a painless swelling to an area of localised numbness. Usually they are found near the opening of thenasolacrimal duct in the inferior meatus. A soft oval palpable mass is usually seen in ala area. Bimanual palpation of nasal floor and labial sulcus often confirms its position.Secondary infection may result in tenderness and pus accumulation followed by spontaneous rupture and drainage into oral or nasal cavity. Rarely, it causes difficulty in breathingor nasal blockage unless greater dimensions are achieved to produce symptoms. Extraorally, the cyst may obliterate the nasolabial fold, often lifting the nasal ala causingdeformity of the nostril. Similar scenario was also observed in our case. Rarely dacrocystitis may occur with epiphora  - 10 ifDiagnosis is usually made by clinical findings with CT or MRI. Several imaging modalities may be used. Since without bone involvement, it is rarely identified in plain radiographs.Though MRI gives good soft tissue definition, CT is preferable as it is less expensive. Ultra sound can also be a diagnostic method and has advantages over other invasive techniques  - 12. Surgical excision is the treatment of choice with less morbidity. An intra oral approach is ideal with good cosmetic results. Other modalities include endoscopic excision or even marsupialization  - 14. TIntra operative nasal mucosal perforation is a common complication which can be sutured or small perforations will heal spontaneously even if left untreated.Such complications were not observed in our case. Recurrence is usually rare on complete removal of lining and malignant transformation has not been reported but cellular changeshave been reported in some cases in literature reviews. Informed consent from patient obtained.Nasolabial cysts are rare soft tissue cysts less commonly seen in the population. The differential diagnosis of normal anatomical variation in the area and other vascular lesionsshould be kept in mind before surgical management. It is usually confirmed by clinical examinations aided with common imaging modalities. Surgical excision gives excellentresults with less morbidity and recurrence.The authors declare that they have no conflict of interest.


Carney complex is a rare genodermatosis characterized by cardiac and cutaneous myxomas, among other tumors. In the majority of cases, cutaneous myxomas precede the diagnosis of cardiac myxomas, which are the main cause of death in these patients. Despite the fact that the diagnosis of cutaneous myxomas is histopathological, high-frequency ultrasonography plays an essential role in the differential diagnosis with other cutaneous and subcutaneous tumors. The authors of the present study describe, for the first time in the literature, the ultrasonographic features of both variants of cutaneous myxomas, superficial and subcutaneous, in a patient with a Carney complex. This gene codes for the regulatory subunit type I alpha of the protein kinase A enzyme, which is a key component of the cAMP signaling pathway, involved in endocrine tumorigenesis. To date, more than 125 pathogenic inactivating mutations have been reported. For the majority of the PRKAR1A-negative CNC patients, the genetic cause remains unknown.Carney Complex (CNC) is a rare autosomal dominant neoplasia syndrome characterized by cardiac and cutaneous myxomas, mucocutaneous pigmented lesions , and multiple endocrine tumors often causing endocrine abnormalities involving various organs.A 20-year-old man with a personal history of surgical excision of various cutaneous lesions  was referred to the present study’s department for evaluation and treatment of acne. Physical examination revealed facial and labial lentigines, a subcutaneous nodule on the left malar area, as well as multiple pedunculated, velvety, soft, and pink papules located on the trunk.Gray-scale and color Doppler US examinations of both the subcutaneous nodule and one of the papules located on the right periareolar region  were perfsX5 mutation in the PRKAR1A gene, which was present in the patient but not in his first-grade relatives. Therefore, it was a sporadic case of CNC. The patient is followed regularly in the present study’s department in order to rule out new-onset associations.With the diagnosis of CNC, the following tests were performed: echocardiogram, thyroid and testicular US, 24-h urinary free cortisol, plasma insulin-like growth factor-1, and PRKAR1A sequencing. No alterations were found except for the presence of thyroid colloid nodules and the p.Val164Glucafé au lait spots, melanocytic nevi, lipomas, and angiofibroma. After cutaneous lentigines and blue nevi, cutaneous myxomas are the third most frequent skin manifestation (30%–55% of patients). They usually present as multiple, asymptomatic, sessile, or pedunculated pink papules located commonly on the eyelid, external ear canal, breast nipples, and genitalia. They can be localized in the dermis, or the subcutaneous layer and they measure less than 1 cm in diameter.4Carney complex is a rare genodermatosis with more than 600 cases reported worldwide. Many skin manifestations are numbered among its diagnostic criteria, including cutaneous lentiginosis, cutaneous or mucosal myxomas, and multiple blue nevi. Other skin lesions associated with CNC are On the other hand, cardiac myxomas are the most common noncutaneous lesions found in CNC (20%–40% of patients). They can appear early in infancy and occur anywhere in the heart. They present with obstructive, constitutional, or embolic phenomena like strokes, heart failure, or sudden death. They are responsible for more than 50% of the mortality of the disease and considering that 81% of patients with cardiac myxomas have previously presented cutaneous myxomas, the existence of the latter should always lead the physician to rule out cardiac involvement.6While cardiac myxomas sonographically present as pedunculated, atrial, hyperechoic masses; ultrasonography of cutaneous myxomas is yet to be described in the literature. The authors have observed that they show different ecographic features depending on their location in the skin. Superficial myxomas present as well-defined, round, homogeneous, and hypoechoic lesions located in the superficial dermis with an elevation of the epidermis. Instead, subcutaneous myxomas appear as ill-defined, elongated, heterogeneous and hypoechoic masses located in the deep dermis and hypodermis. The sonographic differential diagnosis of superficial myxomas should be made with benign skin tumors such as cysts, dermatofibromas, pilomatrixomas, and neurofibromas. Cysts are well-defined, round, hypoechoic, or anechoic lesions and usually present posterior enhancement and bilateral edge shadowing. Dermatofibromas are ill-defined, hypoechoic and heterogeneous lesions. In the US, pilomatrixomas appear as hypoechoic lesions with well-defined margins and typically present multiple small calcifications and posterior shadowing. Color Doppler US generally reveals a single artery that penetrates the lesion and gives rise to multiple intralesional branches.In conclusion, despite the fact that distinctive features have not been identified, the authors believe that high-frequency US is a valuable complementary tool in the diagnosis of cutaneous myxomas in patients suspected of CNC. As far as the authors are concerned, this is the first ultrasonographic description of superficial and subcutaneous myxomas. High-frequency ultrasonography also enables the follow-up of those lesions not tributary to surgical treatment, especially the subcutaneous variant in locations such as the face.None declared.Monica Quintana-Codina: Approval of the final version of the manuscript; critical literature review; data collection, analysis, and interpretation; effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic management of studied cases; manuscript critical review; preparation and writing of the manuscript; study conception and planning.Marina Corral-Forteza: Approval of the final version of the manuscript; data collection, analysis, and interpretation; effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic management of studied cases; manuscript critical review; study conception and planning.Maribel Iglesias-Sancho: Approval of the final version of the manuscript; data collection, analysis, and interpretation; effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic management of studied cases; manuscript critical review; study conception and planning.Montse Salleras-Redonnet: Approval of the final version of the manuscript; data collection, analysis, and interpretation; effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic management of studied cases; manuscript critical review; study conception and planning.None declared.


Arterial tortuosity syndrome (ATS) is a rare autosomal recessive connective tissue disorder chiefly characterized by elongated and tortuosity of the large and medium sized arteries and anomalies of the vascular elastic fibers. Here we reported cases of brother about ATS from the same family on the prenatal ultrasound diagnosis. Reports of this case are rare in antenatally and we draw the vessel simulated diagram to display visually.Prenatal ultrasound scanning at 29 weeks of gestation of the first fetus showed obvious tortuous and elongated of the aortic arch, ductus arteriosus, left and right pulmonary arteries, carotid and subclavian arteries. Three months after delivery, Contrast-enhanced computed tomography images (CTA) were performed to clearly display vascular abnormalities consistent with prenatal diagnosis of ultrasound. Whole exome sequencing (WES) was performed eight months after birth, two heterozygous variants of SLC2A10 gene was detected in newborn and their father and mother, respectively. Prenatal ultrasound scan at 22 weeks of gestation of the second fetus showed similar cardiovascular imaging. After birth the siblings have facial characteristic features gradually as aging. No surgical intervention was performed in the siblings follow up 19 months.The key points of prenatal ultrasound diagnosis of ATS are the elongation and tortuosity of the large and medium sized arteries. Genetic counseling is the process of providing individuals and families with information on the nature, inheritance, and implications of genetic disorders to help them make informed medical and personal decisions.The online version contains supplementary material available at 10.1186/s12884-021-03960-w. Arterial tortuosity syndrome (ATS) is a rare autosomal recessive connective tissue disorder chiefly characterized by elongation and tortuosity of the large and medium sized arteries and anomalies of the vascular elastic fibers, and a propensity for arterial and aortic aneurysm formation with vascular dissection, caused by mutations in SLC2A10 [A 29 weeks fetus suspected fetal heart abnormality was referred to our center for the detailed prenatal ultrasound scanning. Routine scanning demonstrated that the fetus was in good status without any extra-cardiac abnormalities. Echocardiography showed Fig.  obviouslThe newborn presented with respiratory distress after birth. Umbilical cord blood was performed chromosome microarray detection at the time of delivery in the first neonate, indicating no abnormalities of sex chromosome and autosomes with definite clinical significance. Postpartum CT diagnosis: pulmonary hypertension, left and right ventricular wall thickening, the aorta, PA and cervical artery were tortuous, and clinically suspected Marfan syndrome.Three months after delivery in the first neonate, CTA was performed to clearly display vascular abnormalities. The MPA send out RPA and LPA, LPA distinctly tortuous and elongated through the front of the trachea. The aortic arch arises from the ascending aorta and pass backward to the left of the trachea and esophagus to join the descending aorta which lies farther than normal aortic arch system of man . A left The first baby followed up to 19 months, with normal growth and development and had been hospitalized for several times due to severe pneumonia and occasionally cough with dyspnea. Facial characteristic features gradually as aging, such as blepharophimosis or periorbital fullness, downslanted palpebral fissures, micrognathia, large ears, long face and cutis laxa. Echocardiography in child showed the Arch and PA were still tortuous. These extensive tortuous and elongated vessels would be difficult to handle in the operation according to the consultation of pediatric cardiac surgery experts. It has been reported that severe pulmonary stenosis usually requires surgery and/or transcatheter treatment, but aortic arch abnormality does not require intervention in most cases of ATS .Prenatal ultrasound scanning at 22 weeks of gestation of the second fetus showed Fig.  similar ATS was first described by Ertugul in 1967  and BeurDue to the high suspicion of ATS in prenatal ultrasound and genetic confirmation, we summarized our experience and drew a simulated diagram to facilitate understanding and learning Fig. . Both ofMajor clinical findings mainly divided into cardiovascular involvement, craniofacial involvement, skin and connective tissue disorder. In terms of cardiovascular disease, it could happen aneurysms , 10–12, ATS should be differentiated from EFEMP2-related cutis laxa, Loeys-Dietz syndrome , 18, EhlExtra-cardiac abnormalities are difficult to detect, but the great vessel problems are easy to detect prenatally. In summarize, the key points of prenatal ultrasound diagnosis of ATS are the elongation and tortuosity of the large and medium sized arteries. There are great clinical implication of timely diagnostic work-up in patients with unusual appearance of great vessels of the fetal heart during routine morphology examination.Additional file 1. Fetus echocardiography showing the tortuous process of tracing the course and connecting about the great vessels include AAO, Arch, LPA, RPA, DA.Additional file 2. Spatiotemporal image correlation (STIC) showing the correct vascular connection of the tortuous and elongated great vessels about the first fetus in the 22 weeks.Additional file 3. Spatiotemporal image correlation (STIC) showing another view rotate the STIC images 180 degree to observe the backside about the first fetus in the 22 weeks.Additional file 4. Sonogram showing the the tortuous process of tracing the course and connecting about the great vessels include AAO, Arch, LPA, RPA and DA about the second fetus in the 29 weeks.