Cervical cystic lymphangiomas are rare benign tumors that pose a formidable challenge to surgeons confronted with managing this uncommon entity. Due to the intimacy with which these cystic lesions blend with critical cervical structures, a considerable number of patients who undergo surgical management are often plagued with recurrence and morbidity. As a result, doxycycline sclerotherapy has become an attractive, safe, and effective alternative as a primary treatment modality in a select group of pediatric patients. This case report presents an 18-month-old patient with a large cervical cystic hygroma that was effectively treated with exclusive doxycycline sclerotherapy. Cystic hygroma, a cystic subtype of lymphangioma, is a congenital benign lymphatic malformation. It is considered a relatively rare lesion having an incidence of 1 in 2000–4000 live births . Approxi3 in volume, whereas microcysts contained lesions less than 2 cm3. Furthermore, this classification is also capable of predicting the response to sclerotherapy [Several classification systems have been proposed to aid in diagnosing and managing cystic hygromas. Smith and colleagues described the cystic lymphangiomas as either macrocystic, microcystic, or mixed, depending on radiological findings. Macrocysts were defined as lesions with cystic contents of at least 2 cmotherapy . de Serrotherapy .Historically, surgery has been the cornerstone of treatment, but the total excision of the lymphangioma can lead to severe complications such as neurovascular injury and cosmetic deformity. Recent evidence has shown that macrocytic lymphangiomas treated with surgical excision also respond very well to percutaneous sclerotherapy with less surgical morbidity , 8. The An 18-month-old male was referred to the Department of Otolaryngology by his pediatrician for an asymptomatic, aesthetically unappealing swelling. The patient's mother provided an antenatal history of him being born full term via cesarean section and subsequently noticed a small yet visible left-sided neck mass. She was advised to follow-up in the ENT outpatient clinic but defaulted from any further care. A history of an upper respiratory tract infection and progressive left-sided neck swelling that began one month prior was given.On clinical examination, the infant was in no respiratory distress. A 9 × 9 cm nontender cervical neck mass involving both the left anterior and posterior triangles was evident Figures . The necAn ultrasound of the neck demonstrated two distinct complex cystic avascular masses with multiple separations and internal echoes. One mass was located anterior to the external auditory meatus measuring 3.8 × 3.4 × 3.5 cm, and the other mass was located at the left lateral cervical region measuring 7.7 × 3.4 × 7.7 cm. Based on these dimensions of the mass, a diagnosis of a left cervical cystic hygroma was made, and a computed tomography (CT) scan was arranged to acquire more precise radiological details of the mass. CT findings showed a large 9 × 5 × 9 cm well-defined left-sided neck mass extending from the subcutaneous tissues deep to the left parapharyngeal space. The lesion encased the left sternocleidomastoid muscle and contained thin internal septations with a multiloculated appearance. The medial margin of the left lesions abutted the left carotid sheath with mild displacement and compression of the left internal jugular vein. At the level of the glottis, there was encasement of the carotid sheath with a small extension to the region of the left aryepiglottic fold, extending superiorly to the left oropharyngeal region. Superiorly, the lesion extended into the left parotid gland and subcutaneous tissue posterior to the external ear Figures  and 3. AThe procedure was performed at an operating theatre under general anesthesia. The patient was cleaned and draped. Prophylactic antibiotics were given before the procedure and continued for approximately one week. Using ultrasound guidance, the macrocysts were cannulated with a 5 French Yueh Centesis catheter needle . The fluOne hundred milliliters of 10 mg/ml doxycycline solution was reconstituted in sterile water using 100 mg doxycycline capsules. Five milliliters of Ultravist TM 300 was injected to outline the cysts fluoroscopically. The doxycycline solution was then injected into the lesion. The patient was left intubated and transferred to the Pediatric Intensive Care Unit, where he was kept sedated. The doxycycline was retained in the lesion for six hours and then actively drained. Instillation and drainage of the doxycycline solution was repeated the following day, and the catheters were removed. Subsequently, the patient was extubated and sent to the ward for 24 hours of observation and pain control. The entire procedure was repeated twice, 3 months apart Figures –9. ComplCystic lymphangiomas of the head and neck in children are often intimately involved with tissue planes, namely, the critical aerodigestive and neurovascular structures. Though several treatment algorithms for cystic hygromas have been proposed, the ideal model for management has yet to be revealed. Treatment options include watchful waiting, sclerotherapy, or surgical resection. The clinical presentation of cystic lymphangiomas is determined by their anatomic location, size, and the physiological impairment they cause. Sudden expansion may result from infections or spontaneous intralesional hemorrhage. Lesions of the cervicofacial region may not only cause airway compromise, dysphagia, elocution, and facial disfigurement but also be a source of substantial social and psychological concern to both the patient and parents , 7, 9.Despite many inherent dangers associated with surgical excision, it remains the most popular method of treatment in many developing countries , 3. SurgOver the past 30 years, sclerotherapy have surfaced as a favorable alternative for the treatment of cystic lymphangiomas in children in an effort to avoid the precarious complications of surgery . CommonlDoxycycline is a fairly economical and readily accessible tetracycline antibiotic with an established safety profile. Historically used for the pleurodesis of malignant effusions, its effectiveness as a sclerosant for cystic lymphangiomas was first reported by Molitch et al. , 16. SinThere is no standard technique for doxycycline sclerotherapy, and as a result, the index sclerosis procedure was generally adopted from Molitch et al. . Their mDoxycycline is known to cause severe discomfort during injection and postprocedure for approximately 3 hours as the index case was done under general anesthesia. The child was kept intubated and sedated in the PICU in order to seamlessly repeat the process the subsequent day, avoid dislodgement of the percutaneous catheters, rovide adequate analgesics, and closely monitor our novel therapeutic endeavor. Relatively large volumes of doxycycline can be safely used for sclerosis without toxicity as it can be used to treat large cystic lymphangiomas . As demoOur preliminary results with doxycycline sclerotherapy as an alternative form of treatment for cervical cystic hygromas are encouraging. This case report highlights the possibility of safely achieving favorable results with doxycycline sclerotherapy in a developing country. Adequate diagnostic workup, classification and staging of cystic lymphangiomas are paramount for proper individualized treatment selection. The traditional absolute surgical option employed by developing countries need to be revised as the astounding recurrence rates and potential surgical morbidity can be avoided in some patients.


Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. It is a locally aggressive tumor usually seen in infants. Here we report a case of kaposiform hemagioendothelioma in a child who responded to propranolol and steroids.A 3-year-old male child presented with a swelling below his right knee with characteristic violet skin lesion. There was no evidence of Kasabach–Merritt phenomenon. After no improvement with several attempts at debridement and anti-tubercular treatment; a diagnosis of Kaposiform Haemangioendothelioma was reached on the basis of overall clinical picture and histology. The child was treated with propranolol and steroids and had an excellent clinical response and a near complete resolution on imaging at 5 months.These cases are often misdiagnosed and despite a delay in diagnosis have good outcomes with appropriate multimodality management. This case highlights the unique and typical characteristics of kaposiform haemangioendothelioma. Althsumption . The natA 3-year-8-month old male presented with a violet skin lesion and a swelling below his right knee which was first noticed at an age of 7 months. The swelling gradually increased in size over the next two years and was associated with pain, low grade intermittent fever and decreased appetite. At this time a radiograph was obtained which revealed lytic areas involving head and proximal shaft of right tibia and fibula (images not shown). An exploration of lesion was done in December, 2015 and histopathology revealed fibrofatty tissue with evidence of chronic inflammation. A provisional diagnosis of osteomyelitis was made and treatment with broad spectrum antibiotics was initiated. However, there was no symptomatic relief and the swelling progressed in size. After a repeat imaging, 2nd debridement was done in June, 2016 (Fig. He presented to our hospital in August, 2017. The child had a swelling below knee over the right leg which was hard in consistency. The skin over the tumor was of violet color with increased temperature. The child had a poor nutritional status. His complete blood counts were suggestive of anaemia, leucocytosis and normal platelet count. Liver and kidney function tests were normal. Peripheral smear revealed microcytic and hypochromic RBCs. Bone marrow studies were normal. Workup for primary immunodeficiency, bacterial/fungal infection and tuberculosis was negative. A radiograph of right leg showed lytic sclerotic destructive lesion with midzone of transition seen involving right proximal tibia and fibula with soft tissue component Fig. . It was The treatment plan for surgery was discussed with parents. However, parents have preferred to defer any extensive surgery and decided to continue with medical management.KH is set apart from other common and benign vascular lesions of childhood like juvenile haemangioma, cavernous haemangioma, angiomatosis and endovascular papillary angio-endothelioma by its unique histopathological features and locally invasive nature .The most common clinical presentation of KH is a characteristic violet cutaneous lesion which infiltrates the underlying skin on the extremities . Our patDue to rarity of KH, there are no prospective studies and only case series and case reports are available to guide management. For resectable lesions, surgery is the mainstay of management as there is no spontaneous regression in KH. For non-resectable lesions or for those in which surgery will entail significant morbidity pharmacological management is desirable to shrink tumor size. Such pharmacological treatment is also used in patients presenting with KMP to correct coagulopathy and decrease tumor size. Trans-arterial embolization has been used to treat KH of scalp and cervical region , 13. CorIn 2013, Drolet et al. presented consensus guidelines based on limited clinical literature . InitialIn our patient we started with propranolol and steroids with a clinical response at 1 month and an excellent response on imaging at 5 months of treatment Fig. . The lonThere can be a delay in diagnosis of such rare tumours, as in our patient. Since KH is a rare tumor, the delay may be due to lack of expert sarcoma pathologist and dedicated sarcoma oncologists in India . More co


A nine-month-old female baby with normal birth history presented with her mother complaining of a white spot in the baby’s right eye, which the mother had noticed at five months of age. External photograph showed a retrolental fibroplastic membrane visible in the superior half of the dilated pupil. Retcam fundus photo revealed myelinated nerve fibers extending from the disc till the ora superiorly and forming a membranous fold. Intraoperative OCT confirmed thickened RNFL with compact retina. Thus, the retrolental fibroplasia turned out to be a masquerade for myelinated nerve fibers. Since it was not involving the visual axis with no coexisting traction, the mother was reassured regarding the benign nature of the condition. A nine-month-old female baby from rural North India born at 37 weeks with a birth weight of 2.1 kg presented with her mother complaining of a white spot in the baby’s right eye. The mother had accidentally noticed the spot when the child was around five months of age and she was referred to us by her local ophthalmologist with the query to rule out retinoblastoma. The child had central, steady, and maintained fixation in both eyes. Retinoscopy revealed a dull glow in the right eye with a cycloplegic refraction of –18.00 D and a clear red glow in the left eye with cycloplegic refraction of +1.00 D in undilated state. On torch light, leukocoria with normal anterior segment and clear lens was noted. The white reflex was more prominent from the superior one third of the pupil, more evident in the dilated state. Keeping in mind the differential diagnosis of retinoblastoma, retinopathy of prematurity and exudative retinopathy, an examination under anesthesia was advised.On examination under anesthesia, the fundus showed a yellowish white shiny area (Figure 1A Myelinated retinal nerve fiber is a congenital benign condition which is commonly found in conjunction with the optic nerve head and uncommonly noted in isolation over the fundus. It appears as a whitish or yellowish membrane over the retinal surface following the distribution of ganglion cell axons, giving it a frayed appearance .The normal myelination process begins at around eight months in embryonic period and ends before birth .Various hypotheses have been postulated, out of which the most commonly accepted one is the inhibition of oligodendrocyte migration due to cellular factors released from astrocytes just before birth , 3]. If. If3]. IIn our case, the MNF was extending from the ONH till the superior ora serrata and presumably might have formed a membranous fold at that site. This subsequently gave an appearance of retrolental fibroplastic membrane and showed a very typical white reflex on torch light examination. Taking the age and history into consideration, congenital cataract, retinoblastoma, retinopathy of prematurity or exudative retinopathy would be the primary suspects. A dilated fundus examination with thorough peripheral fundus evaluation and photo documentation is imperative in such cases, preferably under anesthesia. To add to our armamentarium of investigations, an intraoperative OCT was done and the thickened RNFL seen in the area of MNF was captured, which ascertained our diagnosis of MNF .Since the retrolental membranous fold was fortunately neither involving the visual axis nor causing any concurrent traction, the observation of the same was advised and the mother was henceforth reassured regarding the benign nature of the condition. Regular use of glasses with amblyopia therapy was advised. A six-monthly follow-up was recommended.MNF usually is a benign localized condition, but can be as extensive as till the ora, giving a pseudoappearance of leukocoria and masquerade conditions like retinoblastoma or retinopathy of prematurity. A thorough dilated fundus examination is a must in all such cases, preferably under anesthesia, to prevent misdiagnosis and mistreatment. As long as the retrolental membrane is neither in the visual axis nor causing any coexisting traction, observation is warranted in such cases.The authors declare that they have no competing interests.The patient has viewed the content and images of this case report and has consented to the submission of the case report for publication.


Described is an atypical presentation of a rare condition. It highlights the importance of thorough algorithm of medical and family history, physical examination, appropriate investigations, and perioperative workup and for surgery. The report demonstrates how even such very rare nonsecreting paragangliomas can be secondary to mass effects. Described is an atypical presentation of a rare condition. It highlights the importance of thorough algorithm of medical and family history, physical examination, appropriate investigations, and perioperative workup and for surgery. The report demonstrates how even such very rare nonsecreting paragangliomas can be secondary to mass effects. Syncope was preceded by 15 minutes of flushing, nausea, and palpitations. She reported similar episodes in the preceding months. Assessment included normal vital signs, cardiopulmonary auscultation, neck examination, cranial nerve examination, electrocardiogram, echocardiogram, thyroid function, CBC, and CMP. Head/neck CT revealed bilateral carotid body tumors (Figure The larger carotid body tumor on the left was excised, and pathology revealed a paraganglioma with positive synaptophysin Figure  and chroNone to declare.DPL: was an author. TDH and MKMS: were reviewers and editors.This manuscript has been reviewed and approved by the IRB and Public Affairs Office.


Tailgut cyst (TGC) is a rare congenital disease that originates from residues of the tail intestine during the embryonic period. Most TGCs are benign lesions and the malignant transition is very rare.A 50-year-old woman attended our department complaining of defecation difficulty for more than 2 months. She reported irregular defecation with a small amount of liquid stool, 3 to 4 times per day.Biochemical analysis showed high levels of carcinoembryonic antigen  and carbohydrate antigen 199 . Abdominal computer tomography and magnetic resonance imaging showed a large cystic mass with enhanced signals. Post-surgical histopathology indicated that the mass was a TGC with adenocarcinoma transition.The cyst was completely resected. Symptomatic treatment was further performed, and the patient recovered well.We reported a rare case of a large TGC with adenocarcinoma transition. CT, MRI, and histopathology are important to diagnose TGC. Complete surgical resection is the first choice to treat TGC. TGCs are often located in the space behind the rectum and ahead of the sacrum. In addition, the upper boundary of the space is the peritoneal reflex; the lower boundary is the levator ani muscle; and the bilateral boundaries are the bilateral ureters, iliac vessels, and sacral nerve roots.,4 TGC was first reported by Middelporf in 1885 and was considered as a remnant of the tail intestine. Most TGCs are benign lesions and malignant transition is very rare. In this study, we report a rare case of a large TGC with adenocarcinoma transition. We summarize its clinical features, diagnosis, and treatment methods.Tailgut cyst (TGC), also known as retrorectal cystic hamartoma, is a rare congenital disease that originates from residues of the tail intestine during the embryonic period.2This study was approved by the Ethics Committee and institutional Review Board of the Second Hospital of Jilin University, Changchun, China. The patient provided informed consent for publication of the case.A 50-year-old woman attended our department with defecation difficulty for more than 2 months. She complained about irregular defecation with a small amount of liquid stool, 3 to 4 times per day. There was no blood or mucus in the stool. One month earlier, the symptoms of irregular defecation worsened, and abdominal distention occurred that was mainly located in the middle and lower abdomen. The abdominal distention was slightly alleviated after defecation. In addition, there was no accompanying abdominal pain. The patient denied any history of hypertension, coronary heart disease, diabetes, or infectious diseases. She had received a Cesarean section 25 years ago and received surgical treatment for teratoma 12 years ago. A digital rectum examination (lithotomy position) indicated a 3 × 3 cm sized mass that showed good mobility upon touch, which was at the 6 o’clock orientation and was 4 cm from the anal edge. The upper margin of the tumor was not tough.The patient's carcinoembryonic antigen (CEA) level was 79.89 ng/mL  and her carbohydrate antigen 199 level was 57.60 U/mL . There were no other abnormal biochemical indicators. A pelvic ultrasound examination revealed a 90 × 80 mm sized echoic mass in the pelvic cavity that had many separations. Abdominal computed tomography (CT) found gallstones Good exposure of surgical field and sufficient operating space are vital for successful surgery. Based on the anatomical site of the TGCs, there are 4 main surgical approaches, including the sacrococcygeal approach, the single abdominal approach, the combined sacrococcygeal approach, and the anal approach.(2)The TGCs should be sharply separated from the outside to inside along the TGC envelope.(3)For cysts that are large, deep, and difficult to remove, punctuation to reduce the cyst volume should be performed.(4)If the cysts have infiltrated nearby organs or tissues, combined organ resection can be performed.(5)The principle of surgery is to completely remove the cyst; however, for patients whose cysts cannot be completely removed, electrocoagulation cauterization can be used. For TGCs with malignant transformation, post-surgical adjuvant therapy is suggested to kill the residual tumor tissues and improve patient prognosis. For TGCs with neuroendocrine tumor transformation, the administration of postoperative somatostatin might improve patient prognosis. In addition, tyrosine kinase inhibitors and an oral mechanistic target of rapamycin inhibitors have been found to increase patients’ progression-free survival of patients. For TGCs with adenocarcinoma and squamous carcinoma transformation, surgical resection followed by radiotherapy and chemotherapy is still the main treatment method. However, few cases of TGCs with malignant transformation are reported; therefore, the metastatic potential of TGCs with malignant transformation is unclear.TGCs with malignant transformation are always diagnosed using postsurgical histopathology. The 3 most common types of malignant transformations in TGCs are neuroendocrine tumor, adenocarcinoma, and squamous carcinoma.In the present study, we reported a rare case of a large TGC with adenocarcinoma transition. CT, MRI, and histopathology confirmed the diagnosis of TGC. Complete surgical resection of the TGC was performed successfully. We summarized the clinical manifestations, diagnostic methods, and treatment experiences of TGC.Jiannan Li and Shuang Wang designed this study. Min Wang wrote this study. Guoliang Liu, Yu Mu, Hongyu He, and Min Wang performed the surgery. Jiannan Li confirmed the final form of this study.


Optic disc melanocytoma is an ophthalmic tumor that arises from melanocytes, and is a variant of the melanocytic nevus. Here we report 2 cases of optic disc melanocytoma in Asian patient: one associated with normal tension glaucoma (NTG), and the other associated with angle closure glaucoma (ACG).Case 1 is a 57-year-old Asian female presented to our department for a general ophthalmic examination. Incidentally, brownish pigmented lesion was found on dilated fundus examination of her right eye. The fundus examination and optical coherence tomography (OCT) examination revealed a mass within optic disc, and superotemporal retinal nerve fiber layer (RNFL) thinning. The Humphrey visual field test showed corresponding visual field defect. Fluorescein angiography showed no leakage around the lesion. Case 2 is a 78-year-old Asian woman presented with complaints of acute bilateral ocular pain. The initial examination revealed shallow anterior chamber. Under the impression of intermittent angle closure attack, prophylactic laser peripheral iridotomy were performed. On dilated fundus examination, black pigmented lesion was found at superior sector of optic disc. Further examination revealed bilateral superotemporal, inferotemporal RNFL thinning on OCT, and spatially corresponding visual field defects.Clinical diagnosis of NTG was made for case 1 patient. Although it was a little distant from typical glaucomatous changes, nevertheless she had RNFL defect compatible with visual field defects. Considering her normal IOP and angle structures, we believe NTG was a probable diagnosis for the patient. In case 2, we made diagnosis of ACG presenting as intermittent angle closure attack because of her presenting symptoms, narrowing of anterior chamber and angle structures found on gonioscopic and slit lamp examinations.In Case 1, we prescribe 0.005% latanoprost ophthalmic solution. In Case 2, at first prophylactic laser peripheral iridotomy was performed. Then, topical eyedrops administration was started, and the patient was examined periodically.In Case 1, at 6 months’ follow-up, OCT and visual field test showed no progression. In Case 2, to this date, the optic disc melanocytoma remains stable for over a 6-year-follow-up period.The fact that NTG and ACG can coexist in patients with melanocytoma of optic disc should be recognized, and the possibility of such should appropriately be evaluated. Melanocytoma typically occurs in the area of optic disc, and it rarely occurs in ciliary body, iris, choroid, even conjunctiva.,4Melanocytoma is a benign melanocytic lesion that arises from uvueal melanocytes of neural crest, and is considered congenital.–7 Hence, we intend to report 2 cases of optic disc melanocytoma that were associated with glaucoma in Asian patients: one associated with normal tension glaucoma (NTG), and another associated with angle closure glaucoma (ACG).Although there has been several case reports regarding melanocytoma found within optic disc, there were only a few reports on its association with glaucoma.22.1A 57-year-old Asian female presented to our department for a general ophthalmic examination. Her medical history and review of systems were unremarkable. On initial examination, her best corrected visual acuity was 20/20 OD in both eyes, intraocular pressure (IOP) was 14 mmHg in the right eye and 18 mmHg in the left eye. Other than mild cataract, slit lamp examination was within normal limits. Furthermore, there was no peripheral anterior synechiae and widely opened angle was observed on gonioscopic examination. On dilated fundus examination, brownish pigmented lesion on optic disc was found. The optical coherence tomography (OCT) examination revealed a mass within optic disc, and superotemporal retinal nerve fiber layer (RNFL) thinning. The Humphrey visual field test showed an inferior nasal step corresponding to superotemporal RNFL defect. Fluorescein angiography showed neither any filling delay in early phase, nor any peripapillary leakage in early and late phase. Thus, under the diagnosis of NTG in right eye, we prescribe 0.005% Latanoprost  ophthalmic solution. At review at 6 months, melanocytoma remained stable, with no evidence of tumor growth. Further superotemporal RNFL thinning, deterioration of visual function was not observed in follow-up OCT, visual field test  of her right eye. Axial length was somewhat short as it was measured to be 22.84 mm in the right eye and 22.89 mm in the left eye. Under the impression of intermittent angle closure attack, immediate prophylactic laser peripheral iridotomy (LPI) was performed.After a successfully performed LPI, dilated fundus examination was performed, in which black pigmented lesion was found at superior sector of optic disc. Further examination revealed bilateral superotemporal, inferotemporal RNFL thinning on OCT, and spatially corresponding visual field defects. Dorzolamide-Timolol (Cosopt) and Brimonidine (Alphagan) administration was since started, and the patient is examined periodically. To this date, the optic disc melanocytoma and RNFL thickness remain stable for over a 6-year-follow-up period Fig. .3 In 1965, Zimmermann reported 2 white cases of iris melanoma, in which it was later revealed to be iris melanocytoma after enucleation. Melanocytoma, in many cases, is asymptomatic and found coincidentally. However, impairment of visual acuity can occur in 26% of the cases due to retinal exudate involving fovea or neuroretinitis, secondary to tumor necrosis. In rare instance, severe visual impairment can occur due to spontaneous ischemic necrosis, malignant change or secondary retinal vein occlusion. Enucleation is usually considered in such cases, but it should be recognized in advance that the impairment can be induced by transient ischemic necrosis or tumor-promoting inflammation, and that it can later show improvement.,8 Intraocular melanocytoma typically occurs within optic disc, but there were only a few reports on its association with glaucoma. Hence, we intend to report 2 cases of optic disc melanocytoma associated with NTG and ACG, respectively.Originally, melanocytoma was thought to be malignant due to its morphological similarity to melanocytosis. Similarly, no significant change in tumor size and corresponding visual field alterations was observed in our cases as well.Visual field abnormalities are seen in upto 90% of optic disc melanocytoma patients, commonly without any significant decline in visual acuity. It can manifest as an enlarged blind spot when the tumor extends beyond the disc margin, whereas arcuate defect is related to compression of nerve fiber bundle. This visual field impairment is known to change with increase in tumor size, but there has been no reports that studies changes of visual field defect according to increased tumor size in Asian population. According to a study of optic disc melanocytoma in Korean population, it was observed that minor tumor growth was observed in 0% of the patients by 1 year, 5% by 2 years, 14% by 5 years and 57% within 8 years. An average tumor enlargement of 1.8 mm was observed in 53 months of observation.In western population, in a review of 116 cases, Shield et al reported that minor tumor enlargement was observed in 11% of the patients within 5 years, and 32% within 10 years. However, there a few limitations to case 1. First, since optic disc melanocytoma is able to induce compressive RNFL defects and subsequent visual field defects that is similar to NTG, the possibility of such cannot be completely excluded in the first case. Second, glaucoma, by definition, is a progressive optic neuropathy characterized by corresponding loss of vision. This entails a long term follow up study is required to make a definite diagnosis of NTG in this patient. In the second case, ACG was associated with optic disc melanocytoma, and to our knowledge, this is the first report of such instance. A case of secondary acute angle closure attack has been reported that was caused by neovascular glaucoma due to retinal vein occlusion secondary to tumor necrosis. However, the relevance between optic disc melanocytoma and ACG has not yet been established.After a thorough literature review, we recognized that our first case is the first reported case of NTG associated with optic disc melanocytoma in Asian patient, other than a case of NTG associated with optic disc melanocytoma recently reported by a Japanese research group. However, ACG in this case may also have been caused by microscopic invasion of melanocytoma into iris or ciliary body, which may not be evident on gonioscopic examination. Therefore, different imaging modalities, such as ultrasound biomicroscopy, may be required to meticulously inspect anterior chamber and structures behind it.In the second case, the patient had shallow anterior chamber depth and short axial length. It suggests a possibility of coincidental occurrence of 2 clinical entities, due to high ethnical prevalence of glaucoma in Asians.,13 Recently, one group reported the case series showing that circulatory disorder caused by the optic dis melanocytoma might be correlated with visual field defect. Therefore, in optic disc melanocytoma patients, long-term monitoring of visual field changes and RNFL, perifovea, ganglion plexiform layer with OCT angiography is necessary. In case of disease progression, correlation between changes of tumor size and visual field defects should be analyzed, to detect progression of either one or both of the conditions.As it was mentioned above, RNFL and visual field defects can be caused by compressive effects of optic disc melanocytoma. Hence it is necessary to differentiate whether the RNFL defect and corresponding visual field changes are due to glaucoma itself or compressive effects of optic disc melanocytoma, though it is very challenging to do so. It would also be important to distinguish whether the visual field progression during follow-up period is due to tumor growth or glaucoma itself. In recent studies, OCT angiography has been used to study decreased perfusion around RNLF layer.4To conclude, 2 cases of optic disc melanocytoma associated with glaucoma, one with NTG and the other with ACG, were presented in this report. A physician should always keep in mind the possibility of the 2 disease entities occurring concurrently, glaucoma evaluation should be performed for such patients for initial and follow-up visits.Conceptualization: DSK, HMP, HWL and WJLData curation: DSK, HMP, HWL and WJLFormal analysis: DSK, HMP, HWL and WJLFunding acquisition: HWL and WJLInvestigation: WJLMethodology: WJLProject administration: WJLResources: WJLSupervision: WJLValidation: WJLVisualization: WJLWriting – original draft: DSK, HMP, and WJLWriting – review & editing: HWL and WJL


Amyand’s hernia is a rare condition approximately 0.4–0.6% of all inguinal hernias. Although rare, the Amyand’s hernia is worthy of discussion since the variable presentation that make clinical challenge to diagnose especially in infant. A mesenteric chylous cyst is rare disease and has not been reported in Amyand’s hernia.We report an unusual case of Type II Amyand’s hernia with an enlarging chylous mesenteric cyst on the retrocaecal in the anulus into canalis inguinalis. A-2-months old infant presented with enlarging mass in the right scrotal. During laparotomy exploration, we found inguinal sac with intestinal and appendix content in the sac. In the edge site of the sac we found enlarging of mesenteric cyst on the retrocaecal in the anulus into canalis inguinalis. Based on the histopathology examination, the morphological feature is suitable for mesenteric chylous cyst appearance.Presentation of mesenteric chylous cyst is rare, and there was no report about it in Amyand’s hernia. This unusual presentation should be considered by the surgeon, especially pediatric surgeon, in Amyand’s hernia cases. Amyand’s hernia is a rare condition approximately 0.4–0.6% of all inguinal hernias where the vermiform of appendix is found in inguinal hernia sac . An incaA-2-months old infant presented with enlarging mass in the right scrotal. On physical examination, the enlarging mass was found with diameter 10 cm non-fluctuant, non-erythematous which was fixed and mildly tender to palpation (Fig. In the emergency department the patient underwent to take plain abdominal radiography. There was intestinal like appearance in the scrotal region and some dilated intestinal but no evidence of intestinal obstruction or aeration in the scrotal compartments Fig. . The advDuring laparotomy exploration, we found inguinal sac with intestinal and appendix content in the sac Fig. a. The inBased on the histopathology examination, the cyst resection is form of a swollen connective tissue (red arrow in Fig. In 1735 at the St. hospital George in London, dr. Claudius Amyand succeeded in carrying out the first appendicitis surgery in the world. The patient is an 11-year-old boy with a diagnosis of inguinal hernia combined with acute appendicitis. During the operation, dr. Amyand finds the appendix in the hernia sac . This raAmyand ‘s Hernia commonly located on the right side due to the location of the appendix. Approximately 0.1% of Amyand’s Hernia cases, appendix could be inflamed . TherefoAmyand’s Hernia commonly performs as incarcerated inguinal hernias that make it very difficult to diagnose only by clinical assesment especially in infant. Preoperative diagnosis of Amyand’s Hernia can be established using investigations in the form of ultrasound or CT scan. Mostly cases of Amyand’s Hernia are diagnosed intraoperatively during the operation process . In thisIn Amyand’s Hernia, the appendix can be found together with the right cecum or colon in the hernia sac. In some cases it can also be found a bladder, ovary, fallopian tube, omentum or meckel diverticulum in the hernia sac . In thisMesenteric cysts is a rare pathologic entity that identified in approximately 1 out of 100,000 adult and 1 in 20,000 paediatric in hospital admissions . A mesenAmyand’s hernia has variability in presentation that makes it difficult to diagnose clinically in infant and mostly identified intraoperatively. Presentation of mesenteric chylous cyst is rare, and there was no report about it in Amyand’s hernia. This unusual presentation should be considered by the surgeon, especially pediatric surgeon, in Amyand’s hernia cases.


Splenic rupture is a potentially life-threatening condition, often associated with chest or abdominal trauma. Spontaneous rupture is very rare and is usually reported as being secondary to underlying pathological conditions. We report a case of atraumatic splenic rupture in a patient with no underlying disease pathology. This case should remind the emergency physician spontaneous splenic rupture should be considered in the differential diagnosis of unexplained acute abdominal pain. Splenic rupture is mainly caused by trauma. But in some rare cases, it can also occur without obvious trauma, known as atraumatic splenic rupture (ASR) or spontaneous spleen rupture. ASR is often life threatening due to the delay of diagnosis and treatment.3. After resuscitation measures and transfusion of 4 units of red blood cells transfusions, the ultrasound examination showed echogenic peritoneal effusion with hypoechognic mass of the left hypochondrium  [Atraumatic splenic rupture was first documented in the 19 [et al.  in 1985. [et al. . The inc (8/251) . ASR are (8/251) . The eti (8/251) . Spontan (8/251) .Spontaneous splenic rupture without a history of trauma is an uncommon life-threatening abdominal emergency. The pathogenesis of the disease remains unclear. In patients with atraumatic left hypochondrial pain and low hemoglobin, splenic rupture should be kept in mind.


Any instrumental examination may lead to unexpected diagnosis that in turn can radically change the clinical pathway of a patient. Any instrumental examination may lead to unexpected diagnosis that in turn can radically change the clinical pathway of a patient. Breast ultrasound is of paramount importance for the prevention of breast cancer. In this report, we describe the unexpected finding of a mediastinal teratoma in a young, asymptomatic woman, who eventually required surgical excision.A 43‐year‐old woman was referred to our hospital after a mediastinal mass was incidentally found on a breast ultrasound performed for screening purposes. Chest computed tomography, magnetic resonance imaging, and transoesophageal echocardiogram revealed a heterogeneous, 10 × 6 cm cystic mass with areas of septal calcifications in the anterior mediastinum Figure  and was Authors have no conflict of interest to disclose.FC and NP: wrote the manuscript. PF: proofread the manuscript and helped with English language editing. MM: performed surgery and supervised the scientific content of the paper. PB: performed surgery and supervised the scientific content of the paper. CC: performed echocardiography and collected related images. AC: performed histologic analysis and collected related images.


Hepatic venous outflow obstruction (HVOO) is a rare complication of hepatectomy. We report a case of HVOO caused by remnant liver migration into the subphrenic space after hepatectomy, which was successfully managed by repositioning of the remnant liver.A 55-year-old Japanese man was diagnosed with a liver tumor on ultrasound. Contrast-enhanced CT revealed early enhancement in the arterial phase, followed by a washout in the late phase. Preoperative diagnosis was hepatocellular carcinoma, and hand-assisted laparoscopic extended posterior sectionectomy was performed. On postoperative day 1, middle hepatic vein (MHV) flow was not detected on ultrasound, and the portal flow was hepatofugal. CT during arterial portography revealed absence of the portal flow to the medial and anterior sections, and remnant liver migration into the subphrenic space. Therefore, we suspected that HVOO was caused by the remnant liver migration and performed redo laparotomy to reposition the remnant liver with suturing of the falciform ligament to the anterior abdominal wall. Postoperatively, contrast-enhanced CT demonstrated that the remnant liver remained in the anatomical position, and the medial and anterior sections were well enhanced.HVOO might occur irrespective of whether the left triangular ligament is preserved. We believe that it is necessary to fix the remnant liver to the abdominal wall in cases with poor venous blood flow confirmed by intraoperative ultrasound. If kinking of the hepatic vein persists, stent insertion should be performed.HVOO after hepatectomy is rare but potentially fatal, and prevention and countermeasures should be discussed. HVOO is a lethal complication in living donor liver transplantation (LDLT); its incidence is 3.3%–12.5% in LDLT .24 /μL, and prothrombin time 105%. The Child–Pugh score was 5. Hepatitis virus serological markers were absent. The tumor marker protein-induced vitamin K absence-II was elevated (315 mAU/mL). Other tumor markers were within normal range: α-fetoprotein 5.0 ng/mL, carcinoembryonic antigen 1.3 ng/mL, and carbohydrate antigen 19-9 25.0 U/mL. Abdominal US revealed a round hypoechoic nodule in segment 7, measuring 44 mm in diameter. Contrast-enhanced computed tomography (CT) revealed early enhancement in the arterial phase, followed by a washout in the late phase  performed during his annual medical check-up. He had a history of appendicitis and fatty liver. On physical examination, there were no abnormalities. Laboratory tests showed no remarkable abnormalities: aspartate aminotransferase (AST) 23 U/L, alanine aminotransferase (ALT) 37 U/L, alkaline phosphatase 165 U/L, γ-glutamyltransferase 36 U/L, total bilirubin (T.bil) 0.9 mg/dL, albumin 4.70 g/dL, platelet count 19.8 × 10te phase . On magnte phase . Additiote phase . He has 3The incidence of HVOO after hepatectomy (0.1%) is lower than that after LDLT 3.3%–12.5%). Some researchers report that this is because the preservation of the supporting tissues such as the left triangular ligament and the main trunk of MHV prevents migration of the remnant liver into the subphrenic space %. Some r. HoweverWe searched the PubMed database using the keywords “hepatectomy” and “outflow obstruction.” This search yielded 15 cases of HVOO after hepatectomy; these cases are summarized in HVOO might occur irrespective of whether the left triangular ligament is preserved. Some researchers recommend routine fixation of the remnant liver to the anatomical position using the falciform ligament for the prevention and treatment of HVOO. In the present case, repositioning was effective. We believe that it is necessary to fix the remnant liver to the abdominal wall in cases with poor venous blood flow confirmed by intraoperative US. If kinking of the hepatic vein persists, stent insertion should be performed.4After right hepatectomy, the remnant liver tends to spontaneously rotate around the inferior vena cava, which can cause HVOO owing to kinking of the hepatic vein. In the present case, HVOO was successfully managed by repositioning. However, in some cases in which repositioning fails to prevent or treat HVOO, stenting is required.The authors declare that they have no competing interest.The authors declare that this work was not supported by any grants or funding.Ethical approval was obtained from the Ethics Committee of Kurume University (No. 2020-038).Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.HK drafted the manuscript. KO and YA supervised the study. AY, YG, and TH performed perioperative management of the patient.Not applicable.Hiroki Kanno.Not commissioned, externally peer-reviewed.


A glomus tympanicum with chronic infectious otorrhea should have a lower threshold for surgical exploration as an occult cholesteatoma may be present in a location difficult to diagnose without performing a tympanomastoidectomy. A glomus tympanicum with chronic infectious otorrhea should have a lower threshold for surgical exploration as an occult cholesteatoma may be present in a location difficult to diagnose without performing a tympanomastoidectomy. Such tumors can occur at any site within the body. It is the most common primary neoplasm of the middle ear, and the second most common tumor of the temporal bone.Although these lesions are histologically benign, they tend to be slow growing, locally destructive, spread along the path of least resistance.Glomus tympanicum Glasscock‐Jackson classification includes type 1 which is a small mass limited to promontory, type 2 which is tumor completely filling middle ear space, type 3 which is tumor filling middle ear and extending into mastoid, and type 4 which is tumor filling middle ear, extending into mastoid or through tympanic membrane to fill external auditory canal and may extend anterior to carotid.In this case report, we highlight an unusual and rare presentation of an extensive glomus tympanicum tumor with a coexisting atticoantral cholesteatoma.2The case report protocol number 1584204‐1, titled Unusual presentation of a large glomus tympanicum with a coexisting cholesteatoma was approved by Franciscan Research Administration institutional review board. A 59‐year‐old male with a past medical history of asthma, hypertension, and ocular migraine presented with a long‐standing right unilateral hearing loss that becomes more apparent about 8 years ago. Hearing loss was nonfluctuating and was profound to the point he could not use a telephone. His right ear had otorrhea ongoing for years that typically was foul smelling and only temporarily resolved with both oral and ototopical antibiotics. Over the past year, he required antimicrobial therapy every 2‐3 months. Patient denies vertigo, headaches, otalgia, and previous ear surgeries. He reported tinnitus that was high‐frequency nonpulsatile in nature. Examination initially demonstrated purulence in the ear canal along with some squamous debris. After debridement of the ear canal and administration of oral and ototopical antimicrobial therapy, further examination demonstrated a polypoid mass completely filling about half of the ear canal representing a glomus tumor. There was no visualization of a tympanic membrane or ossicular chain . A computed tomography (CT) temporal bones without contrast and magnetic resonance imaging (MRI) of brain and internal auditory canal (IAC) with and without contrast were ordered for further evaluation.CT temporal bones without contrast showed partial opacification of the right external auditory canal with complete opacification of the right middle ear cavity and hypoplastic right mastoid air cell. Tegmen tympani was grossly intact. Erosive changes of the scutum as well as erosive changes of the right middle ear ossicles including the malleus, incus, and stapes were noted. There was questionable erosion involving the canal wall of the tympanic segment of the right seventh cranial nerve. The right inner ear structures including the cochlea, vestibule, and semicircular canal appeared to be grossly intact Figure . MRI didAfter reviewing the results, patient agreed to proceed forward with surgery. A right canal wall down tympanomastoidectomy with facial nerve preservation was performed. The glomus tympanicum was completely excised from the ear canal, middle ear, and mastoid. Partial dehiscence was noted along the middle ear segment of the fallopian canal. The ossicular chain was mostly eroded, and the tympanic membrane was completely eroded. Erosion of the scutum was confirmed. The chorda tympani was not found. Adjacent to the glomus tumor was a cholesteatoma in the attic extending into the mastoid antrum, which was completely excised Figure . The cho3Coexisting pathology of the temporal bone has been previously reported. One report demonstrates that 11 percent of temporal bones were found to have more than one coexisting pathologic finding such as otitis media, otosclerosis, endolymphatic hydrops, labyrinthitis, and cancer when studied postmortem in an otopathology laboratory.Our case demonstrated a nonclassical presentation of a Glasscock‐Jackson type 4 glomus tympanicum tumor with a coexisting cholesteatoma. The atypical polypoid appearance of the glomus tympanicum tumor has been previously described where the tumor extending into the external auditory canal mimicked a polyp covered by squamous epithelium.Previous to an Otolaryngology referral, the patient in our case had been repeatedly treated for otitis media due to his ear examination demonstrating otorrhea and symptoms of hearing loss for many years. It is possible the polypoid nature of his ear examination was confused for a bulging tympanic membrane during this time. After treatment, the otorrhea would temporarily improve giving an opportunity for a reliable ear examination upon follow‐up. The delay in diagnosis may have occurred due to the lack of such follow‐up to confirm resolution of otitis media on examination. This demonstrates the importance of a follow‐up examination after treatment versus trusting resolution of nonspecific symptoms such as otorrhea.The glomus tumor was diagnosed in the office only after temporarily clearing the infectious otorrhea using antibiotics. Cholesteatoma coexisting with a glomus tympanicum has only once been previously reported in literature representing a rare clinical entity.Cholesteatomas tend to have some similarities with glomus tumors such as being locally destructive, nonmetastasizing, and causing conductive hearing loss. Cholesteatomas unlike glomus tumors tend to present with chronic otorrhea similar to our case. Extensive glomus tympanicum presentation where the neoplasm extends into the external auditory canal with coexisting chronic infectious otorrhea should have a high clinical suspicion for a cholesteatoma.The primary management of such extensive glomus tumors is surgery. In certain cases, radiosurgery has been performed in patients with high surgical morbidity and mortality. This is due to the fact that glomus tumors are vascular in nature. One retrospective study has shown that stereotactic radiosurgery is an effective upfront option with or without eventual surgical therapy in the management of glomus tumors.4In conclusion, nonclassical presentation of a glomus tympanicum with chronic infectious otorrhea should have a lower threshold for surgical exploration as an occult cholesteatoma may be present in a location difficult to diagnose without performing a mastoidectomy.None declared.Kartike Gulati: performed evaluation and surgical management for the patient mentioned in the manuscript, wrote the manuscript, and took charge of the overall planning and direction.The case report identification number 1584204‐1, titled Unusual presentation of a large glomus tympanicum with a coexisting cholesteatoma was approved by Franciscan Research Administration institutional review board. Written informed consent was obtained from the participant(s) of this study.


This case represents the first report of malignant primary cardiac tumour in a patient with Lynch Syndrome associated with MSH2 pathogenic variant.A 57-year-old woman with previous ovarian cystadenocarcinoma was admitted to the emergency room for hematic pericardial effusion. Multimodal diagnostic imaging revealed two solid pericardial vascularized masses. After pericardiectomy, the final histological diagnosis was poorly differentiated pleomorphic sarcomatoid carcinoma. During follow-up she developed an ampulla of Vater adenocarcinoma. Genetic analysis identified an MSH2 pathogenic variant.This case contributes to expand the tumour spectrum of Lynch syndrome, suggesting that MSH2 pathogenic variants cause a more complex multi-tumour cancer syndrome than the classic Lynch Syndrome. In MSH2 variant carriers, symptoms such as dyspnoea and chest discomfort might alert for rare tumours and a focused cardiac evaluation should be considered. Lynch syndrome (LS) is an autosomal dominant disorder caused by a germline mutation in one of the DNA mismatch repair (MMR) genes  or by a loss of expression of MSH2 due to deletion in the EPCAM gene , 4; indeA 57-year-old woman was admitted to our emergency room for worsening dyspnoea. Her past medical history included salpingo-ovariectomy for an ovarian cystadenocarcinoma in 1996 . Unfortunately, histological details of this tumour were unavailable.At clinical examination the patient appeared sweaty, with low blood pressure and turgor of jugular veins JVP: 12 cmH2O). Transthoracic echocardiography .Chest-abdomen computed-tomography (CT) Fig. b revealeConsidering the echocardiographic stability of the pericardial effusion, in contrast with the CT findings, a cardiac magnetic-resonance (MR) Fig. c was per18F-FDG PET/CT) and a focal area of radiotracer hyper-accumulation in the anterior pericardium recess (SUV max 12.6) was identified . In order to further characterize such lesion, an echoendoscopy was performed showing a 25 mm mass near Vater’s ampulla, infiltrating biliary and pancreatic tracts. Histological analysis indicated an infiltrating adenocarcinoma.Cardiac-MR and The patient underwent pancreaticoduodenectomy and histological examination confirmed the presence of moderately differentiated ampulla of Vater adenocarcinoma with polypoid extension in the biliary tract and in the duct of Wirsung; there was no involvement of the lymph nodes and the surgical margins were neoplasia-free .The patient did not undergo chemotherapy treatment due to the disease staging but continued with close clinical and instrumental follow-up. Chest-abdomen-CT and cardiac-MR performed during follow-up did not show disease relapse either in the cardiac chambers and pericardium or in the duodenum. showed a history of uterine cancer in the paternal grandmother and in two of her sisters, while two paternal cousins were reported to have developed colorectal cancer. Clinical cancer genetic testing (NGS-based 27-gene panel) detected the germline pathogenic variant c.171_172insATCCGGGTGA (p.F58Ilefs*27) of the MSH2 gene, which was diagnostic for LS7. This made it possible to implement a proper endoscopic surveillance programme for the patient and to offer predictive genetic testing to relatives. The daughter, who as a child had been treated for Acute Lymphoblastic Leukemia, was proven to carry the MSH2 variant as well. Targeted testing was then offered to other family members and demonstrated that the variant had been inherited from the father, who developed colorectal cancer shortly after the genetic diagnosis in the proband (at the age of 83), while was absent in the healthy 53-year-old proband’s sister. Among the clinically relevant mutations found in the tumour tissue, the MSH2 variant was the same as detected by germline testing, while the MLH1 and TP53 mutations were confirmed to be somatic by both the germline panel test and targeted Sanger sequencing.Due to the very rare cardiac tumour histotype, a molecular characterization of the cardiac tumour (other tumours tissues were unavailable) was performed by FOUNDATIONONE®CDx, a commercially available Next Generation Sequencing (NGS)-based analysis of hundreds of cancer-related genes. Pericardial lesion analysis showed mutations in five genes: CD79A R131fs*61), SETD2 (F1132fs*22), MLH1 (N168fs*4), MSH2 (F58Ilefs*27), TP53 (R175H), as well as a high tumour mutational burden and an intermediate microsatellite status. In the meantime, the patient was referred to our Cancer Genetics Clinic for genetic counselling because of the personal history of early onset multiple malignancies suggesting an inherited cancer predisposition. Extended pedigree collection 1fs*61, SPrimary cardiac tumour are uncommon, with an estimated prevalence around 0.02–0.056%, among these the prevalence of primary pericardial neoplasms ranges from 0.001 to 0.007% . This isOur case supports this evidence, since the patient did not develop the predominant Lynch malignancies , while she did develop rare LS manifestations  and the unusual cardiac tumour hereby described. The case was further characterized throughout tumour NGS analysis, leading to the identification of additional somatic events, with a high mutation burden which is consistent with the underlying DNA MMR defect . This co


We report a case of intra-articular osteoid osteoma (IAOO) of the elbow, in order to point out the clinical and imaging features which made the case challenging and caused a diagnostic delay. A 9-year-old boy presented to the rheumatology consultation with left elbow pain for the prior three months. The pain was associated with swelling, stiffness, and limitation of movement suggestive of inflammation. The blood work was normal. Mono-articular juvenile idiopathic arthritis (JIA) was suspected, and the patient was referred for left elbow ultrasound, which confirmed synovial thickening , involving commonly the hip and very rarely the elbow. Because of the intra-articular location of the tumor, stiffness, pain and decreased mobility are the most common symptoms, simulating arthritis.Whereas the classical radiological presentation of osteoid osteoma is that of a rich osteosclerotic process along the diaphysis of a long bone and centered on a small osteolytic nidus, the imaging features of IAOO are often atypical and lead to a delay in diagnosis. In IAOO, the osteosclerotic reaction is less abundant, sometimes absent. Osteopenia may appear, because of the articular inflammation associated with IAOO. The nidus is more difficult to detect because of its osteopenic surroundings.Even though it was not performed in our case, CT is the best modality to identify the nidus in osteoid osteoma, once the suspected area of interest has been identified. CT is the method of choice to characterize bone changes and may differentiate between osteoid osteoma and chronic osteomyelitis. The role of MRI is controversial because of the variable appearance of the lesion and the reactive changes in the bone marrow and soft tissues .This case shows the importance of identifying atypical X-ray manifestations in the context of arthritis. The lack of response to adequate treatment must suggest an alternative diagnosis and the search for a possible osteoid osteoma in pediatric patients.


Blunt traumatic gastric perforations in children are rare. Delayed diagnosis will lead to abdominal contamination and may result in morbidity and even mortality. We present a case of an adolescent who sustained blunt abdominal injury in a motocross accident and presented with remarkable hyperdense spherical shaped structures on the computed tomography (CT).A 15-year-old boy arrived at the emergency room with an acute abdomen after a motocross accident. A CT scan of the abdomen demonstrated free air and hyperdense round structures in the stomach, pelvic cavity and right paracolic gutter. During emergency laparotomy a traumatic gastric perforation was sutured, a splenic rupture was treated with a vicryl mesh and multiple spherical food scraps were removed from the abdomen. After surgery, the boy clarified that he had eaten a whole bag of colorful and spherical shaped candy just before the accident.Traumatic gastric rupture in children is rare but physicians should be aware of this diagnosis in case of blunt abdominal trauma with free air on the CT scan. Gastric contents, in this case candy, can present as hyperdense shaped structures in the abdominal cavity on the CT scan. Blunt traumatic gastric perforations in children are rare . An inciA 15-year-old, otherwise healthy, boy arrived at the emergency room after a motocross accident. Trauma screening was performed according to the Advanced Trauma Life Support principles. His airway was free and his cervical spine was immobilized. He had a tachypnoea of 28 breaths per minute, with normal vesicular breathing sounds and a saturation of 100%. He had a tachycardia, with 130 beats per minute, a blood pressure of 160/90 mmHg and a painful abdomen on palpation. His consciousness was slightly decreased with an EMV score of 3–5–4. X-ray of the thorax showed opacification, which was interpreted as a lung contusion. The Focused Assessment with Sonography for Trauma demonstrated free abdominal fluids. CT-angiography showed a grade IV spleen laceration and free air in the abdomen. There was no active bleeding. Also, remarkable hyperdense spherical structures were present in the stomach, pelvic cavity and right paracolic gutter (Fig. Traumatic gastric ruptures are rare with an estimated incidence of 0.02–1.7% of all blunt abdominal injury –11. ThisIn conclusion, traumatic gastric rupture in children is rare but physicians should be aware of this diagnosis in case of blunt abdominal trauma with free air on the CT scan. Moreover, certain released stomach contents—in this particular case candy—can present as hyperdense shaped structures at the CT scan.


Endoscopic retrograde cholangiography related duodenal perforation is an infrequent complication and associated with significant morbidity. The management of such perforations, especially in the setting of malignancy, is not standardized given the paucity of literature.We encountered a patient who was diagnosed with periampullary carcinoma and had a perforation in the duodenum during endoscopy. Emergency pancreatoduodenectomy (EPD) was performed considering it to be a resectable disease with minimal contamination. He had a prolonged hospital course due to surgical site infection and hepaticojejunostomy leak, however, which was managed successfully. At one year follow up, he is healthy with no evidence of recurrence.We conclude that EPD can be attempted for selected iatrogenic duodenal perforations with co-existent resectable malignancy in a stable patient. It may help to avoid the morbidity of a second surgery in the setting of a distorted anatomy and simultaneously preventing the probable upstaging of disease due to peritoneal seedling. Emergency pancreatoduodenectomy (EPD) has been carried out mainly for pancreatic trauma, bleeding aneurysms, iatrogenic duodenal perforations, and hemorrhage within a pseudocyst and has proven to be lifesaving in such instances. The mortality rates for EPD varies from 8-40% ,2. HowevA 53-year-old male presented with complaints of painless progressive jaundice with cholestatic features and intermittent episodes of fever associated with anorexia and weight loss for one month. He had no history of prodromal symptoms, malaise, hematemesis, or melena. His liver function tests were as follows - Total bilirubin 8.4mg/dl, direct bilirubin 5.2 mg/dl, alkaline phosphatase -1031 IU/L, Aspartate transaminase-118 U/L, and Alanine transaminase-153 U/L.Ultrasound of the abdomen showed dilated intrahepatic biliary radicles and common bile duct (CBD). Endoscopic retrograde cholangiography (ERC) and stenting was planned in view of raised bilirubin levels with a history of fever. During the procedure, a periampullary growth was noted and before biopsy could be performed, the endoscopist noted an iatrogenic perforation in the second part of the duodenum. The procedure was abandoned and urgent surgical gastroenterology consultation was sought. Following the procedure, the patient was anxious with tachycardia, however, other vital parameters were within normal limits. His abdomen was distended with guarding noted in right hypochondrium and lumbar region. Subcutaneous emphysema was noted extending to the chest bilaterally Figure . ContrasExplorative laparotomy was performed within four hours following the perforation. Intraoperatively, contamination of the right para duodenal space and mesocolon was seen with ~1 x 1 cm perforation seen on the lateral aspect of the second portion of the duodenum Figure -2c. CBD He was transferred to the Intensive Care Unit (ICU), where he was extubated on postoperative day (POD) one, and the inotropic support was gradually weaned off. Feeding through jejunostomy tube was commenced on POD two and oral feeds were started on POD three which he tolerated well. An amylase level from the abdominal drains on POD three was normal and the drains were subsequently removed.On POD eight, he developed surgical site infection (SSI) which progressed to deep space infection. The wound was managed with daily dressings and antibiotics were upgraded according to culture sensitivity reports of the wound. Further, he started having biliary discharge from the surgical site for which CECT abdomen was done which suggested a hepaticojejunostomy leak without any obvious collection. A wound manager was applied on the midline wound and daily irrigation was done. On POD-16, he developed septic encephalopathy for which he had to be intubated and monitored in the ICU and later underwent tracheostomy on POD-22. He subsequently improved and enteral nutrition was continued through a jejunostomy tube. As his nutritional status improved, the bilious discharge from the wound gradually decreased and the wound showed signs of healing. Later, he was restarted on oral feeds on POD-30 and his tracheostomy was downsized and subsequently removed. Finally, his wound completely healed by secondary intention and he was discharged on POD-45 in a stable condition.His pathology report of the lesion was suggestive of ampullary adenocarcinoma (stage pT1N0Mx) with all resection margins free of tumor. At one year follow-up, he is doing well without any evidence of recurrence.Iatrogenic perforation during ERC varies from 0.01 to 2.1% and is associated with considerable morbidity ,4. StapfAlmost 15-40% of iatrogenic perforations occur in the presence of a co-existent malignancy, however, there is a lack of a defined protocol for their management due to scarcity of published literature ,6. The tSurgical intervention is warranted in patients with overt signs of peritonitis, large intra/retro-peritoneal air, hemodynamic instability, or failure of medical management. Surgical management primarily involves draining intra and retroperitoneal fluid collections, primary repair of the perforation with or without duodenal diversion .Emergency Pancreatoduodenectomy (EPD) has been employed for managing iatrogenic duodenal or bile duct perforation in the background of malignancy. On reviewing the literature, only three cases have been reported where successful early within 24 hours) EPD was performed for pancreatobiliary malignancy in which a scope induced perforation occurred  hours EP. Our casEarly definitive surgery may prove beneficial in such cases but given the limited literature describing such cases, it is difficult to make concrete conclusions. The therapeutic benefit may be speculated since perforation in the setting of malignancy may upstage the disease with the risk of tumor seeding in the peritoneal cavity and disease progression. The initial hospital stay may be prolonged in such a setting but it avoids the morbidity of a second definite procedure in which altered anatomy with dense adhesions might make the pancreatoduodenectomy more complex. However, one must pay attention to the early identification, resuscitation, and prompt mobilization of these patients to the operation theatre as delays may contribute to dismal outcomes. Another important aspect is postoperative morbidity, as such patients are liable to have wound-related (16%) and anastomotic leak related (33%) complications ,9. In thAlternative procedures that might have been tried in the emergency setting were a primary repair of the perforation with pyloric exclusion with/without biliary drainage but the rates of mortality and post-operative duodenal leaks in such cases are extremely high . FurtherMortality rates in EP vary from 8-40% with considerable variability depending on the primary pathology, timely surgical intervention, and whether the surgery was therapeutic or diagnostic -3,5,9. DIn conclusion, EPD can be considered selectively in iatrogenic duodenal perforations of patients with resectable periampullary tumors, stable hemodynamics, and an experienced surgeon/center. It is important to consider this in a selective cohort of patients only, given the high postoperative morbidity and mortality rates. Early surgery before the onset of sepsis will have favorable outcomes, however, the real challenge lies in anticipation and early identification of patients requiring surgery.However, in unstable patients with diagnostic uncertainty, primary closure of duodenum with or without biliary drainage with feeding jejunostomy would be a safer option and definitive procedure should be deferred for the future.


Spontaneous esophageal rupture, also called Boerhaave’s syndrome, is relatively uncommon but may result in high morbidity and mortality. Synchronous presentation of spontaneous esophageal rupture and perforated peptic ulcer was rare and may contribute to the difficulty of achieving a correct diagnosis.We reported two patients with spontaneous esophageal rupture following perforated peptic ulcer. Both patients were successfully treated with thoracoscopic primary repair of esophageal rupture. The first patient underwent peptic ulcer repair via laparotomy. The second patient underwent laparoscopic duodenorrhaphy. Both patients resumed oral intake smoothly and were discharged uneventfully.Minimally invasive approaches are safe and feasible for both esophageal rupture and perforated peptic ulcer in patients diagnosed within 24 h and without shock. Spontaneous esophageal rupture, also called Boerhaave’s syndrome, is relatively uncommon but may result in high morbidity and mortality. The classic presentations are chest or abdominal pain, shortness of breath, and shock after forced emesis or cough. Missed diagnosis as myocardial infarction, pancreatitis, and peptic ulcer perforation were reported due to similarities in symptoms . PneumomA 67-year-old male with history of lung adenocarcinoma and brain metastasis suffered from intermittent epigastric pain for several days before presentation. A sudden onset of sharp left chest pain was noted after several episodes of cough. He was brought to the Emergency Department of Changhua Christian Hospital, Taiwan. Initial physical examinations revealed sinus tachycardia without abnormal rhythm and abdominal tenderness with rebound pain. Acute myocardial infarction was initially excluded based on the electrocardiogram findings (absence of diagnostic wave change) and laboratory data . Chest and abdominal computed tomography findings revealed left-sided hydeopneumothroax and mural wall swelling of the lower third of the esophagus with suspicion of perforation. Furthermore, distended stomach and pneumoperitoneum were also noted with a soft tissue around the pyloric region  and an elevated procalcitonin level of 14.13 ng/mL. Chest computed tomography revealed a right-sided hydropneumothorax with mediastinal fluid accumulation around the lower thoracic esophagus. Additionally, pneumoperitoneum was also noted (Fig. The management for spontaneous esophageal rupture includes conservative treatment, endoscopic treatment, and surgical treatment. The factors that determine the treatment choice include the size of perforation; time interval between perforation and presentation; general condition, including shock and nutrition status; and physicians’ or surgeons’ experience and perspectives. De Schipper et al.  reviewedBoth Boerhaave’s syndrome and perforated peptic ulcer are life-threatening diseases. Only three cases have been reported in the literature, and most of them were managed by resection and reconstruction via laparotomy –4. Our fThis report concluded that spontaneous esophageal rupture following perforated peptic ulcer is uncommon but can be life threatening. This differential diagnosis should be kept in mind, and associated examinations should be performed. Minimally invasive approaches are safe and feasible for both esophageal rupture and perforated peptic ulcer in patients diagnosed within 24 h and without shock.


Lipomas are the most common cause of intussusception in adults. To our knowledge, however, no cases of lipoma and ectopic gastric mucosa with gastritis cystica profunda (GCP) have been reported. We report a case of intussusception caused by a small intestinal lipoma with ectopic gastric mucosa containing GCP-component cells within the inverted Meckel’s diverticulum.A female in her 40s underwent computed tomography for postoperative follow-up of left breast cancer. A tumor, suspected to be a lipoma, was found in the ileum. Since there were no symptoms, the patient underwent regular follow-up. However, gradual enlargement was observed, and surgery was recommended due to the risk of intussusception. After reduction via the Hutchinson technique, laparoscopically assisted partial resection of the small intestine was performed due to suspicion that the tumor was causing intussusception starting from the ileum. Histopathologic examinations revealed proliferation of mature adipose tissue in the subserosal layer, which was diagnosed as lipoma. Furthermore, adipose tissue was found in the stem area and accordingly, we diagnosed lipoma associated with the inverted Meckel’s diverticulum. Moreover, gastric mucosa-like crypt epithelium and proper glandular tissue were identified in the mucosal membrane at the area of onset, and signs of gastric pit dilatation over the submucosa and crypt epithelium hyperplasia were observed. Diagnosis was ectopic gastric mucosa containing GCP component tissue.Intussusception in the small intestine complicated with lipoma and ectopic gastric mucosa with GCP within the Meckel’s diverticulum has not been reported, demonstrating the rarity of our case. Intussusception in adults is a relatively rare condition that is often caused by neoplastic disease . IntussuWe report on a rare case of bowel intussusception due to ectopic gastric mucosa with small bowel lipoma and GCP within inverted Meckel’s diverticulum.A female patient in her 40s presented without a chief complaint. She had undergone a partial left mastectomy for left breast cancer 2 years previously and currently was on adjuvant hormone therapy. Postoperatively, the patient was undergoing follow-up examinations at our breast oncology department. Computed tomography (CT) revealed a neoplastic lesion (lipoma) with invagination in the small intestine.At admission, the patient was 161.8 cm tall, weighed 60.6 kg and had a body mass index of 23.15. The abdomen was flat and soft, with no tenderness or palpable mass. Admission laboratory blood tests revealed no noteworthy findings. Plain abdominal CT showed a fatty mass in the small intestine (Fig. Laparoscopic-assisted partial resection of the small intestine was performed with the patient under general anesthesia. A pedunculated neoplastic lesion was found approximately 30 cm from the terminal end of the ileum on the oral side and intussusception was observed over approximately 20 cm on the anal side. Although edema was noted together with the duplicated intestine, there was good color tone and there were no ischemic changes. The intussusception was released via the Hutchinson technique, and partial ileal resection was performed.The resected specimens revealed a pedunculated, club-shaped mass protruding from the mucosa into the lumen connected to the wall of the small intestine. The transverse and long axis tumor diameters were 36 and 78 mm, respectively Fig. a and an Histopathologically, the mucosal epithelium showed inflammatory cell infiltration in the superficial layer of the area of tumor onset and the submucosa showed proliferation of atypical mature adipocytes Fig. a. GastriImmunohistochemical findings Fig. a–d reveaThe patient progressed well postoperatively, and food intake began on day 4 postoperatively. The patient was discharged from the hospital on day 9.We report a rare case of intussusception of the small intestine caused by lipoma with ectopic gastric mucosa including GCP within the inverted Meckel’s diverticulum.Ectopic gastric mucosa originates from the transposition or invasive migration of gastric tissue, mutation and dysplasia of intestinal mucosa , and vitPolypoid mucosa in the gastrointestinal anastomoses of the remnant stomach has been reported as GCP ; patholoHere, gastric crypt-like epithelium and dilated cystic glandular duct structures were observed in the lamina propria and submucosa. G cells did not produce gastrin in the pyloric antrum of the stomach, but CDX2 was expressed in epithelial cell nuclei in the intestine from the duodenum to the rectum . Mucins MUC6 is expressed in gastric pyloric and cardiac glands, gastric accessory cells, duodenal Brunner glands, and esophageal cardiac glands . CDX2, MWe reported on an extremely rare case of a patient with intussusception caused by a small intestinal lipoma accompanied by ectopic gastric mucosa containing GCP within the inverted Meckel’s diverticulum.


Intraparietal inguinal hernias are a rare variant of inguinal hernia in which the hernia sac lies between the layers of the abdominal muscles. Intraparietal inguinal hernias mimic Spigelian hernias clinically; the diagnosis presents superior difficulties than its treatment. We report a case of a giant intraparietal hernia misdiagnosed as a Spigelian hernia clinically. The patient was 83 years old woman presented with complain of a large swelling over right abdomen for around 25 years. The patient had a huge mass of 25 x 30 cm occupying right flank, right lumbar region extending up to the umbilicus and inguinal region, partially reducible with gurgling sounds. Surgery started with transversal incision over the mass, it was found to be an interstitial variety of intraparietal inguinal hernia with a long viable segment of the small bowel with their mesentery as content of the sac. Hernioplasty with a polypropylene mesh was achieved satisfactorily. The patient was discharged on third postoperative day without complications. It is challenging to diagnose intraparietal hernias preoperatively; intraoperative findings defined its definitive diagnosis and its surgical technique. The inguinal hernia is one of the most common surgical diseases in clinical practice; giant inguinal hernia, however, is more unusual and significantly challenging in terms of surgical management when it is presented as a rare variant . IntrapaAn 85 years old black woman presented with complain of a large swelling over right abdomen for around 25 years. The swelling increased progressively over the years and it is associated with post-prandial discomfort. Her bowel habits were normal and there was no urinary complain. There was no history of trauma or any surgical procedure in the past. Her past medical history included hypertension for the last 10 years ago with regular treatment. Her physical examination revealed a huge mass of 25 x 30 cm occupying right flank and right lumbar region extending up to the umbilicus and inguinal region. Cough impulse was present. The mass was round shaped, soft, mild tender and partially reducible with gurgling sound . PercussIntraoperatively it was found to be intraparietal inguinal hernia. Surgery started with transversal incision over the mass, after external oblique fascia opened careful exploration revealed that the hernia sac was lying in between external & internal oblique muscles; the hernia sac was coming out of a very widely dilated deep inguinal ring of about 5 cm diameter  and exteIntraparietal inguinal hernias form a group of rather unusual hernias located between the layers of the abdominal wall in the inguinal region ,3. The iThe clinical presentation with a partially reducible mass and the location of the mass in our patient mimicked a Spigelian hernia. Although diagnosis may be challenging but treatment is simple surgical exploration. The approach of surgical exploration may be laparoscopic or open depends upon the surgeon´s choice and the facility available in hospital . In our We treated a patient with an interstitial intraparietal inguinal hernia that mimics a Spigelian hernia because of the huge size, anatomy location and ultrasonographic findings. The impossibility of performing a CT scan of the abdomen made more difficult to reach an accurate preoperative diagnosis. While clinical diagnosis may be challenging the treatment is always the surgical exploration; once the surgical anatomy is comprehended, the definitive diagnosis is determined and the surgical repair is simple as in our case.


Primary splenic cysts are very rarely diagnosed in pregnancy, with only thirteen cases described in the literature. We examine the approach towards diagnosing and managing uniquely large abdominal masses that significantly complicate obstetric care.A 37-year-old primigravida woman presented with abdominal distension and discomfort, yet otherwise asymptomatic. On ultrasound, an incidental pregnancy at 25 weeks of gestation and a large pelvic lesion were discovered. MRI defined a 28 × 29 cm lobulated, complex cystic mass in the upper abdomen. The patient underwent two ascitic drainages throughout her pregnancy. At 34 weeks of gestation, she had a classical caesarean section. Then at five-weeks postpartum, she underwent a laparotomy and total splenectomy with 16 L of fluid drained. Histopathological analysis revealed an epithelial cyst of the spleen. Her recovery was complicated by complete portal vein thrombosis.This case describes the largest splenic cyst ever reported in pregnancy and explores the diagnostic dilemmas and treatment challenges associated. We introduce the utility of serial ascitic drainages in prolonging the pregnancy and emphasise the reliance on imaging for surveillance of splenic size and fetal wellbeing. A patient with the largest splenic cyst ever reported in pregnancy undergoes caesarean section at 34 weeks, then an open total splenectomy five weeks postpartum.Magnetic resonance imaging is highly beneficial in defining large abdominal masses in pregnancy especially when splenic cysts are suspected.Careful consideration of patient symptoms, splenic cyst size, and gestational age is advised in counselling patients about timing and mode of delivery, as well as general surgical treatment options.True cysts of the spleen are rare clinical encounters with less than 1000 cases reported worldwide. Clinically, splenic cysts are asymptomatic in the majority of cases, otherwise symptoms are non-specific such as vague abdominal discomfort and distension. The management of splenic cysts remains controversial, however traditionally, a surgical approach is advised for symptomatic cysts greater than 5 cm.We explore the diagnostic dilemma and treatment challenges of a giant primary splenic mass found in pregnancy, a pairing described in only thirteen cases worldwide –13. ThisA healthy 37-year-old primigravida Australian Indigenous woman presented to a rural Australian hospital with increasing abdominal distension and discomfort, otherwise asymptomatic. She denied recent overseas travels. She reported amenorrhoea for approximately five months. Her past medical, surgical and family history was unremarkable. She was an ex-smoker, reported recreational alcohol intake, and denied any illicit drug use.On examination, she was obese, and weighed 127 kg. She had a grossly distended, but soft and non-tender, abdomen. Her initial ultrasound scan (USS) showed an incidental intra-uterine pregnancy of 25 + 4 weeks of gestation, cervix long and closed, as well as a complex pelvic lesion with internal septation and vascularity.The patient was transferred to a major tertiary centre for further investigation and surgical optimisation and planning. Magnetic resonance imaging (MRI) defined a large lobulated, complex cystic mass of unknown origin measuring 28 × 28 × 29 cm in the upper abdomen with nodularity and papillary projections from the wall, and a second 7.0 × 4.6 × 9.8 cm cystic lesion abutting the left kidney with internal septation Fig. . ModeratAt 26 + 1 weeks of gestation, the patient underwent her first ascitic drainage of 1.5 l of cloudy brown fluid, which showed no malignant cells or organisms, and overall features suggestive of an inflammatory reaction. Post-drainage, she had a reassuring pelvic USS at that showed an estimated fetal weight of 1005 g (72nd percentile), with normal AFI and dopplers.Echinococcus granulosus serology which came back negative. At 27 weeks of gestation, a chest computed tomography (CT) scan for staging purposes demonstrated enlarged anterior paracardiac lymph nodes, measuring up to 8 mm, however no other lymphadenopathy. Her case was discussed at gynae-oncology multidisciplinary team meeting and the diagnosis was presumed a metastatic ovarian malignancy. Other differential diagnoses included benign ovarian cysts, other abdominal malignancy such as gastric, hepatic or colonic cancer, lymphangioma, infection or inflammation of unknown aetiology, pancreatic abscess or pseudocyst, and splenic enlargement. The preliminary plan was to aim to prolong pregnancy to at least 28 weeks of gestation and steroids 48 h prior to an elective surgery +/− staging.The diagnostic dilemma prompted an Infectious Disease consultation, who tested for At 27 + 4 weeks of gestation, the patient underwent a second ascitic drainage of 4 l of cloudy brown fluid, which also showed no malignant cells. A follow-up MRI was performed and demonstrated stable size of the 32 × 27 cm lobulated cystic lesion. At 28 + 1 weeks of gestation, USS showed steady fetal growth 1161 g (33rd percentile). Given the patient had normal fetal movements, reassuring ultrasound scans, and felt symptomatically well, the patient was discharged and offered outpatient follow up. Her USS at 32 + 1 weeks of gestation showed steady fetal growth 1765 g (20th percentile), with normal AFI and dopplers. An obstetric plan for caesarean section at 34 weeks of gestation was subsequently made, taking into careful consideration the risk of potential malignant spread and the patient’s intensifying abdominal discomfort, versus, the risks associated with an early delivery.With adequate antenatal steroid-loading, at 33 + 6 weeks of gestation, she underwent an elective midline classical caesarean section and exploratory laparotomy with the gynae-oncology team in attendance. As the mass did not appear to arise from the ovaries, Colorectal and Hepatobiliary Surgical teams attended and recommended further imaging before another general surgical procedure. A baby girl was born at 1719 g and discharged in good health after 31 days in the nursery.A follow-up CT scan demonstrated a mass originating from the spleen. At five-weeks post-partum, she underwent a midline laparotomy for excision of the splenic cyst Fig. . InitialEchinococcus granulosus) or non-parasitic. Morgenstern [Splenic cysts are rare; the reported incidence rate is 0.07% according to a large case series of 42,327 autopsies over a 25-year period . Fowler genstern  subdividgenstern –20. Hormgenstern .In terms of demographics, there is a female preponderance ; two-thiDiagnosis of splenic cysts are often incidental during abdominal ultrasonography and can further be defined by CT or MRI scans, in regards to the composition of cystic fluid, the cyst morphology including septations or calcifications, and the surrounding tissue , 24. On Furthermore, there is reasonable utility in investigating tumor markers pre- and post-operatively, as the epithelium lining of some primary splenic cysts may produce CA 19.9, CA 125, and CEA . This reA wide range of approaches for splenic cysts in non-pregnant patients have been described, often dependent on patient age, symptoms, cyst characteristics and size. Nonoperative measures such as observation and regular serial imaging is recommended for asymptomatic, small (< 5 cm) cysts . HoweverStreptococcus pneumonia, Neisseria meningitides and Haemophilus influenza is crucial, as well as stringent postoperative antibiotic coverage for total splenectomies. Today, the optimal treatment options range from cyst fenestration, marsupialisation and partial cystectomy to total cystectomy with partial splenectomy [Classically, the gold standard surgical approach to splenic cysts has been open total splenectomy. However, due to an evolving understanding of the haematological and immunological functions of the spleen, organ-salvaging techniques have since been developed to mitigate the life-threatening risk of overwhelming post-splenectomy infection (OPSI) , 38. Henenectomy –41.In pregnancy, the majority of early case reports of splenic cysts underwent splenectomy either open or laparoscopically –5. In laSince then, five more cases of splenic cysts in pregnancy have been reported, of which, four returned to traditional methods of total splenectomy in the second trimester with success. Dabrowski et al.  and ForoIn light of these case reports, there appears to be no clear consensus, nor any guidelines, for the management of splenic cysts in pregnancy, as it remains a balance between minimally-invasive approaches of aspiration with risks of re-accumulation and further surgery, versus, organ-preserving approaches of cystectomy, versus, the definitive approach of total splenectomy with greater operative risk and OPSI. Therefore, in counselling obstetric patients about their treatment options, besides general considerations regarding symptoms, cyst size, intraoperative and postoperative risks, additional attention to their gestation, fetal wellbeing, and preference for vaginal delivery or caesarean section is recommended.In our case, given the patient was in her second trimester at time of presentation, temporising measures such as serial ascitic drainages provided symptomatic relief as we aimed to prolong the pregnancy to an adequate gestation. The exact etiology of the recurrent accumulation of ascitic fluid remains unclear, however the authors postulate its origin from the splenic cyst, and/or, hepatic venous occlusion from the concealed complete portal vein thrombosis owing to the combined compressive effects of the splenic cyst and enlarging gravid uterus. Due to the size of the mass and its compressive effects on surrounding organs, its origin remained unclear despite the use of multiple imaging modalities throughout her pregnancy. This posed a significant diagnostic dilemma that considerably influenced the mode and timing of delivery, as well as the type of general surgical procedure. Ultimately, a splenic mass of such size required an open total splenectomy.One of the major strengths in this case presentation is the retrospective analysis of our clinical approach towards diagnosing and managing unusually large abdominal masses in pregnancy. The diagnosis of splenic cyst proved a very unique and unexpected finding for all clinicians involved, hence this case serves as a kind reminder for obstetricians and gynaecologists to consider diagnoses outside of the pelvic region for women presenting with extraordinary abdominal distension. This case presentation not only adds to the limited body of literature on splenic cysts in pregnancy, but also provides insight into an interesting combined case between obstetricians and surgeons where all maternal and fetal treatment options are well considered. The authors advise caution in generalising this rare case report to other more common abdominal masses in pregnancy and acknowledge the advantage of treating such complicated cases in a tertiary hospital where multiple specialties, diagnostic tests and treatment options are readily available.In conclusion, though epithelial splenic cysts are rare, it ought to be considered as part of the differential diagnoses in young individuals presenting with abdominal distension. We have introduced to the literature the notion of serial ascitic drainages to alleviate patient symptoms and prolong the pregnancy. This case emphasises the reliance on imaging for diagnosis of splenic cysts and serial surveillance of fetal wellbeing. Furthermore, this case highlights the importance of tertiary-level care and implementing a multidisciplinary approach towards complex clinical cases, encouraging good communication between the patient, obstetricians and surgical specialities in exploring all treatment options and optimising maternal and fetal outcomes.