Incomplete urachal obliteration during fetal development gives rise to distinct malformations of the median umbilical ligament. Most anomalies are asymptomatic and resolve during early infancy, but some go unrecognized until adulthood. These rare cases can present with acute abdominal symptomatology secondary to infected urachal remnants.A 20-year-old man presented with periumbilical pain. Physical exam showed a warm, erythematous infra-umbilical mass that was tender to palpation. CT revealed an infected urachal cyst. The patient underwent urachal abscess incision and drainage with cyst excision. The patient returned home on postoperative day two. Two-week outpatient follow-up confirmed an uncomplicated recovery.Surgical excision of urachal anomalies in adults is curative and preventive against recurrent infection and malignancy, but preoperative management is currently unstandardized. Current literature recommends a two-stage approach characterized by preoperative antibiotics and cyst incision and drainage followed later by complete surgical excision. Our patient underwent a single therapeutic approach with preoperative antibiotics and definitive operative excision. Our patient had a comparable outcome with no complications and a short hospitalization.Preoperative antibiotics with excision of urachal remnants was effective in our case. Staged approaches are appropriate in complicated cases as a means to reduce post-operative infection rates and hospital stays. Urachal remnant excision is recommended in adults to prevent future malignancy. The urachal canal normally obliterates as the bladder descends into the fetal pelvis, forming a fibrous connection with the ventral abdominal wall known as the median umbilical ligament. Complete obliteration typically occurs during late fetal development or early infancy (<6 months) .When urachal obliteration fails, four distinct embryologic malformations can result: patent urachus, umbilical-urachal sinus, vesicourachal diverticulum, or urachal cyst. In patent urachus, the urachal canal remains open and drains the urinary bladder into the umbilicus. Those with umbilical-urachal sinus possess a single blind opening that may drain into the umbilicus. Patients with a vesicourachal diverticulum have urachal tissue that remains patent inferiorly and creates an outpouching from the dome of the urinary bladder. The process of urachal obliteration can result in a retained urachal cyst along the median umbilical ligament .Many of the aforementioned entities are diagnosed during early infancy and monitored with ultrasonography ,4. AfterHerein, we report a case of an infected urachal cyst in a 20-year-old man discovered on CT scan and treated with preoperative antibiotics and operative therapy. We also discuss the literature for surgical management of urachal disease and associated complications. This case has been reported in line with the SCARE criteria .23 cells/μL and urinalysis was unremarkable. Based on history and physical exam, the patient underwent diagnostic evaluation for suspected incarcerated umbilical hernia.A 20-year-old man presented to the emergency department after four days of progressively worsening periumbilical pain. He was moving heavy boxes for his job when he began experiencing pain and was unable to finish his work. He reported pain with defecation but denied fever, chills, nausea, emesis, weight loss, and recent travel or illness. Past surgical history included branchial cleft cyst excision as a child. On abdominal exam, a one square-centimeter erythematous infra-umbilical mass was exquisitely tender to palpation. Laboratory data on admission demonstrated a WBC count of 10.7 × 10CT abdomen/pelvis revealed a four-centimeter segment of organized periumbilical inflammation with central lucency passing the ventral abdominal wall into the anterior abdominal compartment . The proThe patient was admitted to the surgical floor where he noted his pain was markedly improved. The next day he was discharged to home on post-operative day two with adequate pain control. Two-week follow up in the outpatient surgery clinic confirmed an uncomplicated recovery.3Urachal cyst infections predispose patients to a number of acute complications, including bladder fistula formation, cyst rupture, peritonitis, and sepsis. Surgical excision of embryologic remnants is the therapeutic mainstay for symptomatic urachal disease, but preoperative management strategies often vary. Some advocate for a two-stage management approach focused on treating the urachal cyst infection with antibiotics and ultrasound-guided drainage before operative excision . Others Our patient received antibiotics and prompt surgical excision without experiencing any postoperative infectious complications. His two-day hospitalization was shorter than the average reported with the two-stage management described above. Our approach was nearly identical to a case published in emergency medicine literature that also yielded a good outcome . FactorsSome urachal cyst infections in pediatric patients have been successfully managed with more conservative measures. In a fifteen-patient case series of symptomatic urachal anomalies, three infected urachal cysts were treated with antibiotics and percutaneous drainage (two cases) or laparoscopic drainage (one case) without total excision. The patients in this case series were an average of 3.5 years-old, ranging from four weeks to fourteen-years-old. Urachal infection resolved in all cases, and complete urachal obliteration was confirmed with ultrasonography at a mean follow up of 26 months. These findings suggest that draining infected urachal cysts may be sufficient for the remnants to completely obliterate thereafter. Although symptomatic children can undergo cyst drainage and expect spontaneous urachal obliteration, this remains unclear for adults .Another variable influencing surgical management for urachal disease is the perceived risk of future malignancy. Urachal carcinoma is a rare neoplasm with poor prognosis believed to arise from residual epithelia within urachal malformations from fetal development. Evidence from pediatric surgical literature suggests that asymptomatic anomalies in children do not confer significant risk for malignancy because the vast majority spontaneously resolve with time . The samOur patient’s presentation and physical exam suggested umbilical hernia as the predominant pathology before imaging revealed an infected urachal cyst. The patient was not septic and operative drainage and excision was successful. Follow up in the office showed no untoward outcomes.4We report a case of urachal cyst infection with associated umbilical hernia in a 20-year-old man. He received IV antibiotics and underwent successful urachal cyst excision, local abscess I&D, and primary umbilical hernia repair the following day. After urinary retention resolved on postoperative day one, he was discharged on postoperative day two and made an uncomplicated recovery. Current literature provides evidence to support a two-stage treatment approach as most effective in reducing post-operative complications and prolonged hospital stay. However, our case demonstrated that short term administration of IV antibiotics prior to complete surgical excision was sufficient with no postoperative complications or prolonged hospital stay. This approach can be considered safe with patients with isolated urachal cyst infection. Presentations complicated by urinary bladder fistula, large abscesses, and sepsis are more likely to benefit from a staged approach with prolonged IV antibiotics, ultrasound-guided I&D, and surgical excision after resolution of the acute urachal cyst infection.None.None.This report was conducted in compliance with ethical standards.Informed consent has been obtained and all identifying information is omitted.Adel Elkbuli, Dessy Boneva, Kyle Kinslow, Mark McKenney– Conception of study, acquisition of data, analysis and interpretation of data.Adel Elkbuli, Dessy Boneva, Kyle Kinslow - Drafting the article.Dessy Boneva, Mark McKenney – Management of case.Adel Elkbuli, Kyle Kinslow, John D. Ehrhardt Jr, Dessy Boneva, Shaikh Hai, Mark McKenney – Critical revision of article and final approval of the version to be submitted.This is a case report study.Dessy Boneva.Mark McKenney.Shaikh Hai.Not commissioned, externally peer reviewed.


Mesenteric psuedocysts are rare tumors of the gastrointestinal mesentery that are seldom symptomatic. Although these benign tumors are most commonly found incidentally during work-up for other pathology, they can be troublesome in select patients based off size, location and risk of malignant transformation. This case is reported in accordance with SCARE Criteria .A 24 year-old-male presents with life-long migratory abdominal pain presents with a one week history of acute pain associated with nausea. Computed tomography revealed free fluid in the pelvis and a thin-walled mesenteric cyst within the left, mid-abdominal mesentery measuring approximately 4.3 × 4.0 × 4.0 cm. The patient was admitted for resuscitation and planned delayed operative intervention.Propionibacterium acnes without true cystic wall consistent with an infected mesenteric pseudocyst. These lesions are difficult to diagnose secondary to varied presentation and lack of pathognomonic clinical, laboratory and imaging findings. Mesenteric pseudocyst have a low rate of recurrence after removal; however, surgical management is mandated due to risks of malignant transformation.The patient underwent complete open enucleation secondary to location and in an attempt to limit injuries to or resection of small bowel. Pathological analysis revealed a mesenteric cyst with fluid culture positive for This is a rare case of a mesenteric pseudocyst of small size presenting with lifelong abdominal pain secondary to its location near the root of the mesentery and inflammatory reaction secondary to infection. It is important to maintain a high index of suspicion for mesenteric cyst as many complications may result if misdiagnosed or without proper surgical management. These l2A 24-year-old Caucasian male presented to the emergency department for unremitting left lower abdominal pain for one week that intensified over the last 24 h with nausea. The patient reported a lifelong history of abdominal pain as mild, self-limiting episodes. Past medical history was significant for dilated cardiomyopathy diagnosed 5 years prior to presentation. Past surgical and family history was noncontributory.On clinical exam, all vitals were found to be within normal parameters. The patient’s abdomen was soft, non-distended and tender to palpation in the left abdomen lateral to the umbilicus. No masses, organomegaly, nor hernias were palpated. Laboratory testing was significant for a leukocytosis of 12.4 bil/L and neutrophil count of 10.4 bil/L. Computed tomography revealed free fluid in the pelvis with a thin-walled unilocular cystic structure within the left, mid-abdominal mesentery adjacent to multiple small bowel loops and numerous vessels, measuring 4.3 × 4.0 × 4.0 cm in craniocaudal, AP and transverse dimension. The cystic structure measured Hounsfield units of -4.59 consistent with simple fluid. Differential diagnosis included mesenteric cyst, omental cyst, enteric duplication cyst, lymphoma, or infectious etiology .Fig. 1CoConservative management was attempted based on the patient’s HPI, clinical presentation and imaging. Decision was made to move forward with surgical intervention secondary to progression of the patient’s symptoms. Pre-intervention discussions centered on surgical approach, with consideration for excision of cyst, enucleation, bowel resection, laparoscopic resection, or endoscopic resection. In reflection of the size and location of the mass and surgical yield, decision was made to attempt an open surgical approach.The patient was consented and underwent exploratory laparotomy by a team of experienced surgeons and residents. A mass was found intraoperatvely, initially unable to be delivered through the incision secondary to location and adherent nature to the root of the mesentery. The cyst was eventually mobilized and carefully dissected from surrounding tissue. Secondary to tightly embedded nature within the mesentery and with surrounding intestine, the cyst was unavoidably entered producing thick white drainage then collected and sent to pathology. Decision was then made to perform complete enucleation of the cyst with ligation of all feeding vessels. No resection of bowel was found to be necessary .Fig. 2InThe postoperative period was uneventful and the patient was discharged without complication on postoperative day two. He was seen in office for follow up one week later and with complete resolution of previous symptoms. The patient has no need for further follow-up examinations.Propionibacterium acnes, an opportunistic, gram-positive pathogen found in the normal skin flora, primarily known for its role in acne , obstruction or recurrence. Preferred treatment is complete enucleation, associated with a high cure rate. Excision with enterectomy may be necessary due to intimacy with bowel or vessels feeding the bowel  %, obstr,14. PartIn this case, excision of the cyst would compromise a significant length of bowel secondary to its intimate relation with the mesenteric root vasculature. Many successful laparoscopic cases have been documented. There has yet to be a large study to evaluate the effectiveness or best approach to the technique. Advantages to laparoscopy include decreased postoperative pain, shorter hospital stay, earlier return to normal activity and smaller incision. In many cases, complete excision was achieved without recurrence of the cyst . In the Propionibacterium acnes, an opportunistic gram-positive pathogen found in normal skin flora, primarily known for its role in acne. Reports have also shown that this bacterium is responsible for many cases of postoperative infections and other chronic infections. Reports of P. acnes causing endocarditis of prosthetic and native heart valves, corneal infections, postoperative endophthalmitis, focal intracranial infections and cerebral spinal fluid shunt infections are published [P. acnes isolated in cases of chronic inflammatory disease including chronic prostatitis leading to prostate cancer, chronic recurrent multifocal osteomyelitis, sarcoidosis and sciatica [Propionibacterium acnes has been isolated in a mesenteric pseudocyst.Fluid culture grew ublished . Studiessciatica . The bacsciatica . This ca4Mesenteric cysts are rare tumors seldomly included in the differential diagnosis of a patient presenting with chronic or acute abdominal pain. These lesions are difficult to diagnose secondary to varied presentation and lack of pathognomonic clinical, laboratory and imaging findings. It is important to consider these pathologic lesions due to the morbidity if misdiagnosed or without proper management. These lesions have a low rate of recurrence after removal; however, surgical cure is mandated due to risks of malignant transformation. This case report examines a small mesenteric cyst presenting in a 24-year-old male with reported lifelong migratory abdominal pain and nausea for one week prior to admission. After making the initial diagnosis on CT, successful surgical management with open laparotomy lead to the complete enucleation of the cyst and resolution of the patient’s symptoms.None.IRB Approval exemption has been given from the IRB/Resident Review committee.Consent obtained in person with patient, consent form signed by patient and two physicians.Thomas J Serena, D.O. M.A.: Thomas.Serena@beauont.org, Primary author, corresponding author, study concept/design, analysis and interpretation.Kelly Dinnan, D.O.: Kelly.Dinnan@beaumont.org: Guarantor, Contributing Author.Raisa Gao: Gaoraisa@msu.edu, Contributing Author, review.Retrospective review of case without identification or new/investigational treatments.Registered with Research Registry: researchregistry5170.Kelly Dinnan, DO.Not commissioned, externally peer-reviewed.None.


Lipomas are the most common benign mesenchymal tumors found in humans, with a prevalance rate of 2.1 per 1000 tumors. Most of them are small, weighing only a few grams and measuring less than 2 centimeters in diameter. However, those weighing upwards of 200 grams and exceeding 10 centimeters have only been described in different anatomic locations on occasion in the literature. A 54-year-old man presented with a large soft tissue growth over the lower back, present for the past 20 years and rapidly enlarging over the past 3 years. A diagnosis of giant lipoma was made. Six hours and several surgical teams were required to remove the 14 kilogram mass. During excision, the skin flaps overlying the mass were preserved and were ultimately used to reconstruct the surgical site defect in a layered fashion once intraoperative frozen pathology confirmed the pre-operative diagnosis. Benign lipomas of the size reported in this case have rarely been described in the literature, as lipomatous masses of this size are often found to instead be either atypical lipomatous tumors or high-grade liposarcomas. In this case, we describe one of the largest of these giant benign lipomas ever reported to date. The case also illustrates the use of an interdisciplanary, multi-team approach to undertake the high-risk operation of removing such a mass from a patient safely. Finally, the case describes aninteresting approach toward reconstructing the large soft tissue defect that remained once the tumor had been removed from the patient.  However, given the size of these lesions, the resultant soft tissue defecit can often be quite large and as a result can be challenging for plastic surgeons to reconstruct. In this manuscript, we report the case of one of the largest benign lipomas ever recorded in the literature. The case is not reported beause of the rarity of the condition, since lipomas are one of the most common benign masses, but because of itslarge size. The enormity of the mass highlights some of the unique challenges associated with the diagnosis, surgical resection and reconstruction of these giant benign tumors.A 54-year-old man with a past medical history significant only for hypertension presented to our clinic with a large soft tissue growth on hislower back which had been present for the past 20 years. Over the past three years it had been rapidly enlarging, nearly doubling in size over that time frame. He had recently re-established medical care after having not seen a physician since childhood. At the time of presentation, he denied any pain or tenderness over the mass and denied any systemic symptoms such as fever, night sweats, and weight loss. The patient had an unremarkable physical exam except for the large soft tissue mass over the lower back, with the maxiumum dimension measured to be 38cm  .After evaluation by the surgical oncology and radiation oncology services, an abdominal CT scan was obtained and thisdemonstrated a large , heterogeneous soft tissue mass. A differential diagnosis consisting of teratoma versus liposarcoma was established based on the radiologic imaging. Subsequently, several core biopsies of the mass were performed, all of which revealed fat necrosis with calcifications.Surprisingly, given the size and rapid growth of the mass, a diagnosis of benign giant lipoma was made. Four weeks after presentation, several surrgical teams performed a six hour operation to remove the 14 kilogram mass. After the patient was widely prepped and draped, the skin overlying the central portion of the tumor was shaved and harvested as multiple split thickess skin grafts . SubsequThe specimen was sent for frozen section analysis,which was consistent with a lipoma, and was confirmed on final the pathology. The defect was able to be closed primarily with the preserved skin flaps, which measured greater than 200 cm × 40 cm . The ski4,6The patient in this case had not accessed the health care system for over 20 years and initially presented to a new primary care physician for evaluation of this mass. The initial treating physician promptly referred the patient to our institution given the very uncommon situation of a patient presenting with a mass of this size. As in this case, the identification and treatment of such a large mass is best performed at a tertiary care center where a patient can be evaluated by a multidisciplanary team of physicians with extensive experience in managing large soft tissue tumors.4,6The plastic surgeons have an important role as part of this team, as they are often required to assist with the closure of the large and often complex soft tissue defects that result after resection of such a mass. This can often be a daunting task considering patients can receive radiation therapy to the area and are sometimes malnourished. For these reasons, it is critical for the plastic surgeon to evaluate these patients pre-operatively and be an integral part of the pre-operative planning from the outset.4,6At our center, the longstanding relationship we have with the surgical oncologists enabled this patient to not only receive a prompt diagnosis but also allowed his wound to be completely reconstructed without the need for local muscle flaps, skin grafting or free tissue transfer. Using a combined multi-team approach, the skin envelope surrounding this large mass was able to be preserved during the resection so that it could be used to achieve primary wound closure at the end of the case. Additionally, although we did not ultimately use them, split-thickness skin grafts were harvested from the skin overlying the tumor at the begnining of the case with the plan to use them for reconstruction in the event that the preserved skin flaps might prove insufficient or unsuitable for wound closure due to ischemia or positive resection margins.The ability to perform this patient’s reconstruction was a direct result of the collaboration between both the plastic surgeon and surgical oncologist in the operating room for the duration of the case as well as the meticulous pre-operative planning by an interdisciplanary team of phycsicians. As illustrated by this report, we advocate that plastic surgeons actively participate as part of pre- and intra-operative interdisciplanary teams when undertaking a complex operation to remove such a mass from a patient in the safest manner possiblein order to achieve the best possible outcome.The authors declare no conflict of interest.


Embryonal sarcomas of the liver (ESL) are extremely rare solid tumors appearing mainly in children. The therapeutic standard for an ESL is a margin free resection combined with chemotherapy. The Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) procedure as a surgical therapy offers a curative approach for liver tumors of various origins where the future liver remnant (FLR) would be insufficient after a one-staged (extended) hemihepatectomy.A 19-year-old patient was diagnosed with an undifferentiated embryonal sarcoma of the liver (UESL) in the right liver lobe with oligometastatic spread to the lungs.After neoadjuvant chemotherapy remission was enough to plan a resection of the liver tumor. During the operation we changed our strategy from one-stage hepatectomy to ALPPS because of borderline FLR and macroscopic and histologic liver damage to avoid posthepatectomy liver failure. The interstage and postoperative course of the patient was uneventful beside postoperative bile leakage, which was treated by interventional drainage and stenting.The ALPPS-procedure as a comparatively new surgery was considered over a portal vein ligation or embolization. ALPPS shows a faster hypertrophy compared to standard one-staged hemihepatectomy with decreased or similar proliferation, apoptosis or angiogenesis (at least for CRLM)In experienced centers the ALPPS-procedure is evolving as the safer approach in hemihepatectomys where the FLR is critical. Additionally, ALPPS can serve as an intraoperative option when liver volume and quality seem not to be sufficient and is to be considered when facing new tumor-entities. Most common types of soft tissue sarcomas are rhabdomyosarcoma 57 %), neuroectodermal (10 %) and synovial tumors (8 %). Of primary liver tumors in children, the hepatoblastoma and the hepatocellular carcinoma are the most common, followed by the focal nodular hyperplasia and then the embryonal sarcoma. The undifferentiated embryonal sarcoma of the liver (UESL), firstly identified as an independent clinicopathologic type of sarcoma in 1978, is seen in about 2 % . Typical7 %, neurThe Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) is a two-stage hepatectomy with an extensive regenerative response. The procedure provides the possibility of resecting a liver tumor leaving an otherwise insufficient future liver remnant (FLR) for an one-staged operation by inducing liver hypertrophy via a two-step approach .Purpose of this case report is to show the efficacy of ALPPS as an established method to increase the resectability rate and the important role of intraoperative histological analysis. To our knowledge, we describe the first UESL treated with ALPPS.This paper has been reported in line with the SCARE criteria .2A 19-year-old male patient presented with intermittent shoulder pain and a palpable mass in the right upper quadrant, causing abdominal discomfort. MRI-imaging revealed a hyper intense inhomogeneous mass located in the right lobe of the liver with an approximate size of 9,5 cm in diameter and five nodules in both lower lobes of the lung . The worDefinitive diagnosis was made by biopsy of the pulmonary nodule of the left lower lobe, showing an undifferentiated pleomorphic embryonal sarcoma. The staging was cT2b cN0 cM1 (PUL), UICC IV, Intergroup Rhabdomyosarcoma Studies (IRS) stage 4. The multidisciplinary conference recommended neoadjuvant chemotherapy followed by a radical surgical resection.Neoadjuvant chemotherapy was performed according to the Cooperative Soft Tissue Sarcoma Study Group  for 13 weeks, with four cycles of CEVAIE. Primary tumor and pulmonary metastasis showed a good response and shrinking in restaging. The patient had normal liver laboratory values and a preoperative LiMAx of 479 μg/kg/h. A hemihepatectomy (right lobe + segment 4b) with a sFLR of 28,4 % of segments I-IVa was planned A. IntraoNine days after step I, re-volumetry based on a CT scan revealed a sFLR of 57,3 % B. The foThe patient developed bile leakage, which was treated with interventional drainage and ERC. Otherwise, the postoperative course was uneventful and the patient was discharged in good conditions on POD 14. The pathological work up demonstrates tumor free margin. Extensive sampling of the tumor bed shows broad areas of fibrosis and necrosis with haemorrhage and resorptive changes with infiltrates of foamy histiocytes. Only a few small spots of residual vital sarcoma could be detected , Fig. 6.3Embryonal sarcoma is an exceptionally rare diagnosis with almost no improvement in 5-year-survival-rate over the past 40 years . The higDeveloped in the last decade, ALPPS offers a surgical option for patients with extensive liver lesions, bilobular liver tumors (metastasis invading or tumor masses expanding over both lobes) or a low FLR. After transection of the liver and ligation of the portal vein of the tumor-bearing liver lobe, a rapid hypertrophy of the FLR is induced . TherefoIn this case report, we present the first case of UESL treated with ALPPS. The rarity of this highly malignant disease is also based on its rapid tumor growth and early metastasis. Definitive and early diagnosis is impeded by its nonspecific symptoms, radiographic similarity to hepatic cysts and alveolar echinococcosis linked with the only available validation being histological investigation. Neoadjuvant chemotherapy to ensure possibility for oncological operation will therefore prevail, as margin-free resection is the only known real cure. With a tumor invading the right liver lobe and leaving a sFLR of 28,4 %, the spectrum of possibilities is limited. With the intraoperatively found invasion of the right and middle hepatic vein and because of a firm liver and intraoperative histological evaluation of the parenchyma we did not perform the initially planned one-staged right hemihepatectomy and changed the strategy. To reduce the risk of posthepatectomy liver failure, a complete transection of the liver and ligation of the right portal vein was performed. The aim was a rapid hypertrophy and timely completion of the resection. The risk for complications or mortality in this patient was suspected as very low. For our center it was also the first patient treated with ALPPS at this young age. A FLR growth of approximately 100 % in 9 days strengthened our concept during the course. Other options, such as portal vein embolization or ligation could have failed because of slower hypertrophy and the risk of tumor progression before liver resection. Besides, ALPPS shows a faster hypertrophy compared to standard one-staged hemihepatectomy with decreased or similar proliferation, apoptosis or angiogenesis (at least for CRLM). All under the premises having sufficient technical possibilities and surgeons experience.In conclusion, with this case we have shown the feasibility of the ALPPS procedure in a newly faced tumor-entity. Furthermore, the importance of an intraoperative histological evaluation of the parenchyma to estimate the individual risk for liver failure can be shown in this case. Also, deciding for ALPPS intraoperatively shows to be feasible.Nothing to declare.No ethical approval for Case Report nessecary. Written informed consent from the patient was obtained.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Dmitri Schepelew - writing the paper.Tim Reese - writing the paper.Katja Horling - writing the paper, pathological work-up.Christian Frenzel – patient work up, final approval of manuscript.York von Rittberg – critical review of the manuscript, final approval of manuscript.Georgios Makridis – critical review of the manuscript, final approval of manuscript.Mohammad-H. Fard-Aghaie – critical review of the manuscript, final approval of manuscript.Kim Caroline Wagner – critical review of the manuscript, final approval of manuscript.Alexandros Kantas – critical review of the manuscript, final approval of manuscript.Karl J. Oldhafer – writing the paper, critical review of the manuscript, final approval of manuscript.N/A.Tim Reese and Karl J. Oldhafer.Not commissioned, externally peer-reviewed.Nothing to declare.


Giant mature retroperitoneal teratoma of the adrenal region is quite rare in adults. In most cases, open adrenalectomy is required to ensure complete resection. We describe a case of bilateral giant primary mature cystic teratoma in the region of both adrenal glands in a 22-year-old female patient.A 22-year-old female patient was admitted to our hospital with no complain after detecting to have 2 giant well circumscribed masses in a routine investigation.She was diagnosed with bilateral giant primary mature retroperitoneal teratoma of the adrenal region.The patient underwent en bloc excision of the mass through laparoscopic simultaneous resection.We carefully separated and retained most of the adrenal tissue on both sides during surgery. Pathology reported mature teratomas. Eleven days after operation, the patient made uneventful recovery and left the hospital without any complication.Preoperative imaging and histologic analysis confirmed mature retroperitoneal teratomas. It is feasible to treat such giant benign tumors by laparoscopic simultaneous resection. Until now, only a very few case reports have been documented in literature. In most cases, patients are asymptomatic, have no specific complaints or are identified on routine physical examination incidentally. Open or laparoscopic resection of the lesions is the mainstay of treatment, and can give us an opportunity to determine the definitive diagnosis by histopathological examination. In terms of prognosis, patients with complete lesion resection often have an excellent prognosis with an overall 5-year survival rate of nearly 100%. Bilateral giant mature teratoma of the adrenal region and the treatment strategy has not been documented. Here we report a case of bilateral giant primary mature cystic teratoma in the region of both adrenal glands in a young adult female patient treated by laparoscopic simultaneous resection for the first time.Teratomas are neoplasms of the embryonic tissues that typically arise in the gonads (testes and ovaries) and sacrococcygeal regions of adults and children. Mature teratomas are made up of well-differentiated parenchymal tissues composed of somatic cell types which are derived from 2 or more germlayers .2A 22-year-old previously healthy female patient was found to have 2 giant well-circumscribed masses with rim shadowing in the region of both adrenal regions by abdominal ultrasound in a routine investigation. She was asymptomatic and then referred to our hospital for further examination and treatment. At physical examination, her arterial blood pressure was 128/77 mm Hg. Abdominal examination disclosed 2 global distention with dullness on the both sides of her upper abdomen. No other physical findings were observed. The baseline workups for this patient, including complete blood count, renal, and liver function tests, were within normal limits. Functional evaluations for adrenal hormones, including 24-hour urine cortisol, fractionated metanephrines, and plasma aldosterone to renin ratio, were also within normal limits.During the course of investigations, an abdominal and pelvic computed tomography (CT) scan showed 10.4 × 10.1 × 13.2 cm and 12.3 × 10.5 × 12.5 cm solid and cystic masses in the left and right adrenal region containing bone and multiple soft tissue densities, respectively , most of teratomas arise from the ovary, of which the incidence is 1.2 to 14.2 cases per 100,000 people per year. Some patients can present with complications such as infections (abscess formation), traumatic rupture leading to acute peritonitis, or malignant transformations rarely. Midline teratoma masses with restricted mobility can be easily detected on physical examination. Besides, however, very a few patients are asymptomatic with no complain. In this study, our patient was asymptomatic, and was found to have masses by abdominal ultrasound in a routine investigation. Her teratomas was located in both adrenal regions and shares very similar radiological and histopathological features with the cases reported in other previous literatures.,9,14,15The clinical presentations of teratomas are variable. The most common clinical presentations include nonspecific pain in the abdomen or back, obstructive gastrointestinal, and genitourinary symptoms, as well as lower limb/genital swelling resulting from lymphatic obstruction. Compared with other adrenal tumors , the most characteristic imaging feature of mature teratomas is a more heterogeneous mass containing fluid, fat, and/or sebum in the form of a fat–fluid level and calcification, just as seen in our patient.,17 Specifically, X-ray (plain radiographs) can identify calcified elements in about 62% of cases–20 whereas ultrasound (US) can greatly distinguish between cystic and solid elements. The most valuable examinations might be CT and MRI. On CT or MRI, teratomas are frequently cystic and contain calcifications, including teeth. CT scans can easily differentiate between adipose tissue and bone  masses. By comparison, MRI scans show better resolution of soft tissues (adipose tissue), feasible identification of benign and malignant neoplastic features, and most importantly superior tumor staging assessment. However, it still remains challenging to distinguish teratoma from the more common fatty myelolipoma. Another aspect is the rarity of this disease. In this case, CT and MRI examinations of our patient both recommended diagnosing the masses as retroperitoneal teratomas rather than myelolipomas. In addition to their diagnostic role, such imaging studies provide better preoperative planning and increased likelihood of complete removal of the tumour with less iatrogenic damage. They can display the precise location, morphology, and adjacent structures of the teratoma, which is of utmost importance. Histologic analysis confirmed the diagnosis of teratomas, which showed the possibility that bilateral giant teratoma, other than myelolipoma, arises in adrenal regions in adults. This can help other similar cases in preoperative diagnosis, better preoperative planning, and patient counseling in the future.The preoperative diagnosis of a teratoma of adrenal region is difficult, because of the varied imaging characteristics based on the different composition of the mass. Radiographic investigations, including US, CT, and MRI, play valuable roles in diagnosing teratomas. One defect of laparotomy is the inability to find and preserve the adrenal tissue, which is always displaced and is densely adhered to the giant teratoma. This is especially vital in young adult patients with bilateral lesions. The lack of adrenal corticosteroid is fatal, and lifelong adrenal corticosteroid replacement therapy impairs patients’ life quality seriously. For benign tumors, laparoscopic resection can help surgeons overcome the problems above by offering a better operational view for careful dissection. As long as the tumor has no local invasion on preoperative imaging or is found intraoperative, laparoscopic resection can be performed safely and can replace open surgery in most cases. In addition, the advantages of laparoscopic resection include beautiful incision, small surgical damage and shorten recovery time after surgery. In this case, considering that the masses are more likely to be benign tumors, to retain normal adrenal tissue as much as possible to maintain the function, we performed laparoscopic simultaneous resection and provided a reference for the feasibility of this approach. Careful preoperative evaluation is the key step to help us determine the first operation side. In our opinion, starting from the relatively simple side can help preserve the adrenal tissue. After this, surgeons can handle the difficult side completely regardless of the possibility of adrenalectomy in this step. Of course, preoperative evaluation is the most important point for choosing surgical approach. Laparoscopic surgery is only suitable for benign tumors, which highlights the important role of imaging assessment and surgeon experience.As to the treatment, due to their large size, an open approach is often the chief approach to resect these giant masses when the teratomas are larger than 6 cm. However, regardless of the benign histological nature of mature teratomas and no findings elsewhere, close follow-up is still recommended because the incidence of malignant transformation is about 3% to 6%. In the present case, the patient recovered with no sign of recurrence after 10 months of follow-up.In terms of prognosis, mature teratomas are likely to be benign and carry an excellent prognosis with overall 5-year survival nearly 100% after treatment.From this case, we showed the feasibility of laparoscopic resection to deal with such bilateral giant mature retroperitoneal teratomas, which retains adrenal tissue more effectively. Starting from the relatively simple side is recommended. The limitations of this study include the low incidence and relatively lower accuracy in laparoscopic resection. Maybe more cases in future will help surgeons to deal with such giant teratomas more appropriately.In conclusion, we report a bilateral giant primary mature retroperitoneal teratoma of the adrenal region for the first time. Preoperative imaging and histologic analysis confirmed mature retroperitoneal teratomas. We performed laparoscopic simultaneous resection to carry out en bloc excision and retained most of the adrenal tissue successfully, which suggests the feasibility of this approach. Further characterization of the cytogenetic changes underpinning tumorigenesis in such teratoma may provide additional insights into its prognosis.Conceptualization: Caibin Fan.Investigation: Jianqing Wang, Caibin Fan.Methodology: Jianqing Wang, Jibing Zhang, Chuan Xiao, Caibin Fan.Supervision: Caibin Fan.Writing – original draft: Jianqing Wang.Writing – review & editing: Caibin Fan.Jianqing Wang orcid: 0000-0002-0709-9694.


Mediastinal lipoma/liposarcoma is a rare tumor of the mediastinum.This article reported one case of giant anterior superior mediastinum well-differentiated liposarcoma involving the left thoracic cavity with symptom of dysphagia. The mediastinum liposarcoma was completely resected through a left thoracotomy. Histologic examination and molecular pathological test clarified the diagnosis as well-differentiated mediastinal liposarcoma. There has been no evidence of recurrence during the 8 months follow-up.Molecular pathological examination of the MDM2, CDK4 and p16 gene in tumors provides the diagnostic gold standard in distinguishing well-differentiated liposarcoma from lipoma. Complete surgical resection is the first-line treatment choice for mediastinal lipoma/ liposarcoma. Lipoma and well-differentiated liposarcoma are well-circumscribed mesenchymal tumor which originate from adipose tissue . MediastA 30-year-old man presented with dysphagia over 3 months was admitted to our hospital. Chest CT scan revealed a huge mass with fat density in the anterior superior mediastinum and left lung field, the inferior lobe of left lung was compressed, trachea and heart shifted to the right side Fig. . EchocarMediastinal lipoma and well-differentiated liposarcoma usually grow slowly and remain asymptomatic. When these tumors reach a huge size, which compress the adjacent structures, they will cause symptoms such as superior vena cava syndrome, Horner’s syndrome, dysphagia, dyspnea, cough, spinal nerve paralysis, tachycardia, and heart failure –6.There are five histological types of liposarcomas: well-differentiated, mucus, dedifferentiated, pleomorphic, and round cell. Well-differentiated liposarcoma is the most common type of liposarcoma, which can be further classified into three types: lipoma-like, sclerosis, and inflammatory . SometimNeither lipoma nor liposarcoma is sensitive to chemotherapy or radiotherapy, and complete surgical resection is the first-line treatment choice . When thIn this case, the essential of the operation was to expose the tumor clearly and to resect it completely. In order to achieve those purposes, we chose extensive thoracotomy through the 3th intercostal instead of thoracoscopic approach to provide a better exposure and to ensure the safety of the surgery. The technical key point of excising such a huge mass is to find the pedicle of the tumor in the first place, and then isolate the tumor along the pedicle.Mediastinal lipoma and well-differentiated liposarcoma are well-circumscribed mesenchymal tumors of the mediastinum, which can cause various symptoms due to the tumor mass effect. Chest CT scan and MRI can help clarify the diagnosis, and molecular pathological examination of the MDM2, CDK4 and p16 gene in tumors provides the diagnostic gold standard in distinguishing well-differentiated liposarcoma from lipoma. Complete surgical resection is the first-line treatment choice for mediastinal lipoma/ liposarcoma.


We describe a case of a rare soft-palatal and parapharyngeal mass in an adult woman. A 71-year-old female presented with a huge mass protruding from the soft palate, complaining about difficulty in swallowing for the past 4 months. After inspection and ear nose and throat fibroscopy, in which the mass appeared regular-shaped and with a regular mucosa, the patient underwent a CT scan and MRI examination. The CT scan showed an oval, not-infiltrating, hypodense mass including a discrete irregular-shaped central calcification, while the MRI examination confirmed the non-infiltrating growth and showed that around the calcified core, the lesion was surrounded by fat. After surgical removal, the histopathologic diagnosis was that of an "ectomesenchymal chondromyxoid tumour". Ectomesenchymal chondromyxoid tumour is a rare benign neoplasm arising from the tongue. Although only 45 cases have been reported in the literature, there are several unique features that define this lesion. Ours is the first case with a complete CT scan and MRI with diffusion imaging description. This case has specificity for both imaging description and anatomical localization: the tonsils are an extremely rare site for an uncommon type of tumour; in fact, the majority of the 45 cases of ectomesenchymal chondromyxoid tumours (ECMTs) described in the literature were localizeda th lingual site.A 71-year-old female presented to an ear, nose and throat specialist in November 2014 complaining of obstructive dysphagia for 4 months, without pain. She did not report haemoptysis or other clinical signs or symptoms.During the clinical examination, the physician found a bulky mass localized to the left tonsillar bed, without the presence of mucosal ulceration. There was no clinical evidence of any pathological cervical lymph nodes. A maxillofacial CT scan and MRI were then scheduled.The patient underwent a CT scan with and without contrast media , and a concomitant MRI , which showed a huge oropharyngeal mass with a maximum axial diameter of 3.5 cm, in the left tonsillar bed and prestyloid parapharyngeal space, with heterogeneous tissue structure owing to the presence of fatty and calcified areas ; no areaThis lesion caused dislocation of the soft palate with associated subtle contralateral compression on the ipsilateral pterygoid muscle.T1 weighted images (b” coefficient (maximum b = 800 s mm–²) to assess if the tissue had pathologic restriction of molecular water movement; no pathologic restriction was found  and frequent positivity for S-100 and cytokeratins.Although the immunoprofile was negative for GFAP, S-100, cytokeratin AE1/AE3 and α-smooth muscle actin, most likely owing to the decalcification process, the final histological diagnosis was that of ECMT  and 4a,b3 They described 19 tumours, all localized to the anterior dorsal surface of the tongue. Despite the original description of ECMT, the clinical, histopathological and immunohistochemical features of this tumour have not been thoroughly defined as these are rare tumours, with only 48 ECMTs having been reported in the English literature. The histopathological similarity of several tumours such as myoepithelioma, oral focal mucinosis, soft tissue myxoma, ossifying fibromyxoid tumour, chondroid choristoma, nerve sheath myxoma, pleomorphic adenoma and mucocele results in only a small number of of ECMTs getting diagnosed.ECMTs are rare, benign mesenchymal soft tissue neoplasms usually located in the oral cavity, particularly the tongue. The entity was first described in 1995 by Smith et al.4Clinically, ECMT presents as a slow growing, asymptomatic swelling usually seen on the anterior dorsum of the tongue; however, two cases presenting on the posterior tongue have also been documented. In addition, a case of ECMT on the hard palate has been reported but owing to lack of appropriate supporting documentation, its diagnosis has been the subject of controversy. The size of the lesion usually varies from 0.3 to 2.0 cm and the age of patients ranges from 9 to 78 years, with a mean age of 39 years.3,5 The differential diagnosis of ECMT with other extraskeletal myxoid chondroma, oral focal mucinosis, pleomorphic adenoma, myoepithelioma, mucocele, glial/chondroid choristoma, and ossifying fibromyxoid tumour is not simple.6No sex predilection is reported. The diagnosis of ECMT is based on the clinical as well as histopathological and immunohistochemical confirmation.7Some features were reported in the literature, which allowsa differential diagnosis to be defined; for example, extraskeletal myxoid chondroma (ESMT) usually occurs in the hands or feet and is often seen related to tendon; myoepithelioma of minor salivary gland can show both chondroid and myxoid stroma; and ossifying fibromyxoid tumour shows the presence of bone in the periphery.3ECMT probably arises directly from the neural cells of the tongue or primitive mesenchymal cells that undergo neural differentiation during oncogenesis.8 The tumour is negative for epithelial markers such as keratins and carcinoembryonic antigen; however, other authors have reported variable positivity for cytokeratin.3 No malignant change is reported and rare recurrence has been observed in the literature after complete excision.2The expression of S-100 and GFAP supports a neurogenic origin.The treatment of choice is complete excision with tumour-free margins, and it is feasible because these lesions are small and relatively well-circumscribed.9The other few cases described in the literature did not include a complete integrated radiological work-upof the lesions reported. CT scan and MRI, by themselves, do not allow the diagnosis of ECMT to be hypothesized, although it may be suspected, but prove useful in diagnostic pathology, tissue characterization and therapeutic decision-making.gImaging helps mainly in defining the tumoral boundary, internal composition and determination of eventual infiltration into the surrounding structures.This case is of interest for several reasons: the rarity of the ECMT, the complete imaging work-up and the “tonsillar” site, with the majority of ECMT having been reported, until now, with "lingual" localization.Oropharyngeal benign masses with central calcification and fat are usually considered benign at first, they can be misinterpreted as a teratoma , and the evidence for this rare type of tumour (ECMT) should be considered, in other infrequent cases of found at the tongue site. While imaging helps in defining and confirming the benign nature of a mass, histopathological features confirm its diagnosis.The patient told us that she was very worried about cancer, but after CT scan and MRI, we could calm her because we were quite sure that the lesion was benign. The histopathological analysis allowed us to tell her that it was not malignant, nor would it recur.Written informed consent was obtained from the patient for publication of this case report, including accompanying images.


Mediastinal lymphangiomas are rare benign congenital malformations, but complications can occur, including infection, cystic hemorrhage, superior vena cava syndrome, airway compromise, and chylothorax. Radiologically, lymphangiomas are well-defined masses, with low attenuation ranging from simple to complex fluid and fat. They often encase adjacent mediastinal structures. We present a case of mediastinal lymphangioma in a young female, who presented with recurrent complex pleural effusions, initially thought to represent an empyema and/or necrotic mass. Despite surgical chest tube and interventional radiology drainage, fluid reaccumulated. Upon further review, the interventional and thoracic radiologist concurred that the complex collection was in fact predominantly extra pleural in location. The patient underwent partial resection after it was discovered intraoperatively that the extra pleural cystic mass was contiguous with and extended deeply into the mediastinum. Histopathology confirmed the diagnosis of lymphangioma. Mediastinal lymphangiomas are rare, benign congenital malformations that arise from focal proliferation of lymphatic vessels. They comprise up to 4.5% all mediastinal tumors . The mos9/L and platelet count of 634 x 109/L. A bedside chest tube insertion was undertaken, with subsequent CT thorax demonstrating no change in the size of the large left complex collection , lymphangiomas appear as cysts with a smooth contour and encase adjacent mediastinal structures. Lymphangiomas are homogenously low attenuating, with attenuation ranging from simple to complex fluid and fat , 6.On MR imaging, findings include signal isointensity to muscle on T1 weighted images and hyperintensity on T2 weighted images, with variability depending on the extent of cystic components and proteinaceous contents .Top differential considerations include necrotic neoplasm, thymic cyst, hematoma, seroma, or abscess, as well as mature teratoma. The diagnosis of lymphangioma in patients with typical findings of a uniform cystic mass at the anterior mediastinum is relatively evident. However, in atypical cases that exhibit no convincing evidence of cystic components or present in unusual locations, preoperative diagnosis may not be possible solely based on imaging characteristics.In our case, imaging features were nonspecific, and the clinical finding of recurrent thoracic fluid accumulation raised suspicion for an underlying abscess initially, which did not respond to antibiotic therapy and drainage. Given its failure to result with conservative management, thoracic surgery was performed. Given its intimate association with mediastinal structures, VATS resection may not be optimal, and the recommended treatment is open thoracotomy for surgical resection of cases that extend to encase mediastinal structures . ReporteWe highlight that, in cases of recurrent thoracic fluid accumulation, lymphangioma should remain an unusual consideration. Gross total resection is often curative, with very low recurrence rates.


Pott’s puffy tumor is a rare and severe complication of frontal sinusitis, characterized by the progressive swelling of the frontal soft tissues secondary to a subperiosteal abscess. Radiological imaging with ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) are important diagnostic tools in establishing diagnosis and treatment planning. Early surgery along with intravenous antibiotics are required in order to achieve a good recovery. The authors report a case of Pott’s puffy tumor in an obese 14-year-old male, with a previous history of asthma and a chronic use of steroids, treated with neurosurgical debridement followed by a combined course of intravenous (IV) and oral antibiotics, who had a favorable outcome upon long-term follow-up. In addition, a brief review of the current medical literature was performed for a discussion on the diagnostic and therapeutic features of this pathology. More than 250 years have passed since the British surgeon Sir Percival Pott initially described what is now known as the Pott’s puffy tumor, a subperiosteal abscess of the frontal bone presenting as a progressive forehead edema secondary to frontal osteomyelitis following frontal sinusitis -2. AlthoWe describe herein a case of a male adolescent diagnosed with a Pott’s puffy tumor with an epidural abscess in addition to the classical frontal subperiosteal abscess and discuss the literature regarding the diagnosis and treatment of this pathology.A 14-year-old adolescent was referred to the emergency department (ED) for a neurosurgical consultation with a chief complaint of progressive left forehead bulging in the last three weeks. His parents reported that the patient was seen by a general physician in another institution at the beginning of the symptoms and received a course of oral antibiotics (amoxicillin and clavulanic acid) for the past 21 days. Nevertheless, due to a worsening of the edema on his forehead, CT and ultrasound scans of the head were performed and the patient was referred for a neurosurgical assessment. The patient had a history of asthma, with the daily use of nasal formoterol and budesonide and the chronic use of oral prednisone monthly for up to two to three days. He had recently been referred for a pediatric endocrinological evaluation due to marked obesity (body mass index of 45) with Acanthosis nigricans. There were no reports of head trauma, previous surgery, or hospital admissions.Upon physical examination, marked obesity was noted with Acanthosis nigricans and a compressible soft-tissue swelling in the left forehead Figure , with anRadiological imaging with contrast-enhanced CT and gadolinium-enhanced magnetic resonance scans of the head were performed, showing extensive frontal-ethmoidal sinusitis with bone erosion of the frontal sinus internal and external walls and an epidural and periosteal left-frontal mass with capsular contrast-enhancement suggestive of an abscess Figures -4.After parental consent, the patient was submitted to surgery with a bicoronal skin incision, exposing the infiltrated subcutaneous tissue. After a left frontal craniotomy, an epidural mass compatible with an abscess was found Figure .The left frontal sinus was cranialized with an exenteration of the mucosa in order to avoid late complications, such as mucocele formation, and after an extensive removal of debris and specimen sampling, a pedicled pericranium flap was inserted in the frontal sinus drainage pathway and the bone flap was fixed Figure .The postoperative period was uneventful, and the patient was submitted to a four-week course of intravenous ceftriaxone/ oxacillin/metronidazole followed by four weeks of oral amoxicillin with clavulanic acid after discharge, as discussed with the Institutional Commission for Control of Infectious Diseases, based on the favorable clinical response of the patient. A post-operative contrast-enhanced CT scan showed no signs of complications or residual lesions Figure .The culture of the intraoperative lesion was positive for Peptostreptococcus species. The patient was followed in the pediatric neurosurgical outpatient clinic, with no signs of recurrence. However, 18 months after surgery, the patient was referred for an otorhinolaryngologist consultation following chronic sinusitis.Pott’s puffy tumor is a rare entity; however, there is an increasing number of reports in the medical literature in recent years, particularly in the pediatric population, which may be attributed to delayed or inadequate treatment of frontal sinusitis . ClinicaDifferential diagnosis for any swelling in the forehead includes, but is not limited to, soft-tissue infection, such as carbuncle and infected sebaceous cyst; benign tumors, such as dermoid cysts, lipomas, intraosseous lipomas, lipoblastomas, frontal sinus mucoceles, and superficial temporal artery pseudoaneurysms, and, finally, malignant tumors, such as metastases or aggressive frontal meningiomas -9.The diagnostic workup should include contrast-enhanced CT scan with brain and bony sequences to investigate the presence of an epidural or subdural abscess and to estimate the extent of bone erosion. Also, gadolinium-enhanced MRI may help define the involvement of brain parenchyma or dura mater . Pneumoc, anaerobic bacteria, such as Fusobacterium species and Bacteroides species, and, less commonly, Staphylococcus aureus and Enterococci species [Common causative organisms are Streptococcus species , Hemophilus influenza species .In our case, the patient was referred after an initial clinical suspicion of soft-tissue frontal infection and was shown by the CT and MRI scans to present an epidural abscess associated with frontal osteomyelitis with the erosion of the inner table of the left frontal sinus, although no neurological symptoms were present upon admission. Also, in our case, we wondered if the chronic use of corticoids due to asthma and the association of obesity had any relation with the occurrence of this rare complication; however, we couldn’t find any previous reports of similar clinical scenarios.After diagnosis, the management should include broad-spectrum antibiotics and the surgical evacuation of the abscess, which may be performed via open surgery -2,7 or, Prognosis, in general, is favorable, even if neurological symptoms are present upon admission; obviously, prompt recognition and treatment are mandatory for successful outcomes.Although sometimes depicted as a rare occurrence in the age of broad-spectrum antibiotics, Pott’s puffy tumor must be suspected as a serious complication of frontal sinusitis in both pediatric and adult patients. Early clinical suspicion, diagnosis, and the treatment of the frontal sinusitis and its complications with surgery and broad-spectrum intravenous antibiotics bring up the possibility of good recovery.


Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a malignant tumor of the upper nasal cavity. This report illustrates the case of a 63-year-old woman who presented with intractable headaches. Subsequent radiologic evaluation and correlation with histopathologic analysis confirmed esthesioneuroblastoma. We review herein the typical computed tomographic (CT) and magnetic resonance (MR) imaging findings related to this locally destructive tumor, the prompt diagnosis of which may help prevent long-term morbidity and potentially, mortality. Up-to-date diagnostic criteria, staging, and management considerations are also outlined. Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare and malignant tumor of the upper nasal cavity. The incidence follows a bimodal distribution with peaks at the second and sixth decades of life. Common presenting symptoms include epistaxis and unilateral nasal obstruction; however, the case discussed herein is an unusual presentation involving intractable headaches due to an intracranial extension of the tumor -2. The lA 63-year-old woman with a past medical history of hyperlipidemia, diabetes mellitus, and a remote ischemic stroke presented with intractable headaches of five-day duration. Headaches were described as constant, unrelenting, and throbbing in nature. The pain was described as predominantly occipital, which radiated throughout the rest of her head. The month prior to presentation, the patient underwent a rhinoscopy with nasal polypectomy at an outside facility due to a six-month history of progressively worsening unilateral left nasal passage obstruction. The patient was unaware if histopathologic analysis was performed on the removed specimen.Upon presentation to the emergency department, the patient underwent a CT evaluation of the head without contrast, which demonstrated a midline aggressive-appearing tumor versus infectious process centered in the bilateral nasal cavities, paranasal sinuses, and right orbit with an intracranial extension to the bilateral frontal lobes. Edema was noted with a mass effect and a midline shift of 4 mm to the left Figure .Subsequent evaluation with MRI of the head demonstrated a large, hypercellular mass involving the anterior aspect of the right frontal lobe with erosion through the cribriform plate and lamina papyracea, with additional extension into the superior nasal cavity and superomedial right orbit. An extensive vasogenic edema within the right frontal lobe was also visualized, along with a significant mass effect with a 1.1 cm of right-to-left midline shift Figures -4. PathoEsthesioneuroblastoma, also known as olfactory neuroblastoma, is a malignant tumor of the upper nasal cavity. It is thought to arise from cells in the olfactory epithelium of neural crest cell origin. This cancer exhibits a bimodal age distribution with peaks at the second and sixth decades of life. It accounts for three percent of all intranasal tumors . Over 12Unfortunately, there are several other aggressive processes that can occur in the upper nasal tract and imaging alone is often unable to definitively distinguish esthesioneuroblastoma from these other processes. However, suggestive findings include unilateral origination of the mass from the superior olfactory recess medial to the middle turbinate, which is often completely opacified, and involvement of the anterior and middle ethmoid air cells. Most commonly, the mass will also be seen invading superiorly through the cribriform plate and into the anterior cranial fossa. This aggressive malignancy usually may cause destructive changes of the surrounding bone depending on the extent of disease . Due to Two main classification systems have been employed for the evaluation of esthesioneuroblastoma. The most commonly used system is the modified Kadish classification, which utilizes an A-D scale Table  5]. The. The5]. For classification purposes, it is important to identify the involvement of the cervical and retropharyngeal nodes. Definitive diagnosis is by histopathological evaluation and involves immunohistochemistry. Prognostic factors and diagnosis depend on Hyam’s I-IV grading system that accounts for various pathological findings . ManagemThe prompt identification and treatment of esthesioneuroblastoma is required for a favorable outcome and depends upon the close collaboration of radiology, pathology, and surgery. Staging, management, and prognosis are in large part determined by regional involvement in non-metastatic cases due to the destructive nature and proximity to neurological structures.


Dermatology, due to the peculiar characteristic of visual diagnosis, is suitable for the application of modern telemedicine techniques, such as mobile teledermoscopy. To evaluate the feasibility and reliability of the technique for the diagnosis of pigmented lesions. Through the storage and routing method, 41 pigmented lesions were analyzed. After the selection of the lesions during the outpatient visit, the clinical and dermatoscopic images were obtained by the resident physician through the cellphone camera and sent to the assistant dermatologist by means of an application for exchange of messages between mobile platforms. Firstly, the assistant dermatologist described the visualized dermatoscopic structures and defined its diagnosis and conduct, based solely on the evaluation of the clinical and dermatoscopic images, without having the knowledge of the anamnesis data. Afterwards, the same assistant dermatologist evaluated the patient face to face, defining the dermatoscopic structures, diagnosis, and conduct. The data obtained through teledermoscopy and face-to-face assessments were compared and accuracy was defined as the concordance between the diagnoses. A match rate of 90% between teledermoscopic and face-to-face diagnosis was demonstrated . The reduction of the morbidity and mortality of nonmelanoma skin cancer and melanoma is the greatest current challenge for dermatology and, within this context, this includes the early diagnosis of melanoma, dermatoscopy, teledermatology, and teledermoscopy.Early diagnosis results in a better prognosis for the patient , and dermoscopy is essential, since it consists of a more accurate method for the diagnosis of melanoma than the naked eye one, increasing the detection of early-stage melanoma by up to 49% [Dermatology, due to the peculiar characteristic of visual diagnosis, is ideal for the application of modern telemedicine techniques, with several recent studies proving the viability and reliability of teledermatology and, in particular, teledermoscopy, with high levels of concordance in diagnosis and management plan in relation to face-to-face consultation .The World Health Organization defines telemedicine as the use of health communication technologies for the exchange of medical information for diagnosis, treatment, prevention, research, evaluation, and education. One of the existent ways of telemedicine is teledermatology, which is already well established, whose publications began in 1995 and these ones have been growing exponentially. Within teledermatology, teledermoscopy appears as a promising area for the diagnosis and management of pigmented skin lesions, early detection of skin cancer, and screening .Teledermatology has two distinct operation models, the synchronous, through videoconference and satellite communication, which occurs in real time and the asynchronous, through a storage and routing system, including the use of e-mail, web, and mobile teledermatology, and which provides high levels of diagnostic accuracy, with lower cost, greater convenience, and practicality .Storage and routing teledermatology are constantly growing around the world with improvements in communication and imaging technologies, allowing expert judgment in situations in which access to a dermatologist might be difficult due to geographic distance or excessive demand.Mobile teledermoscopy consists of a new application of teledermatology, in which clinical and dermatoscopic images are captured and transmitted by mobile devices  . The imaIn this mobile teledermoscopy study, the first one developed in Brazil, we aimed to study the feasibility and reliability of the technique for the dermatological diagnosis of pigmented lesions.Patients were prospectively selected from the outpatient clinic of the Department of Dermatology from April to June 2017. The inclusion criteria consisted of men or women, of any age, with pigmented lesions, whether melanocytic or not. After the selection of the lesions during the outpatient visit, the clinical and dermatoscopic images were obtained by the resident physician and sent to the assistant dermatologist before face-to-face assessment.The clinical images were obtained using the cell phone camera  in two panoramic and macromodes (at an established distance of 20 cm from the lesion to be further studied). The dermatoscope which has been used was DermLite DL4 from 3Gen®, San Juan Capistrano, CA 92675, USA; and, for the acquisition of the dermatoscopic images, the camera lens was applied to the DermoLite® MagnetiConnect TM device of the 3Gen® Connection Kit for iPhone6 P / N: DLCKi6-MC, San Juan Capistrano, CA 92675, USA, with the dermatoscope at position 0, in polarized mode, without making use of flash or zoom camera assets. To the large lesions, we performed more than one dermatoscopic image.At first, the images were sent via WhatsApp Messenger, a free messaging application available for the iPhone and other platforms, to the assistant dermatologist with extensive experience in dermatoscopy, which described the dermatoscopic structures visualized, as well as the analysis of patterns, when these ones were applicable; and the diagnosis and procedures were defined, based only on the evaluation of the clinical and dermatoscopic images, without the knowledge of the anamnesis data.At the second moment, the same assistant dermatologist evaluated the patient face-to-face, using the same dermatoscope and having access to anamnesis data , defining the dermatoscopic structures and / or pattern analysis, diagnosis, and procedures.The data obtained through teledermoscopy and face-to-face examinations were compared, the differences between them being analyzed and what was essential being defined, in face-to-face examination, for decision-making. The face-to-face examination was defined as the gold standard for the final procedure. The diagnostic accuracy was defined as the agreement between the teledermoscopic diagnosis and the gold standard. Clinical and dermatoscopic face-to-face diagnoses were considered suitable in patients with clinically and teledermoscopically benign and nonsuspected lesions and no biopsy procedure was performed. The lesions considered malignant or suspicious were studied histopathologically and were correlated with clinical-dermatoscopic diagnosis.The diagnostic agreement between teledermatology/teledermoscopy and face-to-face assessment was calculated using the McNemar test, hypothesis test for paired data. This test works with two hypotheses: the null hypothesis, in which there is no difference between the representativeness between the diagnoses by the two methods and the alternative hypothesis, in which there is statistical difference. At the significance level of 5% (0.05), the null hypothesis is accepted when the p value is greater than 0.05.Forty-one lesions were studied in 31 patients, 22 females, and 9 males. The mean age was 56.5 years (11 to 78 years). The lesions were present for an average duration of 80.4 months (from 1 day to 40 years), excluding those not specifically determined, only reported as present ones since childhood or as time of unknown onset. The specific locations included back (11 lesions), hand , malar (4), upper eyelid (2), cervical (2), thorax (2), breast (2), thigh, plantar (2), infraorbital (1), temporal (1), nose (1), retroauricular (1), armpit (1), abdomen (1), and leg (1 lesion). Eight patients reported local symptoms in the lesions, which consisted of growth (4), bleeding (1), color change (2), and pruritus (1). No patient had previous history of melanoma, one of whom had a personal history of basal cell carcinoma and one that had a family history of melanoma (first degree relative). Nineteen patients reported comorbidities: psoriasis (3), systemic arterial hypertension (8), diabetes (3), bullous lupus (1), cystic fibrosis (1), renal transplantation (1), and Sjogren's syndrome (1). We highlight other relevant aspects in the anamnesis in 10 cases, which consisted of the use of immunosuppressants (2), immunobiological (1), smoking (1), outdoor work profession (1), contact with exogenous pigment (1), inflammatory pigmentation of the underlying disease (1), application of acid to the lesion (1), and nonapplication of acid to the lesion (1).From the clinical images obtained, two were reported by the teledermatologist as not perfectly made ones in focus and in one of the other cases there was an equivocal perception of the lesion size. All the dermatoscopic images were considered excellent.There was no injury to the teleanalysis of the lesions in any of the cases. Teledermoscopic diagnoses and those established after face-to-face analysis are shown in The teledermoscopic diagnoses were pigmented basal cell carcinoma (3), intradermal nevus (6), seborrheic keratosis (4), benign melanocytic lesion (12), blue nevus (3), intracorneal hematoma (1), atypical nevus melanocytic suspicion (2), dermatofibroma (1), congenital nevus (1), solar lentigo (2), and melanoma (4).On the other hand, the face-to-face diagnoses consisted of pigmented basal cell carcinoma (4), intradermal nevus (6), seborrheic keratosis (4), benign melanocytic lesion (14), blue nevus (3), exogenous pigmentation atypical nevus (2), dermatofibroma (1), congenital nevus (1), solar lentigo (2), and melanoma (3).The anatomopathological study was performed on 8 lesions and 4 pigmented basal cell carcinomas , 1 junctional melanocytic nevus (lesion 21), and 3 melanomas  were found.The agreement between the evaluations was 90%, and this reduction of 10 percentage points was not considered statistically significant, once there was no difference between the representativeness of the cases, since the p value was 0.1366. In other words, even though there is a reduction in concordance for teledermoscopy, there is insufficient statistical evidence to prove that this method is inferior, which can be considered equivalent to the traditional method (face-to-face assessment).Discordant results included exogenous pigmentation, a pigmented basal cell carcinoma, and two benign melanocytic nevi.The exogenous pigment lesion (lesion 9) was present 1 day ago in the right second finger of a 57-year-old female patient with a family history of melanoma and consisted of a discontinuous brownish macula, with poorly defined limits and linear medial aspect of the proximal interphalangeal joint to the dorsal distal interphalangeal joint. Dermoscopy revealed spots and globules, some brownish and other blackish ones, in the center and periphery, irregularly distributed along the lesion, with areas without dermatoscopic structures. The teledermoscopic diagnosis was of intracorneal hematoma. In the anamnesis, the contact with synthetic paint was revealed and the diagnosis was of exogenous pigmentation. In this case, the determining factor for the decision making was the data obtained during the anamnesis, from contact with the exogenous pigment. The conduct was expectant.One case of benign melanocytic nevus was a 66-year-old man with a diagnosis of bullous lupus, with a dark brown macula on the back, for an unknown time, with no local symptoms, and with no melanoma history (lesion 16). In the dermoscopy analysis, reticulum-globular pattern, irregular net, and globules were identified with the colors light brown, dark brown, and gray. The diagnosis was of suspected malignant melanocytic lesion and the decision-making to be taken would be the excisional biopsy. In face-to-face assessment, the knowledge of the underlying disease and its pathophysiology , as well as the presence of suggestive lesions of disease activity, has modified diagnosis and decision-making for benign melanocytic lesion with inflammatory pigmentation of the disease and clinical follow-up.The third discordant case (lesion 25) was referred to the male patient, 62 years old, renal transplant, using immunosuppressants, with lumbar lesion for unknown time, whose telediagnostic conclusion was melanoma or recurrent nevi . The face-to-face assessment concluded that this one was pigmented basal cell carcinoma, where arboriform telangiectasia at the periphery of the lesion and leaf structures as well are identified. The anatomopathological study confirmed the diagnosis established in the face-to-face assessment.Finally, the other case of benign melanocytic nevus was a 61-year-old female patient, who had a pigmented lesion in the dorsal median line, for unknown time, with atypical pigmentary network teledermoscopy and striae (lesion 39). In the face-to-face assessment, the dermoscopy was reassuring, in which a homogeneous pigment network was visualized. In this case, it was decided to perform the digital dermatoscopy, which showed a similar image to that of the teledermoscopy one. We suppose that the location of the lesion was the difficult point in the teledermoscopic and digital analysis, due to the difficulty in coupling the dermatoscope in maintaining the focus on the images capture.There was one case where the palpation of the lesion was essential for the diagnostic conclusion, referring to the lesion 23, in which the telediagnostic hypotheses were of solar lentigo associated with blue nevus or melanocytic lesion with eccentric homogeneous pigmentation.We also emphasize the case of lesion 21, which concerned a 22-year-old female patient, with a cystic fibrosis diagnosis, presenting a hyperpigmented macule, blackened in the center, and dark brown in the edges, present for 2 years, which evolved with the darkening of the lesion. The teledermoscopy identified an irregular network, striae, and gray-blue areas. The diagnostic hypotheses were atypical melanoma or melanocytic nevus and excisional biopsy as the conduct to be performed. In the face-to-face assessment, the same dermatoscopic structures were identified, maintaining the same dermatoscopic description, the same hypotheses, and the same conduct; however, the assistant dermatologist reported a more reassuring aspect after a global analysis of the case. The anatomopathological study revealed junctional melanocytic nevus associated with marked degree of melanin pigment incontinenceThe concern with confidence in the diagnosis of skin malignancy with teledermatology is reflected in the current orientation that all suspicion of malignancy of the skin should be seen face to face . HoweverIn 1999, the teledermoscopic study carried out by Piccolo et al.  analyzed pigmented lesions of 66 patients in two groups (face-to-face and asynchronous teledermoscopy) and found agreement in 60 cases (91%) . All lesMassone et al., in Austria in 2008, carried out the first teledermoscopy study using cellular phones for image capture and the storage and referral system for two teledermoscopists, in which diagnostic correspondence was found between the face-to-face examination of pigmented lesions in 89% and 94 % [In 2016, Arzberger et al. showed excellent agreement on recommendations  between dermatologists (face-to-face and teledermatology) in a cohort of 70 patients with moderate to high risk [Another teledermoscopic challenge is the diagnosis of hypo- or nonpigmented lesions, as demonstrated by Fabbrocini in the study of pink-lesions , in which the clinical and dermatoscopic telediagnosis showed less diagnostic accuracy than the face-to-face one , 2. In tAn important factor that should be considered is the standardization of image and service equipment. There are still no universal imaging standards developed and implemented in teledermatology; however, there are systematic reviews that summarize the technology standards and image technique for acquiring digital dermatological images . In 1997The study of basic notions of photography is very important to the training of dermatologists. In Brazil, there are few training centers that offer it in their programs; however, this is already a subject much addressed in the national congresses, where minicourses take place for the dermatologists.In the present study, sending the images by the application generated a resolution reduction of 3264x2448 to 1280x960 pixels, which is still within the established technology standards for the purposes of teledermatology, allowing, along with the standards of the technique, the analysis of an image quality.We selected the WhatsApp to perform the mobile teledermoscopy after the verification that, with the sending of the image, the reduction of the resolution would not compromise the image analysis, since it still remained within the established technology standards for the purposes of teledermatology. The other reasons for choosing were the practicality offered by the application and the greater ease of replication.What the studies clearly demonstrate is that even though the diagnostic accuracy of the lesions may be slightly lower for teledermatology, it has the ability to screen clearly benign lesions, allowing obvious malignancy and suspicious lesions to be properly managed in secondary care facilities . We boldOur study demonstrated a high concordance rate between the teledermoscopic and face-to-face diagnoses, comparable to those described in the medical literature, and it allows us to conclude that teledermoscopy, and in particular mobile teledermoscopy, is a promising method for the analysis of pigmented lesions and we firmly believe that it deserves attention for future applications, especially in our country, where the distribution of dermatologists is irregular and scarce in some regions, besides the nondominance of the dermatoscopic technique by a considerable portion of these ones. The latest medical census, for example, showed that the Southeast region concentrates 58.9% of the specialists, 15.8% throughout the South, 13.8% in the Northeast, 8% along the Midwest, and 3.5% throughout the North region. In absolute numbers, the variation ranges from 5 to 2183 dermatologists per state  .We emphasize that the diagnosis did not influence the final decision making, which was based exclusively on face-to-face assessment, either in the diagnostic definition or in the established behavior; we also declare that there was no identification of the patients through the sent images, which contained only a numerical tag; we also affirm that the sharing of these ones was restricted to the two participating physicians; moreover, we emphasize that the data from anamnesis and physical examination are sometimes essential for this decision-making, especially when faced with doubtful lesions; we declare that the use of the dermoscopic adapter for image capture may have contributed positively to the quality of the images; and, lastly we affirm that the necessary infrastructure for the progress of this study was of minimal cost, with high practicality and functionality.We conclude, hence, that prospective and randomized clinical studies, legal aspects, and systematized protocols are absolutely necessary for the advancement of mobile teledermoscopy in Brazil, once it stands out as a good diagnostic accuracy, practical, and low cost method.


Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is an unusual and special type of Müllerian duct anomaly. Patients usually present in adolescence, soon after menarche, with pain and pelvic mass and rarely with infertility in adulthood. Majority of the cases of OHVIRA have been reported in association with uterus didelphys and the presentation of uterus didelphys with OHVIRA is known as Herlyn–Werner–Wunderlich syndrome. A complete septate uterus with OHVIRA is exceedingly unusual. Less than 30 cases of complete septate uterus with OHVIRA have been reported to date, to the best of our knowledge. We present a rare case of incidentally detected complete septate uterus with OHVIRA in a young woman who presented with acute pain in the right iliac fossa owing to acute appendicitis. Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is an unusual and special type of Müllerian duct anomaly (MDA). Patients usually present in adolescence, soon after menarche, with pain and pelvic mass and rarely with infertility in adulthood. Majority of the cases of OHVIRA have been reported in association with uterus didelphys and the presentation of uterus didelphys with OHVIRA is known as Herlyn–Werner–Wonderlich (HWW) syndrome. A complete septate uterus with OHVIRA is exceedingly unusual. Less than 30 cases of complete septate uterus with OHVIRA have been reported to date, to the best of our knowledge. We present a rare case of incidentally detected complete septate uterus with OHVIRA in a young woman who presented with acute pain in the right iliac fossa (RIF) owing to acute appendicitis.A 22-year-old female presented to the gynaecology department with acute onset of severe pain in the right lower abdomen. The pain was colicky in nature, with associated nausea and vomiting. She attained menarche at the age of 14 years and had a history of mild dysmenorrhoea. She had been married for the last 8 months and cohabited with her husband for the same duration. There was no previous history of an abortion. On physical examination, her pulse rate, blood pressure and respiratory rate were within normal limits. Tenderness was noted in the RIF on deep palpation. On per-vaginal examination, the uterus was anteverted and normal in size. A 6 × 6 cm mass was palpable in the right lower adenexa, which was non-tender and cystic in consistency. This mass was causing a smooth bulge in the right vaginal wall.Clinical differential diagnoses were acute appendicitis, twisted right ovarian cystic mass and ruptured tubal pregnancy.All the biochemical investigations were within normal limits. Urine pregnancy test was negative.She was referred to the radiology department for ultrasonography (USG). Both transabdominal and transvaginal sonography was performed. 3 MHz curved and 10 MHz linear probes were used for transabdominal sonography, whereas transvaginal sonography was performed with a 6 MHz endocavitory probe.On sonography, a tubular, non-compressible, blind-ending structure was seen in the RIF measuring 8 mm in diameter, consistent with appendicitis. A small amount of periappendiceal fluid was also noted. The uterus was normal in size, having two endometrial cavities extending up to the external os. There was evidence of a cystic mass with internal echoes measuring approximately 5 × 4 cm in size on the right side of the vagina and communicating with the right endometrial cavity. No fluid was seen in the endometrial cavity on either side. Both ovaries were normal in size and outline. The right kidney was not visualized in the right lumbar region or the pelvis. The left kidney was enlarged in size, measuring 13.4 × 5.6 cm in size.T2 weighted  sagittal; FS-FSE T2 weighted  sagittal; FSE T2 weighted FS  coronal; FSE T2 weighted  coronal, including abdomen for the kidneys; FSE T1 weighted  coronal; FSE T2 weighted  axial; short tau inversion-recovery  axial; FSE T1 weighted  axial; FSE T2 weighted  oblique coronal T2 parallel to uterus sequences. Intravenous contrast agent was not used as it was not expected to yield additional information. The MRI revealed two endometrial cavities extending up to the external os. The endometrial cavities were separated by a septum showing intermediate signal intensity, isointense to the myometrium . The following pulse sequences were employed: fast spin echo (FSE) ometrium . The uteometrium . The angic fluid  and 4a,bic fluid . The appic fluid .When enquired about the history of backache or neck pain, the patient complained of occasional neck stiffness. A lateral radiograph of the cervical spine was performed during her stay in the hospital, suspecting an associated vertebral anomaly, which revealed a block vertebra involving the C6–C7 vertebrae .Based on the clinical and radiological findings, a diagnosis of complete septate uterus and OHVIRA with acute appendicitis with block vertebra C6–C7 was made.1 proposed an MDA classification in 1979, which was subsequently modified by the American Society for Reproductive Medicine in 1988.2 Uterus didelphys constitutes a Type III anomaly, which is a common MDA. However, uterus didelphys with vaginal septum leading to obstruction of menstrual blood with ipsilateral renal agenesis is a special and unusual malformation known as HWW syndrome. A septate uterus has been classified as Type V MDA and is the most common form of MDA, accounting for 55% cases. The septum can be partial or complete, extending up to the external os. The septum may rarely extend into the vagina. In our case, the septum was extending into the vagina with obstructed right hemivagina. Although the majority of the patients with OHVIRA have uterus didelphys, obstructed hemivagina may also be seen with other MDAs such as a bicornuate and septate uterus. Very few cases of complete septate uterus with OHVIRA have been reported to date. A complete septate uterus with OHVIRA was seen in our case. We could trace fewer than 30 cases of complete septate uterus with OHVIRA in the English literature. Haddad et al3 in the year 1999 reported septate uterus with obstructed hemivagina in 9 out of 42 patients having blind hemivagina. Shavell et al4 in 2009 reported OHVIRA with complete septate uterus in a 19-year-old female. Fedele et al5 could find only 10 cases of septate uterus with OHVIRA in their retrospective study of 87 patients with obstructed hemivagina. Their retrospective study spanned over a period of 30 years, emphasizing the rarity of the disease. They studied 87 patients with double uterus with OHVIRA and found that classic HWW syndrome, that is, uterus didelphys with OHVIRA, was seen in 63 cases (72.4%), 10 out of 87 patients (11.5%) had a septate uterus whereas bicornuate uterus was seen in 9 patients (10.3%). Kim et al6 reported pregnancy in a septate uterus with OHVIRA in 2014.A failure of fusion of the Müllerian ducts or resorption of the uterovaginal septum between 6 and 12 weeks of intrauterine life leads to a complex spectrum of MDAs. The reported prevalence of MDAs is 1–5% in the general public and 13–25% in females with recurrent abortions. Buttram and Gibbonsvia a small communication between the two hemivaginas. Moshiri et al7 also reported a case of HWW syndrome with a partially obstructed hemivagina.Patients with OHVIRA usually present soon after menarche with dysmenorrhoea, pelvic pain or pelvic mass. Rarely, a patient may present in early adulthood with infertility. No such complaints were reported by our patient except for mild dysmenorrhoea. She cannot be labelled as a case of primary infertility as she had been married for the last 8 months only. OHVIRA was incidentally detected while being investigated for RIF pain caused by acute appendicitis. Moreover, she did not have haematometra, haematosalpinx or endometriosis in spite of her late presentation. This might be owing to a partial decompression of haematocolpos into the left hemivagina 8She also had a block vertebra involving the C6–C7 vertebrae. Vertebral anomalies are found in 29% patients with MDA, which include block vertebra, spina bifida or wedged vertebral body.Our patient had an unusual variant of HWW syndrome, having a complete septate uterus with OHVIRA with block vertebra involving the C6–C7 vertebrae.The patient developed an appendicular lump during her stay in the hospital, so she was managed conservatively with intravenous antibiotics and analgesics. Excision of the vaginal septum with drainage of approximately 400 cc of deep brown-coloured haemorrhagic fluid was performed 4 weeks later. Her post-operative period was uneventful.Early diagnosis of MDA is important as it is associated with an increased risk of endometriosis, infertility and abortions.MRI is the imaging investigation of choice for the diagnosis and detailed evaluation of the type and complications of MDA.HWW syndrome is an unusual and special type of MDA, characterized by the presentation of uterus didelphys with OHVIRA.Compete septate uterus with OHVIRA is an exceedingly unusual variant of HWW syndrome. Very few cases have been reported in the literature to date.