Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype (pHGG) is a rare and aggressive brain tumor characterized by a specific DNA methylation profile. It was recently introduced in the 5th World Health Organization classification of central nervous system tumors of 2021. Clinical data on this tumor is scarce. This is a case series, which presents the first clinical experience with this entity. We compiled a retrospective case series on pHGG patients treated between 2015 and 2022 at our institution. Data collected include patients’ clinical course, surgical procedure, histopathology, genome-wide DNA methylation analysis, imaging and adjuvant therapy. Eight pHGG were identified, ranging in age from 8 to 71 years. On MRI tumors presented with an unspecific intensity profile, T1w hypo- to isointense and T2w hyperintense, with inhomogeneous contrast enhancement, often with rim enhancement. Three patients died of the disease, with overall survival of 19, 28 and 30 months. Four patients were alive at the time of the last follow-up, 4, 5, 6 and 79 months after the initial surgery. One patient was lost to follow-up. Findings indicate that pHGG prevalence might be underestimated in the elderly population. Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype (pHGG) is a new brain tumor entity defined by a specific DNA methylation profile. It has recently been introduced in the WHO classification of central nervous system (CNS) tumors of 2021 [PHGG is considered a highly malignant brain tumor (WHO grade 4), mostly occurring in children and adolescents. Histologically it is characterized as a diffusely infiltrating glioma with mitotic activity, showing no mutations in IDH1, IDH2 or H3-genes. Furthermore, the tumor either presents a methylation profile aligned with subgroups pHGG RTK1, pHGG RTK2 or pHGG MYCN or it exhibits molecular features as PDGFRA alteration, EGFR alteration or MYCN amplification [Prognosis has been reported as unfavorable with a median overall survival (OS) of 17 months . SubtypeWe compiled a retrospective series of cases diagnosed between 2015 and 2022 at Charité-Universitätsmedizin Berlin. All patients classified as pHGG by DNA methylation-based brain tumor classification analysis were included . Except We identified eight patients diagnosed with pHGG between 2015 and 2022. In the same period, a total number of 1.339 patients with grade 4 glioma were diagnosed at our institution.Table Table n = 650).Figure Figure Figure Table 2 b.i.d.) and tumor-treating fields (TTF). Six months later, in September 2017, MRI again showed multifocal tumor progression. The patient wished to continue metronomic temozolomide and TTF. Two months later his general condition deteriorated. He was taken to the intensive care unit due to a septic shock caused by an erysipelas of the right lower leg. The patient died in a hospice, 28 months after the initial diagnosis.In October 2015, a 51-year-old male presented with right-sided hemiparesis. Cerebral MRI showed a left central necrotic lesion with perifocal edema see Fig. A, B. NeuIn February 2016 an 8-year-old girl was externally diagnosed based on histomorphology with a disseminated leptomeningeal oligodendroglial tumor after a tonic–clonic seizure. The patient received carboplatin- and vincristine-based chemotherapy. Almost one year later, in January 2017, she experienced a progressive gait disorder and paresthesia in the right hand. MRI demonstrated local tumor progression as well as intramedullary spinal metastases see Fig. C, D. Neu2/day, 21-days on/7-days off) and valproic acid (30 mg/kg/day) were administered from March 2017 to February 2018, when imaging again showed intracranial tumor progression. Chemotherapy was changed to temozolomide  and radiotherapy was administered . In June 2018, the girl presented with progredient hydrocephalus, fever, and neutropenia. Treatment included shunt revision and antibiotics. The patient seemed gradually more apathetic, even though imaging showed no signs of progression. Soon temozolomide had to be paused due to myelotoxicity. The patient was alive at last follow-up, 79 months after the initial diagnosis.Postoperatively, the patient suffered from high intracranial pressure and the insertion of an external ventricle drain was performed. After prolonged convalescence, the girl was able to return to school but reported mild difficulties with writing and concentration. Dose-dense temozolomide -positron emission tomography (FET-PET)-MRI showed an area of increased tracer uptake in the resection cavity. We agreed on the observation of the suspicious lesion and recommended the continuation of temozolomide. Four months later, in April 2021, the patient underwent re-resection of multifocal tumor relapse. Neuropathological analysis confirmed the diagnosis of glioblastoma, IDH-wildtype, MGMT-promoter not methylated. Because of a nuclear loss of PMS2 staining there was the suspicion of a diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype [2 b.i.d.) was initiated.In January 2021, a 22-year-old female patient presented herself at our institution. After a diagnosis of a right-parietal glioblastoma, IDH-wildtype in July 2020 see Fig. G, H, sheFour months later, the patient presented with a right hypoglossal nerve palsy. FET-PET-MRI revealed a multifocal tumor progression. Re-resection was performed in October 2021, which was well tolerated and adjuvant regorafenib was initiated . After t2 b.i.d.) was prescribed after radiotherapy. Five months later, in November 2019, the patient was admitted to our emergency department with left-sided hemiparesis, fever, elevated infection parameters and general malaise. MRI revealed massive tumor progression. Antibiotic treatment of the infection led to rapid reconstitution. The patient was released into outpatient care and died in February 2020, shortly after the initiation of bevacizumab treatment, 30 months after the initial diagnosis.In August 2017, a 71-year-old male patient presented with sudden onset motoric dysfunctions and disorientation. MRI showed a large right temporoparietal hemorrhagic, cystic lesion see Fig. I, J. TheIn February 2022, a 29-year-old female patient was admitted with new onset of high frequent seizures. MRI showed a left insular lesion, interpreted as focal cortical dysplasia see Fig. K, L. FETAfter initiation of the cyclic temozolomide treatment in July 2022, the patients' overall condition started to deteriorate when she developed pancytopenia and a severe SARS-CoV2-infection. Seizure medication had to be escalated and temozolomide had to be paused after the second cycle due to severe nausea, vertigo and headaches. MRI from November 2022, however, showed no tumor progression. The patient was alive at last follow-up, 6 months after surgery.In August 2019, a 15-year-old female patient was referred to our neurosurgery department due to tumor relapse of a right fronto-temporal lesion see Fig. M, N whicA 52-year-old female patient was admitted to our hospital with the chief complaint of severe headache. MRI demonstrated a T1 hypointense and FLAIR hyperintense bithalamic lesion (right > left) with no contrast-enhancement see Fig. O, P. In In 2015, Korshunov et al. presented a molecular analysis of 202 paediatric glioblastomas to investigate the prognostic significance of genomic and epigenetic alterations. The authors found a subset of 59 H3/IDH-wildtype pHGG patients with a median age of 12 years range 1–18 years) [ years [8Two years later, in 2017 the authors identified three distinct molecular subtypes, which differed in genomic and epigenetic profiles as well as in outcome. Although all analyzed tumors showed a dismal prognosis, the authors pointed out significant differences in survival among subtypes. So-called pHGG RTK2 tumors displayed the most favorable median OS of 44 months, followed by pHGG RTK1 with an intermediate median OS of 21 months and pHGG MYCN with the shortest median OS of 14 months .In the same year Mackay and colleagues conducted a molecular meta-analysis of 1067 cases of pHGG and diffuse intrinsic pontine glioma. The median age of the population was 9.8 years, 982 were 21 years old or younger. They found that H3/IDH1-wildtype tumors had a 2-year survival of 23.5% and a median OS of 17.2 months. The authors recommended further investigation of the heterogenous subgroups [Our patient group was significantly older than the published cases thus far and our oldest patient was diagnosed at age 71. As already mentioned in the WHO pHGG article, the prevalence in adults and elder patients might be underestimated [Information on pHGGs radiological appearance is scarce. The WHO describes pHGGs MRI characteristics resembling other high-grade gliomas, showing contrast-enhancing tumors with mass effect . So far,The cases in our series seemed to be universal in appearance, showing hypo- to iso-intensity in T1w, hyperintensity in T2w images and differing contrast agent enhancement behavior. In conjunction with the small case number, it is difficult to work out general MR imaging patterns; MR morphological findings are rather unspecific and not helpful in confirming or refuting a diagnosis.Patients with suspected pHGG should receive a standard CNS tumor assessment protocol including T2w and T1w sequences e.g., TSE or MPRAGE, FLAIR or TIRM, DWI, and postcontrast T1w sequences e.g., TSE or MPRAGE. Additional PET might improve the specificity of diagnosis in cases where no certain tumor signs are seen on MR.In 2020, Varlet et al. conducted a histopathological data analysis of 178 high-grade glioma patients (aged 3–18 years) and found that WHO grade 3 versus 4, including endothelial proliferation and necrosis, had no prognostic value in pHGG. However, a high Ki-67 index (≥ 20%) and midline location were significantly associated with dismal outcome .As brain tumors are increasingly defined by their molecular profile, future subtype classification and evaluation of proliferation markers of pHGG might help to further refine treatment and determine prognosis.In our study group, three patients died from their disease, with an overall survival of 19, 28 and 30 months after initial diagnosis. Four patients were alive at last follow-up, 4, 5, 6 and 79 months after surgery. One patient was lost to follow-up. PFS was 9, 12, 15 and 22 months. Until last follow-up, three patients remained without radiological tumor progress 4, 5 and 6 months after initial surgery.Our data of eight diffuse paediatric-type high-grade glioma cases suggest that this entity is not confined to children and adolescents. Imaging showed no specific features. The diagnostic pathway is constrained to DNA methylation profiling. By reporting detailed procedural information, we might contribute to clinical decision-making until larger case series are published.


Usually, fatty hepatic infiltration is diffuse and homogeneous. However, in some cases, it can be localized simulating benign or malignant tumors. We present a case of a 61-year-old female patient with family history of malignancy: sister with lung cancer, an other sister with colon cancer and a mother with breast cancer; who presented with multiple hepatic nodules at the ultrasonography images. CT scan and MRI were not sufficient to pose a certain diagnosis which was later confirmed by liver biopsy. In some cases, fatty hepatic infiltration may simulate benign or malignant tumors. We present a case of a 61-year-old female patient with family history of malignancy who presented with multiple hepatic nodules at the ultrasonography images. CT scan and MRI were not sufficient to pose a certain diagnosis which was later confirmed by liver biopsy. Liver steatosis is a well defined entity characterized by abnormal accumulation of fat in the liver tissue. Its prevalence is increasing affecting currently 25–30% of the general population . Usually2. The abdominal exam was normal. Laboratory tests showed normocytic anemia: hemoglobin at 8.9 g/dl, thrombocytopenia at 135,000 cell/mm3. The hepatic tests were normal. Abdominal ultrasonography revealed a 17 cm hepatomegaly with multiple nodular hyperechoic lesions that looked unvascular at the doppler ultrasound. A CT scan showed multiple centimetric liver lesions hypoattenuating compared with the adjacent liver without any enhancement suggesting metastatic lesions  and ACR-3 (probably benign) lesions according to the American College Of Radiology and a multinodular goiter respectively. Fine needle aspiration of thyroid nodule was negative. Giving the negativity of all exams, liver biopsy was mandatory. It was practiced by an experimented radiologist using a fine needle and under scannographic control. It revealed heterogeneous fat deposits with mild inflammatory signs and ruled out malignancy .2. The gold standard for diagnosis is liver biopsy. However, since biopsy is an invasive procedure, other criteria have been established to lead diagnosis based on ultrasonography, CT scan and MRI [BRCA1 and BRCA2 mutations [Fatty liver is a well recognized disease defined by macrovesicular fat infiltration affecting more than 5% of the hepatocytes ,4. Its p and MRI ,8. Fatty and MRI ,10. The  and MRI ,11. Mult and MRI . As well and MRI . In the utations . Thus, iThe present case report shows that multifocal hepatic steatosis may simulate neoplastic disease, in particular in patients with underlying history of malignancy. In most of the cases, imaging tools such as CT scan and MRI are sufficient to make the diagnosis. Seldom, we are obliged to practice liver biopsy for a certain diagnosis.Fatty liver affects usually the liver in a homogeneous way (68%).Computed tomography and MRI are some times unable to distinguish between fatty lesion and hepatic tumor.Liver biopsy remains the gold standard for certain diagnosis of hepatic nodular lesions.


Venous malformations affect around 2 per 10,000 newborn and are the most common type of congenital vascular malformation. They are always present at birth and are often misdiagnosed with hemangiomas. Accurate diagnosis and adequate targeted therapy through a multidisciplinary approach is advocated for a successful treatment, considering a combination of modalities in complex cases. We present here the workup and treatment of a venous malformation in a 14 month old child by combining a preoperative sclerotherapy with sodium tetradecyl sulfate (STS) followed by complete surgical excision respecting the calvarium.Case presentation.A large right extracranial fronto-parietal venous malformation and scalloping of the underlying calvarium, attached to the pericranium, surgically excised after preoperative sclerotherapy with STS in a 14-month-old child.The patient had an uneventful postoperative course with complete resection of the lesion, satisfying cosmetic appearance and no complications. Histopathological examination confirmed a venous malformation.We demonstrated the workup and the rationale of the combined sclerotherapy followed by surgical resection of a large growing extracranial venous malformation. Preoperative direct contrast injection and sclerotherapy allowed to rule out significative extracranial to intracranial venous inflow and reduce blood loss for the surgical procedure, respectively. Complete removal of the malformation minimized the impact on future growth of the calvarium. Venous malformations (VMs) have been described by the Mulliken and Glowacki classification of vascular anomalies in 1982 [The revised version of 2018 continues to divide vascular anomalies into vascular tumors and vascular malformations and classifies vascular malformations in simple or combined  rather than high or low flow. Associated syndromes  and specific mutations (TIE2 mutation) have been also included in the classification.VMs are always present at birth by definition, thus have to be considered congenital lesions, and grow in parallel with the child's development , 4, withClinically, these lesions are superficial, soft, without pulsations, thrill, or increase of temperature relative to adjacent skin, in contrast to arteriovenous malformations. The size of the VM can increase with activity, Valsalva, or supine position , 7, 8. CVMs can sometimes be painful, especially in cases of facial VMs, if the temporal muscle is affected (patients can complain of migraine). Patients could also present with swelling and pain due to thrombosis of a portion of the lesion .Diagnostic workup should start first with ultrasonography (US) to identify the classic hypoechoic, heterogeneous, and compressible appearance of VMs. Duplex is then used to show the low flow and identify the feeding vessels and intralesional thrombosis. Duplex ultrasound is also the best modality to differentiate hemangioma (high flow on duplex) from VMs (low flow). This discrimination is important because in case of misdiagnosis, inappropriate beta-blocker therapy may be proposed . MRI T1-Treatment options include medications, surgery, sclerotherapy, cryoablation, and laser photocoagulation; the decision for treatment should be ideally done after interdisciplinary discussion. Small well-localized VMs are often treated successfully with a single modality , whereas larger venous malformations may require a combination of technique –5, 9.Compressive therapy is a simple and efficient option mostly for VMs of the extremities to reduce pain and thrombosis; targeted medical treatment consists of low-dose aspirin, anti-inflammatory drugs, or low-molecular-weight heparin in cases of pain associated with thrombophlebitis , 13.The modality of choice for the treatment of VMs is considered to be sclerotherapy, which can be curative, or used prior to surgery as a neo adjuvant solution, mostly to induce thrombosis inside the lesion to facilitate excision . Many diSurgery alone is sometimes preferred to sclerotherapy, especially when the venous malformation is small and does not involve vital structures; the use of sclerotherapy as an neoadjuvant therapy is often advocated mostly to reduce intraoperative blood loss, to decrease the volume of the malformation to be resected, and to improve visualization of vital structures.We present here the case of a 14-month-old child, without any relevant past medical history, with a large right fronto-parietal soft tissue mass, more prominent in the supine position, and which gradually increased in size with the child growth. Final size was estimated around 7 cm in maximal diameter. The perinatal history was unremarkable, the delivery uneventful, and no previous trauma was recorded. The child’s weight at the time of presentation at our clinic was around 11 kg. The child did not show any particular complaint in relation to this lesion.Clinical examination showed a soft lesion, not mobile to deep structures of the scalp. Recumbent position showed an enlargement of the lesion. The skin was intact, and the rest of the inspection and palpation of the cranial vault were normal  revealed large vascular spaces, with minimal intracranial extension abutting the dural surface through bony lacunae. No extra to intracranial shunts were demonstrated on TOF sequences. Heterogeneous signal, sharp boundaries, and serpiginous content with a low flow were highly suggestive of a vascular lesion with venous characteristics.After a multidisciplinary meeting, including neurosurgeons, interventional neuroradiologists, dermatologists, angiologists, and pediatric surgeons, surgery was offered to parents because of the significant cosmetic discomfort, the tendency of the mass to grow, and the scalloping of the calvarium.We decided to perform the procedure in a hybrid operating room, with preoperative direct contrast injection and sodium tetradecyl sulfate (STS) sclerotherapy. The aim of the direct contrast injection followed by sclerotherapy was to rule out any extra to intracranial venous shunt and to reduce blood loss respectively, prior to surgery.The patient is in supine position; antibiotic prophylaxis with intravenous cefazolin 25 mg/kg is given prior to the endovascular procedure.The angiographic procedure began with a direct injection of contrast (30 mL of Iopamiro 300 mg/mL) into the lesion  and one small frontal cortical vein. After ruling out any extra to intracranial venous shunt, sclerotherapy was slowly performed via direct puncture of the lesion using 4 21-gauge butterfly needles in the different compartments. We injected 4 mL of sodium tetradecyl sulfate foam (Trombovar® 1%) mixed with contrast and air (in proportion of 1:1:1). No diffusion to the previously described veins was seen under fluoroscopy during the procedure , and Evicel® (Ethicon™) were used for hemostasis. The deep plane and subcutaneous plane were closed with Vicryl® 3.0. An intradermal suture of Monocryl® 3.0 was used to close the skin and a pressure dressing was applied for 3 days  1. Immediate postoperative follow-up was unremarkable and patient was discharged at POD 4.To our knowledge, this is the first description of the successful treatment of a cranial venous malformation threatening calvarium development in a pediatric case by combining sclerotherapy to surgical excision. Pre-operative sclerotherapy allowed easier manipulation, decreased the per operative blood loss, and shortened surgical procedure and therefore the length of general anesthesia. Finally, by a direct injection of contrast into the malformation at the beginning of the procedure, we were able to rule out any significative extra to intracranial venous shunt.Management of venous malformations requires a multidisciplinary approach from the diagnostic workup to the treatment. It should involve interventional radiologists or neuroradiologists, surgeons, dermatologists, and angiologist in order to maximize the success rate while avoiding iatrogenic complications.


Dentigerous cysts are rarely reported in young children. They are usually asymptomatic and only identified when becoming significantly large. Treatment by enucleation may damage structures like the inferior alveolar nerve, maxillary sinus, or permanent teeth, thus reducing the child's quality of life. Therefore, conservative surgical treatment such as decompression is indicated. This case report describes the treatment and subsequent complete regression of an inflammatory dentigerous cyst based on the decompression method using a customized surgical tube in a 10-year-old girl. The innervation was preserved, and permanent teeth erupted. The dentigerous cyst (DC), also called a follicular cyst, is odontogenic in nature and includes the crown of an unerupted or impacted tooth.Their origin can be developmental or inflammatory, but their exact etiology remains unclear. An inflammatory dentigerous cyst (IDC) appears around an unerupted permanent tooth due to inflammation spreading from an overlying nonvital primary tooth.Smaller DCs are generally asymptomatic and accidentally discovered, for instance, during a routine radiographic examination. Larger cysts may cause expansion of the bone resulting in facial asymmetry, root resorption, and shifting of adjacent teeth.A follicular cyst radiographically appears as a well-defined unilocular radiolucency surrounding the crown of an unerupted tooth. Inflammatory types usually involve the roots of a nonvital primary tooth and the crown of an unerupted permanent successor that can be displaced.A correct diagnosis requires histopathological analysis because unicystic ameloblastoma and odontogenic keratocysts exhibit similar radiographic features.The DC is treated using enucleation, marsupialization/decompression, or a combination of the two procedures. Enucleation should be done for any cyst that can be safely removed without sacrificing adjacent structures.A 10-year-old girl was referred to the Department of Maxillofacial and Oral Surgery for painless swelling on the right side of the mandible. Intraoral examination revealed a normal-looking mucosa with a thin expansion of the buccal cortical, exhibiting bone elasticity on palpation in the primary mandibular right first molar region. The patient denied any sensory deficit. There was no account of specific systematic diseases or previous traumatic injuries in the affected area.A panoramic radiograph and a cone-beam computed tomography (CBCT) brought in by the mother showed significant unicystic radiolucency, with well-defined margins expanding from the primary mandibular right second molar to the permanent central incisor on the same side. The nonerupted permanent canine was horizontally shifted, and the first premolar mesially inclined, while the second premolar seemed to be typically positioned. The roots of the central and lateral right incisors were tilted, and the inferior alveolar nerve was in contact with the lesion. The primary first molar was significantly damaged by caries and nonvital. The root of the primary canine was resorbed and2.Based on these clinical and radiological findings, a provisional diagnosis of an IDC caused by the primary mandibular right first molar was made.The primary mandibular right canine, including the first and second molars, were extracted under general anesthesia due to the patient's age and fear of the procedure. First, an incisional biopsy for the histopathological examination was performed. Then, a decompression device made from a prefabricated surgical T drainage tube  was used. It was cut precisely to the desired length and width from measurements on a preoperative CBCT, and its vertical end was positioned inside the cystic lumen. Next, the horizontal part (wings) was drilled on both sides, providing an easier fixation on the mucosa. The device was inserted into the extraction socket of the primary first molar and secured with 4–0 nylon sutures .The patient's parents were instructed to irrigate the cyst cavity using 10 mL syringes filled with 0.9% saline solution by inserting the plastic part of the cannula into the tube entrance three times a day. Postoperative follow-up appointments were scheduled to take place every 3 months.A histopathological examination of the lesion confirmed the clinical diagnosis of the IDC .Three months later, the postoperative radiograph showed a more vertically positioned canine with reduced radiolucency . The deA year after the decompression had been done, all permanent teeth involved in the eruption process maintained vitality. The complete regression of the lesion with bone formation was radiographically observed , and thEven though the histopathology of the follicular cyst remains unclear, its connection to inflammation caused by the nonvital primary tooth is obvious. A study involving a histological evaluation of cysts occurring in the mixed dentition stage detected an inflammatory process caused by a primary tooth in 93.6% of the observed follicular cysts.Several authors have shown that decompression is an effective treatment for odontogenic cysts.Reducing intraluminal pressure and facilitating bone formation requires keeping the cyst open. This is done using various devices, such as a simple iodoform gauze, stents, brackets and chains attached to impacted teeth, or using removable partial dentures that act like obturators.Full eruption of the involved permanent teeth and healing of the cystic cavity in our patient occurred after 12 months, which is somewhat longer than Allon et al reported, where the estimated mean decompression period is 7.5 months in children under 18 years of age.Previous case reports, as well as ours, show that the permanent successors, even when badly dislocated, erupt into the dental arch.Not many published studies report DCs treated using decompression in children, which may be due to the low incidence of DCs in that population. Therefore, further studies of this kind should be conducted.IDCs can be treated successfully with minimal intervention using a conservative method like decompression. By extracting the infected primary teeth and ensuring continuous drainage utilizing a device like ours, essential structures can be protected and spontaneous eruption of the permanent teeth achieved, thus reducing the need for prosthetic rehabilitation. The patient should be scheduled for regular follow-ups until the healing process has been completed.


A bladder diverticulum (BD) is an abnormal pouch protruding from the bladder wall, which can be congenital or acquired. Acquired diverticula are more common, usually secondary to outflow obstruction or neurogenic bladder. Spontaneous rupture of a BD is rare, particularly in women. This report is about a female patient who develops abdominal pain and dysuria, progressing to an acute abdomen. The diagnosis of spontaneous rupture of a BD was suspected in pelvic ultrasound and confirmed in computed tomography (CT) cystography. The patient was submitted to diverticulectomy and bladder wall reconstruction. Although rare, this entity should be considered in patients with acute abdomen with unclear etiology, even in women with no evident risk factors for a BD or its rupture. A bladder diverticulum (BD) is a mucosal protrusion through the detrusor muscle defect, composed of urothelial lining without a muscular layer -3. The tThese diverticula may be caused by congenital or acquired defects of the bladder wall. Congenital diverticula usually develop from fetal malformation, such as weakness of the ureterovesical junction or a posterior urethral valve ,7. AcquiBladder diverticula can be present at any age, but mostly are detected in elderly people (over the age of 60 -6), withTypically, the BD is small and asymptomatic. However, the clinical presentation can be haematuria, urinary tract infection, urinary retention, ureteral reflux, or obstruction, calculi, inflammation, neoplasm formation (0.8%-10% of cases), or acute abdomen due to its rupture .Spontaneous rupture of a BD (SRBD) is rare; however, spontaneous rupture of acquired bladder diverticula has been reported more often than in congenital ones . In chilWe report a case of a female adult patient with SRBD who presented with an acute abdomen, and a clinical approach and surgical treatment were performed.A 50-year-old female was admitted to the emergency room (ER), with a one-day history of lower quadrant abdominal pain and dysuria. She had a past medical history of arterial hypertension and a hysterectomy performed 10 years ago due to fibromyomatosis disease. No history of trauma was reported. The physical examination showed discomfort on hypogastric palpation. The blood tests and urinalysis had normal results, and the patient was discharged.After three days with dysuria and growing and progressive diffuse abdominal pain, the patient returned to ER. On physical revaluation, the abdomen was distended with diffuse pain, peritoneal irritation signs, and tenderness in the hypogastric area. The laboratory findings were 11.30 x 109/L of white blood cells, 8.61 x 109/L of neutrophils, 9.33 mg/dL of C-reactive protein, 89 mg/dL of serum urea, and 2.42 mg/dL of serum creatinine.The Doppler pelvic ultrasound identified an anechoic lesion in continuity with the bladder wall, through which a fluid leak could be seen Figure .A computed tomography (CT) cystography was performed, confirming the urine extravasation from a bladder diverticulum formation Figures -3.Clinical presentation and imaging findings lead to the diagnosis of acute peritonitis secondary to intraperitoneal rupture of a BD.An emergency midline laparotomy was performed. We identified a ruptured BD, localized in the bladder dome with fluid collection around. It was performed a diverticulectomy, followed by bladder wall reconstruction with a continuous Vicryl double suture layer. A copious saline lavage of the peritoneal cavity was done, and the perivesical space was drained.The postoperative period was uneventful. She was discharged three days after the surgery, maintaining the urethral catheter, which was removed after five days. No complications were reported. The drain was removed when drainage was minimal.The peritoneal fluid cultures were negative for bacteriological microorganisms. The histopathology revealed a perforated BD with no evidence of malignancy. On the first-year follow-up, the patient remained asymptomatic and complication-free.Urinary bladder rupture is post traumatic in 96.6% of all cases , and it A spontaneous rupture of the bladder (SRB) is less recognized than a traumatic rupture. It is a very rare surgical emergency (< 1%), with an incidence of 1:126.000 and eventually a life-threatening event, with a mortality rate of 47% ,8.It is associated with risk factors such as the weakness of the bladder wall and/or increased bladder pressure ,10. ZangSRBD is an extremely rare entity. Until 2020, only 16 cases have been reported in the literature ,9. We deMost of the cases of SRBD are intraperitoneal and located in the bladder dome ,9-10. InAn accurate diagnosis, followed by surgical intervention, is the key to a successful outcome. A delay in diagnosis of 24 hours (or more) has a related mortality rate as high as 25% for SRB . MisdiagAs reported in this case, early diagnosis is difficult mainly because the clinical presentation is nonspecific and the differential diagnosis includes a large spectrum of more common pathologies. The clinical presentation includes painful abdominal distension, decreased urine output, and voiding complaints, and, in some cases, it can take on the appearance of acute peritonitis with sepsis, oliguria, and acute renal failure due to peritoneal urine reabsorption ,10. In cThe diagnosis was suspected on Doppler ultrasound and confirmed by CT cystography, allowing the patient to receive prompt surgical treatment.CT cystography is the gold standard for diagnosis . The impEuropean Association of Urology guidelines, the management of intraperitoneal bladder rupture requires immediate surgical repair because it might lead to a life-threatening condition due to the risk of abdominal sepsis and peritonitis [According to the itonitis . It can itonitis ,8. A lapitonitis .As reported in this case, the surgery consists of exploring the whole abdominal cavity, diverticulectomy, bladder wall reconstruction, and draining the abdominal fluid ,9-10, liA BD, despite of rare, may be present in women and complicate with spontaneous rupture and acute abdomen. Due to the variable clinical presentation, early diagnosis of a BD rupture may be difficult, enhancing the importance of a high index of suspicion. A CT cystography plays an important role in the diagnosis of bladder rupture and should be used as a first-line exam when it is suspected. The management of this situation requires immediate surgical repair


Mullerian cysts are rare cystic lesions that represent remnants of Mullerian ducts. The clinical presentation usually involves swelling or symptoms of large cyst-size compression of adjacent structures. The preoperative diagnosis is very challenging due to the lack of specific features, and the precise diagnosis is reached with histopathological examination. In this report, we discuss a case involving a 26-year-old woman who visited our clinic with complaints of swelling in her left inguinal region. The patient was operated on as a case of a suspected left inguinal hernia, but the histopathological examination of the excised mass was consistent with the diagnosis of a Mullerian cyst. The female uterus, fallopian tubes, and upper part of the vagina are formed during embryonic development by the fusion of the two Mullerian ducts . MulleriA 26-year-old female patient, married and with children, with a previous diagnosis of familial Mediterranean fever (FMF) and a surgical history of appendectomy and caesarean section (CS). The patient was seen in the outpatient clinic and had been complaining of left inguinal swelling for one year that was non-tender but increased in size with coughing. She was an infrequent smoker, not on any medications, including medications for FMF, and had a negative family history of malignancy. On examination, the patient had a thin built, looked healthy, and had normal vital signs. A mass measuring approximately 2.0 cm × 3.0 cm was seen in the left inguinal region with no overlying skin changes. On palpation, the mass was non-tender, with a positive cough impulse, and partially reducible. The impression was of a left inguinal hernia. Ultrasound (US) was done preoperatively; the differential diagnosis was a cystic lipoma versus a hernia containing bowel loops.A computed tomography (CT) scan was done to confirm the diagnosis Figure . It repoIntra-operatively, a left inguinal incision was made and opened layer by layer down to the inguinal canal. After opening the inguinal canal, the mass was seen with multiple cysts and a thin strip of solid component, measuring approximately 4.0 cm × 2.0 cm × 1.5 cm  are commonly used in diagnosis. CT scans can detect unilocular or multilocular cysts with thin walls containing transparent fluid , whereasSeveral therapeutic strategies, including external marsupialization, internal drainage, and straightforward aspiration, have been proposed. Even though these techniques are minimally invasive, they have been linked to high recurrence rates . For theBecause primary adenocarcinoma can develop from a Mullerian cyst, the presence of malignancy cannot be ignored in these cases . A definDespite their rarity, it is essential to consider Mullerian cysts when diagnosing inguinal cystic lesions. The histopathological examination is the only confirmatory diagnostic test, especially to rule out any evidence of primary malignancy, therefore allowing physicians to provide proper management and follow-up to patients with this pathology.


Syncope is a common chief complaint among patients presenting to the emergency department, the etiology of which can often be discerned with a thorough history and physical examination. Inversely, liposarcomas are rare tumors that frequently pose a diagnostic challenge as the clinical presentation is highly nonspecific and varies greatly depending on the anatomic location and size of the tumor. Here we present a case of retroperitoneal liposarcomas (RLS) presenting to the emergency department (ED) with a sole complaint of syncope, resulting in a diagnostic dilemma. This clinical scenario highlights the significance of thorough physical examination regardless of the presenting chief complaint, as unexpected physical examination findings prompted an extended work-up and thus facilitated the diagnosis, providing the opportunity for early intervention and resection of the tumor. Syncope comprises approximately 3% of emergency department (ED) visits, where up to 50% of cases can be diagnosed with a thorough history and physical examination . InverseA 75-year-old male with a past medical history of well-controlled hypertension and tobacco use disorder presented to the emergency department due to syncope. The patient described several occurrences of lightheadedness following positional changes and reported two episodes of collapse and loss of consciousness. He denied preceding chest pain, palpitations, shortness of breath, headache, blurry vision, paraesthesias, bowel or bladder incontinence, tongue biting, or other associated symptoms. Both episodes lasted a few seconds, and the patient reported an immediate return to baseline mentation after regaining consciousness. Vital signs were stable on presentation. The patient underwent extensive diagnostic evaluation, including comprehensive laboratory examination, 12-lead electrocardiogram and continuous cardiac monitoring, chest x-ray, computed tomography (CT) of the chest with angiography, and CT head without contrast, all of which was unrevealing. A large, indurated, palpable abdominal mass was noted in the right lower quadrant upon thorough physical examination. CT of the abdomen and pelvis with intravenous contrast was ordered and revealed a large 19.6 x 13.8 x 11.2 centimeter (cm) heterogeneous mass appearing to originate from the right adrenal gland  of the adrenal glands with and without contrast further characterized a large, T2 hyperintense, heterogeneously enhancing mass in the right hemiabdomen extending from the right hemidiaphragm into the right upper hemipelvis, abutting the right kidney and right hepatic lobe. In addition, evidence of mass effect was noted with MRI revealing compression of the inferior vena cava (IVC) Figures -4.It should be noted that the patient denied abdominal pain, distension, early satiety, or any other abdominal symptom, with his only complaints consisting of lightheadedness and syncope. Work-up was continued with functional biochemistry , which returned within normal limits. CT-guided biopsy of the mass initially showed a well-differentiated liposarcoma with amplification of the mouse double minute 2 (MDM2) gene at 12q15. Given the case's complexity, the patient was evaluated by surgical oncology, urology, and vascular surgery. Exploratory laparotomy revealed a large retroperitoneal mass enveloping the right kidney and adhering to the inferior edge of the liver, the duodenum, and the inferior vena cava. The patient ultimately underwent radical resection of a 24.6 cm right retroperitoneal mass with en bloc nephrectomy, adrenalectomy, partial right liver repair, and repair of the IVC  which was adherent to but did not invade the renal cortex, liver capsule, or adrenal gland. Margins were tumor-free, and the final staging was pT4N0M0, stage IIIB. Given no evidence of alternative etiology for the initial presentation, of dedifferentiated retroperitoneal liposarcoma was ultimately made due to a sole presenting symptom of syncope, caused by severe preload reduction from mass effect causing IVC compression.Liposarcomas (LS) are rare soft tissue tumors typically occurring in middle-aged to older adults and are most often located in the retroperitoneum or extremities . SeveralContrary to many other malignancies, as most liposarcomas are not F-Fluorodeoxyglucose (FDG) avid, positron emission tomography (PET)-CT scan is not routinely recommended as a component of diagnosis or staging -12. AlthRetroperitoneal liposarcomas are rare soft tissue tumors that often pose a diagnostic challenge due to nonspecific presenting symptoms. Given the poor prognosis and possible metastatic potential, a high clinical suspicion is essential for early diagnosis. This clinical scenario highlights the significance of thorough physical examination regardless of the presenting chief complaint, as unexpected physical examination findings prompted an extended work-up and thus facilitated the diagnosis, providing the opportunity for early intervention and resection of the tumor.


Left-sided appendicitis is usually caused by situs inversus totalis or midgut malrotation. Clinical and imaging diagnoses have been presented relatively fully in the literature. However, this is a rare condition, and each related case should be further reported to help the day-to-day clinician better investigate and understand. Therefore, in this paper, we present a case of left-sided acute appendicitis in an adult male patient with situs inversus totalis. In addition, we also discuss the laparoscopic technique of the left-sided appendectomy as it is technically more difficult because of the mirror nature of the anatomy. There are three types of visceral situs that exist in the body including situs solitus, situs inversus and situs ambiguous. Situs solitus refers to the normal arrangement of organs. Situs inversus refers to a mirror-image arrangement of organs to situs solitus. Situs inversus totalis is a rare congenital disease associated with autosomal recessive inheritance. The condition is a positional anomaly when both the thoracic and abdominal organs develop in the wrong position. The prevalence in the general population is reported to be between 0.001 and 0.01% .A 42-year-old male admitted to the hospital with dull abdominal pain that had persisted for the previous 10 h. The patient stated that the pain started in the epigastrium and then spread to the left iliac fossa. Accompanying symptoms were nausea and shivering fever. The patient had a known history of organ reversal. The clinician suspected acute appendicitis, ordered routine tests, chest radiograph and abdominal ultrasound immediately afterwards. Complete blood count showed an elevated white blood cell count of about 16 000/mL. The chest radiograph showed the existence of dextrocardia . The ultThe surgeons placed three ports according to experience, as long as they feel most convenient. A 10 mm trocar was placed just below the umbilicus; the other two 5 mm trocars were placed in the left iliac fossa and suprapubic region . SurgeonLeft-sided appendicitis is usually caused by situs inversus totalis or midgut malrotation. The prevalence of appendicitis associated with situs inversus totalis ranged from 0.016 to 0.024%. They are reported to occur more frequently in males than in females (ratio 3/2) and the reported age ranges from 8 to 82 years. There were 62% of patients present with left iliac fossa pain, 14% right iliac fossa pain and 7% bilateral pain. This is explained by the corresponding non-translocation nervous system and referred pain .Laparoscopic appendectomy in situs inversus totalis can be performed with three ports; however, there is no agreed standard portal position. Basically, all positions on the abdominal wall can be inserted trocar according to the basic principle of laparoscopy triangulation, as long as it is most convenient for the surgeon to manipulate. At the same time, port placements are modified based on the patient’s condition and the position of the appendix assessed on imaging studies .With the appendix on the left side, the surgeon’s preferred port placements include one in the umbilicus, one in the hypogastrium and one in the right iliac fossa . In our These port placements keep the surgeon comfortable and free from arm and wrist fatigue. Handedness has been reported to affect surgical performance in situs inversus. Left-handed or ambidextrous operators can perform the laparoscopic left-side appendectomy more easily. The resection and ligation of the mesoappendix in situs inversus is also more difficult than usual, so harmonic scalpel or electrocautery should be used to perform this procedure instead of scissors and endoloop .Surgical technique for left appendicitis is not specific. Therefore, the surgeon should base on the position of the appendix on the imaging of each patient to come up with a specific treatment strategy. Surgical techniques should also refer to the literature. However, the technique can be variable and arbitrary, as long as the surgeon finds it convenient and comfortable.


Pleural epithelioid hemangioendothelioma (EHE) is a rare malignancy of vascular origin. It can affect various organs; pleural involvement is rare and lack of suspicion leads to delay in diagnosis. We present a case of pleural EHE with metastasis to lung parenchyma that presents with chest pain and dyspnea. Chest imaging showed loculated pleural effusion, pleural thickening, mediastinal lymphadenopathy, and pulmonary nodules. Pleural effusion is one of the most common diseases involving pleural space. The etiology of pleural effusion is varied and its presence could be due to benign or malignant conditions affecting the pleura. Epithelioid hemangioendothelioma (EHE) of the pleura is a rare subtype of an uncommon malignancy and proves a diagnostic challenge. It reportedly involves bone, meninges, soft tissue, gastrointestinal tract, mediastinum, spleen, breast, testicle, and skin. Pleural involvement is rare .Herein is described a patient who presented with pleural effusion and pleural thickening, who underwent multiple thoracentesis with no clear diagnosis. Eventually after thoracoscopy and pleural biopsy, she was diagnosed with pleural EHE. A 73-year-old African-American female was followed in the pulmonary clinic for shortness of breath that was going on for 3 months. She was found to have multiple pulmonary nodules and pleural thickening that remained initially stable on serial CT imaging. She had progressive fatigue, dyspnea, cough, and pleuritic chest pain and was found to have a small left-sided pleural effusion and abnormal right paratracheal lymph nodes (1.5 cm in size) with increasing pulmonary nodules. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TNBA) of the right paratracheal (station 4R) lymph node was done for diagnostic purposes, but the final pathology on the cell block did not reveal any malignant or diagnostic cells. The patient experienced persistent shortness of breath and was admitted for further evaluation. Imaging Figure . The patient returned to the clinic following her thoracotomy and decortication with symptomatic improvement, reduced need for supplemental oxygen, and scheduled outpatient follow-up with oncology where she was offered a combination of chemotherapy and radiation therapy. Her pleural effusion did not recur and she continued her follow-up with the Oncology team. Epithelioid hemangioendothelioma is a rare endothelial vascular neoplasm of soft tissue, described as an intermediate of hemangioma and angiosarcoma . This neThe patient described in this case presented with non-specific symptoms of cough and dyspnea. Pleural effusion is a prominent feature of pleural EHE and is commonly loculated, like in this case. The pleural effusion is usually exudative due to inflammation causing increased capillary permeability. The differential diagnosis for this constellation of symptoms is broad and includes malignancies, infections, and autoimmune etiologies.CT imaging of this patient demonstrated multiple stable pulmonary nodules, plaque-like pleural thickening, and lymphadenopathy, in addition to unilateral loculated pleural effusion. These findings are typical for pleural EHE, which most commonly features unilateral pleural fluid and nodular pleural thickening; multiple pulmonary nodules may indicate metastases, consistent with the aggressive nature of the pleural variant of this neoplasm .Biopsy of the neoplastic tissue confirms the diagnosis of pleural EHE and differentiates it from other malignancies of the pleura. Histologically, EHE features epithelioid cells with abundant eosinophilic cytoplasm in a fibromyxoid stroma, which may have a signet-ring appearance due to the potential presence of intracytoplasmic vacuole. The cells of this neoplasm share characteristics with non-neoplastic endothelial cells, but are distinguished by immunochemical staining and electron microscopy . PatholoTreatment options for pleural EHE include a complete surgical resection, which in many cases is not possible due to metastasis and patient debility. Other options like chemotherapy with carboplatin plus etoposide have been tried with some success . As for The pleural variant of EHE is an aggressive neoplasm presenting with non-specific symptoms. Other than pleural effusion, it can present with pulmonary nodules and mediastinal lymphadenopathy. The diagnosis requires stepwise approach and can be delayed due to its low prevalence. Diagnosis eventually requires biopsy with immunostaining differentiating it from other pleural neoplasms.


Intralabyrinthine schwannomas (ILSs) are an uncommon finding. Diagnosis is challenging and no gold standard treatment exists yet. In this article, we present a two-cases series and review the latest available literature to assess the best diagnostic and therapeutic scheme.We reviewed the latest available literature assessing most frequent and relevant sets of symptoms, clinical features of the disease, diagnostic tests and imaging, possible treatments and after-surgery hearing rehabilitation techniques. We then compared literature data to our own series ones.ILSs clinical presentation and development may overlap with other, more common otological conditions. Full audiometric battery test, electrophysiological study of VEMPS and MRI with contrast enhancement all appear to be critical to correctly diagnose these tumors. Several treatments exist: radiological follow-up, radiation therapy, full or partial surgical excision. Hearing rehabilitation is mostly accomplished through simultaneous cochlear implantation.Our case-series data matches the available literature. ILSs are a rare type of vestibular schwannomas. Diagnosis in challenging and delayed in time as all the diagnostic tests, yet sensitive, are not specific for ILSs. The most suitable treatment seems to be surgical excision of these tumors followed by simultaneous cochlear implantation to restore hearing. Intralabyrinthine schwannomas (ILSs) are a specific group of 8th nerve schwannomas that are located within the labyrinth. Their low prevalence , togethA 60-year-old female patient came to our attention in late 2021 complaining of recurrent vertigo and left-sided tinnitus since November 2017, and reporting an episode of left sudden sensorineural hearing loss (SSNHL) in March 2018. She had a positive clinical history for patent foramen ovale surgery and an autoimmune thyroiditis. The patient had undergone two Magnetic Resonance Imaging (MRI) scans in 2018 and in 2019, both reported as negative for cerebellopontine angle (CPA), 8th cranial nerve and inner ear pathology. In late 2021 she underwent a third MRI scan that showed a 5 × 3 mm contrast enhanced lesion filling the vestibule and the ampullary end of the posterior semicircular canal  is a common benign tumor, accounting for up to 8% of intracranial neoplasm . VS are ich zone , 5, wherich zone . In 2004ich zone , describPatients suffering from ILS may present with common symptoms, such as hearing loss (HL), vertigo/dizziness and tinnitus . HL is uThe diagnosis of ILS is often delayed in time, due to non-specific symptoms, radiological misdiagnosis, low prevalence, and low growing rate . A complDue to the rarity of these tumors only few cases have been reported so far, hence the lack of gold standard treatment nor guidelines, forcing the otolaryngologist to a case-by-case evaluation of the best therapeutic option. Kennedy et al. suggested that radiologic follow-up with repeated MRI is the best strategy for small ILS causing a non-invalidating symptomatology, given their slow growing rate , 20; nevSurgery represents the best option in case of small tumors that may be completely excised with limited surgical morbidity. Microscopic, endoscopic , 29 as wIn terms of hearing rehabilitation, the translabyrinthine approach allows the intracochlear application of a CI through a posterior tympanotomy approach, while drilling the cochlea may prevent the application of a CI, if the modiolus and spiral lamina are not carefully preserved , 33; Ma Although a case of intracochlear schwannoma excision with hearing preservation was reported , cochleaSo far, CI appears to be a feasible hearing rehabilitation strategy as long as the modiolus and the spiral lamina are preserved, despite the extent of the tumor , 33.In case of cochlear drilling, after CI insertion the lateral cochlear wall should be reconstructed with temporalis muscle fascia, autologous cartilage, perichondrium or bone pâté .Auditory outcomes of CI after ILS surgery have been reported in case series and reports , 32–38, Table In this series we presented two cases of ILS, an intravestibular and an intracochlear subtype. The clinical history of the two patients was similar, as they both developed progressive non-specific audiological symptoms, and experienced a veritable diagnostic delay, due to several MRI scans reported as negative for inner-ear pathologies (counting an average delay of 30 months). Both the patients were finally treated by surgical removal with simultaneous CI insertion, given the small size of the tumors, the degree of HL and the absence of vestibular symptoms. In case 1 the IV localization of the tumor did not represent an issue for CI, as there was no risk of direct damage of the cochlea, while in case 2, the removal of the IC schwannoma required the demolition of the lateral wall of the cochlea and its immediate reconstruction with bone pâté and temporalis muscle. Our results in terms of hearing rehabilitation match the ones reported in previous literature as regards to the IC schwannoma , and are even better in the IV case .ILS is a rare type of 8th nerve schwannomas that may cause severe distress for patients: sudden SNHL, vertigo and tinnitus are very frequent and may be strongly debilitating. Delayed diagnosis appears to be an actual issue, even if modern MRI technologies can early detect this type of lesion. Surgical excision with simultaneous or secondary CI seems to be a valuable therapeutic option. The two cases we present in this paper generally reflect the main features of this pathology found in the literature so far. Further studies are although necessary to establish standard treatment protocols to assure patients the best outcome possible.


Fungal rhinosinusitis (FRS) presents as various phenotypes ranging from asymptomatic colonization to life-threatening infections. Here, we report an atypical case of FRS of the left maxillary sinus that extended to the contralateral maxillary sinus through the nasal septum. An 80-year-old woman with a history of osteoporosis was referred to our hospital for further management of headaches and chronic rhinosinusitis. Computed tomography (CT) of the sinus revealed a mass lesion with calcification in the left maxillary sinus, extending to the contralateral maxillary sinus through the nasal septum. T1-weighted and T2-weighted magnetic resonance imaging revealed a mass lesion with low-intensity signals. Endoscopic sinus surgery was performed for the diagnosis and treatment. Histopathological examination revealed fungal elements in the caseous material of the left maxillary sinus. However, no tissue-invasive fungal forms were found. Additionally, eosinophilic mucin was not observed. Based on these findings, the patient was diagnosed with fungus ball (FB). To the best of our knowledge, there are no reports of a FB extending contralaterally through the nasal septum. This report serves as a reminder that FB can extend into contralateral paranasal sinuses through the nasal septum and the possibility that osteoporosis is a cause of extensive bone destruction. Osteolytic lesions in the nasal cavity and paranasal sinuses should be carefully investigated to exclude the possibility of malignancy. Additionally, it is important to note that fungal rhinosinusitis (FRS) can cause bone destruction and remodeling. FRS represents a spectrum of diseases ranging from asymptomatic colonization to life-threatening infections . GeneralAn 80-year-old woman with a history of osteoporosis, hypertension, and depression was diagnosed with chronic rhinosinusitis (CRS) by computed tomography (CT) of the head at a local hospital after a thorough evaluation of her persistent headache and bilateral nasal congestion for several years. Her medications were alprazolam, paroxetine, and ramelteon for depression. She had no history of sinonasal surgery or trauma. She received low-dose macrolide therapy for two months. As the symptoms did not improve, she was referred to our hospital for further treatment. Nasal endoscopy revealed a polypoid mass with smooth surface occupying the left common nasal meatus and purulent discharge in the right common nasal meatus Figure . A biopsContrast-enhanced CT of the sinus revealed a mass lesion with calcification in the left maxillary sinus extending to the contralateral maxillary sinus through the nasal septum Figure . The nasThe patient underwent endoscopic sinus surgery (ESS) for diagnosis and treatment. Intraoperatively, a caseous mass accompanied by purulent discharge extending from the left maxillary sinus to the contralateral maxillary sinus was observed Figure . There wApproximately 10% of the patients who undergo ESS are diagnosed with FRS. In more than 90% of the FRS cases, a single sinus is involved, and unilateral involvement is present in almost 99% of the cases . The mosTo the best of our knowledge, there are no reports of an FB originating from the maxillary sinus and extending to the contralateral maxillary sinus through the nasal septum. Contralateral involvement in non-invasive FRS is extremely rare. In the present case, the preoperative blood test showed a positive result for Aspergillus fumigatus-specific IgE, whereas eosinophilic mucin was not observed in the paranasal sinuses. Therefore, the patient was diagnosed with FB. Considering that reported cases of FB progressed to the IFRS over a period of one to several years ,6, the pWe need to note that the patients also had osteoporosis. Some reports have described the relationship between osteoporosis and paranasal sinuses. Choi et al.  reportedWe report a rare case of FRS that extended to the contralateral side through the nasal septum. The present case adds to the current knowledge by emphasizing that non-invasive FRS can cause extensive bone destruction, especially in the presence of osteoporosis is present.


Giant cell tumours (GCTs) of the bone often arise in the long bones while occurrence in smaller bones of the hand and feet is very rare. We report a case of GCT in the talus of a 17-year-old male who presented with a six-month history of worsening pain in his left ankle and loss of function, reducing his ability to walk and participate in sports. Radiographs of the ankle showed bony overgrowth on the head and neck of the talus with cortical breaching. MRI revealed possible extension into soft tissue and bone marrow oedema. CT scan also revealed an aggressive lytic lesion at the head and neck of the talus. He was managed with intralesional curettage and autologous bone grafting with bone harvested from the left knee. There was no evidence of recurrence at the six-month follow-up and the patient was able to walk freely. In conclusion, GCTs of the talus tend to occur in younger and healthier patients and have disastrous consequences if they persist, recur, or metastasize. Given the severe negative impact that GCTs have on a patient’s quality of life, they must be ruled out when investigating any ankle pain or reduced mobility. Current treatment options have produced consistently positive results while novel therapies that enable a faster return to weight bearing and reduce recurrence appear promising. Giant cell tumours (GCTs) of the bone are benign but potentially aggressive tumours more commonly seen in women and arise between the ages of 20 and 40 years . GCTs moA 17-year-old male presented with a six-month history of worsening pain in his left ankle and foot with increasing loss of function, becoming most severe two months prior. There was an associated reduction in left lower limb muscle bulk. The patient who was fit and active was now struggling to walk and was unable to play sports for the past six months. He complained of poor sleep and a weight loss of 5 kg. His appetite was normal, and he denied any history of trauma, fever, night-time pain, or night sweats. There was also no right ankle pain and no left hip or knee pain. On examination, there was diffuse dorsal-medial-lateral swelling of the left ankle. He had a full range of motion, intact distal neurovascular status and no overlying skin changes, redness or warmth. He was walking with an antalgic gait and was unable to run.Radiographs of the left ankle showed bony overgrowth on the head and neck region of the talus with cortical breaching Figure . A compuThe expertise and guidance of the tertiary-level orthopaedic oncology centre were sought, and the decision was made to proceed with an ultrasound-guided biopsy to rule out osteosarcoma. An orthopaedic consultant specialist in foot and ankle surgery performed the procedure in consultation with the orthopaedic oncology surgical specialist. This was done under aseptic conditions using a 13 gauge x 16 cm bone biopsy needle to access the lytic lesion of the anterior medial talus. Multiple samples were taken directly to histology. Results indicated that the samples received were non-representative of the lesion in question. A supplementary report further stated that there were some fragments of slightly reactive bone and normal articular cartilage. Although the biopsy did not identify any inflammation or tumour, the radiologists reported there was a significant lesion at the time of biopsy, suspected to be a GCT or chondral blastoma. These results thus fell into the 10% of oncological biopsies that are deemed non-conclusive. Based on this finding, the tertiary centre was contacted again and the decision to operate and remove the lesion was made.A left ankle excisional bone biopsy of the talus with bone grafting and left knee bone graft harvesting was conducted. Under the sterile technique, a tourniquet was inflated to 300 mmHg and the patient was placed in the supine position with standard draping. The talar neck was approached by a standard dorsomedial approach. Samples were curetted and sent for histopathology tests and tissue culturing Figure . The antPostoperative histopathology reported a 2 cm benign bone-forming tumour characterised by numerous bony trabeculae with variable osteocyte rimming and prominent vascular stroma. The tumour was shown to have mixed histology containing a significant amount of osteoclast-type giant tumour cells with no background stromal cells. These were consistent with type III cells found in benign GCTs. Results of soft tissue culturing showed no evidence of infiltration on return.The patient was seen at the outpatient department six weeks post-operatively. The surgical wound was healing well, and he was able to walk freely with no complaints. At the six-month follow-up, the patient was well and there was no radiographical evidence of recurrence or metastasis Figure . A plan A literature search was conducted on PubMed using the following combination of keywords: “giant cell tumour”; or “giant cell tumor”; or “GCT”, and “talus”. Results were restricted to English publications with available full-texts and case reports involving GCTs of the talus without any date restrictions. A summary of 16 cases reported in 15 studies can be found in Table Among 16 case studies, 56% of patients were within the 20-40-year-old age range ,13-16,18Like the patient in this case, the head and neck of the talus were specifically affected in five cases ,7,10,16 Physical examination findings reported include pain, swelling and tenderness ,13,15-18Our case utilised radiography, MRI and CT scans to identify the lesion and assess the extent of its growth and spread. In the literature, radiographs were similarly useful in 14 cases -16,18, wSimilar to how the patient in our case was managed using intralesional curettage and autologous bone grafts harvested from his knee, six cases used curettage and bone grafts taken from sites such as the iliac crest ,10,12,16Follow-up assessments include checking for physical signs or radiographical evidence of recurrence or metastasis, testing for improvement in ankle or subtalar joint range of movement, and assessing the patient’s ability to walk or carry out daily activity -18. RoutGiven the higher risk of GCT in the tarsal bones in patients under 20 and the disastrous effect it can have on their quality of life, it must be detected early and removed before it becomes malignant or metastasizes. Traditional treatments of GCTs of the talus continue to appear effective while non-traditional management and novel therapies have also been reported to produce encouraging outcomes. The treatments may also allow recurrence to be detected earlier, reduce recurrence and enable earlier weight bearing and return to normal walking function.


Numerous techniques for provisionally replacing a single tooth at the aesthetic zone while planning for future dental implant placement, while soft and hard tissues heal, are critical in restorative treatment strategy. The available materials, simplicity, cost, and impact on the potential implant location should be considered when evaluating the treatment choices.Replacement of a single anterior tooth in the aesthetic zone while planning for future implant placement is crucial to the restorative treatment plan. Several methods exist for immediate provisionalisation of the extracted tooth while waiting for soft and hard tissue healing. Although there's a myriad of possible provisionalisation methods available, each option has its advantages and disadvantages. The treatment options should weigh various factors such as the available materials, ease of fabrication, costs, and the effect on the future implant site. This article describes three clinical cases demonstrating a simple yet cost‐effective technique to temporarily replace an extracted single anterior tooth, enhancing patient satisfaction and increasing compliance before receiving the definitive implant restoration. The pro and cons for each treatment option available as opposed to the technique involved in the three cases used are also described. Thus, in such situations, bone grafting procedures may be indicated during the extraction stage itself to prepare for a suitable implant site before implant placement.1.1A 42‐year‐old female patient complained of a mobile and sensitive upper right canine tooth Figure . There i1.2A 48‐year‐old male patient presented with a complaint of mobile and lingually displaced lower right mandibular central incisor, causing discomfort during mastication. There was no contributory medical or social history. Dental history revealed episodes of recurrent infection with a history of pus exudate from that tooth. Upon clinical examination, the mandibular right central incisor was found to be lingually positioned, supra‐erupted, with grade III mobility. Periodontal pocket depths ranged from 5 to 9 mm, and gingival recession of 3–5 mm on six points pocket charting. The adjacent teeth were checked for pocketing and mobility with no significant pathology. Radiographic examination revealed the loss of alveolar bone support beyond the apical region Figure . The dia1.3A 52‐year‐old female patient complained of a mobile crown on her upper right central incisor. The patient is fit and healthy. Dental history revealed multiple episodes of crown dislodgement and recementation dating back 7 years ago. Clinical examination of the affected tooth revealed grade III mobility, the presence of pus discharge, swollen labial mucosa and associated with tenderness upon palpation and percussion Figure . The per1.4This technique requires a laboratory preparation stage before the tooth extraction to reduce the chairside time during the extraction appointment.1.5Step 1A study model of the arch is used, and the impending tooth to be extracted is trimmed off to simulate post‐extraction socket contour. A replacement acrylic denture tooth is trimmed to fit into the horizontal and vertical space of the prepared edentulous area Figure .Step 2A slot/groove was prepared on the palatal/lingual surface of the acrylic tooth with an undercut to accommodate the diameter of 0.7 mm wire. The wire was bent to follow the curvature on the palatal surfaces of adjacent teeth, incorporating the acrylic tooth on the prepared wire.Step 3The bent wire was placed into the undercut slot prepared on the acrylic tooth and secured with composite resin and a bonding agent Figure .Step 4Occlusion was checked using articulating paper on static and dynamic movements to ensure no occlusal interference or any premature contacts that may contribute to the acrylic tooth splint dislodgement.1.6After the extraction of the teeth and hemostasis is achieved, the acrylic tooth splint is adapted onto the lingual/palatal surfaces of the adjacent teeth and fixated onto adjacent teeth, which were treated using etch and rinse adhesive bonding and composite resin.2For all the cases discussed above, the patients reported a pleasing and satisfactory outcome related to the overall duration of the splint in the mouth. None of the patients experienced any dislodgements throughout the splint being in situ; for Case 1 (5 months), Case 2 (6 months), and Case 3 (5 months). All patients have received a definitive implant prosthesis and are currently in the maintenance phase of the treatment.3Immediate replacement of extracted anterior teeth can assuage the psychological and social impact of losing one's teeth.This case series highlighted the usage of acrylic tooth splinted with 0.6 mm, twisted, as reported in case 3‐ and 0.7‐mm diameter wires, untwisted, as seen in Cases 1 and 2. They were placed with composite on the palatal/lingual surface for provisionalisation of a missing/extracted anterior tooth. The advantages and disadvantages of this technique are summarized in Table In preparing the acrylic tooth to be used in the splint, it should ideally be contoured for an ovate pontic configuration on the tooth intaglio surface facing the gingiva to allow the gingiva to heal around the ovate pontic, simulating the emergence profile of a natural tooth.However, this technique of choice requires careful selection and consideration, namely, sufficient occlusal space on the palatal surfaces of the adjacent teeth to avoid interference in occlusion. It would not be suitable in cases with a deep overbite.4The acrylic tooth splint described in this case series illustrates a simple yet reliable technique for replacing a single tooth in the aesthetic zone. With proper case selection to match the patient's requests and needs, this technique may be helpful for clinicians considering immediate replacement of single anterior teeth.Fara Azwin Adam: Conceptualization; data curation; investigation; methodology; writing – original draft. Farah Syazwani Mohamd Tarmizi: Conceptualization; data curation; formal analysis; investigation; methodology; writing – review and editing. Chui Ling Goo: Conceptualization; methodology; resources; supervision; validation; visualization; writing – review and editing.The manuscript was prepared with the support of the Incentive Grant for FGG Researchers, The National University of Malaysia (DD2020‐008).No known conflicts of interest are associated with this publication, and there has been no significant financial support for this work that could have influenced its outcome.All patients' written informed consent with permission to publish was obtained before publishing the article.


A Morgagni hernia is a rare diaphragmatic hernia that is usually asymptomatic but can present with gastrointestinal and chest symptoms and is reported in many cases with strangulation. Here we report a rare case of a Morgagni hernia with transthoracic herniation of the left lobe of the liver and transverse colon that presented with abdominal pain.A 54-year-old Saudi female presented with abdominal pain, vomiting, and shortness of breath. Chest radiography revealed an air-containing viscus and a wide mediastinum. Computed tomography confirmed the presence of a right-sided Morgagni hernia. Reduction of the defect contents and repair of the hernia together with cholecystectomy were successfully performed using the laparoscopic approach. The patient recovered smoothly with complete resolution of preoperative symptoms.A Morgagni hernia is a rare diaphragmatic defect with an increased risk of incarceration. In addition to the omentum, transverse colon, and small bowel, the defect may involve the left lobe of the liver. Surgical repair is recommended in all cases of Morgagni hernia, to avoid the risk of incarceration. Morgagni hernia is rare in adults and accounts for 3–5% of diaphragmatic hernias . It is a2, and oxygen saturation of 95%. Chest auscultation revealed reduced air entry over the anterior lung field toward the bottom and laterally to the right side. The abdomen was distended and nontender with no guarding. Bowel sounds were audible. There were no other findings on physical and neurological examinations. Chest radiography revealed an air-containing viscus with a wide mediastinum . Chest radiography revealed white spot infiltration (pneumonia) and atelectasis Fig. A. InfusiThe main findings in this case report are the presence of huge Morgagni hernia, in an elderly female, with transthoracic herniation of the left lobe of the liver and transverse colon, presented with a combination of gastrointestinal and respiratory symptoms. The uniqueness in this patient is the subacute presentation of abdominal pain and synchronous prolapse of large part of the liver with the transverse colon, which is rarely reported in the literature .Morgagni hernia is one of the three types of diaphragmatic hernias in which the defect is located posterolaterally to the sternum in the anterior and retrosternal locations , 5. It iThe patient presented with shortness of breath, abdominal pain, nausea, and vomiting. Similar presentations of combined gastrointestinal and respiratory symptoms have been reported in adults, including retrosternal or chest pain, which is often relieved by standing, shortness of breath, flatulence, indigestion, and cramping , 10–13. The main contents of the hernia sac in our patient were the omentum, the middle part of the transverse colon, and the upper part of the left lobe of the liver. Various abdominal organs, including the transverse colon, stomach, omentum, and small intestine, have been reported in the hernial sac; however, few cases have reported a part of the liver in the hernial sac , 7, 15.The diagnosis of Morgagni hernia was suggested from the presentation, physical examination, and chest radiography, and confirmed by the computed tomography scan without contrast. Conventional radiography is usually helpful in the diagnosis of Morgagni hernia, with occasional missed diagnosis , 16. ComIn this case, a laparoscopic approach was used, with reduction of the hernia contents and repair of the defect using mesh and polypropylene sutures. Surgery was preferred because the patient was symptomatic with a subacute presentation of abdominal pain and vomiting. Repair is recommended, even in asymptomatic patients, to avoid incarceration , 8. SomeMost patients are usually discharged 3 days after surgery after an uneventful recovery , 14. In A Morgagni hernia is a rare congenital diaphragmatic defect. It is usually asymptomatic but may present in late adulthood with characteristic symptoms of chest pain, abdominal pain, and vomiting. The defect is most commonly composed of omental fat and the small and large intestines; however, it may also include the liver and other organs. Owing to the increased risk of incarceration, surgical repair is recommended in all cases of Morgagni hernia.


An epignathus is caused by a continuous spectrum of masses of the oral cavity or oropharynx ranging in its entity from mature teratoma to the exceedingly rare fetus-in-fetu. Due to its location, regardless of the entity, the occurrence of an epignathus is frequently associated with life threatening airway obstruction. Here we demonstrate a case of a fetus-in-fetu presenting as an epignatus. We describe its successful management and review the available literature. Early diagnosis and knowledge of the preoperative workup are essential to enable a multidisciplinary management. Once the airway is secured, surgical excision is the treatment of choice often resulting in a good clinical outcome and prognosis. Heteropagus, with an incidence of less than 0.1 per 100,000, is defined as conjoined twins, in which a severely defective and dependent twin (parasite) is connected to its mostly intact twin (autosite) . They arEpignathus itself is a heterogenous group of oropharyngeal masses with an incidence of 1 in 35,000 to 1 in 200,000 births and it can be caused by a broad spectrum of entities ranging from mature teratoma to a real fetus-in-fetu , 6. HoweIn the following we report the case of a fetus-in-fetu presenting as epignathus and discuss the interdisciplinary management of the case as well as the available literature.2.P3), head circumference 30 cm (P < 3). At all times, the respiration was without restraints and oxygen saturation as well as blood gas analysis were within the normal range. Upon physical examination, a 12 cm × 8 cm × 5 cm stalked fetiforme mass protruding out of the mouth was observed (A 29-year-old woman had her second pregnancy. The family history of both Caucasian parents had been unremarkable except of the documentation of a stillborn child. Apart from insulin dependent gestation diabetes the beginning of the pregnancy had been without any noticeable medical events. Exposure to teratogenic agents was denied. In gestation week 30, a routine ultrasound examination was performed leading to the diagnosis of a large tumor originating from the oral cavity with a diameter of 6–7 cm and cystic and solid parts. The main differential diagnosis at that point was a teratoma/epignathus. For further specification, a subsequent fetal MRI scan led to the diagnosis of a large epignathus originating from the right palate and masticator space . The airobserved . Otherwiobserved . Vasculaobserved . IncisioIn the histopathological preparation, the specimen presented as a pedunculated tumor that was completely covered by skin, showing multiple round nodular distensions . A rudimThe postoperative course including airway situation and the gradual normalization of oral feeding was always uneventful and the patient discharged on postoperative day 15.Follow-up clinical controls and ultrasound investigations were performed after 1, 10 and 22 months. The ultrasound investigations showed stable, non-perfused residual cystic alterations in the submandibular area on the right side with a non-definable right submandibular gland on that side. Clinical examination of the thriving child was unremarkable except for an asymmetric soft palate and elongated uvula. Breathing, swallowing and voice production were not affected in any way.3.An epignathus is a rare condition caused by a palatal or pharyngeal mass that can continuously range in its entity from a mature or complex teratoma to a real fetus-in-fetu. Attachments of the masses to the oral cavity, the pharynx or the area of Rathke pouch with intracranial extension have been described , 7, 8. OEarly prenatal diagnosis of an epignathus is vital for the outcome and prognosis. Depending on the size and the location of the attachment of the epignathus to its autosite, intrauterine complications ranging from preeclampsia to polyhydramnion have been reported , 14. PosDuring EXIT procedure, intubation or tracheotomy might be performed while uteroplacental circulation with neonatal anaesthesia and controlled uterine hypotonia are maintained to avoid asphyxia , 18. IndAs for our case, diagnosis could be made early in gestation week 30 by intrauterine MRI scan, which confirmed a patent airway . In part4.Epignathus can be caused by a continuous spectrum of anomalies ranging from mature teratoma to the exceedingly rare fetuses in fetu and many ENT surgeons might not encounter it at all in their careers. Regardless of the entity, by originating from the palate or pharynx, epignathus frequently causes life threatening airway obstruction. Hence early diagnosis and knowledge of the preoperative workup are essential to enable multidisciplinary management. Once the airway is stable, radical excision is the treatment of choice often resulting in a good clinical outcome and prognosis.


Neonatal spontaneous colonic perforation in term neonates is a rare phenomenon, with caecal perforation being seldom reported. Therefore, this case report presents a rare case of spontaneous caecal perforation in a term neonate who presented with vomiting and abdominal distension on day two of life. On exploration, a single large full‑thickness cecum perforation was noted. Histopathologic samples were negative for necrotizing enterocolitis and Hirschsprung’s disease. Clinical awareness of this rare entity could help in preventing delays in imaging and prompt surgical management. A gastrointestinal perforation is a neonatal surgical emergency. Although small intestinal perforation is frequently seen in newborns, reports of colon perforation are rare. In preterm infants, colonic perforation is caused mostly due to necrotizing enterocolitis (NEC). It may be associated with Hirschsprung disease, small left colon syndrome, meconium plug syndrome, idiopathic perforations, anorectal malformations, stercoral perforations, and cystic fibrosis -3. In teA two-day-old female with complaints of vomiting and distension since day one was referred to our hospital. She was a term baby weighing 2.6 kg born to a 25-year-old primigravida by cesarean section. The baby cried soon after birth but had not passed meconium in the first 24 hours. Since the mother had decreased secretions, the baby was fed water mixed with sugar. The last few months of pregnancy went without incident. There was no evidence of any family history of the condition. Upon clinical examination, she displayed decreased activity, and her heart rate was 180 beats per minute with dehydration. Her abdomen was firm but not red, and she did not have any bowel sounds. A rectal examination indicated no clutching and an empty rectum. A significant pneumoperitoneum was visible on both sides of the abdomen on a plain X-ray taken with the patient's abdomen upright  was identified in the cecum, and the rest of the bowel was found to be dilated Figure .Resection of the terminal ileum, appendix, and part of caecum with double barrel ileostomy was done. Rectal biopsies were taken. However, the biopsy report did not identify any particular pathological diagnosis. The post-operative period was uneventful; the stoma became functional on postoperative day one and oral feeds were started on postoperative day five. The patient responded well to the treatment regimen.Hirschsprung disease is perhaps the only condition where a routine correlation to a cecum perforation has been made in case studies ,2,4. TheMost of these cases are diagnosed after the possibilities of a probable etiology have been carefully excluded. Resch et al. suggested that the combination of episodes of hypoxia, resulting in ischemic injury to the gut, together with the immaturity of the intestine could result in perforation in these infants . TheorieAn optimal management plan depends on the site of perforation, degree of contamination, and hemodynamic stability of the neonate. In a hemodynamically stable patient with a well-localized and single perforation primary closure or resection and anastomosis can be performed. In cecum perforations, unless Hirschsprung disease is ruled out, a primary repair becomes impossible, and a stoma is usually done. Biopsies are recommended in all cases . AvailinSpontaneous perforation of the cecum in a neonate without a demonstrable or known cause is sufficiently rare. It is extremely important to perform a thorough history and histopathological evaluation in all cases. The management varies depending on the cause. Even with the best care, babies with colonic perforation tend to have a poor prognosis.


Among congenital anomalies of the portal venous system, prepancreatic postduodenal portal vein (PPPV) is very rare and has only been reported to date. Herein, we report a case of PPPV identified in preoperative examinations for hepatocellular carcinoma and a literature review.A 63-year-old man was admitted to our hospital for treatment of a liver tumor. After examination, he was diagnosed with hepatocellular carcinoma with a diameter of 40 mm in segment 8. Contrast-enhanced computed tomography scan showed a portal vein passing between the duodenum and pancreas, hence called PPPV. At the hepatic hilus, the portal vein branched off in a complicated course with some porto-portal communications. We determined that anatomical resection with manipulation of the hepatic hilum in this case resulted in major vascular injury. Therefore, we performed partial liver resection, and the patient was discharged uneventfully on postoperative day 14.Although PPPV is an extremely rare congenital vascular variant, it is important to carefully identify vascular patterns preoperatively and to recognize the possibility of such an anomaly to avoid misidentification and inadvertent injuries during surgery. Although the frequency of portal system anomalies is lower than that of the bile ducts and arteries, its anatomical recognition is very important for safe gastrointestinal surgery, especially hepatobiliary–pancreatic surgery. Among congenital malformations of the portal system, the preduodenal portal vein (PDPV) is occasionally reported as a comorbidity of other visceral malformations , 2; howeA 63-year-old man complaining of discomfort in the upper right abdomen was referred to our hospital because of a liver tumor diagnosed by another hospital.Previously, he had undergone an appendectomy, and had a history of untreated chronic hepatitis C and a daily habit of drinking. His physical examination revealed no notable abnormalities. The abnormal values in his blood test findings were as follows: aspartate transaminase, 71 IU/l; alanine transaminase, 108 IU/l; alpha-fetoprotein, 5626.0 ng/ml; and protein induced by vitamin K absence or antagonist II, 642 mAU/ml. Other blood counts, biochemical laboratory findings and coagulation factors were within the normal ranges. The retention rate of indocyanine green at 15 min was 10%.Contrast-enhanced computed tomography (CT) showed a 40-mm tumor in segment 8 (S8) of the liver, which was enhanced in the early phase and washed out in the delayed phase, suggesting hepatocellular carcinoma (HCC) Fig. . His porBecause of this anomalous configuration, the Glissonean approach at the hepatic hilum or anatomical resection was judged to be dangerous; therefore, we performed partial resection of S8. During the operation, the liver had a chronic hepatitis pattern and ascites was not observed. No morphological malformations were detected in the liver or in other organs. Intraoperative ultrasonography showed worm-like meandering of the intrahepatic portal veins, but no occlusion findings due to a thrombus Fig. A. We perHere, we report a case of PPPV, which is an extremely rare congenital portal vein anomaly. Portal vein development begins at 4 weeks of gestation and is formed by the paired vitelline veins with three anastomoses that traverse the foregut (duodenum). As the weeks pass, the lower part of the right vitelline vein and the upper part of the left vitelline vein disappear, and the intermediate anastomosis on the dorsal side of the duodenum forms the portal vein main trunk , 4 Fig.A, B. TheIn our case, PPPV and the complex morphology of the portal vein could be identified by evaluating the contrast-enhanced CT and 3D-constructed images as preoperative examinations for HCC. His portal vein had an inverted L-shaped formation and ran along the ventral side of the bile duct, which is consistent with previous reports. In addition, the portal vein was dilated at the hepatic hilum, complicatedly meandering, bending, and branching, similar to cavernous transformation, with some porto-portal communications observed. Among the previously reported PPPV cases, the complex morphology of this case is extremely rare. We chose S8 partial resection as the operative procedure instead of anatomical resection to avoid manipulation around the hepatic hilum because of the anomaly of the portal vein and the location of the HCC on the liver surface. However, anatomical liver resection using the staining method without touching the hepatic hilum was an option to consider. At the time of resection, we considered the possibility that his portal vein wall was thinner and more fragile than usual, as pointed out by previous reports, and did not perform the Pringle maneuver, even though there was no scientific evidence that this technique was dangerous for PPPV. Fortunately, no complex meandering or irregular dilation was observed in the peripheral portal vein branches. Therefore, a partial hepatectomy for the tumor on the surface of the liver could be performed without affecting the malformation of the portal vein. If the tumor was located near the hepatic hilum, the risk of resection was thought to be markedly increased.Based on this review and our case, PPPV cases may have positional abnormalities, fragility and thinness of the portal vein wall, strong adhesion with surrounding tissue, and complicated portal branching abnormalities. Therefore, it is important to carefully identify the running and branching morphology of the portal vein on preoperative images to avoid misidentification and inadvertent damage during surgery, especially when performing surgery around the pancreatic head region, or those requiring dissection of the hepatoduodenal ligament, or major hepatectomy for patients with PPPV.


Xeroderma pigmentosum is an autosomal recessive disorder with various ocular manifestations of which bilateral limbal stem cell deficiency is a rare manifestation. Timely diagnosis and meticulous management are vital in these cases to prevent irreversible ocular sequelae.Bilateral limbal stem cell deficiency (LSCD) can be a rare manifestation in patients afflicted with xeroderma pigmentosum (XP). The authors report a rare case of a 12‐year‐old boy who presented with redness and defective vision and was diagnosed with bilateral LSCD and hyperpigmented lesion over the face and trunk suggestive of XP. Kirandeep Kaur: Conceptualization; data curation; formal analysis; investigation; methodology; project administration; resources; software; supervision; validation; visualization; writing – original draft; writing – review and editing.There are no conflicts of interest.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.At our institute case reports, images and case series are exempted from IRB approval and the research followed the tenets of the Declaration of Helsinki.