Intralesional bleomycin injection treatment of intra-abdominal lymphangiomas presenting with acute abdomen in children.

BACKGROUND: We evaluated the results of urgent intralesional bleomycin injection (IBI) treatment of intra-abdominal lymphan-giomas (IAL) presenting with acute abdomen in children. METHODS: The records of patients who underwent urgent IBI due to acutely presenting IAL between January 2013 and January 2020 were reviewed retrospectively in terms of age, presenting symptoms, cyst type, number of injections, pre- and post-treatment cyst volume, clinical response, complications, and follow-up. RESULTS: Six patients with a mean age of 4.3 years (2-13 years) were treated. Presenting symptoms were acute abdominal pain (n=4), abdominal distention (n=1), hypoproteinemia and chylous ascites (n=1). Lesions were of macrocystic type in four and macro and micro cystic in two patients. The median number of injections performed was 2 (1-11). Mean cyst volume reduced dramatically from 567 cm3 (range 117-1656) to 3.4 cm3 (range 0-13.8) after treatment (p=0.028). Treatment response was excellent in four patients with complete resolution of the cysts, while good in the remaining two. No early or late complications or recurrence was observed in a mean follow-up period of 40 months (16-56 months). CONCLUSION: IBI is a safe, fast, and easily applicable method with satisfactory results in the treatment of acutely presenting IAL. It may be recommended in primary as well as recurrent lesions.

[Duodenum perforations in children: case series]. / Çocuklarda duodenum perforasyonlari: Olgu serisi.

To analyse patients those admitted to our clinic due to perforation in duodenum. Fourteen patients those have been admitted to our center with perforation in duodenum between 1990 - 2014 analysed retrospectively. Ten patients (8M, 6F) those have been admitted to our clinic between 1990 - 2014 have a mean age of 6.2 years (25 days - 16 years). Two of cases admitted directly to our clinic and the rest referred from another hospitals. Mean time for appliance to our clinic was 3.2 days (1day - 1 week). Ulcer in duodenum was the cause of perforation in 10 cases while in 3 the cause was trauma and in 1 case was surgical complication of infantly persistant hyperinsulinism (IPHH). The case with hyperbilluribinemia after near total pancreatectomy due to IPHH had been explored and perforation in deuedenum diagnosed. Resection in first two parts of duodenum and pylor, choledochojejunostomy, gastrojejunostomy and jejunojejunostomy was performed. Primary repair was performed in the remaining patients. In one case with primary repair gastrostomy was performed, while in 9 cases omentoplasty were performed. In the follow-up 12 cases has no problems and doing well. Two patients died. Perforation in duedenum is a rare entity that pediatric surgeons should encounter and keep in mind. Primary repair (duodenoraphy ± omentoplasty) is safe and reliable surgical treatment modality.

Effectiveness of extracorporeal shock wave lithotripsy on intrahepatic biliary calculi developing after choledochal cyst surgery: A case report.

The development of intra- and extrahepatic bile duct stones has been reported as one of the most serious complications after choledochal cyst excision with biliary-enteric reconstruction through Roux-en-Y hepaticojejunostomy (HJ). Here, we report our experience with extracorporeal shock wave lithotripsy (ESWL) in a case of giant intrahepatic stones developing after choledochal cyst surgery. ESWL is an excellent therapeutic modality for large intrahepatic biliary calculi, and after dilating the HJ anastomosis percutaneously, it can be offered as first-line therapy to these patients.

The impact of share wave elastography in differentiation of hepatic hemangioma from malignant liver tumors in pediatric population.

OBJECTIVE: In children it is crucial to differentiate malignant liver tumors from the most common benign tumor, hepatic hemangiomas since the treatment strategies are quite different. We aimed to evaluate the efficiency of shear wave elastography (SWE) technique in differentiation of malignant hepatic tumors and hepatic hemangiomas. METHODS: Twenty patients with hepatic tumor were included in our study. Two radiologists performed SWE for 13 patients with malignant hepatic tumors including hepatoblastoma (n=7), hepatocellular carcinoma (n=3), metastasis (n=2), embryonal sarcoma (n=1) and 7 patients with hepatic hemangioma. All of our patients were between the age of 1 and 192 months (mean age: 56.88 months). Receiver operating characteristic analysis was achieved to evaluate the diagnostic accuracy of SWE and to determine the optimal cut-off value in differentiation hepatic hemangioma from malignant hepatic tumors. RESULTS: The mean SWE values (in kPa) for the first observer were 46.94 (13.8-145) and 22.38 (6.6-49.6) and those for the second observer were 57.91 (11-237) and 23.87 (6.4-57.5), respectively for malignant hepatic tumors and hepatic hemangiomas. The SWE values of malignant hepatic tumors were significantly higher than those of hepatic hemangioma (p=0.02). The inter-observer agreement was almost perfect (0.81). The area under the receiver operating characteristic curve of SWE for differentiating the hepatic hemangioma from malignant hepatic tumors was 0.77 with a sensitivity of 72.7% and a specificity of 66.7% at a cutoff value of 23.62 with 95% confidence interval. CONCLUSION: Shear wave elastography can be helpful in differentiation of malignant hepatic tumors and hepatic hemangioma.

Successful management of hepatic mucormycosis in an acute lymphoblastic leukaemia patient: a case report and review of the literature.

We present a case of hepatic mucormycosis in a 9-year-old boy with acute lymphoblastic leukaemia. Despite long-term use of combined liposomal amphotericin B and posaconazole therapy, the lesion persisted and could only be treated by surgical excision. After surgery, antifungal treatment was continued with posaconazole. On follow-up, the patient had two episodes of ascending cholangitis which were responsive to intravenous antibiotics. He is doing well at the moment in remission for 2.5 years. Mucormycosis was long regarded as a fatal infection with poor prognosis. With early medical and surgical management, survival rates increase. Isolated hepatic mucormycosis is rare and only seven cases were reported in the literature up to now. We wanted to emphasise the role of early surgery in patients with hepatic mucormycosis in view of the literature.

Spontaneous splenic rupture in a patient with congenital afibrinogenemia.

Afibrinogenemia is a rare bleeding disorder which is observed with an incidence of 1:1 000 000. It is an autosomal recessive disease and occurs as a result of mutation in one of the three genes which code the three polypeptide chains of fibrinogen. Basic clinical findings include spontaneous bleeding, bleeding after minor trauma or due to surgery. Splenic rupture in afibrinogenemia has been reported only in 6 cases so far. In this article, we present a 15-year old congenital afibrinogenemia patient with spontaneous splenic rupture.

Comparison of color doppler US and CT portography in evaluating portal hypertension in children.

OBJECTIVES: To compare grayscale and color Doppler ultrasound (CDUS) findings to that of multidetector computed tomography (MDCT) portography in the evaluation of portal hypertension in children. METHODS: Thirty children (mean age, 11.4 years) with definitive clinical and laboratory diagnoses of portal hypertension were included in the study. Liver parenchymal changes, portal vein thrombosis, cavernous transformation, splenomegaly, recanalization of the paraumbilical vein, ascites collateral vessels, and hemodynamic parameters were evaluated prospectively. RESULTS: Liver parenchymal heterogeneity (n = 21) was detected more often by CDUS than MDCT (P = 0.002). CDUS and MDCT established similar results for increased right/left lobe ratio, lobulation of the liver contour, ascites, and splenomegaly. Portal vein thrombosis, cavernous transformation, and recanalization of the paraumbilical vein were comparable between CDUS and MDCT (κ = 1, P < 0.01). Collaterals were detected more by MDCT portography than by CDUS. Esophageal collaterals and azygous vein dilatation were seen only by MDCT portography (P < 0.01, P = 0.024; respectively). CONCLUSIONS: CDUS was superior to MDCT in identifying parenchymal changes and hemodynamic parameters. MDCT was superior in detecting abnormal collateral circulation. The combination of these two modalities provides more comprehensive information than either alone in the diagnosis and follow-up of portal hypertension.

Multidisciplinary approach to congenital multiple arterio-porto-caval malformation: case report.

A case of intrahepatic arteriovenous malformation connected with umbilical, hepatic, portal, and arterial systems in a fetus diagnosed at 36 weeks of gestation is presented. Prenatal color Doppler ultrasonography in the fetal liver demonstrated complicated vascular connections fed by arterial and portal branches. Postnatal color Doppler, power Doppler, 3D power Doppler, abdominal multidetector computed tomography (MDCT), and angiography CT verified the prenatal diagnoses. Extended right hepatectomy was performed successfully on postnatal day 19.

Radiological evaluation with Doppler sonography and multidetector CT angiography in congenital hepatic arteriovenous malformation in a newborn.

Although hepatic arteriovenous malformations are rarely reported, they frequently have life-threatening complications such as cardiac failure and are associated with a high mortality rate. Consequently, accurate prenatal and early postnatal diagnosis is important and therapeutic procedures depend on the imaging features. We report the early postnatal sonographic, Doppler sonographic, multidetector CT and CT angiography findings of a congenital hepatic arteriovenous malformation in a newborn.

Primary hydatid cyst of the head and neck diagnosed with ultrasound and computed tomography: a report of two cases.

Hydatid cysts of the head and neck are rare, even in countries where echinococcal infestation is endemic. This report presents two patients with hydatid cyst, diagnosed using ultrasonography and computed tomography, in whom the cysts were located between the right cervical paravertebral muscles and below the superficial muscles of the right temporal region.

Hepatobiliary mucinous cystadenoma in a child.

The authors present a 4-year-old boy who had a large mucin-hypersecreting hepatobiliary cystadenoma. The tumor caused a hepato-colo-cutaneous fistula, which produced a large amount of external fluid loss. Total excision and the repair of the fistula could be possible after shrinkage of the tumor with the use of selective embolization of the feeding artery by interventional radiology.

Intestinal rotation anomalies in childhood: review of 22 years' experience.

PURPOSE: We review our experience of treating intestinal rotation anomalies in infants and children in the 22-year period between 1978 and 2000. METHODS: The type of operation performed, postoperative complications, and mortality were compared in three age groups. Group 1 consisted of neonates <1 month old, Group 2 consisted of infants aged <1 year old, and Group 3 consisted of children aged >1 year old. RESULTS: There were 101 infants and children, with a female : male ratio of 2 : 1. Of the 101 patients, 72 (71%) were neonates, with a mean age of 11.8 days (range 1-28 days); 20 (19.8%) were under the age of 1 year, with a mean age of 6.7 months (range 1-12 months); and 9 (8.9%) were >1 year of age, with a mean age of 6 years (range 1-9 years). Eighty-five (84%) patients underwent emergency procedures. Ladd's operation was performed in all patients, with various additional procedures. The most frequent postoperative complications were adhesive intestinal obstruction, stoma necrosis, evisceration, and short bowel syndrome. The mortality rate was 36% in Group 1, 20% in Group 2, and 0% in Group 3. CONCLUSIONS: In this series surgery was usually performed as an emergency procedure, with higher morbidity and mortality in newborns than in older infants and children.

Pyloric atresia: 15-year review from a single institution.

BACKGROUND: Pyloric atresia (PA) is a rare pathology. Calder presented the first pyloric atresia case in 1749 and Touroff, Sussman, Meltz, and their colleagues presented the first successful operation in 1940. PA has 3 types of anatomic variations: (1) type A, pyloric membrane or web; (2) type B, the pyloric channel is a solid cord; and (3) type C, in which there is a gap between the stomach and duodenum. Associated anomalies also have been described. Epidermolysis bullosa (EB) and intestinal anomalies occur most often with this condition. METHODS: Charts of 16 cases of congenital PA, aged 1 to 30 days and admitted to our department between 1986 and 2001, were studied retrospectively in regard to sex, prenatal diagnosis, presence of polyhydramnios, time of admission, pathology, type of operation, associated anomalies, and mortality rate. RESULTS: In the study group, the male to female ratio was 5:3, the mean birth weight was 2,312 g and the mean age of admission was 6.5 days. The distribution of the anatomic variations was type A in 9 (56.3%) and type B in 7 (43.7%) of cases. Associated anomalies were present in 7 cases (43.8%). Familial occurrence was a prominent feature of our series. Seven of 16 cases occurred in 3 families. We performed 9 web excisions together with Heineke Mikulicz (H-M) pyloroplasty, 5 atresia excisions and gastro-duodenostomy, and 2 H-M pyloroplasties alone. Stamm gastrostomy was supplemented in 3 cases. The overall mortality rate (n = 9) was 56.3%. CONCLUSIONS: Pyloric atresia can be managed successfully if it is diagnosed early. In this group of patients, congenital anomalies or septicemia are the main causes of mortality.

Management of portal hypertension in children: a retrospective study with long-term follow-up.

OBJECTIVES: Data regarding the management of the portal hypertensive haemorrhage in the paediatric patients have yielded conflicting results. The purpose of this study was to evaluate the efficacy of beta-blocker (propranolol) alone, sclerotherapy alone and beta-blocker + sclerotherapy combination in the management of portal hypertension in the paediatric population. METHODS: Medical information was retrieved from the records of 62 children with portal hypertension who were under treatment during at least two years of follow-up period. Data collected included diagnosis, type of portal hypertension, age at initiation of therapy, bleeding episodes before and during therapy. RESULTS: Sixteen of 62 patients were diagnosed as extrahepatic portal hypertension, 46 as intrahepatic portal hypertension. The mean age of study population was 7.6 +/- 4.2 years, 45 percent being females. The mean duration of follow-up under therapy was 5.2 +/- 2.5 years. Among the patients with intrahepatic portal hypertension, 29 received propranolol + sclerotherapy, 12 received only propranolol and 5 received only sclerotherapy. There was no significant decrease in bleeding episodes during propranolol or sclerotherapy. However patients under propranolol + sclerotherapy, showed significant decrease in bleeding episodes during therapy (23/29 before therapy, 15/29 during therapy, p < 0.05). Rebleeding index in patients with IHPH was significantly long in the group treated by propranolol + sclerotherapy (p = 0.0001) compared with before therapy. Because the numbers of patients in the groups are small, Kaplan Meier estimation suggest that propranol treatment is more effective. But there isn't significant difference when the results were compared with those of before therapy, except in the combined treatment group. CONCLUSION: The monotherapy is not sufficient for longterm follow-up of portal hypertensive patients. The combination therapy with propranolol + sclerotherapy appears more encouraging in the prevention of portal hypertensive haemorrhage, but this needs to be assessed in randomized trials.

Equality of the left and right renal venous flow predicts the severity of variceal bleeding in portal hypertensive children.

Portasystemic collaterals develop as a result of portal hypertension. The collaterals in the cardioesophageal region is the leading cause of bleeding from esophageal varices. Some of the portal hypertensive patients present with bleeding episodes but the others do not, and some of the bleeders do not respond to endoscopic sclerotherapy procedure, although the underlying pathology is the same. The capacity of the natural collateral vessels might be a determining factor about the hemorrhagic events. Since the first step of portasystemic collateralization takes place in the naturally existent vascular channels, the present study, with its anatomic and clinical parts, was focused on these venous structures.

Arterial carbon dioxide markedly increases during diagnostic laparoscopy in portal hypertensive children.

UNLABELLED: Several factors are responsible for hypercarbia during laparoscopic procedures. This study was undertaken because we observed a sudden increase in PaCO(2) in children with portal hypertension (PHT), which was unusual in healthy children undergoing laparoscopic procedures. Fifty-seven children underwent laparoscopic procedures under general anesthesia and were mechanically ventilated. Arterial blood samples were obtained 5 min after intubation (T(0)), 15 min and 30 min after CO(2) pneumoperitoneum (T(15) and T(30)), 5 min after desufflation (T(end)), and 10 min after extubation (T(ext)) for blood gas analysis. The changes in PaCO(2), pH, and ETCO(2) were statistically significant during the study periods in both groups (P < 0.05). The percentage of PaCO(2) increase between T(0) and T(15) was 11.5% and 20.1%, respectively, in the control group and the PHT group (P < 0.05). This increase reached 36.8% at T(30) in the PHT group, whereas the control group had a 17.2% increase (P < 0.05). ETCO(2) presented similar changes. The variability in base excess, bicarbonate, PaO(2), arterial oxygen saturation, and SpO(2) was not significant in either group (P > 0.05). The PaCO(2) increased remarkably in children with PHT undergoing laparoscopy, with no difference in intrahepatic or extrahepatic origin. Limiting the duration of CO(2) pneumoperitoneum and intraabdominal pressure and adjusting ventilatory variables to accommodate hypercarbia are of the utmost importance for such cases. IMPLICATIONS: We compared children with portal hypertension with systemically healthy children during laparoscopy. The increase in arterial and end-tidal CO(2) was remarkable in children with portal hypertension, regardless of bicarbonate changes. Managing ventilation to accommodate hypercarbia is of the utmost importance for such cases.

Esophageal variceal bleeding secondary to portal hypertension: endoscopic sclerotherapy as the first-step treatment.

BACKGROUND: Variceal bleeding from the esophagus is an important cause of mortality and morbidity in children with portal hypertension (PHT). PATIENTS AND METHODS: A series of 69 PHT cases (41 intrahepatic, 28 extrahepatic) have been evaluated in our department since 1990. According to the Child-Pugh classification, 49 cases were in class A, 16 cases were in class B, and 4 cases were in class C at admission. In our protocol, endoscopic sclerotherapy is performed in all patients, and the diagnosis is achieved directly by diagnostic laparoscopy and fine-needle liver biopsy. The procedure is applied under general anesthesia, and 1% aethoxysclerol (polidocanol) is injected paravariceally and intravariceally with the use of a flexible endoscope. RESULTS: The Sugiura procedure was performed in nine patients who presented with recurrent bleeding episodes despite the strict sclerotherapy protocol. Liver transplantation was performed in two patients who were in Child class C. The total mortality rate in this series was 7% (5/69). CONCLUSION: Endoscopic sclerotherapy, as presented herein, decreases the need for additional surgical interventions in children with PHT.