An intraductal papillary neoplasm involving the biliary tree is an unusual premalignant condition of epithelial origin, identified by its cystic dilatation of the biliary channels. Being a slow-growing tumor, surgery offers the best curative rate, especially in the setting of a low-grade disease. Here, we present a case of a localized, low-grade, intraductal papillary neoplasm of the bile duct (IPNB), residing in the liver, which was treated with resection of the liver lobe. The adjuvant treatment and prognosis highly depend upon the presence of dysplasia or a co-existent invasive malignancy. To the best of our knowledge, being a rare entity, this is the first case to be reported from Pakistan. Intraductal papillary neoplasm of the bile duct (IPNB) is a type of bile duct tumor that is identified as a precursor lesion to invasive cholangiocarcinoma. It originates from the epithelial cells lining the bile ducts and is characterized by a predominantly papillary growth pattern in dilated bile ducts . It is aThere are two distinct subgroups of IPNB, which differ in their location and histology. Type 1 IPNB predominantly involves the intrahepatic ducts, whereas type 2 IPNB is more commonly found in extrahepatic locations. Type 1 IPNB has fewer invasive components, while type 2 IPNB demonstrates more complex papillary structures . There iHere, we discuss a case of a 63-year-old gentleman from Pakistan, who was incidentally diagnosed with IPNB after undergoing partial hepatic resection for a space occupying liver lesion.A 62-year-old gentleman, a resident of Karachi, Pakistan, a civil engineer by profession, having Eastern Cooperative Oncology Group (ECOG) score of 1, and known to have hypertension and dyslipidemia, presented to the outpatient clinic of a tertiary care hospital in Pakistan with complaints of abdominal distension along with abdominal pain for the past three months with no history of fever, nausea, or vomiting. His vital parameters were within normal limits. He denied any history of allergies or addiction. He also denied any history of familial diseases. His systemic examination revealed a distended abdomen with mild tenderness in the right upper quadrant. He got an ultrasound (US) of the abdomen, which showed a suspicious lesion involving the left liver lobe.This was followed by contrast-enhanced computed tomography (CT) of the abdomen, which showed a heterogeneous infiltrative area in the left lobe of the liver with atrophy and intrahepatic biliary dilatation, suggestive of atypical hemangioma versus focal intrahepatic cholangiocarcinoma Figure . His labContrast-enhanced magnetic resonant imaging (MRI) of the abdomen demonstrated slight left liver lobe atrophy with a focal enhancing mass lesion of 78 x 32 mm, with multiple tortuous flow voids, arising from the left lobe. A small exophytic component of 15 x 9 mm, arising from this lesion anteriorly, was also appreciated, being isointense in the arterial phase and has a faster washout relative to the rest of the liver with prominent biliary ducts suggestive of local cholangiocarcinoma (Figure A US-guided liver biopsy was performed, but histopathology showed a scanty tissue sample. After completion of the initial workup, the case was discussed in a multidisciplinary tumor board in our institute, and the patient was advised surgical resection. He subsequently had a left hepatectomy. Intraoperatively, a shrunken left lobe of the liver was appreciated with surrounding parenchymal fibrosis, without any gross ascites and any peritoneal, omental, or bowel deposits suggestive of metastasis. Surgical histopathology showed an intraductal papillary neoplasm of the biliary tree with low-grade dysplasia, confined to the intrahepatic biliary ducts. There was no lympho-vascular invasion or perineural invasion. The bile duct margin and hepatic parenchymal margin were 1 and 0.1 cm, respectively. Post-operatively, the patient was discharged in a stable state. After an initial visit for post-operative care on day 7 of his discharge, he was advised to follow up in the clinic with a post-surgical CT abdomen in four weeks, which did not show any residual or recurrent disease Figure . His CA IPNB is a pre-malignant condition that could be referred to as the biliary counterpart of pancreatic intraductal papillary mucinous neoplasm. It was initially called “papillary carcinoma of the bile duct” by Albores-Saavedra, and the term “IPNB” was adopted after being defined by the WHO Classification of Tumors in 2010 . IPNB orUpfront widespread disease is rare at presentation. In a meta-analysis, compared to the Western population, IPNB in patients residing in Asia was mostly intrahepatic and was less aggressive . Thus, aThe clinical spectrum can vary with patients presenting with vague symptoms, such as abdominal discomfort, jaundice, weight loss, and fever. In a review of 28 patients by Wu et al., approximately 46% of the patients were found to have abdominal discomfort as the only symptom at presentation . The diaSurgical resection is the mainstay treatment for IPNB. Various studies have demonstrated the efficacy of intrahepatic resection, predominantly left hepatectomy with or without biliary duct resection with survival rates ranging from 47% to 68% at five years . In a reDespite being a precursor of invasive cholangiocarcinoma, IPNB generally carries a favorable prognosis. Several factors have been associated with early recurrences and worse survival outcomes, including the presence of invasive carcinoma, lymph nodal involvement at diagnosis, R1 resection, lympho-vascular invasion, and extra-ductal spread of tumor .IPNB is an uncommon, potentially curable biliary tract disease that has propensity to progress to invasive cholangiocarcinoma. The malignant transformation is highly dependent upon the degree of dysplasia. Patients harboring low-grade dysplasia IPNB follow an indolent course and respond well to surgical resection alone with acceptable long-term survival outcomes.


Cutaneous B-cell pseudolymphoma (CBPL) may appear in the face, chest, or upper limbs, and it can be asymptomatic or in the form of nodules, papules, or masses. In most cases, it is idiopathic. However, some causes that have been identified are trauma, contact dermatitis, injected vaccinations, bacterial infections, tattoo dyes, insect bites, and certain drugs. Since the histology and clinical presentation of cutaneous pseudolymphoma (CPSL) are similar to those of cutaneous lymphomas, the diagnosis is usually based on an incisional or excisional biopsy. In this paper, a 14-year-old male patient with a two-month-old mass in the right lateral thoracic region is taken as a case study. He had neither symptoms, nor a past medical history, nor a family history. He had an insect bite a month ago and was fully vaccinated. However, the mass was some centimeters away from the insect bite. A biopsy was taken. The products of it were two paraffin cubes and two histological slides (H&E). The diagnosis was cutaneous B-cell pseudolymphoma. The total removal of the mass was decided since, in idiopathic cases like this, CBPL is not usually healed with topical and non-invasive treatments. Follow-up examinations were suggested since a further antigenic reaction is possible. If cutaneous B-pseudolymphoma is early diagnosed and treated, it does not cause serious problems. In some cases, it even resolves on its own. Cutaneous B-cell pseudolymphoma (CBPL) encloses several terms, including lymphocytoma cutis, cutaneous lymphoid hyperplasia, sarcomatosis cutis of Spiegler-Fendt, and Bafverstedt syndrome ,2. CBPL In an attempt to define this heterogeneous pattern of the disease, various classification approaches have been suggested, taking into account its clinical and histologic presentation. More specifically, Mitteldorf and Kempf introduced the following four categories of the classification system for CPSLs: (1) Nodular PSLs, consisting of solitary or multiple nodules and bearing clinical and histologic resemblance to cutaneous lymphomas; (2) Mycosis fungoides and other cutaneous T-cell lymphomas (CTCLs) simulating PSLs that imitate mainly the histopathology of Mycosis fungoides or other CTCLs and include a large number of clinical entities; (3) other PSLs, a category containing discrete cases reported as PSL; (4) intravascular PSLs regarding reactive lymphocytes of atypical morphology accumulating inside small lymphatic vessels .Even though the majority of the cases are considered idiopathic, several non-malignant stimuli have been attributed to causing CBPL. These stimuli can include trauma, contact dermatitis, injected vaccinations, bacterial infections, especially by Borrelia burgdorferi, viral infections by herpes simplex/zoster virus and HIV, tattoo dyes, and insect bites, but also certain drugs, including anticonvulsants, antibiotics, antipsychotics, anxiolytics, antirheumatics, antihypertensives, antiarrhythmics, and non-steroidal anti-inflammatory drugs. It is worth mentioning that idiopathic cases seem to be connected with photosensitivity, and tattoo-induced CBPLs primarily concern the usage of red dyes ,2,4-7.Clinical doctors encounter difficulties in diagnosing pseudolymphomas over lymphomas since the histology and clinical presentation of CPSL emulate cutaneous lymphomas. However, there are some epidemiological and clinical characteristics that can differentiate the clinical entities of primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous follicle center lymphoma (PCFCL) that need to be excluded before setting the diagnosis of B-PSL. In contrast with pseudolymphoma, both PCMZL and PCFCL are more frequent in females  and appear at later ages, concerning the mid-fifties and early-fifties, respectively. PCMZL shows a different body distribution, affecting mainly the upper trunk, followed by the upper extremities, and lastly the face, but PCFCL follows a similar pattern to that of PSL, with lesions being found mainly in the head and face, followed by the upper trunk. Additionally, PCMZL and PCFCL usually present as solitary or multiple dome-shaped papules, nodules, or erythematous plaques. The workup includes a thorough medical history, especially concerning exposure to one of the causes of B-PSL, along with a detailed clinical examination, including a thorough inspection of the patient and palpation of the lymph nodes, and laboratory blood count and serology tests, whose results are indicative of certain PSL causative agents . A deep Treatment options vary depending on the etiology and include the removal of the underlying causative agent and the prevention of re-exposure. Common drugs used in treating CBPL are topical or intralesional corticosteroids. Additional therapies include surgical excision, radiotherapy, and even cryotherapy ,5,7. RarHerein, we present a case of a child with idiopathic CBPL that was surgically removed and sent over for biopsy in order for the diagnosis to be established.A 14-year-old male patient presented to the dermatologist with a two-month-old mass in the right lateral thoracic region. He stated that there is no pain or any other associated symptoms like itchiness, bleeding, or rash, and no other past medical history (including epilepsy) or family history that is of note. The child was also asked about allergies, piercings, or jewelry in that area, any recent or associated trauma, or any permanent or short-lasting tattoos, and he refused to have taken any recent medications or drugs. Despite that, he mentioned an insect bite near the area of interest in August, but he did not identify its species. As an adjunct, he has completed all the necessary vaccinations for his age frame, including the third dose of the COVID vaccine, which was done in September.During the examination, we did not detect any signs of folliculitis, and we observed a firm, non-pigmented mass with fixed borders, which, according to the patient, first appeared as a dark, flat spot resembling a mole Figure .A biopsy was taken for further investigation with the immunohistochemical markers CD20, CD79a, CIg, bcl-2, bcl-6, CD10, CD21, CD23, CD3, and Ki-67. The products of the biopsy were two paraffin cubes, two histological slides (H&E), and one histological slide Pax5, which, by the microscopic way of evaluation, displayed local hyperkeratosis and the presence of dense lymphocytic infiltration and more specifically spared the reticular dermis without the involvement of the epidermis Figures .The immunophenotype of the specimens was B- and T-lymphocytes, with the former being bcl-2 negative Figure  and bcl-There was also the presence of polyclonal plasmacytes and a plethora of polymorphonuclear leukocytes with highly eosinophilic cytoplasm as the outer surface congregation. Taking the above into account, the diagnosis of cutaneous B-cell pseudolymphoma (cutaneous lymphoid hyperplasia) is confirmed due to a previous or persistent reaction to an immunological trigger.In such cases, the treatment considered is as follows: follow-up examinations, including frequent re-evaluation of the patient, due to the possibility of deterioration since B-pseudolymphoma has an erratic biological character that cannot exclude further antigenic reactions, which in turn could exacerbate the lesion, leading to either recurrence of a previous lesion or enlargement and worsening of an already existing one. All of this is done after surgical excision has taken place Figure .Cutaneous B-pseudolymphoma is an unusual skin disorder that is characterized by the presence of multiple or solitary small, red-brown papules and nodules on the skin. It is a benign, self-limited disorder, which means that if it is diagnosed early and treated, it does not usually cause any serious medical complications; in some rare cases, it has been documented that it resolves on its own, without treatment ,6. The eThe current literature on this topic is rather limited, but both idiopathic and secondary presentations of the disease have been documented. Nnebe et al. record a case of seemingly idiopathic pseudolymphoma, appearing as a red mass on the scalp, as no exposure to possible causative agents was mentioned or realized, which showcased no other symptoms other than the mass and no abnormalities on test results. Special characteristics of the said case were also the age of the patient, being 12 years old, and the T-cell predominance >90%) of the lesion described by matching biomarkers (CD3), which differentiates it from the case we are presenting. Surgical excision was performed after laboratory examinations, which also included a CT of the skull and a PET-CT scan, were done to ascertain the diagnosis, and no relapse was observed . On the 0% of theAll in all, pseudolymphoma, although quite rare, is a present clinical entity affecting mainly the skin and one that closely imitates other important diseases, notably lymphoma. This case highlights PSL’s variability regarding prevalence and laboratory results, making it even more difficult to distinguish from other prominent pathologies. Thus, when suspected, the presence or absence of the disease should be aptly justified because it greatly impacts both the treatment and the patient's well-being.To conclude, CBPL is mostly due to an idiopathic cause. It usually appears on the face in the form of nodules, papules, and tumors. However, there are cases where CBPL appears in other parts of the body. In our case presentation, the patient examined was a 14-year-old boy who had a two-month-old mass in the right lateral thoracic region. Since there were no symptoms such as itchiness or rash, a biopsy was taken along with the surgical removal of the mass. Follow-up examinations were suggested so that further antigenic reactions would be prevented.


Uretero-colonic fistulae are a rare disease resulting from pathologic connection between the ureter and colon, which can be difficult to diagnose. This case report reviews the case of an 83-year-old female with a history of ovarian cancer treated with surgery, radiation, and chemotherapy, who developed a uretero-colonic fistula at a previous colon anastomosis site, which was later diagnosed by ureteroscopy. She was treated with stent placement followed by loop colostomy and was discovered to have metastatic ovarian cancer. She received palliative care consultation and was advised to follow up as an outpatient with the oncology and urology services. Although uretero-colonic fistulae are treatable, treatment depends on patients’ overall clinical picture. Ureteral fistulae are rare, abnormal connections between the ureter with a neighboring organ, with previous case reports describing uretero-arterial, uretero-cutaneous, and many other types of fistulae -3. Clini2 of 97% on room air. Physical examination including pelvic examination were unremarkable except for left-sided costovertebral angle tenderness. CT imaging revealed a 4-mm obstructing stone in the distal left ureter with mild left-sided hydroureteronephrosis  source of infection. However, the patient was hesitant to undergo colonoscopy. Urology service opted for emergent left percutaneous nephrostomy tube placement by interventional radiology, and the patient was later transferred to the intensive care unit after developing hypotension.An 83-year-old female with a past medical history of ovarian cancer treated with exploratory laparotomy including bilateral salpingo-oophorectomy, colon resection with sigmoid-sigmoid colonic anastomosis and no obvious surgical complications, chemotherapy, and radiation in 2017 presented to the emergency department with complaints of left-sided upper abdominal pain, back pain, nausea, and vomiting for several days. One week prior to presentation, she had a stent removed, which was initially placed the month prior for hydroureteronephrosis at a different institution. She denied dysuria, hematuria, or obvious changes in urine character. Vital signs demonstrated a blood pressure of 126/57, temperature of 98.2 °F, heart rate of 101 beats per minute, respiratory rate of 19 breaths per minute, and SpOOnce stabilized, she underwent endoscopic urology evaluation. Cystoscopy findings were unremarkable, and the left ureter was cannulated using a guidewire. Attempts to advance the wire to the collecting system were made; however, the guidewire appeared to turn and enter a lumen significantly medial to the ureter. A ureteroscope was then used to obtain direct visualization. While the distal ureter was normal, 2 cm proximally there was no discernable normal urothelial mucosa, and calcifications on the surgical staple line suspected to be from prior bowel anastomosis were discovered. The guidewire was able to be used to access the collecting system, and an open-ended catheter was advanced over the wire. Retrograde pyelogram confirmed kidney access and the wire was used to place a double-J ureteral stent. Flexible sigmoidoscopy was notable for a large ulceration with significant central necrosis starting from 6 cm into the rectum and extending to 12 cm. There were scattered anastomotic staples as well as exposure of blue tubing, indicative of the ureteral stent and thus confirming the presence of the fistula. (Figure The patient was evaluated by colorectal surgery and underwent laparoscopic loop colostomy with excision of uretero-colonic fistula and omental mass, histology consistent with metastatic carcinoma with papillary features and psammoma body, likely due to the patient’s known history of ovarian high-grade serous papillary carcinoma. The patient eventually underwent palliative care consultation and was advised to follow up as an outpatient with the oncology and urology services.The first case of uretero-colonic fistula was described as early as 1956 [The majority of ureter-associated fistulas are uretero-vaginal fistulas in patients with a history of hysterectomy . In our Identifying a fistula on examination can be difficult as there are not many obvious presentations or manifestations of the disease, and identification on imaging is not always clear. Previous cases of uretero-colonic fistula have described symptoms of generalized lower abdominal pain as well as urinary symptoms such as dysuria, both of which were absent in this case . IdentifDiagnostic evaluation should include CT imaging to evaluate location and size of the fistula; however, it may not always be definitive, as seen in this case. In an ideal scenario, CT imaging can identify fistula, and proceeding to treatment can be expedited. Most notably, urine culture should be obtained, as infectious findings of GI bacteria on culture would strongly support the likelihood of a fistula formation between the GI and genitourinary tracts .Management of uretero-colonic fistula depends on renal function as well as etiology. In cases with poor renal function, nephroureterectomy with excision and closure of the fistula is an option. In cases with good renal function such as this case, stent placement across the fistula followed by surgery is an option. There has also been prior mention of management only using ureteral stent in nonsurgical candidates . UltimatUretero-colonic fistulae are rare and can be difficult to diagnose due to various clinical presentations and nondefinitive imaging findings. Associated symptoms are often nonspecific, with many differential diagnoses and symptoms. In this case, direct visualization with a ureteroscope was required to diagnose the fistula, as CT findings were more consistent with what was believed to be a stone as opposed to a fistula, and urinalysis was unremarkable. Importantly, urine and blood cultures were positive for bacteria of GI origin. This was suspicious for some sort of fistula or anatomic abnormality between the ureter and GI tract. Management options often depend on the patient’s renal function and overall clinical picture. In this case, management included ureteral stent placement followed by surgical resection with colostomy.


Duplication of the alimentary tract is a rare malformation that can occur in any portion of the gastrointestinal tract. Rarely diagnosed in adulthood, it is usually an incidental finding due to non-specific gastrointestinal symptoms. Approximately 80% of cases are diagnosed in infants less than two years old. The most common location is the ileum, and the least common location is the rectum. Embryological theories discussing the etiology of alimentary tract duplications include failure of internal vacuolization during the sixth week of fetal development and/or the yolk-sac dorsal protrusion may adhere to the ectoderm during somite development. Environmental factors such as trauma or hypoxia affecting various intestinal fragments receiving blood supply can evolve into duplication. Excision with consideration to the common blood supply to protect the native bowel is the preferred treatment approach. We present the case of an elderly 70-year-old male with a bowel obstruction complicated by sigmoid duplication. After preoperative assessments, the patient underwent a robotic sigmoidectomy. This case report highlights colonic duplication as a differential diagnosis in the bowel obstruction of an elderly patient. Duplication of the alimentary tract is a rare malformation that can occur at any point from the oropharynx to the anal canal. The most common location is the ileum, accounting for 30% of cases, and the least common location is the rectum, accounting for 5 to 5.5% ,2. AlimeA 70-year-old male presented to the emergency department with constant lower abdominal pain, rectal pain, and constipation for four days. He had a past medical history of irritable bowel syndrome, multiple intrahepatic hemangiomas, masses in his spleen, and hypothyroidism, and a patient stated a surgical history of polyp removal during a colonoscopy, umbilical herniorrhaphy, and inguinal hernia repair. It was not specified by the patient if duplication was noted during the prior colonoscopy and polyp removal. The patient has a family history of Hodgkin’s lymphoma and no history of bowel diseases. The patient was a former smoker. On physical examination, the patient was positive for generalized tenderness to palpation of the lower abdomen. The rest of his exam and vitals were unremarkable. Labs on admission showed elevated white blood cell count (12.5), elevated neutrophil percentage (81.7%), increased absolute neutrophils (10.2), and elevated bilirubin (1.6). An abdominal CT revealed moderate dilatation of the sigmoid colon and descending colon. A transition point was seen at the level of the distal sigmoid colon. The findings were suggestive of an underlying mass versus stricture. There were surrounding inflammatory changes consistent with impaction Figure .The abdominal CT was not able to discern the sigmoid duplication. A gastrografin enema was performed due to continued, significant abdominal pain and a stricture of the distal sigmoid or rectosigmoid junction of indeterminate etiology Figure . A colonoscopy and sigmoidoscopy were performed for direct visualization. The colonoscopy demonstrated severe diverticular disease of the left colon causing functional obstruction and strictures. A colonic stent was placed to treat the bowel obstruction utilizing fluoroscopic guidance. The colonoscopy was performed and the stent was placed the same day. This obstruction prompted a recommendation for an elective resection of the sigmoid colon. A low anterior robotic sigmoidectomy with primary anastomosis was performed three days after the colonoscopy and stent placement. Intraoperatively, two complete lumens were noticed, indicative of a sigmoid duplication Figure .His post-operative recovery was uneventful, and the patient was to follow up as an outpatient. Examination of the colon histopathology showed benign colonic mucosa with reduplication of the colon segment and diverticulosis. The margins of the surgical resection were benign colonic mucosa and a viable bowel wall. There was one benign lymph node and no malignancy present. The confirmed diagnosis via pathology was sigmoid colon duplication.Alimentary duplication is rare and occurs in only one of 4500 live births at a 2:1 ratio of female to male -8. ThereThe embryological origin of alimentary duplication is the gut tube that develops from the yolk sac’s endodermal lining and therefore the mucosa and submucosa. This is engulfed by the mesoderm and forms the mesenteric vessels, adventitia, lamina propria, and muscularis mucosa. Neural crest cells migrate to the submucosa and become the enteric nervous system. The gut develops into three sections: foregut, midgut, and hindgut. Each section's blood supply is from the celiac artery, superior mesenteric artery, and inferior mesenteric artery and their branches, respectively . ConcomiThe few case reports of adults with gastrointestinal tract duplication range in age from 20 years old to 52 years old ,8,13. DuThe case of a 70-year-old male with a sigmoid colon duplication highlights the possible relevance of alimentary tract duplication in the elderly and the need for surgical removal due to bowel obstruction. The patient initially presented with abdominal pain, constipation, and rectal pain with a history of a colonic mass. Preoperative assessments of the patient deemed him a good candidate for robotic sigmoidectomy. In order to avoid complications, excision with consideration to the common blood supply to protect the native bowel is the preferred treatment approach ,4,8. OurResecting alimentary tract duplications is a rare treatment, but it may be necessary to eliminate or avoid complications that may arise. Alimentary tract duplications are sparsely reported in the literature. Most cases represent infants less than two years of age, and adult cases are especially rare. This case report emphasizes the possible relevance and importance of awareness of an alimentary tract duplication of the sigmoid colon in an elderly patient. Robotic sigmoidectomy was chosen as the surgical modality due to patient-specific preoperative assessments. Alimentary tract duplication should be noted as a differential diagnosis for bowel obstruction in elderly patients.


Carotid body tumors (CBTs) are rare neoplasms of the neuroectoderm accounting for 0.6% of head and neck tumors, with a 2%-12.5% risk of malignancy. While surgical resection has been associated with a high rate of neurologic and vascular complications, it remains the mainstay of treatment for malignant CBTs. We present the case of a 40-year-old female with a 5-year history of progressively enlarging right-sided neck mass, with MRI and MRA showing a Shamblin grade III CBT encasement of the internal carotid artery (ICA). Blood flow was absent in the petrous segment of ICA, with great collateralization of brain blood supply, enabling en bloc resection of the tumor with a carotid bulb and ligation of the common carotid artery (CCA) without vascular reconstruction. Further, we describe the characteristics and current management for malignant CBTs, including surgical management, pre-surgical embolization, and adjuvant radiation therapy. Carotid body tumors (CBTs) are rare neoplasms of the neuroectoderm arising from a gangliocytoma at the carotid bifurcation, accounting for 0.6% of head and neck tumors, with a 2-12.5% risk of malignancy .Malignant CBTs most commonly present with a slowly growing firm neck mass without other symptoms, but compared to benign CBTs, they are more likely to be symptomatic presenting as a painless, mobile, pulsatile mass. Infiltration of cranial nerves IX, X, XI, and XII and the carotid artery can lead to hoarseness, ear pain, dysphagia, dizziness, headache or orbital pain, stridor, and Horner's Syndrome . BilaterThe mechanism of developing carotid body paragangliomas is not fully understood. However, chronic hypoxia in cases such as cardiopulmonary disease, living at high altitude, or disruption of oxidative phosphorylation such as mutations in the mitochondrial succinate dehydrogenase complex (SDH) has been suggested as an underlying cause of carotid body hypertrophy leading to CBT , 4.The initial diagnosis of CBTs is based on imaging findings showing a low-resistance blood flow pattern. Doppler ultrasound and CT/MRI are mainstays of imaging. CT and MRI are methods of choice for assessment of size and degree of invasiveness. CT or MRI angiography is the gold standard for diagnosing tumor relation with vessels. Shamblin classification, which indicates the extension of the tumor around the great vessels of the neck, does not correlate with malignancy, however, it is an indicator of intra-operative and post-op complications.There is no consensus on histopathological markers of CBT malignancy. Instead, the definitive diagnosis of malignant CBTs is established with the regional invasion of lymph nodes, blood vessels, and perineural space, as well as skull base structures or distant metastasis in tissues where chromaffin cells are normally absent . FurtherComplete surgical resection, unless contraindicated, is the gold standard for the treatment of paragangliomas and can be combined with pre-embolization and adjuvant radiotherapy. Watchful waiting and radiation therapy, with or without conservative surgery are also possible treatment pathways based on clinical context and goals of care. We will cover treatment options in more detail in the discussion section. Malignant CBTs of the neck follow a more indolent clinical course compared to paragangliomas of other sites with higher 5-year survival rates reported from 60% to 85% for nodal involvement. Although distant metastases are exceedingly rare, they are observed more commonly in bone, lung, and liver, and less commonly in pancreas and brain , 7-8. MeA 40-year-old female presented with a 5-year history of progressively enlarging right neck mass. Her previous workup from an outside institution with fine needle aspiration biopsy was inconclusive, and MRI and magnetic resonance angiography (MRA) of the neck revealed a 3.2 cm x 3 cm x 5.5 cm mass encasing the right common carotid artery and proximal internal and external carotid arteries Figure  with sevPre-operative embolizationThe patient was taken to the angio-suite for preoperative embolization before the day of surgery. Initial diagnostic angiogram demonstrated absent antegrade flow of the right internal carotid artery  and extensive tumors such as class C and D jugular paragangliomas and Shamblin class III CBTs . In thesSurgical resection and neck dissectionThe surgical treatment of choice for malignant or potentially malignant CBTs is resection of the primary tumor and neck dissection . Neck diDue to proximity to neurovascular structures in the neck, carotid body tumor resection is associated with multiple complications. In a large case series of 229 CBT patients with 16 malignant CBTs, malignant tumors were associated with a higher rate of vascular reconstruction or repair 63% vs. 8%), neurologic complications (69% vs 37%), and significantly longer procedure time % vs. 8%,. In caseFor rare and challenging malignant tumors which require extensive resection and ligation of the internal or common carotid artery to achieve R0 resection, vascular reconstruction using vascular prostheses or saphenous vein can be used to maintain sufficient blood supply to the brain . LigatioAdjuvant radiation therapyRadiotherapy is an effective alternative treatment to surgery for early-stage benign tumors or in patients with extensive tumor or high operative risk and can decrease tumor size or stop growth . HoweverMalignant CBTs are rare neoplasms. Surgical resection is the mainstay of their treatment due to the potential for metastatic disease and higher risk of mortality. Extensive vascular involvement in malignant CBTs is challenging and associated with significant intra-operative and post-operative neurovascular complications. Pre-operative embolization of the tumor blood supply can significantly lower blood loss and operative time. In rare cases with severe narrowing of ICA, such as the case presented here, chronic collateralization of cerebral blood flow may enable en bloc resection of the tumor without the need for vascular reconstruction and with excellent postoperative outcome.


This is a review of two cases of neonatal giant scalp congenital hemangioma. Both patients were treated with propranolol using a similar multi-step approach that included transarterial embolization of the supplying arteries followed by surgical resection of the lesion. In this report, we discuss the treatments, complications, and clinical outcomes of interventions and surgical procedures. Congenital hemangiomas (CHs) are rare benign tumors that develop at birth. CHs develop primarily in Caucasian females born prematurely to mothers who underwent chorionic villus sampling . These lInfantile hemangiomas (IH) are on the contrary the most common benign vascular tumors of infancy. They usually involute without significant residua. Indications for intervention include ulceration, prevention of disfigurement, and impairment of function or vital structures . There aGiant CHs are often associated with clinical complications, including high output cardiac failure, consumptive coagulopathy, and permanent cosmetic concerns . TherefoHere, we document our experiences with two children who were diagnosed with extracranial giant CHs located in the temporoparietal and retro auricular regions, respectively. Both children were treated initially with propranolol and subsequently with TAE before surgical excision. g) was delivered at our hospital via cesarean section during the 38 + 1 gestational week. An antenatal ultrasound examination in the 35th gestational week revealed a lesion on the head accompanied by high output cardiac failure. Elective fetal magnetic resonance imaging (MRI) revealed a large extracranial lesion measuring 6.2 cm × 2.2 cm × 5.6 cm in the temporoparietal region with no apparent impact on brain development.A newborn male  lesion with a circumference of ∼6 cm. Echocardiography at birth documented right heart failure accompanied by pulmonary hypertension and grade I tricuspid valve regurgitation.Physical examination revealed a heterogeneous raised  and N-terminal pro-brain natriuretic peptide . Abdominal, spinal, and cranial ultrasound examinations were performed to rule out additional deformities. A repeat MRI study revealed that the highly vascular lesion was supplied primarily by the right external carotid artery (Laboratory results included elevated levels of troponin I (76d artery . Blood d mg/kg/day) commenced immediately after birth. This treatment was well-tolerated and the patient's vital signs remained stable; the dose was increased to 2 mg/kg/day on the following day. Echocardiographic studies revealed that signs of early heart failure diminished during the 14 days to follow.Pediatric cardiology, pediatric surgery, and the interventional neuroradiology departments were consulted for insight into potential treatment approaches. Systemic treatment with propranolol  and Lipiodol®  in a 1:2–3 ratio was introduced to reduce the risk of intraoperative blood loss. After the procedure, the patient was treated with a continuous intravenous infusion of heparin at 400 IU/kg for 24 h.Although the sheath in the left groin used for the embolization procedure was removed, the patient developed acute limb ischemia. Doppler ultrasound of the upper regions of the lower left limb revealed thrombosis in both the external iliac and inguinal arteries. The Hematology team recommended a conservative approach  to address this complication.As no significant reduction in lesion size was observed, surgical resection of the CH was performed two days after the embolization procedure was completed. The size of the lesion was measured followed by a fusiform incision. To preserve skin for primary closure, the lesion was dissected outwards until the cranium was reached . CarefulA Doppler ultrasound examination performed five days later revealed improved vascularity of the popliteal artery due to collateralization of the left iliac artery. Low-molecular-weight heparin was reduced from therapeutic to prophylactic doses after three weeks and continued for six months.A histopathological evaluation confirmed the diagnosis of CH based on glucose transporter (GLUT)-1 negativity . This an g) was transferred to our hospital after a cesarean section during the 37 + 2 gestational week. Non-invasive respiratory support with positive pressure ventilation was required at birth due to poor pulmonary adaptation and APGAR scores of 4/8/10. The patient recovered and began to breathe spontaneously.A newborn female  with audible murmurs. The lesion exhibited a firm elastic consistency; dilated veins were prominent on the lesion surface. Echocardiography was performed to rule out cardiac failure. ng/dl) but no other indication of consumptive coagulopathy. Abdominal, spinal, and cranial ultrasounds were performed to rule out additional deformities.Laboratory results included elevated D-dimer levels  was initiated on the second day after birth; the dose was subsequently increased to 2 mg/kg/day. Propranolol treatment was suspended after a drop in the diastolic blood pressure was observed. The condition of the mass remained unchanged.Propranolol therapy (1™ catheter (Medtronic) with a 0.007-inch Hybrid microguidewire  was used for precise embolization of the branches of the right external carotid artery using a combination of Magic Glue  and Lipiodol® (Guerbet) in a 1:2–3 ratio. Percutaneous sclerotherapy (PS) with Glubran®2/Lipiodol® and Magic Glue/Lipiodol® in a 1:2 ratio was used to ablate the extensive blood supply to this lesion  was performed with a 4-F catheter . A 0.008-inch Marathons lesion . ContinuNo signs of limb ischemia were observed upon removal of the sheath from the femoral artery. Prophylactic antibiotic treatment with cefuroxime was administered after serous secretions were found on direct puncture sites on the hemangioma. Although we observed no size regression, the CH developed a softer consistency after these procedures. Ultrasound examination revealed persistent hypervascularization of the lesion after TAE and PS.Surgical resection of the lesion was performed eight days later. Despite the previous embolization procedures, the patient sustained substantial blood loss from the smaller feeder vessels ; a bloodHistopathological examination revealed overexpression of both CD31 and CD34, confirming its identity as a vascular tumor. The absence of GLUT-1 confirmed the diagnosis of CH which was confirmed as entirely removed. The follow-up evaluation revealed minimal surgical scarring and an excellent cosmetic outcome.The outcomes and complications of 38 patients from 4 different institutions were tabulated and accessed based on the types of embolization and sclerotizing agents used. Skin necrosis is one of the common complications after transarterial embolization. Demuth  reportedIn Jianhong's  case, thPatel  also demPlascencia  supporteUnder the ISSVA classification of vascular tumors, CH falls under benign vascular tumors. It is important to consider other forms of benign vascular tumors which also fall under this category i.e., tufted angioma or Kaposiform hemangioendothelioma. These diagnoses were excluded because of their clinical appearance, as well as mismatching of hematological characteristics such as the absence of profound and sustained thrombocytopenia with profound hypofibrinogenemia and consumptive coagulopathy.Giant CHs may be associated with heart failure that can be attributed to arteriovenous shunting. Propranolol is a member of the class of drugs known as ß-blockers; the ß2 inhibitory effects of these drugs block the release of vasodilatory transmitters and thus promote vasoconstriction and reduced blood flow to the hemangioma . PatientPropranolol also has inotropic and chronotropic effects on the heart and can influence both the heart rate and blood pressure . PatientUnfortunately, patient case #1 suffered limb ischemia following TAE. Infant blood vessels are short and thin and thus prone to arterial spasm, dissection, and thrombosis. In 1997, Saxena et al. discovered that although intra-arterial heparin was administered during the intervention, thrombosis can occur in about 9% of cases. Fewer attempts at arterial puncture, use of a smaller sheath, maintaining a minimum procedure time, and ensuring that back bleed is present can prevent future occurrences of post-catheterization thrombosis . A revieAt this time there are no standard embolic agents recommended for use in TAE or PS. Patel et al.  reportedA giant scalp CH is a rare vascular anomaly that can be accompanied by high output heart failure. Systemic therapy with propranolol is a safe and effective means to treat heart failure in these cases. CHs do not typically respond to this medication. Although both patients in our study underwent treatment with propranolol, its efficacy in this situation remains unclear.TAE/PS is a secondary approach to CH as it is an effective method that can be used to eliminate the blood supply to these lesions. The decision to proceed with surgical resection after TAE/PS should be decided on a case-by-case basis. Ample time should be allotted to provide the lesion with sufficient opportunity to shrink before considering surgical resection. However, skin necrosis warrants prompt resection. Interdisciplinary cooperation is crucial when designing appropriate methods to treat patients with giant scalp CHs.


Background: Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm typically diagnosed in older adults and localized to the extremities or retroperitoneum. Because of poor response to therapy and high rates of recurrence, this neoplasm is associated with a poor prognosis.Case Report: A 12-year-old female presented with weight loss, abdominal pain, fatigue, and diarrhea. She was profoundly anemic with occult blood–positive stools. On endoscopy, a fungating cecal mass was biopsied and diagnosed as malignant sarcomatoid neoplasm. The neoplasm was resected with clear margins during subsequent surgery, and on final pathology was diagnosed as UPS. A suspicious lung nodule was also removed via video-assisted thoracoscopic surgery and found to be a granuloma positive for Histoplasma capsulatum for which the patient received antifungal therapy. The patient did not receive additional chemotherapy or radiotherapy and was doing well without signs of recurrence at 12 months postresection.Conclusion: This report of cecal UPS in a 12-year-old is rare because of the patient's age and tumor location. We have identified only 2 other case reports of pediatric gastrointestinal UPS. This case illustrates the need for a broad differential and prompt workup in pediatric patients presenting with weight loss and abdominal complaints. More information regarding the management and outcomes in cases of gastrointestinal UPS is needed to assist providers in determining the best treatment course and to allow for better prognostication. To our knowledge, only 14 cases of cecal or ascending colon UPS are reported in the literature.14 The classification and subdivision of these tumors went through several iterations until the World Health Organization 2002 classification eliminated the term MFH and replaced it with UPS.1Undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade pleomorphic neoplasm without any definable line of differentiation.15 and account for approximately 20% of all soft-tissue sarcomas.17 UPSs are more common in males than females with a 2.4:1 ratio.7UPSs most commonly occur later in life, usually the sixth and seventh decades,18 The cellular origins of UPSs are unclear, but they possibly arise from primitive mesenchymal stem cells that retain both fibroblastic and histiocytic potential and may present with markers and behaviors of both cell lines.11Risk factors for the development of UPS include genetics, radiation or chemotherapy exposure, chemical carcinogens, chronic postoperative repair, trauma, surgical incisions, and lymphedema.8 Compared to other types of colon cancer, UPSs present more frequently with a right-sided mass, less frequently with constipation, and in up to 25% of cases patients report fevers.10 Laboratory findings at diagnosis may be normal or may show elevated inflammatory markers, leukocytosis, and anemia.9A UPS typically presents as an enlarging lump that is often excised early when located on an extremity. However, intestinal UPSs are often discovered late when they have substantial tumor bulk. Presenting symptoms may include abdominal distention and pain, altered bowel habits, weight loss, anemia, blood in the stool, or palpable abdominal mass.19 Immunohistochemical staining is sometimes positive for vimentin, actin, CD68, alpha 1-antitrypsin, alpha 1-antichymotrypsin, and laminin mRNA.19 Importantly, UPS has no reproducible immunophenotype or pattern of protein expression that allows for further classification of the tumor,16 and exclusion of pleomorphic variants of other neoplastic lines is required.17Diagnosis is based on a combination of microscopic features and immunohistochemical staining techniques used to rule out other cell lines of origin. Lesions are typically characterized by pleomorphic, spindle-shaped cells with bizarre cytology and nuclear atypia.19 Masses are sometimes large and lobulated and may also have calcifications, hemorrhage, myxoid degeneration, necrosis, or tissue invasion.16Computed tomography (CT) typically shows a well-circumscribed and homogeneous mass or a low-density mass secondary to necrosis and hemorrhage.11 An increasing number of reports suggest that adjuvant chemotherapy or radiation may improve prognosis in certain clinical scenarios.19 Information regarding outcomes is primarily based on case reports or retrospective case series, and most reported cases of intestinal UPS were treated with surgery alone.20Standard treatment for UPS is early complete surgical resection with negative resection margins and en bloc lymph node dissection. The role of chemotherapy and radiation in the treatment of UPS is debated and without strong evidence.7 A review of 200 cases found a 2-year survival rate of 60%, a 5-year survival rate of 47%, and an overall recurrence rate of 44%. Metastasis occurred in 42% of cases, most commonly to the lungs (82%) but also to lymph nodes (32%).15The prognosis for UPS is generally poor because of regional invasiveness, distant metastases, and frequent recurrence.A 12-year-old female presented to an urgent care clinic with 1 week of nausea and vomiting. She reported fatigue and chronic intermittent cramping abdominal pain for 4 months and unintentional weight loss of 25 pounds over the last 3 months. On examination, she had pale conjunctiva, tachycardia, and mild abdominal pain.Initial complete blood count (CBC) revealed hemoglobin 7.3 g/dL , hematocrit 29.7% , mean corpuscular volume 62 fL , red blood cell distribution width 18.9% , and platelets 708 K/μL . Several days later, outpatient workup for anemia revealed serum iron of 10 μg/dL , total iron binding capacity 304 μg/dL , iron saturation 3% , and ferritin 5 ng/mL . The patient was found to be fecal occult blood–positive.On outpatient recheck of CBC 6 days later, the patient's hemoglobin had dropped to 5.9 g/dL, and she was admitted to a community hospital where she received further workup and a blood transfusion. C-reactive protein (CRP) at admission was 3.01 mg/dL . An initial concern was that the patient may have inflammatory bowel disease given her iron-deficiency anemia, elevated CRP, and positive fecal occult blood test with weight loss and diarrhea. Nonsteroidal anti-inflammatory drug (NSAID)–induced gastritis was also considered, given her daily NSAID use for abdominal pain. The patient was transferred the next day to a larger children's hospital to undergo further evaluation.Upper gastrointestinal endoscopy showed no abnormalities. Colonoscopy showed a large, fungating, nonobstructing, approximately 7-cm cecal mass . The remThe mass was biopsied 18 days later, and the initial pathology diagnosis was malignant sarcomatoid neoplasm. Tumor cells were weakly positive for SATB2 and negative for keratin OSCAR, keratin AE1/AE3, desmin, myogenin, SMA, SOX10, S100, CD34, WT1, SALL4, and EMA.A formal right hemicolectomy was performed without complication 6 days after the pathology results were received. At the time of surgery, an intraluminal cecal mass was noted to be causing colo-colonic intussusception. On final pathology 15 days following resection, the neoplasm was diagnosed as UPS  and 4. THistoplasma capsulatum, and the patient completed an 8-month course of itraconazole 300 mg daily. Chest x-rays after 6 months of treatment and several months after discontinuation of therapy were both negative for lung abnormalities concerning for recurrence.Ten days after the final pathology results were available and 25 days after colectomy, the patient underwent video-assisted thoracoscopic surgery with wedge resection of the pulmonary nodule. Pathology of the nodule 8 days later identified a 1.1-cm necrotizing granuloma. Small yeast with narrow-based budding without a mucicarmine-positive capsule was confirmed with Grocott methenamine silver and periodic acid-Schiff for fungus cytochemical stains. These findings were consistent with Follow-up magnetic resonance imaging (MRI) of the abdomen at 2, 10, and 12 months postoperatively showed no evidence of disease. Multiple rechecks confirmed that the patient's anemia had resolved. Plans for long-term follow-up include annual endoscopy and MRI with biannual oncology visits for 5 years. After this period, the patient will be followed by oncology on an annual basis.21UPS is a diagnosis of exclusion reserved for sarcomas with a distinct combination of immunohistochemical and microscopic features, made only after careful consideration of other differential diagnoses. This case is unique both for being localized to the gastrointestinal tract, with only 14 cases reported of cecal or ascending colon UPS, as well as the young age of the patient. To our knowledge, only 2 other cases of gastrointestinal UPS have been reported in the pediatric population.Histoplasma capsulatum. The patient will be followed by oncology to monitor for recurrence through periodic imaging and laboratory workup.Our patient developed a nonobstructing right-sided colonic mass, with systemic features of disease including elevated inflammatory markers, anemia, and weight loss, which are consistent with the presentation of UPS. Despite the tumor's large size at diagnosis (7 cm), chemotherapy was not given in this case as the tumor was resected with clear margins and the patient had no lymph node involvement or metastatic disease. A lung lesion that was initially suspicious was resected and found to be positive for Additional research into the role of radiation and chemo-therapy for abdominal UPS is needed, especially for cases in which surgical resection is not possible. Continued monitoring and close follow-up are essential for a good long-term outcome because of the relatively high recurrence rate for UPS.Although malignancies of the gastrointestinal tract in pediatric patients are rare, this case exemplifies the need to maintain a broad differential. The case further shows the value of prompt referral and workup by appropriate medical specialists to limit morbidity and mortality. The treatment and long-term outcomes for pediatric UPS are an area with limited published literature that would benefit from further study of the long-term patient outcomes to guide clinical management.


Testicular torsion accounting for 25% of acute scrotal disease, is an acute surgical condition. Atypical presentations of testicular torsion may lead delay diagnosis.A 7-year-old boy was admitted to the pediatric emergency department with continuous and progressive left scrotal pain for 2 days, associated symptoms and signs included left scrotal swelling and erythema. The pain started 4 days ago as left lower abdominal pain which then migrated to the left scrotum.Physical examination showed left scrotum skin redness, swelling, local heat, tenderness, high-riding testis, absence of the left side cremasteric reflex and a negative Prehn’s sign. Subsequent point of care ultrasound of scrotum revealed increased volume of the left testicle, inhomogeneous hypo-echoic left testis, and no detectable flow in the left testis. Left testicular torsion was diagnosed.Surgical examination confirmed testicular torsion showing 720° counterclockwise rotation of the spermatic cord with ischemic changes in the left testis and epididymis.The patient was stabilized and discharged after left orchiectomy, right orchiopexy and antibiotic therapy.Symptoms of testicular torsion may be atypical, especially in prepubertal age. Detailed history, physical examination, point of care ultrasound usage and timely urologist consultation and intervention are important for prompt rescue to prevent testicular loss, testicular atrophy, and eventual impairment of fertility. The most common causes of acute scrotal pain in children and adolescents include testicular torsion, torsion of the appendix testis, and epididymitis. In some studies, torsion of testis accounted for up to 25% of acute scrotal diseases in pediatrics and could be considered as a critical emergency condition. The annual incidence of testicular torsion is approximately 1 per 26,000 in the pediatric population. It presents with a bimodal age distribution, with 1 peak in the perinatal period, and the second peak around adolescence.,4 Testicular torsion can be present in outside the common age of occurrence and an atypical pattern. Although rare, bilateral testicular torsion can happen in the pediatrics.,7 Additionally, atypical presentations with abdominal pain, groin pain, nausea, and torsion in the abdomen or inguinal area may cause misdiagnosis or delay diagnosis.,9 Testicular torsion is an urgent condition that can lead to testicular loss, and eventual impairment of fertility which makes prompt surgical exploration and treatment essential. This case reports an atypically presenting prepubertal male with testicular torsion.Acute pediatric scrotal pain often requires prompt surgical intervention.A 7-year-old boy presented to the pediatric emergency Department due to the continuous and progressive left scrotum pain for 2 days. Associated symptoms and signs included left scrotal swelling and erythema Fig. . The pat,12 causing the equalization of venous and arterial pressures resulting in testicular ischemia. Additionally, the reperfusion injury that occurs on the release of the twisted cord adds to the principal pathophysiology of the condition. According to experimental research, testes start to infarct around 2 hours of blood re-striction with irreversible ischemia beginning after 6 hours leading to complete infarction after 24 hours. Contrarily, a systemic study revealed that there is 90% survival of the affected testicular tissue at 0 to 12 hours after the onset of ischemia, half survival at 13 to 24 hours, and up to 18.1% survival after 24 hours. However, Howe et al concluded that 15 hours of symptoms and 860 degrees of torsion gives testes a 50% salvage rate. For maximizing the chances of testicular survival, prompt diagnosis and treatment of testicular torsion is crucial.Testicular torsion is a relatively common acute scrotal condition in the pediatric emergency Department. The disease happens when there is a twisting of the spermatic cord along its longitudinal axis obstructing venous return,16 and the source of pain radiates from scrotal area. Testicular torsion should always be involved in lower abdominal pain’s differential diagnosis in males. Prepubertal children are more likely to present with atypical symptoms than post pubertal children, resulting in delayed diagnosis and intervention. In children presenting with abdominal groin pain and empty ipsilateral scrotum, torsion of undescended testis should be considered. Torsion of cryptorchidism is accounted for about 5% in all testicular torsion cases, and the incidence of torsion of cryptorchidism appears to be higher than torsion of normally descended testis.,21 Physical examination is crucial in diagnosing testicular torsion. Unilateral testicular tenderness, high riding testis, trans-verse testicular orientation, anterior palpation of the epididymis, absent cremasteric reflex, and negative Prehn’s sign are some of the key observations.,23 Physicians should also examine abdomen or inguinal canal with a tender mass and missing testis in the ipsilateral inguinal region. Testicular Workup for Ischemia and Suspected Torsion (TWIST) score is a useful tool for testicular torsion diagnosis since it encompasses testicular swell-ing, hard testis, nausea/vomiting, absent cremasteric reflex, and high riding testis, which encompasses common signs of testicular torsion. Meta-analyses revealed the TWIST score achieves a high sensitivity (0.984) and high specificity (0.975). However, TWIST does not capture erythema or abdominal pain, highlighting that this case is atypical at first. Point of care ultrasound can be crucial in diagnosis due to its high sensitivity (100%) and specificity (99.1%) and shows relative decrease or absence of blood flow to the affected testis, which significantly increases the predictivity.,27Testicular torsion is diagnosed clinically with symptoms of abrupt onset testicular or scrotal pain, scrotal swelling, nausea, and vomiting. Patients may also present with nonspecific symptoms such as fever or urinary problems. Not all cases of testicular torsion present scrotal pain or discomfort. The most common atypical symptoms are lower abdominal pain, inguinal pain, and nausea/vomiting. The lower abdominal pain or inguinal pain may move to the scrotum few hours after the onset of the initial abdominal presentation, Manual detorsion is considered a “time buying procedure” and a pre-liminary to surgical intervention. Manual detorsion should be performed along with surgical exploration and orchidopexy, which remains the gold standard in the diagnosis. Orchiectomy is performed on non-viable testis to prevent the formation of anti-sperm antibodies which may negatively affect the non-affected testis. About 40% of boy undergoing surgery for testicular torsion performed orchiectomy, and rate of orchiectomy is higher in torsion of cryptorchidism than in torsion of descended testis.,29 Another study revealed poor rates of surgical salvage in torsion of cryptorchidism. It was because the atypical presentations made parents underestimate the children’s condition. The probability of orchiectomy is highest for children in prepubertal age,17,31 and the risk for orchiectomy decreased by 14% each subsequent year. Additionally, orchiopexy is per-formed on viable testis to prevent retorsion, and prophylactic orchiopexy of the unaffected testis should also be performed to prevent future torsion. However, half of the patients undergoing only salvage therapy still develop testicular atrophy within 2 to 14 months after the procedure. Predictive factors include pain lasting over 1 day and heterogeneous echogenicity on ultrasound. Post-op follow up is crucial.Surgical treatment includes orchiectomy or testicular salvage with detorsion and orchiopexy.In conclusion, although testicular torsion is less common in the preschool and school attending age group, it is more likely to present with atypical symptoms at this age. Physicians need to perform detailed history taking and physical examination. Point of care ultrasound should be used to promptly diagnose the condition necessary for emergent urologist consultation and intervention to decrease the necessity of orchiectomy and subsequently to prevent testicular loss, testicular atrophy, and eventual impairment of fertility.Data curation: Bei-Cyuan Guo.Methodology: Bei-Cyuan Guo.Resources: Bei-Cyuan Guo.Supervision: Han-Ping Wu.Writing – original draft: Bei-Cyuan Guo.Writing – review & editing: Han-Ping Wu.


Porocarcinoma is a rare skin malignancy that should be suspected in patients with chronic skin lesions. Although it usually affects the elderly population in sun‐exposed areas, it may be seen in younger populations and in nonexposed areas such as the groin. Porocarcinoma is a rare skin malignancy that should be suspected in patients with chronic skin lesions. Although it usually affects the elderly population in sun‐exposed areas, it may be seen in younger populations and in nonexposed areas such as the groin.  We report a rare case of porocarcinoma of the groin in a 43‐year‐old gentleman that was successfully managed by wide local excision.Porocarcinoma is a rare skin cancer arising from the intraepidermal portion of the eccrine sweat glands.The largest review of porocarcinoma‐reported cases, which included 453 patients, showed that porocarcinoma occurs with equal gender predilection and a tendency to affect the elderly population more commonly in the seventh and eighth decades of life.We present a rare groin porocarcinoma in a 43‐year‐old gentleman successfully managed by early local resection. This case is peculiar because primary porocarcinoma is rarely seen in the fourth decade of life. In addition, only a few cases of primary porocarcinoma have been reported to affect the groin area.2A 43‐year‐old Indian gentleman, previously healthy, presented to dermatology clinic complaining of a suspicious skin lesion on his right groin. The lesion started as a painless nodule 4 years ago and slowly increased in size. Four months before the presentation, the patient started to feel pain at the site of the lesion with pus discharge. There was no history of other swellings, previous illness, radiation exposure, loss of weight, or family history of cancer.On examination, there was a 2 × 3 cm oval erythematous ugly nodule on the right groin skin with superficial erosion and overlying pus discharge Figure . The lesHistological examination showed marked full‐thickness atypia of the epidermal tissue. Large pleomorphic cells with prominent nucleoli and high mitotic activity were identified forming endophytic lobules that appeared to be of sweat duct origin, suggesting porocarcinoma in situ.The case was discussed in the skin cancer multidisciplinary meeting, and the patient had wide local excision of the skin tumor with a 2‐cm safety margin followed by primary closure by the dermo‐oncology plastic surgeon.Histological examination showed marked full‐thickness atypia in the surface epithelium associated with luminal/glandular formations Figure . Large p3The case we report is the first report in the MENA region of a groin porocarcinoma. This rare skin cancer usually affects the senior‐aged population's sun‐exposed areas and lower extremities.Eccrine porocarcinoma is a rare skin cancer originating from the intraepidermal part of the eccrine sweat glands. The tumor can either start de novo or on top of a previously benign lesion.Regarding the diagnosis, porocarcinoma has a myriad of differential diagnoses. The list includes metastatic adenocarcinoma, Bowen's disease, pyogenic granuloma, fibroma, seborrheic keratosis, and amelanotic melanoma.The histological appearance of porocarcinoma varies widely.Wollina et al. evaluated the histopathological and immunohistochemical differences between poroma and porocarcinoma. In this study, porocarcinoma showed a widely variable response to cellular markers compared with benign poroma. Porocarcinoma showed strong CEA expression by lumen‐forming single vacuolated cells and mature duct cells. Such expression was limited in single poromas lumen formations. Porocarcinoma cell clusters showed scattered reactivity with antibodies Cam 5.2 and Vim 9(1), for which poromas remained unstained. In both poroma and porocarcinoma, scattered cells stained positive for S100A. These cells showed well‐formed dendrites in poroma, while porocarcinoma failed to show dendrites and showed irregularly shaped cells..As per the management, wide surgical excision is the most common treatment option for the local disease, with curative rates from 70% to 80%.4Eccrine porocarcinoma is a rare skin malignancy. Although it is primarily seen in the head, neck, and extremities of elderly patients, it can be found in the younger population and nonexposed areas, such as the groin. Diagnosing this rare cancer is challenging, so clinicians should have a high index of suspicion when facing such lesions. It is crucial to reach an early histological diagnosis for this malignancy, as wide local excision can be the only intervention needed for the localized disease.Heba Megahed: Conceptualization; data curation; project administration; validation; writing – original draft; writing – review and editing. Ayda Al‐Hammadi: Data curation; writing – original draft. Noura Al‐Nauaimi: Data curation. Sameer Alhyassat: Data curation; writing – review and editing. Mahmoud Al‐Thalthini: Supervision; writing – review and editing.The authors declare no conflicts of interest with the subject of this article.Approved by Surgical Research Center, Hamad Medical Corporation REF: SR/RE/2022/41.Written consent for the publication of the clinical details of this case report is present upon request.


Gingival cysts of adults (GCAs) are a relatively rare type of odontogenic cysts developing from the remnants of the dental lamina. Although GCAs generally affect individuals below the age of 65, their findings in older patients may exceptionally occur. Usually asymptomatic, they present as small, generally solitary nodules, located on the attached gingiva, primarily in the mandibular canine and premolar regions. This report highlights a rare case of a solitary GCA in an 82-year-old patient, who presented with a discrete nodule located between the right mandibular canine and first premolar. An excisional biopsy was performed, and the diagnosis of GCA was confirmed by the histological examination. No recurrence was observed during the 18-month follow-up period. Additionally, a literature review was conducted to analyse the clinical and pathological characteristics of GCAs in patients aged 65 and older. By providing details of this rare entity, our case study highlights the importance of considering GCAs when diagnosing gingival lesions in geriatric patients. By increasing our understanding of GCAs in the elderly population, our findings can help improve diagnosis as well as management strategies for these lesions. Gingival cysts of adults (GCAs) are slow-growing, odontogenic, developmental cysts originating from the rests of dental lamina . GCAs arPanoramic and intraoral radiographic examinations usually do not reveal bone involvement, unless significant bone resorption has occurred due to cystic pressure , 8, 11.Microscopically, GCAs are characterized by an uninflamed, connective tissue wall, lined with a thin, squamous, or cuboidal epithelium. Focal areas of epithelial thickening, appearing as plaques, may be present, sometimes containing glycogen-rich clear cells , 5, 7.The treatment of choice for GCAs is surgical excision, with a favorable prognosis and extremely rare recurrence .This article describes the case of an 82-year-old presenting with GCA, with the aim of providing a comprehensive overview of the clinical, radiological, and histological findings. The primary objective is to assist practitioners in recognising and effectively managing this particular pathology. Furthermore, a literature review focusing on GCAs in the elderly population has been included to provide a comprehensive overview.An 82-year-old female patient presented to the dental office for a routine checkup in August 2020, reporting the presence of a slow-growing, painless swelling on the lower right gingiva, which she had noticed several months prior. The patient did not recall any history of trauma in that area. She had previously undergone some dental therapy at the nursing home but no recent treatment. She had a poor dental status, including tooth wearing, discolorations, old resin fillings, and multiple missing teeth, which had not been replaced. Despite no periodontal problems detected, a slight loss of attachment was found. Her medical history included colorectal adenocarcinoma resection 5 years earlier, which prompted her to seek dental advice in order to investigate the exact nature of the gingival swelling. Her medical history also included non-insulin dependent type II diabetes, hypercholesterolemia, and hypertension, all of which were untreated. The patient reported no history of smoking or other tobacco use.On clinical examination, a small, solitary, translucent, yellowish nodule measuring 3 × 3 mm with a smooth round surface was found. The lesion was located on the attached gingiva below the interdental papilla, between the lower right mandibular canine (tooth #43) and first premolar (tooth #44). It appeared firm and asymptomatic with a sessile base . Pulp te™ 4-0, Ethicon® Inc., Somerville, NJ, USA).Because of the size of the lesion, an excisional biopsy was performed under local anesthesia . Injections were made at a distance of approximately 3 mm from the lesion in order not to damage and therefore impinge on the lesion tissue and to ensure good microscopic analysis. Two injection points were chosen, the first above the lesion, at the level of the papilla, and the second below, in the vestibular region. Approximately, 0.5 ml of solution was administered. A spindle-shaped incision was made with a blade scalpel (blade n° 15) extending approximately 1 mm beyond the lesion edge in healthy tissue. The overlying mucosa and the underlying connective tissue were removed, and a translucent, yellowish liquid discharge was observed during the procedure. The excised specimen was fixed in 10% buffered formalin solution and submitted for histopathological examination. Two sutures were placed for wound closure (Vicryl RapideMacroscopic inspection of the excised tissue revealed a grayish nodular fragment of soft tissue, measuring approximately 4 × 3 × 2 mm. Histological analysis of the gingival tissue sections stained with haematoxylin and eosin solution revealed a cystic cavity lined with two or three layers of cuboidal cells of non-keratinized stratified squamous epithelium Figures . The fibThe sutures were removed after 2 weeks, and the patient reported no postoperative complications. Although sutures are usually removed 7–10 days after the intervention, we decided to wait until the patient's next scheduled appointment for other dental treatments, having taken into account her age and in order to minimize the number of appointments.Regular follow-up appointments have shown no signs of recurrence for over 2.5 years .GCAs are rare developmental lesions, with about 170 cases described in the literature over the past 70 years worldwide , 12, 13.In the 15 reported cases of GCAs, the patients' age ranged from 68 to 80 years. Our case represents, to the best of our knowledge, the eldest reported patient with a GCA, the patient being 82 years old.The majority of the cases occurred in the mandibular region and were located on the attached gingiva in the lateral incisor–canine–premolar area. The size of the lesion was reported in 10 cases, normally being less than 5 mm. Only two reports described larger lesions, measuring 1.5 and 2 cm, respectively . Eight cIn our case, there was no radiographic evidence of bone involvement, and it seems that most other studies share this finding, although information is lacking for five cases. Another case showed radiographic radiolucency , which mBased on the clinical presentation, the differential diagnosis of GCA included other lesions presenting as gingival swellings, such as a fibroma or a retained foreign body. However, our patient had no history of trauma in the region. A lipoma may also present with the same aspect of the above described lesion, although it is extremely rare in the attached gingiva, and the histopathological diagnosis is unequivocal . AlthougGCAs are treated by simple excision with minimal margin involvement and submitted for histopathological examination to establish the diagnosis, with usually no tendency to recur .In conclusion, GCAs are considered uncommon, slow-growing, benign, developmental cysts of odontogenic origin, typically located in the mandibular attached gingiva in the canine–premolar area. Although they are rare in the elderly, they should be included in the differential diagnosis of gingival swellings.


Apert Syndrome (AS) is a rare form of acrocephalosyndactyly. The aim of the manuscript was to underline the challenging squint management in a case of Apert Syndrome. A 1.5-year-old male with craniosynostosis, diagnosed at birth, with history of incomplete closure of eyes, more so in the right eye, and squinting of left eye since birth, was brought to eye OPD by the mother. Presence of acrocephaly, prominent forehead with bony irregularity, chin down with left head tilt, fused cervical vertebrae, marked proptosis, cleft palate, dental anomaly and syndactyly confirmed the diagnosis of AS. Old serial photographs of the child were examined to look for progression of squint and proptosis. Squint evaluation revealed 70-75 PD exotropia with 10PD right hypertropia in primary gaze. The right hypertropia increased further in the left gaze, whereas a left hypertropia was noted in the right gaze. The patient underwent bilateral LR recession of 9 mm with full muscle width transposition (upshift) with Inferior Oblique recession of 4:1 mm in the right eye and 3:2 mm in the left eye. Post-operative follow-up after 2 months showed that V pattern collapsed with residual exotropia of 20 PD. Post-operative follow-up after 1 year showed improvement in head posture with pattern collapsed. However, recurrent exotropia was noted on evaluation, for which bilateral medial recti resection was done later. The management of squint in AS and other craniosynostosis poses a multitude of challenges for the ophthalmologists. Frequent follow-ups are needed in patients with AS for the timely management of its ocular manifestations and better visual rehabilitation. It is an autosomal dominant inheritance disorder with complete penetrance, but variable expression. The prevalence reported is 1 in 65000 live births with a total of 300 cases until 2019 [3]. The disorder occurs due to a gain of mutation in fibroblast growth factor receptor protein on chromosome 10q. Restricted anterior skeletal growth occurs due to early fusion of coronal sutures leading to shallow orbits and proptosis. Ocular features of AS included down-slanting palpebral fissure, hypertelorism, strabismus, proptosis, ptosis, lid malpositions, nasolacrimal dysfunction, refractive errors, papilledema, optic atrophy, and exposure keratopathy [4-6]. Strabismus is the most common association in patients with craniofacial dysostosis. V-pattern horizontal strabismus is the most common strabismus reported [7]. Excyclorotated orbits cause aberrant insertion or malrotation of extra ocular muscles leading to incomitant horizontal strabismus, most commonly exotropia . Herein, we aimed to present the challenging squint management in a case of Apert Syndrome.Apert Syndrome (AS), a rare form of acrocephalosyndactyly, which was first described by Wheaton in 1894, is a congenital abnormality characterized by craniosynostosis, midface hypoplasia, and syndactyly of limbs. The syndrome is named after the French pediatrician Eugene Apert, who first described nine people with similar characteristics in 1906 . Another theory suggests that a pseudo IOOA is created since the medial recti pull the eye up, whereas the lateral recti pull the eye down by the virtue of their shifted insertions in a malrotated orbit [11]. Moreover, the lack of inferior support to the inferior oblique in a shallow orbit may also cause an IOOA. In our case, we did not find any torsion on fundus imaging, thus ruling out the presence of true IOOA. Thus, we assumed shifted insertions of the horizontal recti to be the cause of V pattern in our case and upshifted both the lateral recti. However, in addition we also performed a bilateral inferior oblique recession since the V pattern was too large to be corrected by lateral recti transposition alone.Strabismus is a known ocular manifestation of AS. Excyclorotated orbits cause aberrant insertion or malrotation of extra ocular muscles leading to incomitant horizontal strabismus, most commonly exotropia [11]. The management of squint in AS and other craniosynostosis poses a multitude of challenges for the ophthalmologists. In our case, surgical management was a challenge due to the left head tilt at all times as a result of cervical vertebrae fusion, down slant palpebral fissure - as a result of which appearance did not match with measurement, attention deficit aggressive child- making squint measurement even difficult, frequent associated ocular comorbidities and multiple systemic surgeries, which had already demotivated the parents, who did not understand the importance of strabismus correction. What was added to the agony was the changing proptosis and strabismus with cranial surgery and orbital growth. In fact, children may not present with proptosis at birth; the onset of symptoms may occur postnatally, which may progress over the first few years of life [9]. A study undergone by Diamond et al. showed that only 9 out of 140 cases had changes in squint after cranial surgery and summarized that early surgery should be done to restore binocularity [12]. Clement et al. stated that proptosis increases with age, changing the squint pattern. Hence, it is advisable to wait until later [13]. Miller et al. stated that 18-24 months of age is the ideal age for strabismus surgery [14]. Clement et al. also proposed that not only the surgery of the rectus muscle, but also the weakening of the oblique muscle should be performed to ensure an effective result [13]. In our case, the surgery was performed at 2.5 years old, that is, one year and 9 months after the craniotomy surgery. This age was selected in our case since the squint had been stable for the past 6 months and adequate occlusion therapy had been done to ensure free alternation of fixation between the two eyes.This makes the appropriate timing for surgical intervention for strabismus a controversial issue. The decision to delay surgery to wait for progression may be at the cost of loss of binocularity in such children. Various studies have advocated different timings of surgery in cases of AS. Morex et al. stated that 6 months to 1 year after cranial and mid facial surgery represents the ideal time for squint correction [There are numerous theories pertaining to the etiology of V-pattern strabismus and oblique muscle dysfunction in patients with craniofacial dysostosis. The condition seems to be a result of complex anatomical anomalies. The associated strabismus complication might change patterns with the advancing age of patient, due to progressive bone alterations. Hence, sequential and titrated surgeries at least a few months after an early craniotomy surgery are advised for better ocular alignment and adequate visual and binocularity development. To conclude, frequent follow-ups, including age based visual acuity assessment and prescription of glasses along with management of amblyopia, orthoptic evaluation and management, appropriate prevention or treatment of exposure keratopathy and regular fundus examinations to monitor optic atrophy, are needed in patients with Apert Syndrome for a timely management of its ocular manifestations and better visual rehabilitation.Conflict of InterestThe authors state no conflict of interest.Informed Consent and Human and Animal Rights statementsWritten informed consent has been obtained from the guardians to share the information, medical records, including pictures of the patient. Authorization for the use of human subjectsEthical approval: The research related to human use complies with all the relevant national regulations, institutional policies, is in accordance with the tenets of the Helsinki Declaration, and has been approved by the institutional review board/ committee of Armed Forces Medical College, Pune, Maharashtra, India.AcknowledgementsNone.Sources of FundingNone.DisclosuresNone.


Pigmented basal cell carcinoma is a rare cause of vulvar neoplasms that has previously been treated with wide local excision or vulvectomy.This article highlights the importance of genital examination in skin examinations, describes the presentation and mimickers of vulvar-pigmented basal cell carcinoma, and most importantly, encourages the use of Mohs micrographic surgery for vulvar pathologies to minimize disfigurement, as well as psychological and sexual consequences of other surgical approaches.A 52-year-old Hispanic female with a history of undifferentiated connective tissue disease and autoimmune hepatitis presented to a dermatology clinic for a bleeding pigmented lesion on her labia majoris. The patient initially noted the growth 3 years prior, when it bled for several days. She denied a history of preceding trauma, radiation, or surgical procedures in the area. Past cervical screening tests were negative for HIV and high-risk human papillomavirus subtypes. She had no personal or family history of skin cancer. 11 months before presentation, she began azathioprine for autoimmune hepatitis.Physical examination revealed a 1.4 cm irregularly shaped, inflamed plaque with black pigmentation on the right labia majora  and often presents with a delay of several months before patients seek care (2 months–20 years). On evaluation, the pigmented lesions vary in size from 2.5 mm to 6 cm (2.13 cm average). Approximately 66% of patients (18/27) were of Asian ethnicity (Table 11 Two out of 27 (7.4%) pigmented vulvar BCC cases reported either perineural or perivascular invasion and were at least 6 cm at presentation. Conventionally, vulvar BCCs have been treated with local excision or vulvectomy, which have higher risks of disfigurement, and psychological and sexual consequences.12 Moreover, the risk of recurrence with local excision is 20%, and metastases have been reported.13 Our patient is the first pigmented vulvar BCC case reported in the literature that has been treated with MMS, an efficacious method for all vulvar malignancies.12 No recurrences of vulvar BCC have been reported with MMS.12Vulvar BCC can sometimes be an aggressive neoplasm with deep local extension and perineural invasion.In summary, we present a case of a pigmented BCC on the vulva of an immunosuppressed Hispanic patient following a 3-year delay in diagnosis, which was treated with MMS, and review the current literature of other cases. Given the atypical location and presentation, pigmented vulvar BCCs can be a diagnostic challenge. We encourage clinicians to consider pigmented BCC on their differential for atypical pigmented vulvar neoplasms. Importantly, skin cancer screenings should include thorough genital examination in all populations, especially in immunosuppressed patients. Additional studies are necessary to clarify the pathogenesis and optimal management of pigmented vulvar BCC.None.None.N/AMP: Performed the literature review and drafted the manuscript. KL and MON: Critically revised the manuscript.Consent for publication of the patient photographs was obtained at the time of article submission. A copy of the submission was made available for the patient.


Old traumatic disjunction between the cervix and the uterine corpus is very rare case. In most cases, it is not immediately noticed until the onset of other symptoms, such as amenorrhea, periodic abdominal pain and so on. Scanty cases of anastomosis surgery via laparoscope have been reported.We report here a 23-year-old young woman with the primary amenorrhea due to traumatic cervico-isthmic disjunction. The patient had a closed pelvic fracture at the age of 4 and has experienced periodic lower abdominal pain since the age of 17 years. A complete disjunction between the cervix and the uterine corpus was diagnosed. Laparoscopic cervico-isthmic anastomosis was performed to restore the continuity of the endometrial cavity and cervical canal. After this surgery, normal menstruation was resumed without cyclic abdominal pain.Laparoscopic cervico-isthmic anastomosis could reconstruct the uterine outflow tract successfully, alleviate symptoms, and achieve a good short-term outcome. Cervico-isthmic disjunction has two types, congenital dysplasia and traumatic. Both types are extremely rare. The former is mostly accompanied by other genitourinary developmental abnormalities, while the latter is mostly caused by pelvic trauma and might be combined with the other damage on the genital organs. Traumatic disjunction between the cervix and the uterine corpus is usually unnoticed in childhood due to the absence of symptoms. While growing up, patients have the secondary sexual characteristics in normal level. As a consequence of the uterine outflow tract obstruction, in adolescence the related clinical symptoms may appear, such as amenorrhea, periodic abdominal pain, endometriosis, adenomyosis or even infertility, etc.A 23-year-old nulligravida with a history of primary amenorrhea was referred to our hospital for surgical treatment. Her main complaint was that since the age 17 she has had the cyclic lower abdominal pain for 2–3 days once a month. She had normal breast development at age of 13 and never had cyclical menstrual symptom. She reported a history of a pelvic trauma during a vehicle accident at age of 4, which was handled nonsurgically. When consulting in another hospital in Shanghai in July 2021, her pelvic magnetic resonance imaging (MRI) suggested a complete disjunction between the cervix and the uterine corpus combined with the bilateral adnexal cysts  was initiated, in order to accelerate the wound healing in uterine and completely restore the function of the uterus. After three artificial menstrual cycles, combined oral contraceptives were used to prevent endometriosis recurrence. At one month after surgery, the ultrasound examination showed that the myometrium of the lower uterine segment was continuous Fig.  and therCervico-isthmic disjunction is extremely rare condition. The patient with a normal development of the cervix and the corpus but a transection isthmus, indicated that this transection occurred after the fusion and lumenization of the bilateral müllerian ducts had completed. Thus these findings were not consistent with müllerian hypoplasia. Given the history of pelvic fracture in childhood, old traumatic disjunction between the cervix and the uterine corpus was suspected.The female reproductive organs are protected by the pelvic bony structure and rarely subjected to injuries. Pelvic fracture, a hallmark of higher energy trauma, may deform the pelvis and damage the pelvic organs. Up to date, only six similar cases were reported  or CT urography (CTU) were performed to check the urinary system for the presence of any abnormalities. Tumor markers are mostly within the normal range, and blood CA125 levels may be elevated in the presence of endometriosis. The Chromosomal examination was normal. Female sex hormone levels are mostly within the normal range.Traumatic disjunction between the cervix and the uterine corpus needs to be differentiated from complete obstructive genital malformations, such as congenital cervical atresia, transverse vaginal septum, imperforate hymen, etc. Detailed clinical history and imaging modalities such as ultrasonography or MRI are helpful for diagnosis and differential diagnosis.Once cervico-isthmic disjunction is confirmed, it should be treated surgically as soon as possible. The aim of surgery is first to relieve symptoms and second to preserve fertility function. Compared with trauma, the delay in surgery does not affect the likelihood of anatomical recovery, however, early diagnosis can avoid severe pelvic endometriosis and infertility. Among patients with congenital cervical atresia, early diagnosis and surgery seem to be necessary to prevent endometriosis . EvidencPostoperatively, prompt management is warranted to prevent adhesion of the anastomotic site and cervical stent seems to be a good choice. In patients with congenital cervical atresia, the cervical canal with no columnar epithelium but fibrous connective tissue is highly prone to adhesion, stenosis, and atresia postoperatively. It has been reported that 60% patients with a preserved uterus underwent a second surgery because of re-obstruction of the neocervical canal in patients with congenital cervical atresia . Our casIn patients who are not currently planning to conceive, medical therapy is usually administered after surgery to reduce the rate of postoperative cyst recurrence and pain recurrence . Most puIn summary, amenorrhea with cyclic lower abdominal pain in patient with pelvic traumatic history should be on the alert for old traumatic disjunction between the cervix and the uterine corpus. It is not so difficult to make the diagnosis from the time it was evoked. Laparoscopic cervico-isthmic anastomosis is a minimally invasive way to reconstruct the uterine outflow tract successfully, alleviate symptoms, and obtain better short-term outcomes. Further case studies and longer follow-up are needed to better understand whether laparoscopic approach has advantages over laparotomy in the recovery of postoperative fertility.


Kernohan–Woltman phenomenon is a rare and paradoxical neurological situation in which a transtentorial lesion leads to compression of the contralateral cerebral peduncle responsible for compression of the descending corticospinal fibers with clinical consequence of a motor deficit ipsilateral to the primary lesion. This phenomenon should attract the attention of clinicians in order to avoid unfortunate incidents such as wrong‐side craniotomy in neurosurgical practice. In this work, we report a similar situation.The Kernohan–Woltman notch phenomenon is a rare and paradoxical neurological situation in which transtentorial damage is observed leading to compression of the contralateral cerebral peduncle responsible for compression of descending corticospinal fibers with the clinical consequence of a motor deficit ipsilateral to the primary lesion. This phenomenon has been found in several situations including tumors and cerebral hematomas after craniocerebral trauma. In this work, we have reported the case of a 52‐year‐old man with hemiparesis ipsilateral to a large chronic subdural hematoma. It was first described by Kernohan and Woltman in 1929, through postmortem studies of 297 patients following cases of false localization observed at the time.The causes of this phenomenon are multiple and among them, we have slowly evolving supratentorial extraparenchymal mass lesions; such as neoplasms or chronic subdural hematomas.2We report the case of a 52‐year‐old man who, 1 month before his admission to our hospital, received a blow to the head in the right parietal region, then fell, the mechanism of which was not specified by those accompanying him, and presented with a brief initial loss of consciousness. One week after the fall, he began to present with holocranial headaches. Due to a lack of means to seek medical attention, he attended a prayer room with no change in symptomatology. Six hours before his admission, he presented with an abrupt alteration in consciousness, which motivated his relatives to bring him to our hospital for better management. On admission, he had a Glasgow score of 12 (O3V4M5), anisocoric pupils with reflex mydriasis to right light. He also had right central facial paralysis and right hemiparesis.A brain scan was performed which showed a large right hemispheric subdural hematoma with subfalcoral involvement Figure  and a KeOn the second day, the drain was removed after bringing back a total of 150 cc of serohematic fluid, and on the fifth day, he was discharged without any neurological deficit.3The corticospinal pathways originate from the motor cortex in the frontal lobes and descend through the internal capsules and then through the midbrain and annular protrusions, before the majority of these motor fibers (80%) undergo decussation in the medulla oblongata.The Kernohan–Woltman notch phenomenon Figure  is a decThis paradoxical neurologic sign or false localization was first described in 1929 by Kernohan and colleagues during autopsy examination of a patient with a brain tumor whose brain was nicked by the peduncle of a contralateral hernia.Compression of the contralateral peduncle is thought to disrupt fibers of the corticospinal tract above the decussation, resulting in hemiparesis ipsilateral to the primary lesion.Other clinical signs, apart from hemiplegia or hemiparesis, may be observed, including ipsilateral mydriasis, divergent gaze, or ptosis secondary to the primary lesion with oculomotor nerve involvement, and ipsilateral paralysis of the superior motor nerve of the face due to involvement of corticobulbar fibers of the cerebral peduncle.A good neurological examination combined with medical imaging plays an important role in the diagnosis. Brain magnetic resonance imaging may show T2 hyperintensity of the contralateral cerebral peduncle.It is very crucial to recognize this syndrome because of the risk of error in the neurosurgical procedure by operating on the wrong side with respect to the location of the lesion. Wolf RFE and colleagues4The Kernohan–Wotman phenomenon is a rare, paradoxical situation but not to be ignored in clinical neurosurgical practice. Several factors can lead to this phenomenon among which we have cerebral hematoma after cranioencephalic trauma. A good neurological examination and magnetic resonance imaging or CT scan are necessary for diagnosis. This phenomenon should attract the attention of clinicians in order to avoid unfortunate incidents such as craniotomy on the wrong side in neurosurgical practice.Romo Bujiriri Murhega: Conceptualization; writing – original draft; writing – review and editing. Maheshe Balemba Ghislain: Supervision; validation. Paterne Safari Mudekereza: Supervision; validation. Sudi Musilimu: Writing – original draft. Igega Bisimwa: Visualization; writing – original draft. Paul Munguakonkwa Budema: Supervision; validation. Léon‐Emmanuel Mubenga: Supervision; validation.The authors have no conflict of interest to declare.None.This case report received ethical clearance from the Ethical committee of the catholic University of Bukavu.Written informed consent was signed by the patient prior to the publication of this paper.