Surgical management of a 25-cm giant hepatic haemangioma: First case report from Bangladesh with literature review.

INTRODUCTION AND IMPORTANCE: Giant hepatic haemangioma (GHH) is defined as a hepatic haemangioma (HH) of >10 cm in diameter. Its association with thrombocytopenia and consumption coagulopathy is quite rare. CASE PRESENTATION: Here, we present a case of a 39-year-old man with a rapidly enlarging 25-cm GHH arising from the entire left hemiliver. Laboratory findings suggested pancytopenia but normal liver and renal functions. He was diagnosed with Kasabach-Merritt syndrome (KMS). After three units of aphaeretic platelet transfusion, the patient underwent left hepatectomy. Postoperative recovery was uneventful, and his regular follow-up revealed no recurrence even after two years. CLINICAL DISCUSSION: HH predominantly affects females, but males can also be affected, as seen in this case. With observation, it can grow over time, particularly in patients under 50 years of age. Surgical management should be considered when HH causes symptoms or is larger than 10 cm. The evolving understanding of GHH and the critical role of surgery are important, particularly when they complicate haematological or coagulation profiles and lead to thrombocytopenia. CONCLUSION: Our case report highlights the significance of surgical intervention in GHH, and a disease-free outcome can be expected for patients with this condition in the future. To our knowledge, this is the first such case report from Bangladesh.

Kasabach-Merritt Syndrome: a case study of successful treatment with vincristine and propranolol.

Kasabach-Merritt syndrome is a rare condition, characterised by the presence of an enlarging vascular tumour associated with thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy. The syndrome manifests in infancy, with high morbidity and mortality rates. No standard guidelines have been established for the treatment of Kasabach-Merritt syndrome to date. To existing literature we add this report of a four-month-old female child with Kasabach-Merritt syndrome who was successfully treated with propranolol and vincristine. This drug combination helped reverse the severe thrombocytopenia as well as decrease in size of her haemangioma. Management of Kasabach-Merritt syndrome continues to be a challenge, with varying response to first line drugs. Early diagnosis and initiation of treatment in a closely monitored setting is essential to ensure good outcomes. Since this is a relatively rare condition and large studies are not feasible, documenting treatment experience for single cases or small series becomes even more important.

Laparoscopic cholecystectomy for symptomatic cholecystolithiasis (CCL) in "Kasabach-Merritt syndrome" (KMS) (Kaposi-tumor like hemangioendothelioma with case-specific perioperative management).

Objectives: The Kasabach-Merritt syndrome (KMS) is characterized by the occurrence of hemangioendothelioma (giant hemangioma with thrombosis leading to thrombocytopenia), which can be associated with disseminated intravasal coagulation. Specific aim: Based on (i) selective references from the current scientific literature and derived recommendations as well as (ii) own experiences obtained in the diagnostic and perioperative management of a representative case from daily practice in abdominal surgery, the specific case undergoing elective cholecystectomy (CCE) in KMS is to be described by means of scientific case report. Case presentation: (Patient-, finding- and treatment-specific characteristics): - Medical history: 72-years old female patient with a known KMS of the left arm and upper thorax, recurrent thrombophlebitis of the left arm and thoracic veins, previous upper GI bleeding (Mallory-Weiss syndrome in 2006, chronic anemia in lack of vitamin B12, type-A gastritis, former bleeding complications after teeth extraction/open appendectomy 1962/Caesarean section 1968 with need of transfusion [60 red blood cell packages]), intraabdominal adhesions, hypothyreosis, initial liver cirrhosis. - Symptomatology: Characteristic for cholecystolithiasis (CCL). - Diagnostic: Abdominal ultrasound shows CCL, fibroscan does not confirm suspicious cirrhosis. Laboratory parameters showed: Activation of intravasal coagulation with elevated prothrombin fragments, D-dimers and reduced antiplasmin concentration. Accelerated fibrinolysis capacity; currently, no secondary thrombocytopenia or factor-13 decrease. In addition, fibrinogen concentration within normal range, no hint onto the manifestation of an aquired von-Willebrand's syndrome. - Diagnosis: Chronic fibrosing cholecystitis in CCL after former acute cholecystitis (3 months ago) with indication for surgical intervention. - Therapy: Laparoscopic CCE including careful exploration of upper abdominal cavity for KMS manifestation (with no revision of bile duct) and peritoneal adhesiolysis (histological finding, chronic fibrosing cholecystitis with thickening of the wall of the gall bladder but no hint of malignancy) under perioperative prophylaxis with antibiotics and temporary cessation of platelet medication for 7 d preoperatively, "bridging" with low molecular weight heparin (Clexane, 1 × 40 mg s.c.; Sanofi-Aventis, Frankfurt/Main, Germany); 1 h preoperatively, 15-20 mg/kg body weight Cyclocapron i.v. (once again 6-8 h postoperatively; thereafter, 500 mg of Cyclocapron 4×/d until the 3rd postoperative day). - Intraoperatively: Congestion of veins but not at the immediate surgical field (gall bladder, hepatic bed of the gall bladder, Calot's triangle). - Outcome: Uneventful, in particular, no (bleeding) complications. Conclusions: If surgical approach is indicated, the intervention should be thoroughly planned (in particular, under elective circumstances) with regard to hemangioma site and extension as well as distance to the surgical field and possible surgical alternative options (surgical access site, open/laparoscopic approach etc.) to prevent - at the best possible rate - bleeding complications intra-/postoperatively and, thus, to provide adequate patient safety.

Kasabach-Merritt Syndrome, an underdiagnosed swollen leg in newborn with anemia and jaundice.

Key Clinical Message: Kasabach-Merritt syndrome is a rare disease. Early recognition, timely transfer, and proper management are crucial in reducing bleeding complication and mortality. Abstract: The authors report a rare case of Kasabach-Merritt syndrome in a Cambodian neonate presenting with anemia and jaundice at Day 5 of life. Since the mass was not recognized at birth, the diagnosis and treatment were delayed, thus leading to demise.

Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon in an infant girl.

Key Clinical Message: We report a successful treatment course of an infant with mediastinal Kaposiform hemangioendothelioma. As the current complex of diseases is rare and calls for highly specialized treatment, large prospective studies are lacking. This case provides an example of balanced treatment complicated by Kasabach-Merritt phenomenon, life-threatening infections, and pericardial effusion. Abstract: Kaposiform hemangioendothelioma (KHE) and tufted angioma are vascular benign tumors that can be associated with the rare condition Kasabach-Merritt Phenomenon (KMP). KMP is characterized by consumption coagulopathy with severe thrombocytopenia, hypofibrinogenemia, and elevated D-dimer. We here report successful treatment of a female infant with a mediastinal KHE where treatment was complicated by KMP, life-threatening infections, and pericardial effusion. Due to the absence of randomized clinical trials, there is currently no standardized treatment protocol available for KHE. In our case, the infant was treated successfully with prednisolone, vincristine, and sirolimus.

Kaposiform hemangioendothelioma of the thigh: A case report.

Kaposiform hemangioendothelioma is a rare, locally aggressive or borderline vascular tumor that typically affects infants. It presents as a purpuric cutaneous lesion and may be associated with life-threatening coagulation disorders, such as the Kasabach-Merritt phenomenon. The differential diagnosis can be challenging based on clinical presentation alone. Imaging plays a crucial role in the diagnostic workup, particularly magnetic resonance imaging. We present a case report of a 4-month-old patient with an enlarging vinous cutaneous mass on the thigh and coagulation abnormalities. Magnetic resonance imaging revealed a large, infiltrative, soft-tissue lesion with poorly defined margins and heterogeneous enhancement, that involved all muscle compartments of the thigh and was associated with lymphedema, stranding of the subcutaneous fat and cutaneous thickening. These findings were consistent with kaposiform hemangioendothelioma of the thigh and the diagnosis was confirmed by histopathological characterization.

Kasabach-Merritt syndrome complicating a giant hemangioma of the liver: A case report.

Liver hemangiomas are the most common benign tumors of the liver with a prevalence of 1%-20% according to autopsy series. They can in some cases reach measurable sized. These giant hemangiomas can have fatal complications such as hemorrhaging, intraperitoneal rupture, mass of effect and Kasabach-Merritt syndrome. We report an adult case in which recent right quadrant pain revealed a liver hemangioma associated with Kasabach-Merritt syndrome.

Case Report: CTC1 mutations in a patient with diffuse hepatic and splenic hemangiomatosis complicated by Kasabach-Merritt syndrome.

Diffuse hemangiomatosis of the liver and spleen is rare. Currently, few studies are available on diffuse hepatic and splenic hemangiomatosis accompanied by Kasabach-Merritt syndrome (KMS). The conserved telomere maintenance component 1 (CTC1) gene contributes to telomere maintenance and replication by forming the telomeric capping complex. Herein, we report a case of diffuse hemangiomatosis in the liver and spleen accompanied by KMS in a 59-year-old woman who carried two novel heterozygous CTC1 variants: c.435+9A>C and c.3074C>T (p.Ala1025Val). Using next-generation sequencing, we detected mutations in the CTC1 gene in our patient, who had chief complaints of fatigue and abdominal distension complicated by severe thrombocytopenia and consumptive coagulopathy. Clinical symptoms, laboratory tests, and imaging findings led to the diagnosis of diffuse hepatic and splenic hemangiomatosis accompanied by KMS. The patient was treated with prednisone, thalidomide, and sirolimus, and her general condition was ameliorated at the 4-month follow-up with improved platelet count and coagulation function. A CTC1 gene mutation may be involved in the pathological process of vascular diseases. A combination treatment regimen of prednisone, thalidomide, and sirolimus may be effective for KMS.

Kaposiform haemangioendothelioma of the spine associated with fixed hyperlordotic deformity and Kasabach-Merritt Syndrome: a case report and literature review.

Kaposiform haemangioendothelioma (KHE) is a rare childhood disease classified by the International Society for the Study of Vascular Anomalies (ISSVA) as a locally aggressive vascular tumor. It has been reported to affect any site, with a predilection for the extremities and trunk. Although it typically manifests as an enlarging cutaneous or soft tissue lesion, less than 10% of cases have no skin involvement, with the retroperitoneum being the most frequently involved extracutaneous site. Approximately twenty cases of KHE with bony involvement have been reported in the literature to date, with only five of those cases involving the spine specifically.We present a, rare case of KHE who presented with progressive fixed hyperlordotic deformity, multiple non-specific spinal lesions, and abnormal blood tests, posing a clinical and radiological diagnostic challenge. Additionally, we conducted a thorough review of the literature to compare and contrast the various multimodality imaging manifestations of KHE involving the spine.

Cocktail therapy with prednisolone, vincristine and sirolimus for Kasabach­Merritt phenomenon in 10 infants.

Kasabach-Merritt phenomenon (KMP) is a life-threatening condition caused by rare vascular tumors. To reduce drug resistance observed in monotherapy of KMP with prednisone, vincristine (VCR) or sirolimus, the present study evaluated the efficacy and safety of triad therapy in the treatment of KMP. A total of 10 KMP infants managed with prednisolone, VCR and sirolimus in The Second Affiliated Hospital of Xi'an Jiaotong University (Xi'an, China) between April 2017 and August 2021 were retrospectively reviewed. The three female and seven male infants with KMP underwent cocktail therapy with prednisone, VCR and sirolimus. At diagnosis, the infants, aged 49.1±41.0 days, showed laboratory test results with platelet counts 22±15.4x109/l, fibrinogen 81.7±26.9 mg/dl and D-dimer 38649±13443.6 ng/ml. The average maximal diameter of the tumors at diagnosis was 84.5±25.1 mm. KMP risk is increased by large tumors with deep lesions infiltrating the muscle. Platelet counts normalized after a median 10 days (range, 5-69 days) of treatment. With combination therapy maintained for 46.8±24.4 days, ultrasound showed that the thickness of the tumors decreased by 51% from 28.9±12.1 to 13.9±6.2 mm. Neutropenia and gastrointestinal disorders were the most common adverse effects. The present study found that the cocktail therapy with prednisolone, VCR and sirolimus has favorable tolerance and efficacy for life-threatening KMP. Once a stable condition has been achieved, cocktail therapy should be replaced by sirolimus monotherapy to reduce potential side effects.

A Case of Blue Rubber Bleb Nevus Syndrome With Kasabach-Merritt Syndrome and Heart Failure.

A 48-year-old Mongolian man developed bilateral leg edema after suffering from a fever for three months. He lost his appetite, and the edema gradually spread from the legs, becoming systemic. In addition, he had difficulty in moving. He had a history of being diagnosed with numerous venous malformations and Kasabach-Merritt syndrome when he was a child. On arrival, he had numerous venous malformations over pale skin, edema at each extremity, and anemic conjunctiva. Chest roentgen showed bilateral pleural effusion, and cardiac echography findings showed a left ventricular ejection fraction of 30% with diffuse hypokinesis. The results of a blood analysis showed coagulopathy, which was compatible with disseminated intravascular coagulation and pancytopenia. He was diagnosed with blue rubber bleb nevus syndrome with Kasabach-Merritt syndrome and heart failure. Use of diuretics, thiamine, iron, phytonadione, carbazochrome, and tranexamic acid, in addition to intermittent transfusion resulted in the improvement of his Kasabach-Merritt syndrome. Radical management of blue rubber bleb nevus syndrome was deemed impossible by dermatologists due to the large amount of venous malformations. We encountered an extremely rare case of blue rubber bleb nevus syndrome with Kasabach-Merritt and heart failure. Multimodal therapy might help manage Kasabach-Merritt syndrome following improvement in coagulopathy and pancytopenia.

A rare case of Kasabach Merritt Syndrome presenting with an infantile hemangioma: A case report.

Introduction: and importance: Infantile hemangioma, being a benign tumor of the blood vessel, is part of a triad composed of also thrombocytopenia and hypofibrinogenemia as part of Kasabach Merrit Syndrome. Case presentation: We report the case of a 2 months old female Saudi infant referred due to respiratory distress, thrombocytopenia, and enlarging hemangioma on right upper chest, neck, and lower cheek. Diagnosis of kaposiform hemangioendothelioma complicated by Kasabach Merritt thrombocytopenia was done based on the clinical triad of thrombocytopenia, bleeding tendency, and the presence of a vascular tumor. Conclusion: Vincristine and CTA embolization are lines of management that showed to be the most efficient in the improvement of the clinical picture of KMS in our patient.

Long-term outcomes of low-dose radiotherapy in Kasabach-Merritt syndrome.

PURPOSE: Reports on results of radiation therapy (RT) for Kasabach-Merritt syndrome (KMS) are limited. We performed a retrospective study to evaluate the response rates and late complications and to determine the adequate RT dose for patients with KMS patients. MATERIALS AND METHODS: We studied 11 patients who received RT between October 1988 and September 2008 for KMS refractory to pharmacologic therapy. All patients had external hemangiomas and received the diagnosis of KMS within 12 months of birth. All 11 patients received steroids as the first-line therapy; eight patients additionally received interferon-α therapy, and one patient underwent surgery. Nine patients underwent single-course RT with a total dose of 4.5-8 Gy (1.5-2 Gy/fraction). Two patients received multiple courses of RT, with a cumulative total dose of 12 Gy (2 Gy/fraction) and 18Gy (1.5 Gy/fraction), respectively. RESULTS: The median follow-up period was 156 months (interquartile range [IQR], 75 to 226 months). The median total dose of RT was 6 Gy, and all patients maintained complete remission until the last follow-up. An additional course of RT was performed for refractory cases or cases of local relapse after initial RT. Rapid platelet count increase after RT was seen in most patients, which returned to normalcy in a median of 20 days (IQR, 5 to 178 days). However, seven patients experienced radiation-related long-term complications. CONCLUSION: Low-dose RT is effective and yields rapid response in patients with KMS. However, given growth-related late complications, RT should be carefully considered.

Fetal neck giant hemangioma associated with postnatal arising Kasabach-Merritt syndrome.

We report a prenatal ultrasound dia-gnosis of giant neck hemangioma at 30+1 weeks in a fetus resulting in the postnatal development of Kasabach-Merritt syndrome. Ultrasound scan revealed a large isoechoic mass occupying the whole neck, infiltrating the nasopharyngeal cavity, tongue, lower lip and mandible. Complex sonographic visualization with 2D and 4D was helpful in the process of parental counseling.

Primary Kaposiform Hemangioendothelioma of the Humerus: A Case Report.

Kaposiform hemangioendothelioma (KHE) is a neoplasm originating mainly from vessels and has a mild proclivity for malignancy. This neoplasm mainly involves somatic soft tissue and retroperitoneum. Histological findings include a nodular arrangement of oval-to-spindle cells containing pale cytoplasm. Vascular spaces are in the form of slit-like channels in which red blood cells are evident. Here, we report the case of a two-year-old male who presented with Erb's palsy and a mass lesion in the right humerus. Tissue biopsy features were compatible with KHE.

The Impact of COVID-19 On Comorbidities: A Review Of Recent Updates For Combating It.

Coronavirus disease is caused by the SARS-CoV-2 virus. The virus first appeared in Wuhan (China) in December 2019 and has spread globally. Till now, it affected 269 million people with 5.3 million deaths in 224 countries and territories. With the emergence of variants like Omicron, the COVID-19 cases grew exponentially, with thousands of deaths. The general symptoms of COVID-19 include fever, sore throat, cough, lung infections, and, in severe cases, acute respiratory distress syndrome, sepsis, and death. SARS-CoV-2 predominantly affects the lung, but it can also affect other organs such as the brain, heart, and gastrointestinal system. It is observed that 75 % of hospitalized COVID-19 patients have at least one COVID-19 associated comorbidity. The most common reported comorbidities are hypertension, NDs, diabetes, cancer, endothelial dysfunction, and CVDs. Moreover, older and pre-existing polypharmacy patients have worsened COVID-19 associated complications. SARS-CoV-2 also results in the hypercoagulability issues like gangrene, stroke, pulmonary embolism, and other associated complications. This review aims to provide the latest information on the impact of the COVID-19 on pre-existing comorbidities such as CVDs, NDs, COPD, and other complications. This review will help us to understand the current scenario of COVID-19 and comorbidities; thus, it will play an important role in the management and decision-making efforts to tackle such complications.

Transarterial chemoembolization for Kasabach-Merritt syndrome caused by hepatic angiosarcoma: A case report.

Hepatic angiosarcoma is a rare disease, and hepatic hemangiosarcoma with Kasabach-Merritt syndrome (KMS) is even rarer. Although there have been several reports about KMS caused by hepatic angiosarcoma, there has been no mention of successful treatment regimens for hepatic angiosarcoma with KMS. A 64-year-old female patient presented with right upper abdominal pain and multiple cutaneous purpuras for 10 days. Blood analysis revealed that hemoglobin, platelet and fibrinogen were significantly decreased, prothrombin time was prolonged, fibrinogen degradation products were increased. Contrast-enhanced computed tomography scan of the abdomen demonstrated a large mass in the right lobe of the liver, which is pathologically suggestive of hepatic angiosarcoma. Based on the above examination, the patient was diagnosed with KMS caused by hepatic angiosarcoma. Repeated transfusion of blood products could only temporarily improve the coagulation function of the patient.  After transarterial chemoembolization, the patient experienced a long-term improvement of blood clotting, and the patient's survival increased by six months. Transarterial chemoembolization should be considered one of effective therapies for hepatic angiosarcoma with KMS.

Surgical Management of Giant Hepatic Haemangioma - Need for Redefining the Nomenclature According to the Size.

INTRODUCTION: Haemangiomas are the most common benign tumours of the liver. Most of them are asymptomatic. Giant hepatic haemangioma is defined as size greater than 5 cm in diameter. The surgical treatment for giant hepatic haemangioma is not very well defined and reported. Here we analysed the treatment outcome of giant hepatic haemangiomas and redefined the nomenclature according to the size and proposing an algorithm for management of all hepatic haemangiomas. MATERIAL AND METHODS: Retrospective analyses of 6 giant hepatic haemangioma more than 10 cm in size treated by a single surgeon were included. The clinical characteristics, diagnosis, managements and outcomes were recorded. Review of literature was done for definitions, management strategies and outcome of giant haemangiomas and an algorithm was proposed. RESULTS: Five patients were female and the mean age was 36.6 years (range 32 to 45). Pain abdomen was the most common symptoms (100%). Ultrasound abdomen was the initial diagnostic modality followed by contrast enhanced computed tomography. The mean size of haemangioma was 17.4cm (range 12cm to 32cm). Four patients had haemangioma in the left lobe and two had in the right lobe of liver. Formal hepatectomy was done in three patients and enucleation was done in three patients. The mean operating time was 3.66 hours (range 2.5 hours to 5 hours). The mean blood loss was 840ml (range 300ml to 1500ml). There was no surgical morbidity or mortality. On follow-up no haemangioma related complications are reported. CONCLUSIONS: Giant hepatic haemangioma is mostly symptomatic. Hepatectomy and enucleation can be done without significant morbidity and mortality. There is a need for redefining the nomenclature of giant hepatic haemangiomas according to the size.

Tratamiento con propranolol en el síndrome de Kasabach-Merritt secundario a hemangioma hepático congénito. Caso clínico / Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case

El hemangioma hepático es el tumor benigno de hígado más frecuente. Puede ser congénito o infantil, con diferentes evoluciones y complicaciones. La evolución clínica es muy variable, desde pacientes asintomáticos hasta cuadros de gravedad con insuficiencia cardíaca, síndrome de Kasabach-Merritt o síndrome compartimental. El diagnóstico se basa en la historia clínica y los estudios por imágenes, especialmente, la ecografía y el examen doppler en manos experimentadas. Resulta fundamental el diagnóstico diferencial con otras lesiones hepáticas, sobre todo, el hepatoblastoma. En los pacientes sintomáticos, el propranolol surge como primera línea terapéutica con buenos resultados y baja frecuencia de efectos adversos. Se presenta el caso de un recién nacido con hemangioma hepático asociado a síndrome de Kasabach-Merritt, con excelente respuesta y tolerancia al propranolol

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach-Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

[Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case]. / Tratamiento con propranolol en el síndrome de Kasabach-Merritt secundario a hemangioma hepático congénito. Caso clínico.

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach- Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

El hemangioma hepático es el tumor benigno de hígado más frecuente. Puede ser congénito o infantil, con diferentes evoluciones y complicaciones. La evolución clínica es muy variable, desde pacientes asintomáticos hasta cuadros de gravedad con insuficiencia cardíaca, síndrome de Kasabach- Merritt o síndrome compartimental. El diagnóstico se basa en la historia clínica y los estudios por imágenes, especialmente, la ecografía y el examen doppler en manos experimentadas. Resulta fundamental el diagnóstico diferencial con otras lesiones hepáticas, sobre todo, el hepatoblastoma. En los pacientes sintomáticos, el propranolol surge como primera línea terapéutica con buenos resultados y baja frecuencia de efectos adversos. Se presenta el caso de un recién nacido con hemangioma hepático asociado a síndrome de Kasabach- Merritt, con excelente respuesta y tolerancia al propranolol.