Ganglioneuromas are benign slow-growing lesions that arise from sympathetic ganglion cells. They are usually found incidentally. Ultrasound and magnetic resonance imaging (MRI), provides only an unspecified diagnosis and it has to be confirmed by pathologic studies. Complete surgical excision is believed to be the curative treatment for symptomatic lesions. In the literature, the pelvic location reported is exceptional. We report a case of laparoscopic assisted excision of a retrorectal presacral ganglioneuroma for 22-year-old female patient. Ganglioneuromas (GN) are benign slow-growing lesions that arise from sympathetic ganglion cells. They are usually asymptomatic and found incidentally. Surgical excision is believed to be the treatment of choice for symptomatic lesions. In the literature, the pelvic location reported is quite limited. We describe a challenging surgical approach for a huge retrorectal ganglioneuroma.th sacred vertebra. T1-weighted images show intermediate signal intensity  were at normal ranges. On pelvic ultrasonography (US), we found a hypo echoic presacral lesion without adnexal mass or cyst. Pelvic magnetic resonance imaging (MRI) revealed the presence of an elliptic retrorectal presacral mass measuring 10 x 7 cm. The mass represses the rectosigmoid junction with mass effect and a close contact with the 4ntensity , whereasntensity .Prior to excision of the lesion, the patient underwent colonoscopy to ensure the absence of bowel involvement. Initially laparoscopic exploration was performed using three trocars. The sigmoid and the uterus and his adnexa were retracted to expose the tumor in pelvis . The couIn the deep pelvic floor, the mass seems to be attached to the posterior wall and the coccyx. Considering the difficulty in handling of the tumor through laparoscopic instruments, we decide to convert to laparotomy. A subtotal resection of the mass was performed via a midline incision. A residual tumor was left behind in an attempt to preserve the nerve root and prevent possible laminectomy for this young girl. The peritoneum was closed with a running suture and a pelvic drainage was performed in order to prevent residual hematoma. The patient had a good postoperative recovery and was discharged home seven days following surgery. The pathology exam confirmed a benign ganglioneuroma with no evidence of neuroblastoma . At one According to the International Neuroblastoma Pathology Classification, ganglioneuromas are benign slow-growing lesions that arise from sympathetic ganglion cells predominantly composed of Schwannian stroma with individually distributed maturing/mature ganglion cells . Also, iDiagnosis: for a considerable time, ganglioneuromas still clinically silent until they reach a large size and becomes symptomatic [ptomatic . The locptomatic , also, tptomatic . ConsideRadiology: ganglioneuromas is difficult to distinguish from other tumors due to lack of specific image findings. Ultrasound is regarded as the preferred initial screening and diagnostic modality for children with peritoneal tumors because it is non invasive, safe and readily available. But additional diagnostic tools include computed tomography (CT) and magnetic resonance (MR) imaging, which can provide more excellent visualization of tumors and reveal helpful information for surgical approach. The computed tomography cannot confirm the diagnosis but it may be helpful to determinate the extent of the tumor, organ of origin, regional invasion, vascular encasement, adenopathy and calcification. Considering the few findings about retroperitoneal GN and absence of any characteristic radiologic features, the definitive diagnosis is made after a histological study of the surgical piece.Management of GN: the complete surgical excision still the curative treatment [et al. has demonstrated surgery does not need to be radical if only minor residuals are left <2 cm [reatment -8. Neoadreatment  only if reatment . A radicreatment ,8. Recenft <2 cm .In the most publications the anterior intra peritoneal approach was considered the gold approach in pelvic GN. Other approaches reported in the literature include successful use of the posterior trans sacral approach. This was indicated due to the involvement of sacral nerve roots. In the literature, the laparoscopic approach still under experience and there is no consensus about the place of the mini-invasive surgery in retrorectal mass. Laparoscopy remains possible and even privileged for small well-defined masses without intimate relation to the large vessels ,10. It iPrognosis and follow up: the prognosis is good [ is good  and ther is good .The pelvic ganglioneuroma are rare. The surgical resection is the only curative treatment. In our experience, laparoscopic assisted resection of the pelvic tumor is technically feasible. It is preferred to adopt the surgical approach according to the surgeon experience and each individual patient case, taking account the tumor position, its relationship with the pelvic components and MRI characteristics.


Angiographically confirmed complete surgical excision of brain arteriovenous malformations (bAVMs) is conventionally considered curative. Recurrence in adults is rarely encountered; only 18 cases have been reported in the English literature over the past 30 years. The potential for recurrence and consequent need for routine long-term follow-up are important considerations in the management of these lesions. We report a case of a 23-year-old female with a recurrent bAVM discovered incidentally on routine imaging three years after complete surgical excision. We review the existing literature and discuss the options for surveillance and management. Brain arteriovenous malformations (bAVMs) are congenital anomalies of dysplastic blood vessels with direct connections between arteries and veins without intervening capillaries which form a tangle of abnormal dilated channels called a nidus. Most of these lesions are discovered incidentally during imaging for other indications; symptomatic bAVMs most commonly present with haemorrhage and seizures -3. bAVM A 23-year-old right-handed lady with no medical history presented in April 2017 to a district general hospital with acute onset left brachiofacial hemiparesis. A computed tomography (CT) scan of the brain showed a right frontal intraparenchymal haematoma Figure  and CT aShe was taken to theatre emergently for decompressive craniectomy, evacuation of the haematoma and excision of the bAVM. Intra-operative indocyanine green (ICG) angiography revealed two main arterial feeders on the cortical surface and one main draining vein to the superior sagittal sinus. The bAVM was excised en-bloc and post-excision ICG showed no remnant which was confirmed on immediate post-operative DSA Figure .She was transferred to our rehabilitation centre 19 days after surgery. While undergoing rehabilitation she had a generalised seizure attributed to scar epilepsy which was controlled with levetiracetam. She recovered well; her Functional Independence Measure (FIM) score improved from 28 to 108, and she had a cranioplasty implant four months later. She remained clinically well on six-monthly follow-up apart from another seizure in June 2018. A non-enhanced CT showed no haemorrhage and she had no further seizures after the dose of levetiracetam was increased. A routine CTA in August 2020 showed partial recanalization of the same bAVM nidus, now supplied via tiny right middle cerebral artery (MCA)  cortical branch(es) with venous drainage towards the superior sagittal sinus Figure .In light of her age and good functional status, repeat surgical resection was recommended although the option of stereotactic radiosurgery was also offered. After consulting with a radiosurgeon, she decided to proceed with surgical resection. A stereotactic CTA repeated in February 2021 showed stable size of the bAVM. She underwent resection of the recurrent AVM in March 2021 with stereotactic navigation via CTA. Intra-operatively, small cortical feeders were seen which were taken circumferentially; no large draining vein was identified. Post-operative day 1 CTA showed no remnant bAVM Figure . She hadbAVM recurrence in adults after angiographically demonstrated complete excision is a rare phenomenon. Only sporadic case reports/series exist; just 18 cases have been reported in the English literature over the last 30 years  have been found in adult samples , and higThere is little evidence to guide the duration, schedule and modality of follow-up imaging in adult patients after complete surgical resection; there is no consensus even in the paediatric literature ,31. BaseThe appropriate treatment strategy is similarly poorly established, and the risk of re-rupture of these recurrent bAVMs is unknown. Just over half the adult recurrences were treated by surgical excision, stereotactic radiosurgery (SRS) was the next most common option. A systematic review in 2018 that included paediatric cases found that surgery (59%) and SRS (37%) were the most common methods of treatment  and a rebAVM recurrence in adults after complete surgical resection is a rare but potentially important phenomenon. Routine follow-up imaging over an extended duration may be warranted, especially in cases with one or more risk factors for recurrence. New or progressive clinical manifestations should similarly trigger vascular imaging. Non-invasive imaging modalities may be a viable alternative to DSA for surveillance. Repeat surgical excision and SRS were the most common methods of treatment.


Abdominoscrotal hydrocele is an uncommon condition characterized by an hourglass-shaped scrotal hydrocele with an intra-abdominal component connected by an isthmus within the inguinal canal. We report a rare case of an adult patient with giant abdominoscrotal hydrocele. Despite recent trends toward less invasive treatments, in this case, the surgical approach through an inguinal incision was the better therapeutic option with a satisfactory outcome. Abdominoscrotal hydrocele (ASH) is an uncommon condition characterized by an hourglass-shaped scrotal hydrocele with an intra-abdominal component connected by an isthmus within the inguinal canal -3. This Patients are generally asymptomatic, but ASH can have complications related to the compression of adjacent structures, such as hydronephrosis, hydroureter, testicular dysmorphism, testicular torsion, altered spermatogenesis, spontaneous rupture or hemorrhage and testicular malignant transformation ,6. As spWe report a rare case of an adult patient with giant ASH.3. The results of testicular tumor markers revealed normal alpha-fetoprotein, increased human chorionic gonadotropin beta and increased lactic dehydrogenase. Computed tomography of the abdomen revealed a voluminous hourglass-shaped cystic formation involving the left testicle and extending into the abdominal cavity through the ipsilateral inguinal canal. The intra-abdominal component had an approximate volume of 2,067 cm³ and exerted compression on the bladder, rectum and sigmoid colon. The left testicle had increased dimensions and density and heterogeneous enhancement . The sample was sent for histochemical analysis, which confirmed the diagnosis of seminoma.The patient is currently in outpatient follow-up due to the diagnosis of seminoma and hypogonadism.An ASH is a collection of fluid in the tunica vaginalis that extends from the scrotum into the abdominal cavity . It is tThe nature of the lesion becomes apparent when a mass is felt above the inguinal ring and fluid is seen moving between the abdomen and scrotum upon compression of any of the structures ,10.The diagnosis of ASH is made through a clinical examination assisted by imaging methods. A positive transillumination test and cross-fluctuation between abdominal and scrotal collection are clinical benchmarks for the diagnosis. Ultrasonography is the first choice for demonstrating communication between the two components. However, computed tomography with contrast and magnetic resonance imaging can be used to demonstrate the extent of the hydrocele from the inguinal canal to the abdominal cavity -11.Treatment is normally surgical, which is the recommended intervention, as a spontaneous resolution is rare and ASH can cause complications ,7. DiffeASH is a rare form of hydrocele reported mainly in few case reports. ASH affects the pediatric population more than adults. In such cases, the lesion seems to be congenital. There are different hypotheses regarding its etiology as well as multiple clinical-pathological variants. A physical examination and ultrasonography are generally sufficient for the diagnosis, but further diagnostic tools may be indicated.Despite recent trends towards less invasive treatments, surgical excision through an inguinal incision remains the standard approach to ASH. In this paper, we report a clinical case and diagnosis of a patient with ASH treated through the surgical approach.


Late-presenting or “Adult-Onset” Diaphragmatic Hernia is uncommon, especially in individuals with no history of trauma. The non-traumatic diaphragmatic hernia may be a Congenital Diaphragmatic Hernia [CDH] lately manifested as a sequela to an iatrogenic intervention or prolonged infections. We aim to explore the genetic correlations in “adult-onset” CDH, with an insight into the indirect contribution of the COVID-19 pandemic towards the fatal outcome.In this report, we present a case of an adult female who died from the complications of an undiagnosed adult-onset diaphragmatic hernia, deemed completely preventable, if not for the global COVID-19 pandemic. There was no prior history of physical trauma or medical history of any relevance.Early diagnosis and rapid surgical intervention remain the keystone management for successfully treating individuals affected by this entity. The decedent in question presented with symptoms demanding hospital stay for investigations that would have aided in timely diagnosis and prevented death. However, the excessive fear of COVID-19 prevented the patient from undergoing hospitalization and follow-up, delaying the diagnosis and leading to death. She had two similar episodes in the past 3 months, but it subsided without medical intervention. The patient presented with these severe symptoms during the nationwide lockdown imposed by the Central Government after the invocation of section 6, Disaster Management Act. Physical examination and radiological examination of the abdomen was done during the earlier presentation; however, the same proved to be unremarkable. Hence, she was managed conservatively with gastritis as the most likely diagnosis.Two days later, she presented again with severe epigastric pain followed by eight episodes of vomiting, fever, and breathlessness. Routine blood tests and ECG were normal. Along with the regular workup for evaluating the fever, gastrointestinal complaints and breathlessness, the prevalent pandemic conditions alerted the resident doctor at the primary health center to order a COVID-19 test. Swabs for COVID-19 tests were taken, and reports were awaited. Doctors with a basic medical degree usually oversee the Primary Healthcare Centers (PHCs) in India, referring cases needing detailed workup to a Tertiary Healthcare Center. The PHCs usually lack facilities like laboratories and radiological equipment. Even though the treating doctor referred her to a tertiary care center and advised admission, she refused to stay in hospital premises, fearing COVID-19 pandemic, and demanded conservative management.The treating doctor also advised her to undergo a repeat-radiological examination of the chest, abdomen, and pelvis and emphasized isolation. Again, fearing the pandemic and rules of compulsory isolation, the patient refused to acknowledge the referral and took discharge against medical advice. Soon, she became extremely dyspneic and expired at home. Her COVID-19 RT-PCR reports turned out to be negative.The decedent was autopsied five hours after her death. External examination was unremarkable, with neither injuries nor abnormalities. She had fused earlobes, with relatively small fingers and a short stature [body length=148 cm]. Internal examination revealed a loop of gangrenous small intestine upon opening the ribcage A. FurtheAlthough her external appearance with a non-traumatic etiology of diaphragmatic hernia warranted a genetic evaluation, neither the institution nor the medico-legal system had the necessary provisions to carry out a genetic study. The family was advised to do the same in a private set up to rule out any inheritable genetic diseases. The family refused to do the test, citing financial constraints.,Diaphragmatic hernia refers to a defect in the diaphragm that allows the abdominal contents to migrate into the thoracic cavity. Such defects are common after sustaining blunt or penetrating trauma to the chest and abdomen,,In the absence of trauma, a diaphragmatic hernia is a rarity in adults, usually preceded by a weakening esophageal hiatus leading to hiatal hernias. Even more unusual is when a CDH manifests in adulthood in non-traumatic conditions. CDH occurs consequent to incomplete fusion of pleuroperitoneal folds during the first trimester of gestation,,-The genetic etiology of CDH could be related to a single gene, with the mode of inheritance being either autosomal dominant , autosomDubbed as one of the most common syndromes associated with CDH as well as congenital dysplasia of lungs and craniofacial anomalies in infants, Fryns syndrome is reported in up to 10% of cases with a diagnosed CDH.In the present case, the presence of fused earlobes and short stature  warranted a syndromic approach to the autopsy, no significant morphological changes pertaining to congenital anomalies were noted other than the diaphragmatic hernia itself.,Morphologically prevalent among the CDHs are “Bochdalek hernias”, where the posterolateral muscles of the diaphragm fail to fuse. If the same occurs in the anterior mediastinum towards the right side, it is known as Foramen of Morgagni hernia . BochdalMales are more affected than females, with herniation occurring through the left hemidiaphragm in 70%–90% of the cases.Interestingly, a small foramen of Bochdalek may go unnoticed with spontaneous resolution, proving to be virtually harmless, whereas the wider ones could be fatal, as seen in the current case. It is only logical to assume that a small foramen can increase in size later in adult life, consequent to blunt trauma or exertion, which may explain the delayed presentation of a congenital diaphragmatic hernia. We believe was present in this case. Bochdalek hernias may remain dormant throughout childhood, only to be depicted incidentally on routine computed tomography scans.-,,,Studies and relevant literature reveal that the majority of adults with dormant CDHs who become symptomatic were subjected to some form of physical trauma.Early diagnosis has always remained an issue when it comes to catastrophes consequent to diaphragmatic herniation. However, recent years have seen an increment in the detection and treatment of many asymptomatic Bochdalek’s herniae, possibly due to a higher frequency of abdominal imaging.One of the key differences between the diagnoses of CDH in various age groups lies in its presenting symptoms. Neonatal CDH presents as pulmonary problems due to overcrowding of the thoracic cavity. On the contrary, in older children and adults, gastrointestinal pain or discomfort forms the chief complaint, reflecting the features of obstruction more often than respiratory complications.The adults presenting with a diaphragmatic hernia usually have a history of previous traumatic or physically strenuous events. In the present case, we found no evidence of trauma at the time of autopsy. Domestic abuse being common in the lower socioeconomic class, it is possible that an event of blunt trauma that occurred four months back could have caused the herniation. However, the husband denied the occurrence of any violent act upon questioning. Ingestion of a large meal was ruled out since the stomach contained fluid material only. There was no history of any recent pregnancies either.However, physical exertion as a cause could not be ruled out. An initial episode of gastrointestinal symptoms 3 months back suggests a slow progression of the herniation. Since her occupation is that of a laborer, there are more chances that she was exposed to physical exertion, which is generally overlooked in the lower socio-economic class. It can be safely assumed that this may have been the root cause that precipitated the herniation.,-Notwithstanding the etiology or presentation, prompt CT diagnosis could have saved the patient in the present case, assuming there was a rapid surgical intervention without postoperative complications. The fear of the COVID-19 pandemic led the patient to avoid hospital admission or routine investigations for her ailment, which was common during the initial wave of the SARS-CoV-2 infection. Hospitals recorded a major fall [up to 50%] in critical cases.Most cases of CDH remain asymptomatic and present as incidental findings in routine investigations. As a routine, laparotomy or thoracotomy, or a combination of both, form the basis of diaphragmatic hernia repair. Recent advances in minimally invasive surgery have made laparoscopic repair a more favorable option.Despite the lack of advanced genetic or chromosomal tests in our institute, we recommended the deceased’s family undergo further evaluation that may lead to the identification of genetic anomalies, which usually requires locus-specific DNA probes, FISH assay or alternate techniques. Citing financial constraints, they refused to perform the same. Although forensic pathology in developing countries is struggling to keep up pace with modern advances, it is imperative to implement protocols regarding collection of genetic data from all confirmed cases of congenital diaphragmatic hernia to gain clarity into the genetic causes as well as modes of inheritance of severe fetal anomalies such as CDH. Such a move would require legislative measures and funding from the government’s side. Alternatively, the government could commission private labs to analyze and report on the autopsy samples to expedite the otherwise slow process of awaiting the much-desired results.In the present case, although CDH-associated anomalies didn’t seem to play a major role in death, it is safe to assume that if the excessive fear towards COVID-19 pandemic was eliminated, the female in question would have proceeded with the investigations and, assuming that a diagnosis was established, prompt surgical intervention could have prevented the fatal outcome. On a related note, not only is the government responsible for ensuring proper awareness of lethality pertaining to COVID-19, but also it is essential to curb disproportionate fear of a pandemic. This can be achieved by the dissemination of appropriate information and encouraging the citizens to seek help for any medical conditions that may affect them. An audit across the country for similar incidents focusing on deaths due to treatable medical conditions is also strongly recommended to understand the indirect effects of the pandemic.


Wandering spleen (WS) is a rare disease caused by the looseness of the splenic ligaments. A 29-year-old female patient presented to the emergency department with complaints of abdominal pain and a palpable mass in the abdomen. A diagnosis of WS was made as a result of preoperative imaging. We performed urgent laparoscopic splenopexy with non-absorbable mesh in a patient with torsioned WS. WS is a disease that must be operated on urgently because it causes ischemia and necrosis in cases where it causes torsion in the splenic pedicle. Many researchers also recommend surgery in asymptomatic patients. While splenectomy was previously recommended for WS, current recommendations advocate for splenopexy. As a result, the only and definite treatment option in the case of WS is surgery. Splenopexy with minimally invasive techniques should be the first choice if possible. Splenopexy with non-absorbable mesh is an inexpensive and feasible method to prevent re-torsion. The use of non-absorbable mesh in laparoscopic splenopexy has not been shared before in the literature. Wandering spleen (WS) is a rare condition and was first described on an autopsy in 1667 by a Dutch doctor, Van Horne. In this disease, there is laxity or absence of splenic ligaments. Apart from its normal position, the spleen can be found anywhere in the abdomen, from the bottom of the left diaphragm to the pelvis . For theA 29-year-old female patient presented to the emergency department with complaints of abdominal pain and a palpable mass in the abdomen. Her past medical history was unremarkable. There was a 20*15 cm mass extending from the umbilicus to the right lower quadrant on physical examination. Body mass index: 17.3 kg/m². The patient's blood tests were normal. Abdominal ultrasound revealed the absence of spleen in the left upper quadrant and a mass compatible with a 22*18 cm spleen in the right pelvis. Triphasic abdominal tomography revealed a mass compatible with the spleen in the pelvis, 720° torsion in the splenic vein, and free fluid in the lower abdominal quadrants, and there was blood flow in the splenic artery. WS and related torsion were considered. A single dose of prophylactic antibiotic was given before surgery. During the operation, the patient was in the supine position, the surgeon and the camera assistant were placed on the right side of the patient, with the monitor on the left side of the patient, 10mm trochar inferior umbilicus, 10mm trochar from the right midclavicular line level, from the lateral umbilicus and 5mm trochar from the right upper quadrant. It was observed that the spleen was located in the pelvis together with the distal pancreas, and there were two torsions in the pedicle. It was found that the spleen was viable and larger than normal. It was observed that the spleen dimensions returned to normal after being detorsion , it is seen ten times more in women than men after the age of ten . While aIn symptomatic patients, splenopexy can be performed if the spleen is viable, but splenectomy should be performed if there is necrosis in the spleen. Both splenectomy and splenopexy can be performed laparoscopically . In asymSplenectomy is required in 50% of patients with acute ischemia with torsion due to WS . TherefoAs a result, the only and definite treatment option in the case of WS is surgery. The first and most important step in diagnosis is to suspect this disease. Splenopexy with minimally invasive techniques should be the first choice in possible patients. Splenopexy with non-absorbable mesh is an inexpensive and easily applicable method to prevent re-torsion.


A 46-year-old healthy man presented with recurrent epigastric pain and nausea of 1-month duration. He denied other gastrointestinal symptoms. Physical examination was unremarkable. Laboratory tests showed the following abnormal liver enzymes: SGPT 460 IU/L, SGOT 332 IU/L, and alkaline phosphatase 462 IU/L. Magnetic resonance cholangiopancreatography showed the gallbladder, common bile duct (CBD), common hepatic duct, and proximal right and left hepatic ducts packed with gallstones, with a dilated CBD measuring 12 mm (Figure 1 Biliary stenting is associated with a reduction in the number and size of CBD stones.2 Studies have identified multiple potential gene mutations responsible for gallstone formation, such as CYP7A1, ABCB4, and AOPE.3 Their role in promoting gallstone development in humans is still not fully understood. The patient has no obvious risk factors or family history of gallstone disease. A genetic workup was not performed. We report a rare case of numerous gallstones filling the biliary tree. To the best of our knowledge, no other similar case was previously reported in the literature.Finally, a plastic stent was placed in the common bile duct. The patient underwent a laparoscopic cholecystectomy the next day and was discharged home the day after with an uneventful hospital stay. The management of large and/or multiple CBD stones requires usually endoscopic sphincterotomy followed by balloon dilation.Author contributions: T. Nammour and M. Shmais wrote the article. A. Soweid edited the article and revised the article for intellectual content. T. Nammour is the article guarantor.Financial disclosure: None to report.Informed consent was obtained for this case report.


We present the first, to our knowledge, case of a dedifferentiated low-grade central osteosarcoma (LCOS) of the mandible. A 48-year-old Japanese woman underwent enucleation under general anesthesia after a diagnosis of ossifying fibroma. At the second recurrence, the pathological diagnosis after biopsy was of sarcoma with MDM2(+) and CDK4(+) immunohistochemical staining results. Hemimandibulotomy, supraomohyoid neck dissection, and free-flap reconstruction with a rectus abdominal flap were performed. A retrospective reevaluation of the first specimen with additional immunohistochemical staining for MDM2 and CDK4 yielded a final diagnosis of dedifferentiated LCOS. The patient showed no recurrence or lung metastasis 3 years after the final surgery. LCOS represents less than 2% of all OSs reported in the literature . In genegrade OS , to our A 48-year-old Japanese woman who showed radiolucency of the left mandible on panoramic radiography was referred to the Department of Oral and Maxillofacial Surgery, University of Tsukuba Hospital. Her medical history included hypertension and an ovarian cyst. Her face was symmetrical, and slight paresthesia of the lower lip and mentalis was noted. Diffuse elastic hard swelling was observed from the left canine to the second molar. Panoramic radiographs revealed a posterior well-demarcated radiolucent area and an anterior irregular area . A well-Primary recurrence occurred 1 year and 3 months after the first enucleation. A soft tissue mass-like granuloma appeared in the left molar area intraorally. The biopsy indicated granulation tissue with spindle cells. Panoramic radiography revealed an increase in the radiolucent area to the right side of the mandible. Bilateral mandibulectomy and reconstructive surgery with fibula were performed, and the spindle cell-like fibroblast tumor cells were found to be pathologically complicated and thick, forming irregular eosinophilic osteoid. The tumor had destroyed the cortex of the mandible, and the surgical margin was free. The nuclei were swollen and mildly oval. The atypia was mild, and 3 mitoses/50 HPFs were observed. The retrospective immunohistochemical staining findings were MDM2(+), CDK 4(-), and a Ki-67 index of 15%.α-SMA(+), desmin(-), h-caldesmon(-), pankeratin(-), S-100(+: sporadic), CD34(-), β-catenin(-), MDM2(+), CDK4(+), and Ki-67 (40%–50%)  .th) was made. Postoperative chemotherapy and radiotherapy were not performed because of patient refusal. There was no recurrence or lung metastasis, and the patient's status has remained uneventful for 3 years after the final surgery.Retrospective reevaluation of the previous specimen with additional immunohistochemical staining for MDM2, CDK4, and Ki-67 was performed, and a final diagnosis of dedifferentiated LCOS (pT1N0, stage IIA: UICC 8LGOS is rare and subdivided into parosteal OS and LCOS. Parosteal OS comprises a surface tumor representing 4%–5% of all OSs; it usually develops in the posterior surface of the distal femur in the third decade and in 15% to 43% of cases dedifferentiates into high-grade OS . Only onTriantafillidou et al.  previousLCOS is also more likely to be misdiagnosed and inappropriately treated with an intralesional procedure because of overlap of its pathological characteristics with those of benign bone tumors . Our patDujardin et al. reported that MDM2 and/or CDK4 immunoreactivity was present in 89% of LGOS specimens. Although dedifferentiated LGOS and conventional high-grade OS involve different oncogeneses, they share histological features. Therefore, dedifferentiated LGOS is histologically indistinguishable from conventional OS. MDM2 and CDK4 are also markers for distinguishing between dedifferentiated LGOS and conventional high-grade OSs . Toki et


Prostate abscess (PA) is an uncommon clinical manifestation that typically presents in the fifth to sixth decade of age and sporadically affects neonates. These characteristics, coupled with the atypical presentation, represent a clinical dilemma and a challenging diagnosis. A detailed history depicting the clinical course and the presence of risk factors is imperative to alert the clinician of the possibility of a PA. In this case report, we present a surgically confirmed PA, a diagnosis that is rarely encountered within the paediatric age group. Allison in 1842 initially described a fatal case of a PA complicated by spontaneous urethral rupture into the recto prostatic fascia.2Prostate abscess (PA) occurs as a result of the focal accumulation of pus in the prostate gland and is commonly related to the spread of Gram-negative bacilli from the urinary tracts.3 New at-risk populations and the wide range of local and systemic symptoms present a clinical challenge in diagnosis.3Prior to the use of antibiotics, gonorrhoeal infection was the most prevalent aetiology. However, with the advent of antibiotic use, Gram-negative bacteria are the most common organisms encountered and are responsible for 60% – 80% of cases.A 6-year-old male with a medical history of post streptococcal glomerulonephritis presented to the hospital with clinical symptoms of headache and pelvic pain for 2 days.Haematuria was noted in his urinalysis. The patient’s symptoms persisted over 2 days progressing to fever and suprapubic pain. An ultrasound of the lower renal tracts  revealedThe voiding cysto-urethrogram was normal with no features of vesicoureteral reflux or posterior urethral valves. Magnetic resonance imaging (MRI) of the thoracic and lumbar spine  was condStaphylococcus aureus was cultured and intravenous antibiotics were administered. The blood cultures and nasal swabs excluded haematogenous and distant focus of infection. Improvement in symptoms was noted following drainage of the abscess. On follow-up, the patient demonstrated a clinical response to treatment with resolution of the symptoms. Follow-up sonographic images were not available as they were performed at another institution, however the clinician documented resolution of the hydronephrosis.The patient underwent drainage of the abscess. Paediatric prostatic abscess is an uncommon condition and continues to remain a difficult clinical diagnosis to consider. In this case, it was only considered as an incidental finding on the limited images through the bladder base. This highlights the importance of considering PA formation in paediatric patients who present with signs and symptoms of renal tract infection and bladder outlet obstruction. Early diagnosis and treatment of the condition has significant benefits in terms of the relief of patient symptoms and prevents further damage to the renal tracts.This article followed all ethical standards for research. Data and images in the manuscript were anonymised.4 In the presence of bilateral hydronephrosis, vesicoureteral reflux and bladder outlet obstruction are the most likely causes. The common causes of bladder outlet obstruction may be divided according to the level of the obstruction. At the level of the bladder, bladder neck hypertrophy and neuropathic bladder may be considered.4 Urethral valves, strictures and polyps are common causes for the urethral cause of obstruction.4 Prostatic abscess with mass effect and obstructive uropathy represents a rare cause of obstructive uropathy. Other causes of cystic prostate lesions in the paediatric age group include a prostatic utricle cyst and a Müllerian duct cyst.5Urinary tract obstruction can result from congenital or acquired lesions that may occur at any point from the calyx to the tip of the urethra. Most childhood obstructive lesions are congenital.6 The symptoms include dysuria, increase in frequency, fever, urinary retention and leucocytosis.6 The persistence of the symptoms despite antibiotic treatment should alert the clinician to the possibility of a PA.6Prostatic abscess is a rare clinical entity and typically presents in the fifth to sixth decade of life. The diagnosis is often challenging as it mimics a lower urinary tract infection.1 Other causes of immunosuppression including human immunodeficiency virus (HIV), liver cirrhosis, renal failure and haemodialysis have been cited as predisposing factors for prostatic abscess.1 Chemotherapy, organ transplant or previous genitourinary procedures also present a higher risk of prostatic abscess.3In the background of a low prevalence of the clinical entity, as well as the wide range of local and systemic symptoms, it is imperative to understand the risk factors and maintain a high index of suspicion within these patient profiles. In adults, diabetes mellitus has been identified as the most common co-morbidity, with a reported co-prevalence of 63%.Escherichia coli and other coliform bacteria are the commonest causative organisms. The less common mechanism of infection is secondary to haematogenous spread from a distant focus. This includes a variety of organisms, including S. aureus, Mycobacterium tuberculosis, E. coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, Burkholderia pseudomallei and Candida spp.1Two mechanisms for the pathogenesis of prostatic abscess have been described. The first is via the retrograde flow of contaminated urine through the prostatic duct and subsequent infection of the prostate. Bladder outflow obstruction and immunosuppression are predisposing factors and enteric organisms such as 1 The diagnosis may be difficult because flocculence is present in only one third of patients. In addition, rectal examinations are not routine for infants.7 More than 90% of patients will present with a leucocytosis and almost all patients will demonstrate a leukocyturia.8The symptoms overlap with symptoms of other lower urinary tract pathology, and the diagnosis of a prostatic abscess is often delayed. Hence it is often only diagnosed after the failure to respond to medical treatment.1A prostatic abscess may complicate, fistulate and drain into one of the surrounding pelvic structures. Abscesses located at the base of the prostate gland commonly fistulate to the bladder or prostatic urethra, and those near the apex result in a rectal or perianal fistula. Severe cases of a prostatic abscess may extend into the seminal vesicle and spermatic cord.9 A prostatic abscess appears as a hypoechoic or anechoic lesion with more or less defined edges and a peripheral hyperechoic halo.10 They are often of varying sizes, contain internal septations and are typically located in the transitional and central zones.3In the paediatric population, ultrasound is the initial diagnostic modality of choice to evaluate the paediatric lower urinary tract. A 5-megahertz (MHz) to 7-MHz transducer is used transabdominally and, when high resolution is required, a 7-MHz to 12-MHz transducer may be used transperineally. The paediatric prostate is visualised through a full bladder as a hypoechoic, elliptically shaped, soft tissue structure at the bladder base.9 A prostate abscess appears hypointense on T1-weighted MRI and hyper-intense on T2-weighted images.1Magnetic resonance imaging defines the cross-sectional anatomy of the prostate with greater tissue differentiation compared to computed tomography (CT). The prostate shows homogenous signal intensity on MRI imaging that is isointense to muscle on T1-weighted images and iso- to hyper-intense to muscle on T2-weighted images before puberty. After puberty, the signal characteristics resemble those of the adult prostate.11 Collins et al.7 reviewed 13 cases of neonatal prostatic abscess and described Staphylococcus spp. in 77% (10 out of 13) of the reviewed cases. Staphylococcus spp. is a rare cause of paediatric urinary tract infection, suggesting that paediatric prostatic infection likely arises from haematogenous spread rather than an ascending urinary tract infection.7 Chao and Yang12 described a case of a large prostatic abscess in an adolescent successfully treated with antibiotics. Prior to this, only three cases of prostatic abscess in adolescents have been reported.12Reports of prostatic abscesses in neonates have been published. Mann described three cases of acute staphylococcal prostatitis causing urinary obstruction in neonates.S. aureus, specifically methicillin resistant S. aureus (MRSA), have increased recently.13 Kiehl et al.6 reported the case of a 15-year-old male with an MRSA prostatic abscess. In that case, the patient was noted to have a history of MRSA abscesses and recent hospitalisation.6 Foster et al.13 documented the first reported case of methicillin-susceptible S. aureus (MSSA) prostatic abscess in a paediatric patient beyond the neonatal period.Reports of prostatic abscess secondary to 13Prostatic abscess in the paediatric population is a rare occurrence. The referenced cases and our case report highlight the importance of a systematic radiological analysis of the upper and lower urinary tracts in a patient presenting with bilateral obstructive uropathy. Interestingly, our index patient did not demonstrate haematogenous spread or a distant focus of infection and responded completely to medical and surgical intervention. Upon exclusion of the common causes for bilateral obstructive uropathy, a high level of clinical suspicion is needed to diagnose this entity, which in the paediatric population likely arises secondary to haematogenous spread.Although rare, few cases of paediatric PA have been reported in neonates and the finding of a PA in a 6-year-old is an even rarer event. The few case reports describing this clinical entity suggest that prostate infection likely arises from haematogenous spread. Prostatic abscess represents a diagnostic and therapeutic challenge, and in the systematic evaluation of an obstructive or non-obstructive aetiology of bilateral hydronephrosis, it is imperative to consider a PA or prostatic lesion as a possible aetiology, as it is often overlooked because of its rarity.


We present a 14-year-old boy with peritoneal epithelial malignant mesothelioma (PEMM). While pathology is required to make this diagnosis, radiology plays a crucial role throughout the clinical course of this disease. The key imaging characteristics of peritoneal mesothelioma have been previously well-described in the adult population, but there are rare reports in the pediatric population. This pediatric report highlights the multidimensional use of imaging in this disease, from the initial evaluation to therapeutic supplementation and subsequent follow-up. Mesothelioma is an exceedingly rare tumor involving the serosal cells of body cavities, occurring 0.5–1.0 cases per 10 million in children. Peritoneal mesothelioma specifically involves the serosa of the peritoneum and represents 20% of these cases. Peritoneal mesothelioma is divided into various subtypes based on clinicopathologic characteristics and consists of malignant mesothelioma, cystic mesothelioma, and well-differentiated papillary mesothelioma. Within the pediatric population, peritoneal epithelial malignant mesothelioma (PEMM) occurs more commonly in females and, unlike the adult population, there is no clear association to asbestos or radiation [The diagnosis of PEMM is made through pathology and can be supported by molecular testing; however, radiology plays a crucial role throughout a patient's clinical course. In particular, the pretherapeutic assessment of lesions with imaging is crucial to evaluate for resectability. Magnetic resonance (MR) imaging is the imaging modality of choice, as it is efficient in evaluating the extent of disease. Computed tomography (CT) can also be used in the evaluation of these patients who commonly present with abdominal pain. Key imaging findings include peritoneal thickening, nodularity, and enhancement as well as ascites . PositroA 14-year-old male with a history of growth hormone deficiency, gastroesophageal reflux disease, and asthma presented to his primary care physician with chronic abdominal pain, and over 20-pound weight loss in a 9-month period. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were elevated at diagnosis, 14.6 mg/mL  and 47 , respectively. MR enterography (MRE) of the abdomen and pelvis was performed to evaluate for inflammatory bowel disease, as per our institutional MRE protocol: Breeza was administered orally for small bowel distention and 4 cc of intravenous contrast was administered based on patient weight. MRE revealed a thickened omentum along with diffuse, smooth peritoneal enhancement and thickening. A moderate to large volume of ascites was also present Figures . The nodAfter initial imaging, interventional radiology performed an ultrasound-guided omental core biopsy and diagnostic paracentesis. The core biopsies consisted of scant fibroconnective tissue. The cytology slides and cell block revealed a hypercellular specimen composed of benign and reactive mesothelial cells, scattered macrophages, and moderate inflammation, but the sample was deemed too small to be diagnostic. Oncology was consulted, and tumor markers were done. CA-125 was elevated at 171 U/mL , while CA 19-9, CEA, Beta-HCG, and alpha-fetoprotein were normal.Subsequently, laparoscopic omental and peritoneal biopsies were performed to evaluate for inflammatory diseases, malignancy, and possible infection. The biopsy specimen revealed sheets of neoplastic mesothelial cells with foci of chronic inflammation . Stains The patient subsequently underwent 5 cycles of intravenous (IV) chemotherapy with cisplatin, pemetrexed, and Avastin. His tumor was negative for PDL1 and mismatch repair protein defects. Next-generation sequencing (NGS) failed due to inadequate specimen; therefore, we could not assess tumor mutational burden (TMB). Because of a negative PD-L1 status, we did not use PD-L1 inhibitors [The patient underwent debulking surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) with cisplatin. Due to persistent active disease on pathology in the first debulking surgery, the patient had a second HIPEC with cisplatin with peritoneal catheter placement followed by early postoperative intraperitoneal chemotherapy (EPIC) with 4 cycles of intraperitoneal (IP) cisplatin alternating with mitomycin , 6. Of nPrimary peritoneal malignancies, like PEMM, are exceedingly rare but an important diagnosis to consider in cases of pediatric peritoneal pathology. The initial symptoms of mesothelioma can be nonspecific, so diagnosis is often delayed. As in our patient, the initial workup for the nonspecific symptoms including nonspecific elevated inflammatory markers may be targeted more toward looking for inflammatory bowel disease and it can take time to come to the correct diagnosis . ImagingOf note, while imaging clearly plays an important role in the clinical course of PEMM, pathology is required to confirm the diagnosis of peritoneal mesothelioma. Ultrasound-guided biopsies must be supplemented with surgical biopsies in PEMM, as in our case. The molecular testing helped confirm the diagnosis. Studies have shown deletions of the CDK2NA  locus in patients with a malignant pleural and peritoneal mesothelioma, whereas deletions of this region were not observed in patients with reactive mesothelial hyperplasia . GermlinThere are a number of key imaging features of PEMM, which can be seen across various imaging modalities. CT, MR imaging, and US reveal thickened peritoneum; peritoneal nodules; omental, mesenteric, and serosal surface plaque-like masses; and ascites . HoweverThe limitations of the case include that this is the report of an individual's disease process and treatment, and these things may vary depending on the patient. The value of this report is to make clinicians aware of the entity, although the details within this particular case may not be generalizable to a cohort. While radiology findings can support or suggest the diagnosis, they cannot be used alone and must have pathology and molecular testing for final diagnosis.Although quite rare, a diagnosis of peritoneal mesothelioma should be considered when presented with the imaging findings illustrated in this case. An interdisciplinary approach is essential for successful diagnosis and treatment of PEMM. The radiologist should be familiar with key imaging findings among various imaging modalities in order to help guide initial investigation and subsequent management of this disease. This report demonstrates the valuable role of the radiologist, the typical radiographic features, and the numerous mechanisms of imaging in a case of PEMM.


Collision tumors are rare neoplasms defined by the presence of two distant tumors in the same organ without any histological intermixing. Ovarian tumors are often asymptomatic during the early stages and become symptomatic when increased in size causing vague abdominal pain, abdominal distention, vomiting, and frequent urination. We report here a case of a 28-year-old female who presented with a history of worsening abdominal pain and distension. An abdominopelvic ultrasound scan showed a huge complex mass occupying the pelvic area with non-visualization of the left ovary suggesting an ovarian origin; further characterization by cross-sectional imaging by both CT and MRI were performed confirming a left ovarian complex mass containing multiseptated cystic and fat component at the same time along with massive ascites. After surgical resection of the mass, histopathology revealed mucinous cystadenoma coexisting with cystic teratoma. A collision tumor is defined as the coexistence of two different tumors within the same organ with no histological intermixing. Collision tumors have been described in various organs including the stomach, esophagus, liver, kidney, bone, lungs, and brain but once it comes to the ovaries; they are rare . TeratomOvarian mucinous cystadenoma represents approximately 80% of the ovarian mucinous neoplasm and 20%-25% of all types of benign ovarian tumors. Generally, mucinous cystadenomas are larger than serous cystadenomas and they are rare to present bilaterally (2%-5%) . In thisA 28-year-old, nulligravid lady, presented with abdominal pain and abdominal distension. On examination, her vital signs were normal and she was clinically stable with normal general physical examination apart from a palpable pelvic mass. She was initially investigated with an abdominopelvic ultrasound scan and serum human chorionic gonadotropin (hCG) test to exclude pregnancy.Her ultrasound scan revealed a massive 21 cm x 11 cm abdominopelvic mass originating from the left ovary, it was a complex multiseptated cystic mass associated with massive ascites; her carbohydrate antigen (CA) 125 level was 94 U/mL , CA19.9 was 182 U/mL , human chorionic gonadotropin (hCG) was 0 IU/L, AFP was 1.2 KIU/L , and CEA was 83.9 . Contrast‐enhanced CT showed a large midline cystic multilocular left adnexal mass with few foci of macroscopic fat component and calcification, the septa were thick. It was measuring 28 cm x 16.8 cm x 20 cm . Also, there were associated large ascites causing scalloping on the liver surface (Figure Abdominal MRI revealed the same findings of the huge left ovarian mass with thick enhanced septa, containing fat and few foci of calcification. The cystic part of the mass showed dark T1, bright T2 signal intensity, the fatty component was bright on the non-fat saturated T1 and T2 weighted images and suppressed on T1 fat-saturated pre-contrast images. The right ovary was normal. Along with the massive ascites scalloping the liver, there were multiple- peritoneal deposits, the largest measured 1.8 cm (Figure The patient went for open surgery for left adnexectomy, omentectomy, and appendectomy. On histopathology, the ovarian mass was confirmed to be a mucinous cystadenoma with focal epithelial proliferation mixed with mature cystic teratoma, negative for malignancy. The omentum showed fibroconnective tissue with acellular mucin, negative for malignancy. And the appendix was normal without dysplasia or malignancy. The peritoneal deposits were sent for histopathology as well which showed multiple foci of acellular mucinous deposition with exuberant inflammation. And the diagnosis of collision tumor was made based on the radiological and histopathological findings.Collision tumors are uncommon neoplasms that represent two distant tumors in the same organ without any histological intermixing. Collision tumors have been reported in different organs such as the gastrointestinal tract, central nervous system, lung, skin, adrenals, etc. However, collision tumors are uncommon to involve the ovary. Collision tumor is more common to be unilateral, and its size ranges from 5.5 to 200 cm, and mostly the patients' ages range from 17 to 66 years . TeratomCystic teratoma is the most common ovarian germ cell tumor representing 20% of all the ovarian tumors in adults, and it is the most common benign ovarian tumor among women less than 45 years old . CT and Ovarian epithelial neoplasms represent around 60% of all ovarian tumors and 40% of benign ovarian tumors. Ovarian cystadenomas are common benign epithelial neoplasms and represent less than 5% of all epithelial ovarian malignancies . They arOvarian mucinous cystadenomas usually manifest as unilateral, multilocular, cystic, and ovarian lesions . The mosIn conclusion, the most common form of ovarian collision tumors in the literature is the combination of teratoma. Although ovarian collision tumors are extremely rare, the coexistence with other benign ovarian tumors is rarer. Multiloculated cysts have to be extensively examined radiologically and histopathologically, not to miss any component which might have a bearing on prognosis of the patient.


OFD1, a gene responsible for a X-linked ciliopathy, the oral-facial-digital syndrome type 1 . This case expands the complex phenotype of OFD1 syndrome and suggests a possible involvement of OFD1 gene and Shh pathway in the pathogenesis of these anomalies.Morning glory disc anomaly is a congenital abnormality of the optic disc and peripapillary retina reported as an isolated condition or associated with various anomalies, including basal encephaloceles and moyamoya vasculopathy. However, the co-occurrence of these three entities is extremely rare and the pathogenesis is still poorly understood. Moreover, data on the surgical management and long-term follow-up of the intracranial anomalies are scarce. Here, we describe the case of a 11-year-old boy with morning glory disc anomaly, transsphenoidal cephalocele, and moyamoya vasculopathy, who underwent bilateral indirect revascularization with encephalo-duro-myo-arterio-pericranio-synangiosis at the age of 2 years, and endoscopic repair of the transsphenoidal cephalocele at the age of 6 years. A rare missense variant  was found in  Morning glory disc anomaly (MGDA) is a congenital abnormality of the optic nerve characterized by an enlarged and funnel-shaped excavation of the peripapillary fundus with glial tissue overlying the center of the disc, an annulus of chorioretinal pigment, and spoke-like vessels radiating outward from the edge of the anomalous disk . Most caAnother interesting association of MGDA is with basal cephaloceles, rare malformations characterized by evagination of intracranial structures through a congenital defect of the anterior skull base and dura . In partOf note, the co-occurrence of MGDA, moyamoya vasculopathy, and basal cephalocele is extremely rare with only three patients described so far –20. The Here, we describe the clinical, neuroimaging, and genetic features of an 11-year-old boy with MGDA, mild midfacial defects and digital anomalies, who underwent bilateral indirect revascularization for moyamoya vasculopathy at the age of 2 years and transsphenoidal repair of a spheno-nasopharyngeal cephalocele at the age of 6 years.The boy is the first child to a healthy Brazilian mother and an Italian father. He was born full-term without perinatal complications. During the neonatal period, rotational nystagmus was noted in both eyes. Fundus examination revealed bilateral MGDA. Brain MRI and head CT performed at 6 months of age showed bilateral MGDA associated with a small transsphenoidal cephalocele Fig. . At thatThree weeks after the ischemic event, the patient underwent left indirect revascularization with encephalo-duro-myo-arterio-pericranio-synangiosis (EDAMPS), followed 3 months later by right EDAMPS. The patient showed a good recovery and regression of neurological symptoms. Serial follow-up brain MRI and MRA with perfusion studies showed bilateral development of pial collateralization in the sites of surgical revascularization with marked improvement of brain perfusion Fig. .Fig. 2BAt the age of 6 years, the patient started complaining of headache with no other neurological symptoms or endocrine deficits. Brain MRI and MRA did not show new ischemic lesions, vascular changes, or worsening of the brain perfusion, while the transsphenoidal cephalocele was increased in size. Therefore, transsphenoidal repair of the spheno-nasopharyngeal cephalocele was performed. In details, using endoscopic endonasal approach, the sac was isolated and partially removed, and the defect was reconstructed with a nasoseptal flap to prevent the risk of cerebrospinal fluid leaks. The postoperative course was uneventful, and the headache resolved. The postoperative brain MRI demonstrated marked reduction of the cephalocele volume.Two years after surgery, the patient showed reduced growth velocity with low IGF1 levels . Glucagon stimulation test showed a GH peak value of 0.38 ng/ml, compatible with GH deficiency. Treatment with recombinant human growth hormone (rhGH) was therefore started. At the age of 9 years, TSH level was found to be low and treatment with levothyroxine was started.OFD1 was carried out. A novel hemizygous missense variant, c.1081T>C,p.Tyr361His (NM_003611.2), was detected in the boy and in his healthy mother. This variant has never been reported in the literature or in any public database, including gnomAD. The p.Tyr361His is a change of an evolutionary conserved amino acid and is predicted deleterious by several predictive tools . Although c.1081T>C,p.Tyr361His is classified as a variant of uncertain significance according to the American College of Medical Genetics and Genomics (ACMG) guidelines, it holds two criteria, namely PM2 (absent in gnomAD) and PP3  that suggest a possible pathogenic role. However, given the lack of other clinical features of OFD1 and the finding of MGDA and moyamoya vasculopathy never reported before in this syndrome, we performed whole-exome sequencing (WES) through a trio-based approach to seek other possible pathogenic variants. Data were processed using in-house analytical pipelines that include publicly available tools and custom scripts. We looked at non-synonymous exonic and splicing variants with a minor allele frequency ≤ 0.001 in gnomAD database and we did not identify any possible deleterious variants in any other genes.At last evaluation, at the age of 11 years, his height was 98 cm (< 3rd centile), his weight was 18.5 kg (75th centile), and he had an OFC of 48.5 cm (< 3rd centile). On physical examination, he presented rotational nystagmus, ptosis, hypertelorism, a pit in the midline of the philtral groove, short neck, brachydactyly, clinodactyly of the V digit bilaterally, short toes, broad hallux bilaterally, and micropenis , a X-linked-dominant male-lethal ciliopathy characterized by malformation of face, oral cavity and digits, cystic kidney disease, and a wide spectrum of brain malformations [OFD1 mutations have recently been associated to novel phenotypes outlining the wide phenotypic spectrum of ciliopathies [OFD1 mutations. To date, the majority of OFD1 mutations are small insertions or deletions, whereas missense mutations are rarely reported [The etiology of MGDA, basal cephaloceles, and moyamoya vasculopathy is poorly understood, also due to the lack of genetic testing in subjects with this peculiar phenotype. Here, we describe the case of an 11-year-old boy with MGDA, mild midfacial defects, digital anomalies, moyamoya vasculopathy, and sphenoid cephalocele, who harbored a new rare missense variant in rmations . Of noteopathies . Howeverreported . TherefoOfd1 inactivation leads to defective sonic hedgehog (Shh) [Ofd1-/- mouse demonstrates a crucial role of Shh signaling in the closure of the craniopharyngeal canal [Ofd1-/- mutant mouse show a wide basisphenoid defect with inferiorly displaced pituitary tissue [OFD1 mutation [OFD1 variants may lead to midline cranial defects, such as the persistence of the craniopharyngeal canal and transsphenoidal cephaloceles. Of note, hedgehog signaling also regulates optic fissure and stalk formation during vertebrate eye morphogenesis [Ofd1 inactivation by injection of antisense morpholinos in zebrafish causes the incomplete fusion of the choroid fissure of the eye resulting in a retinal coloboma similar to MGDA [OFD1 variants [OFD1 might also indirectly interfere in the closure of the posterior aspect of the fetal optic fissure, leading to MGDA. Finally, disruption of the Shh signaling may also be implicated in moyamoya vasculopathy, taking into account the pivotal role of this gene in vessel formation and remodeling. Indeed, an increased vascularization of neuroectoderm after Shh overexpression has been reported [As for other ciliary proteins, it has been demonstrated in vivo and in vitro that og (Shh) . Interesal canal . In mammy tissue . Moreovemutation . Taken togenesis . Ofd1 in to MGDA . Interesvariants , 28. Thereported . On the reported . Interesreported , 32, indreported .Regarding the clinical and neuroimaging features of this patient, it is noteworthy that the first brain MRI performed at the age of 6 months did not reveal any vascular abnormality, except for two fetal variants. Subsequently, the rapid onset and progression of moyamoya vasculopathy lead to a cerebral ischemic infarct at the age of 2 years of age. This raises an important point related to the screening and surveillance of intracranial vascular anomalies in individuals with MGDA and basal cephaloceles. Indeed, moyamoya arteriopathy is associated with the highest odds of having recurrent ischemic strokes in children, with relevant consequences for the clinical outcome and quality of life . MoreoveHeadaches may arise from intra and/or extracranial painsensitive structures such as skin, muscles, blood vessels, dura mater, and cranial nerves. In the present case, the onset of headache at 6 years of age was not associated with worsening of the moyamoya vasculopathy and/or brain perfusion. Conversely, an increase in size of the transsphenoidal cephalocele was observed, in the absence of endocrinological problems. Of note, headache has been reported in subjects with transsphenoidal cephaloceles, likely related to the mass effect or the traction on adjacent structures, such as the optic chiasm and pituitary gland . MoreoveOFD1 in MGDA, basal cephalocele, and moyamoya vasculopathy. However, functional studies are needed to shed light on the role of this gene and Shh signaling in the pathogenesis of this rare triad. Regarding the clinical and surgical management, this case underlines the importance of screening and surveillance of moyamoya vasculopathy in individuals with MGDA and basal cephaloceles, especially considered the possibility of early revascularization that might improve the clinical outcome. Finally, surgical management of the basal cephalocele might be pondered in the presence of symptoms that may not be attributed to other causes.In conclusion, this case provides insights on the potential role of


Cavernomas are rare cerebrovascular malformations that usually occur in sporadic forms with solitary lesions located most often in the hemispheric white matter, but also in the infratentorial or spinal region. Multiple lesions at different CNS levels are considered a hallmark for the familial form of the disease. The diagnostic modality of choice for cerebral cavernous malformations (CCMs) is magnetic resonance imaging (MRI). We present an intriguing case of a 65-year-old male admitted to our hospital with tetraparesis and cognitive impairment where highly sensitive MRI sequences identified many cerebral cavernous lesions at the supra-, infratentorial and cervical–thoracic spine levels, some of them with recent signs of bleeding in a patient with oral anticoagulant therapy due to atrial fibrillation. The mechanism of cognitive impairment in this patient is most probably the interruption of strategic white matter tracts, as it is known to happen in other subcortical vascular pathologies. MRI can be helpful not only in mapping the anatomical distribution of lesions, but also in weighing the risks and making decisions regarding whether or not to continue oral anticoagulant therapy.


A persistent Müllerian duct syndrome is a rare disorder of sexual differentiation characterized by the presence of the female reproductive system in a normal male.Herein, we report a case of a 35-year-old father with persistent Müllerian duct syndrome and seminoma in the right undescended testis. The exploratory laparotomy was performed and revealed a mass in the right undescended testis and Müllerian duct structures.For patients with cryptorchidism and inguinal hernia, the persistent Müllerian duct syndrome should be considered, and radiological evaluation of the genitourinary system is recommended for early diagnosis of persistent Müllerian duct syndrome. The persistent Müllerian duct syndrome is usually detected during a surgical operation, and it is considered a risk factor for developing testicular malignancies. Persistent Müllerian duct syndrome (PMDS) is a sexual development disorder characterized by the presence of female reproductive organs in individuals with both normal chromosomes  and a normal phenotype of a male. It could be accidentally found during orchidopexy, laparotomy, or routine inguinal hernia repair in patients presenting with cryptorchidism (undescended testis).Usually, one testis is typically positioned, and the other is undescended. However, it is conceivable to be bilateral as well , 2. The A 35-year-old mute father with one child presented to the Department of General Surgery with persistent spastic abdominal pain in the right iliac fossa (RIF) treated with antispasmodic and analgesic medications. The patient has a typical male appearance with regular secondary sexual characteristics and a medical history of right inguinal hernia repair 15 years ago. Apart from the abovementioned medical history, an ultrasound (US) demonstrated a large echogenic mass located in the RIF. No clinical signs of acute appendicitis were found which excluded the diagnosis of appendicitis. Consequently, a computed tomography (CT) scan was performed and revealed a hypodense homogeneous mass between the cecum and the urinary bladder with a diameter of 8 cm compressing the latter and allowing the diffusion of the contrast product  Fig. . The finPMDS was first described in a male with an inguinal hernia in 1939 by Nilson, presenting as hernia uteri inguinal. It may be caused by an absence of AMH released from Sertoli cells of the male fetus from the seventh week of gestation, it is responsible for the regression of the Müllerian duct. When AMH is not secreted, that may lead to the persistence of female reproductive organs in the males with PMDS , 7.Two anatomic variants have been described: male and female. The male type is most common, and it is classified into two subcategories: hernia uteri inguinal and transverse testicular ectopia. The majority of cases present with unilateral cryptorchidism, and contralateral inguinal hernia . In thisPMDS patients are rarely fertile and most of them usually suffer from infertility and inguinal hernia. Causes of infertility are various as testis hypoplasia, and ejaculatory duct obstruction due to compression by MD structures. In comparison, our patient was married and had a child .The diagnosis of PMDS is often controversial, because there are no specific clinical symptoms, it is discovered incidentally either hernia repair or surgery for undescended testes. The risk of malignant transformation increases after puberty; therefore, early diagnosis should be recommended Fig.  11, 12], 1211, 1Seminoma is the most common testicular germ-cell tumor. In PMDS, the incidence of malignant transformation in the undescended testis is similar to the incidence of testicular carcinoma in patients without PMDS. Moreover, Müllerian malignancies are more probable to develop than testicular cancer in PMDS , 13.Usually, the undescended testis is detected in the inguinal channel or intra-abdominally, as in our case the right undescended testis was situated intra-abdominally and the left one was in the scrotum. When testicular malignant is suspected the appropriate surgical procedures of orchiectomy followed by removal of MD structures may be discussed .As a long-term follow-up, the palpation of the testis and radiological examinations should be mandatory for any inconsistencies in patients with PMDS . In stagOf note the resection of the Mullerian remnants is not standardized as a treatment approach due to the high risk of malignancy; however, a recent outcome analysis showed the effectiveness of performing a laparoscopic approach to treat the patients with PMDS .This publication aims to emphasize the importance of profound clinical examination that may help the diagnosis of PMDS associated with seminoma, even in males with typical appearance and secondary sexual characteristics. The correlation between these two diseases is still unclear. Further diagnostic procedures are required while dealing with an inguinal hernia and cryptorchidism patients to early detect PMDS and to prevent relevant complications.


Mucinous cystic neoplasm of the Liver is rare tumors with malignant potential that occur in the biliary epithelium. Because of its rare presentation, it is often misdiagnosed before surgery.A 63-year-old female patient presented with intermittent upper abdominal pain for three months. Laparoscopic hepatectomy of Segment 7 was conducted based on the preoperative diagnosis of space-occupying lesion in the right lobe of the liver. Postoperative pathology showed a low-grade mucinous cystic neoplasm in the right posterior lobe of the liver. The preoperative CA19-9 level was significantly increased while the postoperative CA19-9 returned to the normal range.The diagnosis of mucinous cystic neoplasm of the liver is closely related to the thickening of the cystic wall or the increase of CA19-9, which has great significance and deserves clinical attention. Mucinous cystic neoplasm (MCN) of the liver was officially proposed in the fourth edition of the World Health Organization’s (WHO’s) new classification of digestive system tumors in 2010 . MCN is A 63-year-old female patient was admitted to our hospital with intermittent epigastric abdominal pain for the past three months. Results of the physical examination on admission indicated no icteric sclera. The abdomen was soft with no palpable abdominal mass. The patient had experienced a weight loss of about five kg in the past two months and had no history of hepatitis B or C. She had been exposed to dogs and sheep and denied any history of exposure to infected cases from the epidemic area. After admission, CA19-9 was measured and was 796.20 U/mL. No serological examination for echinococcosis was performed because of the limited conditions of our hospital. Test results from pelvic ultrasound indicated menopausal uterus and uterine fibroids. Test results from gastroscopy showed chronic non atrophic gastritis. Colonoscopy revealed multiple polyps in the large intestine ; intestinal histopathology  showed severe chronic inflammation of the mucosa and adenomatous hyperplasia of the individual glands. Contrast-enhanced computed tomography of the upper abdomen , CK19 (+), and CEA (−); stromal cells ER (+), PR (+), α-inhibin , vimentin (+), desmin (+), and actin (+) Fig.  A–C. ThiMucinous tumors can occur in the ovary, pancreas, and retroperitoneum, but are rare in the liver. Therefore, close attention should be paid to cases with a preoperative imaging diagnosis that is solid, no other mass, and a significantly increased level of CA19-9 level. The preoperative CA19-9 level, in this case,was significantly increased, which may be related to cholestasis and tumor compression of the bile duct. MCN contains ovarian-like interstitial receptors expressing progesterone and estrogen, and most about 75%) liver MCNs are located in the left lobe of the liver . Compare% liver MMucinous tumors of the liver are often misdiagnosed and treated improperly, but the prognosis is good after complete resection . At presMCN is a rare but very challenging disease. Making a definitive diagnosis before the operation is often difficult. CA19-9 serum levels are elevated in the minority of cases with mucinous cystic liver neoplasms. If not diagnosed and treated properly, the tumor may recur or even metastasize; thus, preoperative diagnosis is particularly important for the treatment outcome, and attention should be paid to hepatic cystic lesions with a thickened cyst wall or elevated CA19-9 level. Preoperative needle biopsy is difficult to perform because of the lack of solid tissue in hepatic cystic lesions. Once a malignant tumor is suspected, the most appropriate treatment is complete resection performed in strict accordance with oncological criteria.


A surgical margin is the apparently healthy tissue around a tumor which has been removed. In oral cavity carcinoma, a negative margin is considered ≥ 5 mm, a close margin between 1 and 5 mm, and a positive margin ≤ 1 mm. Currently, the intraoperative surgical margin status is based on the visual inspection and tissue palpation by the surgeon and intraoperative histopathological assessment of the resection margins by frozen section analysis (FSA). FSA technique is limited and susceptible to sampling errors. Definitive information on the deep resection margins requires postoperative histopathological analysis.ex-vivo MRI we reported the jaccard index.We described a novel approach for the assessment of intraoperative surgical margins by examining a surgical specimen oriented through a 3D-printed specific patient tongue with real-time Magnetic Resonance Imaging (MRI). We reported the preliminary results of a case series of 10 patients, prospectively enrolled, with oral tongue carcinoma who underwent surgery between February 2020 and April 2021. Two radiologists with 5 and 10 years of experience, respectively, in Head and Neck radiology in consensus evaluated specimen MRI and measured the distance between the tumor and the specimen surface. We performed intraoperative bedside FSA. To compare the performance of bedside FSA and MRI in predicting definitive margin status we computed the weighted sensitivity (SE), specificity (SP), accuracy (ACC), area under the ROC curve (AUC), F1-score, Positive Predictive Value (PPV), and Negative Predictive Value (NPV). To express the concordance between FSA and ex-vivo MRI showed SE of 100%, SP of 100%, F1 of 100%, ACC of 100%, PPV of 100%, NPV of 100%, and jaccard of 100%. These results needed to be validated in a larger sample size of 21- 44 patients.Intraoperative bedside FSA showed SE of 90%, SP of 100%, F1 of 95%, ACC of 0.9%, PPV of 100%, NPV (not a number), and jaccard of 90%, and ex-vivo MRI of the fresh surgical specimen.The presented method allows a more accurate evaluation of surgical margin status, and the first clinical experiences underline the high potential of integrating FSA with  A surgical margin is the apparently healthy tissue around a tumor that has been surgically removed. Most commonly, in oral cavity carcinoma, a margin larger than or equal to 5 mm is considered as “negative”, a margin between 1 and 5 mm as “close”, and a margin less than 1 mm as “positive” . RadicalCurrently, the intraoperative surgical margin status is based on the visual inspection and tissue palpation by the surgeon during surgery and intraoperative histopathological assessment of the resection margins by frozen section analysis (FSA). FSA technique is limited and susceptible to sampling errors , 5. DefiThe margin revision of initially positive margins to ‘‘clear’’ based on FSA guidance does not equate to an initially negative margin and does not significantly improve local control. Prospective studies should determine what system of resected specimen analysis best predicts completeness of resection .Real-time Magnetic Resonance Imaging (MRI) on surgical specimen has been used in a few previous studies , 8. In pWhen studying correlations between imaging and histological data, the different spatial resolution of the two methods can increase bias, and the evaluation of the piece of the organ as opposed to the whole organ without orientation references could be challenging for the pathologist. The introduction of a 3D-printed anatomic model of the tongue of the patient, obtained from staging MRI for surgical specimen orientation, reproducing the anatomic context from which the specimen has been excised could improve surgeon, pathologist, and radiologist communication in the assessment of margins.The employment of MRI to examine the surgical specimen oriented through the 3D model could allow for a better macroscopic radial margin evaluation and measurement of the distance to all margins, avoiding sampling errors of FSA.The purpose of this paper was to report the preliminary results of the diagnostic accuracy of FSA and MRI in evaluating intraoperative surgical margins in oral tongue carcinoma, by examining the surgical specimen oriented through the 3D-printed specific patient tongue model.We described the steps of the process in a sequential workflow, diagrammed in a flow chart Figure 1http://www.itksnap.org/pmwiki/pmwiki.php). The tongue tumor and tongue parenchyma were segmented as separate anatomical regions of interest (ROIs). For all ROIs, both threshold and manual editing were performed to ensure that only the anatomy of interest would be selected. Each ROI was converted to a separate 3D object and combined into a 3D virtual model. The segmentation data, in DICOM format, were converted to STL format so that the 3D printer could recognize them. Surgeons and radiologists segmented tongue tumor, and a 3D virtual model (We obtained a 3D-printed model of the patient’s tongue on which the area which should have been resected has been reproduced. We implemented the model by the 3D post-contrast fat suppressed gradient-echo T1weighted sequence (VIBE) of staging MRI examinations on a 1.5T MRI System  using a phased array surface coil. 3D post-contrast anonymized images demonstrating a good contrast between the tumor and the tongue parenchyma were selected and transferred into ITK-SNAP, a software application used to segment structures in 3D medical images  between February 2020 and April 2021. We prospectively included all the cases with an intraoperative margin status evaluation by FSA and Ex-vivo MRI examination was 22.7 minutes (range 16-40 minutes).The mean time duration of For the qualitative analysis, two radiologists , in consensus, evaluated the image quality of the acquired MRI series, enabling visualization of even small structures.They, blinded to histological results, radially measured the distance (in millimeters) from all the margins to the tumor Figure 5To differentiate tumor from edema they calculated ADC values within ROI drawn on the tumor and surrounding tissue.<1 mm.They considered a margin clear if > 5mm, close if =1–5 mm, and involved if The patients were stratified into three groups depending on the analysis of their margins. The three groups were negative, close, and involved.A dedicated head and neck pathologist with more than of ten years of experience (BF) without knowledge of the MRI results annotated tumor location and measured the distance from the tumor to the margins, which was considered the gold standard.ex-vivo MRI of specimen oriented on the 3D-printed tongue model , specificity(SP), accuracy(ACC), area under the ROC curve (AUC), F1-score, Positive Predictive Value (PPV), and Negative Predictive Value (NPV), where the weights are proportional to the cardinality of each class.To compare the performance of the initial bedside FSA and ex-vivo MRI, we reported the jaccard index, computed as the number of equal classifications, divided by the number of total samples. Final pathological diagnosis was the gold standard.In addition to this, to express the concordance between FSA and In a study published in the literature , 640 conex-vivo MRI, margin status was negative in nine cases and positive in one case (revision was not performed in this case because FSA was negative). The final pathological T status was T1 in one case, T2 in seven cases, and T3 in two cases. In the 10 cases, the mean maximum diameter of the tumor at the final histological diagnosis was 22.3 (range 10-37 mm). The average time taken for each MRI examination was 24.7 minutes (range 16-38 minutes). The T2 series of MRI was therefore the series that was matched to histology and was subsequently annotated.We enrolled 10 patients  with a histological diagnosis of squamous cell carcinoma of the oral cavity (OSCC) who underwent surgery  for oral tongue squamous carcinoma between February and April 2021. The definitive margin status was negative in nine cases and positive in one case. In MRI succeeded in margin prediction in all the cases, and FSA failed in one case.ex-vivo MRI showed SE of 100%, SP of 100%, F1 of 100%, ACC of 100%, PPV of 100%, NPV of 100%, and jaccard of 100%.In this group of patients initial bedside FSA showed SE of 90%, SP of 100%, F1 of 95%, ACC of 90%, PPV of 100%, NPV (not a number), and jaccard of 90%, and These results needed to be validated in a larger sample size of 21- 44 patients.Intraoperative assessment of the resection margins can provide valuable information, enabling additive resection to obtain negative margin status. Despite this, the clinical value and the method of assessing the intraoperative margin are not well defined , 9, 10. Several techniques aiming for intraoperative assessment of surgical margins in oral cavity/tongue squamous cell carcinoma have been investigated. These include elastic scattering spectroscopy  fluorescex- vivo MRI, and to improve surgeon and pathologist communication by introducing a 3D-printed tongue model to allow pathologists to understand specimen orientation and to learn what margins will be revised based on gross impression.With our study, we have found it helpful to report and demonstrate to our surgical team the gross distance to all margins by using the intraoperative To our knowledge this is the first study in which a surgeon provided orientation of the specimen on the 3D-printed model of the tongue with the reproduced bed of resection. Generally, the surgeon provides orientation by designating one or two points on the specimen.The specimen orientation on the 3D-printed model from patient MRI facilitates review and correlation.By maintaining the orientation of the specimen, the pathologist is facilitated in noting the distance of the tumor from each margin and in communicating the site of positive and close margins to the submitting surgeon.ex-vivo MRI to outcome the limit of the lack of a true measurement of the distance of the invasive tumor from the resection margin. According to FSA, a margin can only be determined as positive or negative and the margin presented separately can be thin <5 mm in thickness. Additionally, without a defined margin orientation it is not clear how the separately submitted margins reflect the true areas of the en block specimen that are noted to be close or positive. In our preliminary experience, ex vivo MRI was more accurate than intraoperative FSA in predicting margin status.While most surgeons sample margins only from the surgical bed without margin assessment from the resection specimen, as demonstrated by a survey of American Head and Neck Society members , we intrex-vivo MRI assessment of the pathologic en bloc specimen could direct intra-operative defect-derived FSA margin assessment and margin revision, determining the closest margins on gross assessment and requiring separately submitted tissues of correct size to have negative margins. The orientation of the specimen on the 3D model could allow a better match between the separately submitted margins and the true areas of the en bloc specimen that are noted to be close or positive. This could be because the revised margin is not taken from the correct location  can be used to evaluate the rate of microscopic water diffusion within tissues. DWI may be measured by means of apparent diffusion coefficient (ADC). Areas of decreased ADC values within tumors could be used as a powerful imaging biomarker of cancer (The limitation of f cancer , 25.ex-vivo MRI of the specimen after formalin fixation. We performed MRI after formalin fixation in one case but the changes of the signal intensity of the specimen did not allow us to evaluate the effect of shrinkage.Tumor shrinkage after formalin fixation could lead to an underestimation of tumor margins . This coin vivo applications of MRI but not in ex-vivo MRI of tongue resection specimens, and the fact that only one pathologist evaluated histopathology.Other limitations were the inexperience of the MRI readers, who had experience with Ex-vivo MRI is a costly and time consuming process. It requires the use of MRI equipment, subtracting time for performing diagnostic tests. It also requires excellent communication and coordination between surgeons, operating room staff, pathologists, and radiologists to avoid additional operating time. Considering that the operating times for these types of complex resections and reconstructions, with the maximum operating time of 160 minutes in which flap reconstruction was not required and 700 minutes of microvascular reconstruction, MRI does not unreasonably lengthen the time in the operating room (ing room . This inex-vivo MRI of the fresh specimen for evaluation on macroscopic proximity of the tumor to the margins of resection, this intervention can be performed with some planning and coordination between the radiologists, pathologists, and surgeons in a selected subset of patients most likely to benefit by avoiding adjuvant radiotherapy. Finally, we would recommend consideration of intraoperative MRI tumor margin assessment for selected cases potentially be cured with surgery alone, i.e. T1/T2N0 tumor.In conclusion, considering the staffing and expensive nature of integrating FSA with The raw data supporting the conclusions of this article will be made available by the authors by contacting the corresponding author.The study was reviewed and approved by the Humanitas Research Hospital, Rozzano. The patients/participants provided their written informed consent to participate in this study.CG, LB, GS, and GM conceived of the presented idea. CG developed the theory and performed the computations. CG, GM, LB, and FF verified the analytical methods. GS encouraged CG to investigate margin status and supervised the findings of this work. All authors discussed the results and contributed to the final manuscript. CG, GM, LD, FC, MC, FG, RB, and BF carried out the experiment. CG wrote the manuscript with support from GM. RB and PO fabricated the 3D model sample. AD, FR, AE, FF, and LT helped supervise the project. CG and EC developed the theoretical formalism, performed the analytic calculations and performed the numerical simulations and contributed to the interpretation of the results. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


An open abdomen with frozen adherent bowels is classified as grade 4 in Björck’s open abdomen classification, and skin grafting after wound granulation is a typical closure option. We achieved delayed primary fascia closure for a patient who developed open abdomen with enteroatmospheric fistulas due to severe adherent small bowel obstruction. We present here the details of his management.A 52-year-old man suffered acute abdominal pain during a flight and received an emergency laparotomy due to adhesive small bowel obstruction. Repeated laparotomies were required, and later open abdomen and proximal site jejunostomy were selected. After negative pressure wound therapy, he was transferred to our institution. Two enteroatmospheric fistulas emerged on the exposed intestine, and we diagnosed the condition as a Björck grade 4 open abdomen. After 8 months of wound care and parenteral nutrition, we decided to attempt primary wound closure because the patient required permanent oral restriction and total parenteral nutrition due to short bowel syndrome. A circular incision along the circumference of the exposed bowel allowed us to take a safe approach into the abdominal cavity. We removed the intestinal adhesions completely and resected the bowels, including the fistulas and anastomosed parts. Finally, the abdominal wall defect was reconstructed using the component separation technique, and the patient was discharged without an ostomy.Primary fascia closure for grade 4 open abdomen is hard, but leaving a long interval before radical surgery and applying pertinent wound management may help solve this adverse situation. The open abdomen (OA) is a life-saving surgical procedure for abdominal rigidity due to severe abdominal trauma, sepsis, or abdominal compartment syndrome (ACS), which involves exposing the intra-abdominal organs to the external environment . The manThe severity of OA is classified according to the presence of enteral effluent contamination, the degree of adhesion, and enteroatmospheric fistula (EAF) formation . The mosWe report a case of Björck grade 4 OA after repeated laparotomy for adhesive small bowel obstruction (SBO), which was managed with complete adhesiolysis. Appropriate wound management with negative pressure wound therapy (NPWT), a sufficient resting period to let the peritonitis subside, and nutritional support made it possible to perform a successful elective primary fascia closure.A 52-year-old man suffered acute abdominal pain while on a flight to the European continent. Upon landing, he was transferred to a regional hospital, where an emergency laparotomy was performed. He had undergone open laparotomy twice before for a complicated appendectomy and recurrent ileus, after which he was diagnosed with adhesive SBO. The first operation revealed several serious bowel adhesions, which were excised. Early recurrent ileus occurred a few days after the first surgery, and more than 10 repeated laparotomies were required. According to the hospital report where he was treated previously, the surgeon decided to construct a terminal jejunostomy due to ubiquitous, unsolvable, strong adhesions in the sixth operation. Finally, OA was selected as a solution. Considering his situation, the patient chose to return to his home country, so he was transferred to our institution.NPWT was introduced for OA on admission to our hospital, and terminal jejunostomy was performed in the right upper abdominal quadrant Fig. A. GranulAs a surgical intervention was impossible considering his clinical status, we decided to perform continuous NPWT management and total parenteral nutritional (TPN). After 3 weeks of NPWT, the granulating tissue had gradually protruded to cover the crevice between the intestine and the abdominal wall, but the EAFs did not close Fig. A. The inWe planned a two-team surgical approach, with a gastrointestinal surgeon and a plastic surgeon for radical surgery of the severely adhesive bowels and the large defect of the abdominal wall. A circular surgical incision along the abdominal wound circumference was made to avoid injury to the frozen adhesive intestine Fig. A. After This report demonstrates that primary wound closure with complete adhesiolysis can be successful in a patient with severe adhesive Björck grade 4 OA with EAF after over 10 reoperative surgeries for SBO. We emphasize that the following two key management points made it possible to solve this adverse situation: pertinent wound management, including NPWT, and a long interval prior to elective surgery (> 6 months).Adhesive SBO is not usually considered an indication for OA management. The World Society for Emergency Surgery (WSES) guidelines hardly recommend OA application for severe peritonitis or extensive visceral edema due to a concern about possible ACS . In our Managing a patient with OA is a great challenge, and the best way to manage a case remains unclear. A classification system was proposed by Björck et al. to help clarify the challenges related to OA . OA gradThe most severe, grade 4, OA is defined as adherent/fixed bowel unable to be closed surgically, with or without fistula, and the key feature of its management is prevention . HoweverA few cases of primary fascia closure for Björck grade 4 OA have been reported previously. Yetisir et al. wrote up a case series on the operative management of Björck grade 4 OA . Eğin etThe key surgical management points are delayed primary closure with an adequate interval to ensure the eradication of abdominal sepsis and a lateral approach, which means a circumferential incision around the granulated tissues 8]. We s. We s8].In conclusion, Björck grade 4 OA is a severe surgical complication, and prevention is essential for reducing the related morbidity. However, if a patient suffers such an adverse event, appropriate wound care and delayed surgery may contribute to the patient’s satisfactory recovery.