Abdominal Ultrasound Scan Research Articles

Pancreatic hydatid cyst in children diagnosis and surgical management

The pancreatic hydatid cyst location is exceptional, even in countries where hydatid disease is endemic. We report three cases of hydatid cyst of the pancreas revealed by abdominal pain and cutaneous jaundice. The diagnosis was based on abdominal ultrasound and CT scan that objectified a clean-walled cystic mass in the head of the pancreas. Hydatid serology (Elisa) was positive for all cases. Surgical treatment consisted to resect of the protruding dome in three cases and drainage. Through these three observations and a review of the literature, we discuss the diagnostic difficulties and the management of this rare localization of hydatid cyst in children.

Bilateral polyotia: case of a rare and atypical congenital malformation in a three-month-old infant.

Polyotia is an extremely rare congenital malformation of the external ear, characterised by the development of an accessory auricle. It is more often unilateral. We report the case of a Cameroonian infant whom we received at the age of three months. The infant´s mother had noticed a deformity concerning both ears since birth and did not observe any other associated symptom. On physical examination, we found an additional auricle fusing with the tragus on both sides. This unusual structure consisted of skin and cartilage. Their greater diameters measured 2.5cm and 2cm on the left and right respectively. Otoscopic evaluation and general physical examination were normal. A computerized tomography (CT)-scan of the temporal bones as well as an abdominal ultrasound scan were requested to explore other possibly associated anomalies. These imaging studies were normal. The diagnosis of isolated bilateral polyotia was made. An ear reconstructive surgery was proposed to the parents. An informed consent was obtained from the parents, pre-operative work-up was requested and the procedure was planned. A bilateral otoplasty was done under general anaesthesia. The procedure was simple, consisting of excision of the excess tissue to give a natural aesthetic result. Post-operative evolution was favourable, with good scarring on day 10, as illustrated in the figure. Nine months following the procedure, the parents seemed satisfied with the aesthetic result. Polytia is a rare congenital malformation. Management requires adequate and comprehensive investigations to exclude enchondroma or associated malformations, prior to ear reconstructive surgery.

Autoimmune hepatitis in a Northern Nigerian woman

Autoimmune hepatitis is an unrelenting inflammation of the liver of unknown etiology. It is a relatively rare disease that commonly affects women and mostly present as chronic hepatitis. It is basically classified into types 1 and 2. The patient is a 56 year old Northern Nigerian woman with background thyroid disease and rheumatoid arthritis who presented with jaundice and fatigue and elevated liver enzymes with increased serum total protein and low serum albumin. Further evaluation showed non-reactive hepatitis B and C markers, features of chronic liver disease on abdominal ultrasound scanning, elevated anti-nuclear and anti-smooth muscle antibodies and increased serum IgG. Liver histology showed features of chronic hepatitis consistent with AIH. Diagnosis of AIH type 1 was eventually made and patient was started on prednisolone and azathioprine combination therapy with improvement of liver function test few weeks after commencement of therapy. To the best of our knowledge, only 2 cases of AIH has been reported in Nigeria, all from Ibadan, Southern Nigeria. No report of AIH from Northern Nigeria. We therefore report a case of AIH type 1 to increase awareness among clinicians.

Acute Intestinal Invagination of the Child at the Teaching Hospital of Bouaké: Ultrasound Diagnosis and Clinical-Echo-Surgical Correlation

Background: Acute intestinal invagination or intussusception is the most common abdominal surgical emergency in infants, but it can occur at any age. Performing an ultrasound scan at the slightest clinical suspicion contributes to early diagnosis and therapeutic management. Objective: To show the relevance of ultrasound in the therapeutic management of the child’s intussusception through a correlation between the ultrasound diagnosis and the clinical and/or per operative diagnosis. Patients and Methods: It was a retrospective study of 24 cases from July 2017 to September 2020 (30 months) in the Medical Imaging and Paediatric Surgery departments of Teaching Hospital of Bouake. We included only patients from 0 to 15 years old who had digestive symptoms, an abdominal ultrasound scan. These patients were eligible for surgery. Data analysis was performed with Epi info 7 software. Results: Median age was 17.2 months [02 - 120]. Male gender predominated (83.3%). Clinico-biological data were dominated by abdominal pain (79.2%), vomiting (75%) and rectorragies (33.3%), with Ombredanne’s triad in 33.3% and hyperleukocytosis (70.8%). Ultrasonography showed invaginated coves (95.8%) sitting in the right angl iliac fossa, peri-umbilical and right flank in 73.9%. Cockade image coupled with the sandwich image accounted for 95.6%. Superficial adenopathies (45.8%) and peri-lesional fluid effusion (20.8%) were associated with it. An occlusive syndrome complicating intussusception was observed in 41.67% associated with intra-peritoneal effusion of fluid (25%). There was a significant diagnostic agreement between the clinic, ultrasound and surgery according to the Fisher’s exact test (p = 0.002). That between ultrasound and surgery was calculated at 95.4%. Conclusion: This study allowed us to show a good clinical-echo-surgical concordance. Ultrasound is therefore an undeniable diagnostic, prognostic and therapeutic tool in the exploration of intussusception, hence the interest of its prescription in the shortest possible time by the clinician.

Unusual Cause of Dysuria: A Large Primitive Retrovesical Hydatid Cyst

Hydatidosis is endemic in Morocco. Pulmonary and hepatic localisations are the most frequent. The primary retrovesical location is exceptional. It is considered an “aberrant” or “ectopic” location and represents less than 1% of all cases of hydatid disease. Its diagnosis is sometimes difficult, requiring radiological and serological examinations. The aim of this case report is to present a specific management of a primitive retrovesical hydatid cyst and to compare it with the review of literature. We report an exceptional case of a large primary retrovesical hydatid cyst in a 45-year-old patient with no significant pathological history, of rural origin with a notion of contact with stray dogs. Symptoms are of late onset and represented essentially by voiding disorders associated with episodes of constipation and a feeling of heaviness. Abdominal and pelvic Ultrasound and CT scan identified a rounded, multivesicular retrovesical cystic mass. Because of the existence of intimate adhesions with neighboring organs, we opted for treatment with a partial cysto-pericystectomy. The post-operative follow up was without incident with a good long term evolution. There was no local recurrence and the patient did not complain of any urinary disorder. In order to reduce the incidence of hydatid disease in endemic countries, the best treatment remains primary prevention.

Recurrent Group B Streptococcal Infection in a Neonate Associated with Infected Breast Milk

Group B Streptococcus (GBS) infection is known to be the leading cause of sepsis and meningitis in newborns, but the relapse rate is very rare, less than 1%. Recurrence has been reported in association with in cases of untreated abscess, undiagnosed congenital heart disease, maternal mastitis, and reinfection through breast milk. We report a 20-day-old full-term female infant who visited the emergency room due to fever and was diagnosed with recurrent GBS sepsis. She had been treated for 10 days with antibiotics for early onset disease and sepsis caused by GBS, and her mother was negative for GBS on prenatal screening. She was breastfed and her mother had no mastitis. Echocardiography, abdominal ultrasound, brain ultrasound, and bone scans were performed to find the cause of GBS reinfection and were normal. Culture of the breast milk yielded GBS. We report the relationship between recurrent GBS infection and breast milk with literature review.

EPS2.02 Characterisation of liver USS findings in pre-school children with cystic fibrosis

Objectives: European consensus guidelines recommend annual screening for cystic fibrosis-related liver disease (CFLD) with annual clinical, biochemical and ultrasound (USS) evaluation. Abdominal USS is initiated from age 5 in many centres across the UK;however, it has been our practice to initiate from age 2. In this study we aim to determine the incidence of liver abnormalities in young children as recent reduced access to radiological services due to the COVID-19 pandemic has prompted us to re-evaluate our practice. Methods: We retrospectively evaluated abdominal USS reports of consecutive children aged 2–5 years with confirmed diagnosis of CF attending the NI regional CF centre between 2012 and 2020, along with relevant demographic and clinical data and liver function tests. All data was retrieved from the Northern Ireland Electronic Care record (NIECR). Results: Seventy 2–5-year-olds underwent a total of 199 scans between 2012 and 2020. In 2-year-olds, 43% of scans detected an abnormality, increasing to 70% in 5-year-olds, RR 1.61, 95% CI 1.03, 2.65, p = 0.03. 107 (53.4%) scans had one or more abnormality reported. Increased echogenicity of liver and/or pancreas was the most common abnormality identified in 72 of 107 scans (67.2%);hepatomegaly in 46 (42.9%);coarse heterogeneous echotexture in 24 (22.4%);increased periportal echogenicity in 6 (5.6%) and other incidental findings in 10 (9.3%) of scans, including cholelithiasis, splenic cyst, renal scarring, renal stones, and micro gallbladder. Conclusion: Abnormalities are present in over half of abdominal ultrasound scans in 2–5-year-olds. Increased echogenicity, liver enlargement and coarsened echotexture are indicative of hepatic steatosis, which has unclear significance in this age group. Longitudinal studies are needed to determine the role of early USS in detection of liver disease, and whether any particular abnormalities may identify those at risk of development of severe CFLD.

Mesenchymal hamartoma of the liver

A case report of a 7-year-old girl who presented with a 4-year history of painless progressive abdominal swelling that became painful 2 weeks before presentation. Examination revealed a girl in painful distress with distended abdomen and a tender palpable firm right upper quadrant abdominal mass measuring 28 cm × 22 cm with well-defined borders. Abdominal ultrasound scan showed a cystic liver mass filling the abdomen which was multilocular. Liver function tests show mildly elevated liver enzymes and mild prolongation of the prothrombin time. She had repeated aspiration of the cyst content in various peripheral hospitals with transient relief of symptoms. She thereafter had abdominal exploration through a right upper transverse incision and was found to have a cystic mass involving segments V, VI, and VII of the right lobe of the liver measuring 30 cm × 26 cm. Marsupialization of the cyst was done and an incisional biopsy of the cyst wall was taken. Histology of the cyst wall showed a mesenchymal hamartoma of the liver (MHL). The aim of this study is to highlight the clinical features, diagnosis, treatment, and differential diagnosis of MHL

The Prevalence, Predictors, and In-Hospital Mortality of Hepatic Encephalopathy in Patients with Liver Cirrhosis Admitted at St. Dominic Hospital in Akwatia, Ghana.

Background Hepatic encephalopathy (HE) is one of the most debilitating complications of cirrhosis leading to death. Decrease in HE mortality and recurrence has been linked with timely identification and early treatment. There is a need to document the burden, predictors, and treatment outcomes of HE in an adult population with liver cirrhosis in our setting as only reports from resource-endowed countries abound in the literature. This study aimed therefore to determine the prevalence, predictors, and treatment outcomes of patients with liver cirrhosis admitted at St. Dominic Hospital (SDH) in Akwatia, Ghana. Materials and Methods A prospective study was conducted involving one hundred and sixty-seven (167) patients admitted at the medical wards in SDH with liver cirrhosis from January 1st, 2018, to March 24th, 2020. The demographic and clinical features of the patients were collected using a standardized questionnaire. Biochemical, haematological, and abdominal ultrasound scans were done for all patients. Patients were then followed up until discharge or death. Results There were 109 (65.3%) males out of the 167 patients with a mean age of 45.8 and 47.5 years for those with and without HE, respectively. The prevalence of HE was 31.7% (53/167). Out of 53 participants with HE, 75.5% (40/53) died. There was a strong association between HE and death (p < 0.001). The major precipitating factor of HE was infection (64.2%). Severe ascites (OR = 0.009) were clinical feature independently associated with HE, whereas high creatinine (OR = 0.987), blood urea nitrogen (BUN) (OR = 1.199), Child–Pugh score (CPS) (OR = 5.899), and low platelets (OR = 0.992) were the laboratory parameters and scores independently predictive of HE. Conclusion HE was common among patients with liver cirrhosis admitted at SDH with high in-patient mortality. The commonest precipitating factor for HE was infection(s). Severe ascites, low platelet count, high creatinine, BUN, and CPS were independent predictors of HE.

Man With Persistent Vomiting

An 80-year old man with a history of hypertension and Parkinson disease presented to the emergency department with persistent vomiting and vague abdominal pain for 2 days. His vital signs were stable, and he was afebrile. An abdominal radiograph (Figure 1) showed a severely distended stomach. After decompression by nasogastric tube, a large amount of bilious material was drained. The blood tests revealed only mild anemia (hemoglobin, 11.4 g/dL) and hypokalemia (3.09 mmol/L). A point-of-care abdominal ultrasound scan was performed (Figure 2).

Correlation of International Prostate Symptom Score With Intravesical Protrusion of Prostate in Patients With Benign Enlargement of Prostate

Objective: To determine the correlation of international prostate symptom score with Intravesical protrusion of prostate. Methods: This prospective clinical study was performed on 60 elderly patients presented with LUTS suggestive of Benign Enlargement of Prostate. Their evaluation included DRE, IPSS, Uroflowmetry (Qmax), serum PSA measurements and trans abdominal ultrasound scan. Statistical analysis was performed by SPSS version 13 using Chi square test and scatter plots together with Spearman’s correlation coefficients were used to assess the relationship between IPP and IPSS. Results: Mean age of the patients was 66.7+9.85 years, IPSS 23.6+6.53, mean prostatic volume was 60.23+38.16 mL, Qmax 7.98+3.87, PVR was 163.18+141.73 mL. Fifty percent of patients had severe degree (&gt;10mm) of intravesical protrusion of the prostate, 30% had moderate and 20% had mild IPP. There was significant positive correlation between IPSS and IPP (r=0.698, P &lt; 0.001). Conclusion: From this prospective clinical study it is revealed that IPP had strong correlation with IPSS. Bangladesh Journal of Urology, Vol. 22, No. 2, July 2019 p.151-154

Helicobacter pylori is not a contributing factor in gallbladder polyps or gallstones: a case-control matching study of Chinese individuals.

ObjectiveTo investigate the relationship between Helicobacter pylori (H. pylori) infection and gallstones or gallbladder polyps.MethodsThis retrospective analysis included 27,881 individuals who underwent health examinations that included a H. pylori test and an abdominal ultrasound scan. Patients were divided into four groups: gallbladder polyp (P group), gallstone (S group), gallstone and gallbladder polyp (SP group), and no gallbladder disease (N group). Case–control matching was used to select the participants in the control group.ResultsThe mean ages of participants in the P, S, and SP groups were all significantly higher than the mean age of participants in the N group. The proportions of participants with each type of body mass index significantly differed between the N and P groups, and between the N and S groups. In total 45.7% of participants exhibited H. pylori infection. After case-control matching, the proportion of participants with H. pylori infection did not significantly differ according to the presence or absence of gallbladder polyps. Similar results were observed regarding gallstones, as well as gallstones and gallbladder polyps.ConclusionH. pylori infection might not be related to gallbladder polyps or gallstones.

Sonographic Assessment of Some Abdominal Organs in Children with Sickle Cell Disease in Ilorin, Nigeria.

Background:The irregular sickle cells can block blood vessels, causing tissue and organ damage and in most cases presented with pain as a result of organ ischemia. Ultrasonography is an easily available instrument for investigating abdominal organs which are most commonly affected by sickle cell disease (SCD). This study was aimed to assessing liver, gall bladder, spleen, and kidney in children with SCD in Ilorin, Kwara State.Methods:A prospective cross-sectional study was conducted among children with SCD in Ilorin from September 2017 to April 2018. An ethical approval was obtained from the Human Research and Ethics Committee of the Kwara State Ministry of Health. In this study, 106 children with SCD and 106 normal children for control were included. Abdominal ultrasound scan was performed on all the selected subjects and the findings were recorded on the data capture sheet. Descriptive and inferential statistic was used in the data analysis, SPSS version 20 was used and the P = 0.05.Results:In the children with SCD, hepatomegaly was found in 45 (42.5%), 29 (27.4%) had coarse echo pattern of liver and 16 (15.1%) had hyperechoic liver, 5 (4.7%) had gallbladder stone, 2 (1.9%) had gallbladder sludge, and 6 (5.6%) had thickened gallbladder wall. Twenty-five (23.6%) had splenomegaly, and 7 (6.6%) had autosplenectomy. Four (3.8%) showed reduced corticomedullary differentiation in each kidney, five (4.7%) in each kidney had mild calyceal dilatation. One (0.9%) had mild calyceal dilatation with reduced corticomedullary differentiation with accompanying loss of renal sinus fat.Conclusion:Abdominal ultrasound imaging for patients with SCD showed a high incidence of abdominal abnormalities, especially in solid organs such as liver, kidney, and spleen.

Sonographic Assessment of Portal Vein Diameter among Healthy Adults in Saudi Arabia

Background: The purpose of this study was to evaluate the mean values of the portal vein diameter (PVD) and establish its ultrasound reference values in healthy Saudi adults. Methods and Results: A total of 124 healthy adult volunteers (45.2% males and 54.8% females, a mean age of 44.8 +/- 16 years), presenting at the US Department of El-Karama Medical Center in Riyadh, Saudi Arabia, were recruited between November 2018 and May 2019 for this prospective study. Abdominal ultrasound scans were conducted on a Hitachi Aloka F37 machine with a curvilinear transducer with a frequency of 3.5 MHz. The data collection sheets completed for each subject included the following variables: sex, age, height, weight, and BMI (kg/m(2)). The mean PVD was 10.40 +/- 1.22 mm and higher in men (10.7 +/- 1.1 mm) than in women (10.1 +/- 1.2 mm) (P<0.05). Study results showed that PVD did not correlate with age and BMI. Conclusion: Further large-scale studies are needed to establish ultrasound reference values for PVD in healthy Saudi adults.

Fístula Colovesical de Origen Diverticular. Resolución Laparoscópica con Nuevo Recurso Técnico

Introducción: La enfermedad diverticular de colon es una patología de alta prevalencia en nuestro medio. Su presentación inicial como fistulas no complicadas no es frecuente sin antecedentes de episodios de diverticulitis previa.Objetivos: Presentar detalles técnicos de la cirugía de resección y tratamiento laparoscópico de una fistula colovesical de origen diverticular.Materiales y métodos: Se presenta el caso de un paciente varón de 63 años con dolor en hipogastrio sin alteraciones evacuatorias ni cirugías previas. Sin antecedentes de diverticulitis. Se realizó ecografía abdominal y tomografía computada donde se constata aire en vejiga. El urocultivo es positivo para escherichia coli y el colon por enema detecta pequeñotrayecto fistuloso entre colon sigmoides y vejiga. Se decide resección laparoscópica.Resultados: Se realizó una colectomía sigmoidea laparoscópica con identificación de trayecto fistuloso a la vejiga que se aisló y seccionó entre clips de polímero. Se completó con anastomosis primara colorrectal y sondaje vesical prolongado que se retiró a los 15 días. Los parámetros postoperatorios fueron favorables con egreso a las 72 hs.Conclusión: La fístula colovesical es una complicación de la enfermedad diverticular de colon aunque es rara su debut como forma de presentación sin epidosios de diverticulitis previa. Se destaca su identificación como trayecto único y no hemos encontrado mención al respecto en la bibliografía. Su resolución de ligadura entre clips es una opción terapéuticaefectiva y rápida para su resolución definitiva. El abordaje laparoscópico electivo es de elección y su tratamiento mediante el procedimiento propuesto resultó efectivo y seguro.

Ultrasonography is insensitive but specific for detecting aortic wall abnormalities in dogs infected with Spirocerca lupi

BackgroundSpirocercosis is caused by the nematode Spirocerca lupi (S lupi). The disease mainly affects dogs and is typically diagnosed by oesophagoscopy or faecal examination; however, these diagnostic tests may deliver...

Differential Outcomes and Clinical Challenges of NAFLD With Extreme Obesity.

Nonalcoholic fatty liver disease (NAFLD) is closely associated with obesity. The prevalence of extreme obesity, defined as body mass index (BMI) of 50 kg/m2 or higher, is rising more rapidly than overall obesity. We aimed to compare the clinical outcomes and performance of noninvasive fibrosis assessment tools in NAFLD with or without extreme obesity. A retrospective analysis was performed in 304 patients with NAFLD with extreme obesity and compared them to patients with NAFLD with BMI of 40 kg/m2 or less, matched for age, gender, race, and liver fibrosis stage. The mean age of the NAFLD with extreme obesity cohort was 55.9 years, BMI 55 kg/m2, and 49.7% had cirrhosis at initial evaluation. Baseline cirrhosis and coronary artery disease were associated with increased risk of death, and dyslipidemia with decreased risk of mortality. Age, insulin use, hypertension, albumin and platelet count were associated with cirrhosis. Fifteen percent of patients had weight‐loss surgery, but this was not associated with survival or risk of cirrhosis. Of the 850 abdominal ultrasound scans performed in 255 patients, 24.1% were deemed suboptimal for hepatocellular carcinoma screening. The mean NAFLD fibrosis score (NFS) in the extreme obesity cohort, versus a propensity‐matched cohort with BMI of 40 kg/m2 or less, was significantly different for both low fibrosis (F0‐F2) (0.222 vs. −1.682, P < 0.0001) and high fibrosis (F3‐F4) (2.216 vs. 0.557, P < 0.001). Conclusion: NAFLD with extreme obesity is associated with increased risk of liver‐related and overall mortality. Accurate noninvasive assessment of liver fibrosis, low rates of weight loss surgery, and high failure rate of ultrasound were identified as clinical challenges in this population.

Rozpoznanie i leczenie nowotworów typu angiomyolipoma (AML)

Angiomyolipoma (AML) is the most commonly occurring tumour from the PEComa family (PEC tumours; perivascular epithelioid cell tumours), a rare group of neoplasms of mesenchymal origin. AML may occur sporadically or in the course of tuberous sclerosis and lymphangioleiomyomatosis. The sporadic type form is the most common subtype of benign kidney tumours and is four times more frequent in women. Kid ney tumours of the angiomyolipoma type are most commonly detected by chance during an abdominal cavity ultrasound scan, during which they are visible as hyperechogenic tumours, and in most cases they are not a diagnostic problem. AML growth is slow, and complications are rare. The main AML complication can be bleeding to the retroperitoneal space or to the pelvicalyceal system. The typical method of AML care is active surveillance (AS). Asymptomatic tumours with a diameter under 4 cm require control by ultrasound examination every 12 months whereas tumours with a diameter of less than 2 cm are considered not to require control ultrasounds. AML with a diameter of over 4 cm require more frequent ultrasound scans — every six months. The size of the tumour, the presence of symptoms (e.g. pain in a tumour projection, haematuria), planned pregnancy, or suspicion of a malignant tumour are critical in therapeutic decisions. Active treatment options include: embolisation, ablation techniques, nephron-sparing surgery (NSS), and radical nephrectomy. In adult patients with tuberous sclerosis, who require treatment but do not require rapid surgical treatment, everolimus is used. In the case of AML, initially doses of 1 × 10 mg per day should be used (an appropriate dose decrease is required in the case of liver insufficiency), and subsequently treatment may be individualised after determining the lowest effective dose with acceptable adverse effects. A rare epithelioid variety of AML (EAML) shows the potential for a malignant course. The basis of EAML treatment is radical resection, ensuring a high percentage of cures. For non-resectable EAML, chemotherapy, mTOR inhibitors, and VEGFR inhibitors (pazopanib, apatinib) are used, but objective responses have been described only in a very small percentage of patients

Prenatal ultrasound diagnosis of duplication gallbladder: a multicenter study.

Gallbladder duplication is a rare anatomic anomaly characterized by the presence of an accessory gallbladder. To appraise the prevalence and significance of prenatal diagnosis of duplication of gallbladder in a multicenter study. This was a multicenter case series with literature review. Clinical records of all consecutive pregnant women with a prenatal diagnosis of duplication of gallbladder, who were referred to our Centers were included in this study. The diagnosis of duplication of gallbladder was based on the evidence of double gallbladder in the standard abdominal circumference plane using grey scale. Postnatal magnetic resonance cholangiopancreatography (MRCP) 3D and postnatal neonatal abdominal ultrasound scan were offered soon after birth to confirm the diagnosis of double gallbladder. The systematic review was conducted using electronic databases from inception of each database through December 2019. Five studies, including a total of seven cases, were identified as relevant and included in the systematic review. Gestational age at diagnosis ranged from 20 to 32weeks of gestation. Associated findings were reported in only one case, where the fetus presented with a left-sided gallbladder, and bilateral renal agenesis with Potter sequence. None of the included cases reported abnormal karyotype. Our cases series included nine cases (0.03%) of double gallbladder with postnatal confirmation, with an overall incidence of this anomaly of 0.03%.Associated findings were reported in only two cases, one with IUGR and omphalocele, that opted for I-TOP, and one with single umbilical artery. Except for the I-TOP, neonatal outcome was favorable in all cases. Duplication of the gallbladder is a very rare malformation with only seven cases reported in the literature diagnosed prenatally. This anomaly is not associated with abnormal karyotype, and the neonatal outcome is favorable if there are no other associated abnormalities.

Propranolol for infantile hepatic hemangioendothelioma: Clinical evaluation of drug efficacy and safety using a single-center patient cohort

Introduction and objectivesInfantile hepatic hemangioendothelioma (IHHE) is a benign liver tumor, associated with hypothyroidism and vascular malformations along the skin, brain, digestive tract and other organs. Here, we determined a single-center patient cohort by evaluating the effectiveness and safety of propranolol and sirolimus for the treatment of IHHE. Patients and methodsWe performed a monocentric and observational study, based on clinical data obtained from 20 cases of IHHE treated with oral propranolol and sirolimus at the Shanghai Children's Medical Center (SCMC), between December 2017 and April 2019. All cases were confirmed by abdominal enhanced CT examination (18/20, 90%) and sustained decrease of alpha fetoprotein (AFP) (2/20, 10%).Propranolol treatment was standardized as once a day at 1.0mg/kg for patients younger than 2 months, and twice a day at 1.0mg/kg (per dose) for patients older than 2 months. Sirolimus was used to treat refractory IHHE patients after 6 months of propranolol treatment, and initial dosing was at 0.8mg/m2 body surface per dose, administered every 12h. Upon treatment, abdominal ultrasound scanning was regularly performed to evaluate any therapeutic effects. All children were followed up for 6–22 months (mean value of 12.75 months). The clinical manifestations and therapeutic effects, including complications during drug management, were reviewed after periodic follow-up. ResultsThe effective rate of propranolol for the treatment of children with IHHE was 85% (17/20). In most cases, the AFP levels gradually decreased into the normal range. A complete response (CR) was achieved in 3 cases, partial response (PR) for 14 cases, progressive disease (PD) for 2 cases and stable disease (SD) was only detected once. Lesions decreased in two PD patients after administration of oral sirolimus. No serious adverse reactions were observed. ConclusionThis study indicates that both propranolol and sirolimus were effective drugs for the treatment of children with IHHE at SCMC.