Sclerotherapy with bleomycin versus surgical excision for extracervical cystic lymphatic malformations in children.

PURPOSE: Sclerotherapy (ST) with bleomycin is an effective treatment for cervical cystic lymphatic malformations (LM) in children. However, its efficacy for treating extracervical cystic LM in children has not been investigated adequately. This retrospective study compares the efficacy of ST with surgery for treating extracervical cystic LM in children. METHODS: The subjects of this study were children treated for extracervical cystic LM at our hospital between 1970 and 2013. We evaluated retrospectively the hospital records of these children for age, gender, presenting symptoms, location of the lesion, radiological findings, treatments, complications, duration of hospitalization, and outcome. RESULTS: We analyzed the records of 70 children (M:F = 1:9) with a mean age of 52.57 ± 54.87 months (range 1-204 months). The number of children treated by surgery alone, ST alone, and surgery plus ST was 53 (77 %), 13 (18 %), and 4 (5 %), respectively. Surgery comprised total excision (n = 41), near-total excision (n = 9), partial excision (n = 6), and incisional biopsy (n = 1). The complication and recurrence rates were lower, the complete response rate was higher, and the length of hospitalization was shorter in the ST group than in the surgery group (5 vs. 15 % and 8 vs. 17 %, respectively, p < 0.05; 91 vs. 77 %, respectively, p = 0.05; and 2.42 ± 1.67 vs. 13.57 ± 16.24 days, respectively, p = 0.03). CONCLUSION: ST is as safe as surgery for extracervical macrocystic or mixed LMs in children, but is much more effective with higher success rates and lower recurrence rates. Thus, ST provides a cost-effective and appropriate mode of treatment for children with extracervical cystic LM.

The management of primary nonparasitic splenic cysts.

Primary nonparasitic splenic cysts (PNSC) are rare and their management in children has been controversial. We conducted this study to discuss various treatment modalities. The medical records of patients with PNSC (1991-2008) were evaluated retrospectively, including age, sex, history of trauma, presenting symptoms, physical examination and radiological findings, therapeutic approaches, and outcomes. Six patients, between 3 to 12 years of age with a male/female ratio of 2, were included. The presenting symptom was abdominal pain in all but one asymptomatic patient. Physical examination findings were unremarkable in all except for palpable spleen in two patients. Cyst sizes ranged from 3 x 4 cm to 10 x 12 cm. The patients were treated with aspiration-sclerotherapy (n=2), total splenectomy (n=2), partial splenectomy (n=1), and cyst excision (n=1). The pathological diagnoses were epidermoid cyst (n=5) and lymphangioma (n=1). The postoperative course was uneventful except for postsplenectomy fever (n=1), recurrence (n=1) and residual cyst (n=1). PNSC larger than 5 cm in diameter or those that are symptomatic should be treated surgically. Total splenectomy should not be done in children to avoid infectious postsplenectomy problems unless there is a mandatory condition like intraoperative bleeding. Aspiration-sclerosis is not recommended because of recurrence.

Management of pancreatic lithiasis in a 5-year-old girl.

Pancreatic lithiasis causing chronic pancreatitis is a very rare entity in childhood. A five-year-old girl presenting with pancreatic lithiasis was treated successfully with a well-organized diagnostic and therapeutic algorithm. The authors emphasize that early diagnosis followed by appropriate therapeutic pancreatographic procedures instead of primary invasive surgical approach can certainly ameliorate progressive and irreversible pancreatic damage. The clinicopathologic features of this rare entity are discussed, with emphasis on diagnosis and treatment.

Portal vein thrombosis after splenectomy in pediatric hematologic disease: risk factors, clinical features, and outcome.

BACKGROUND/PURPOSE: A retrospective study was performed to evaluate risk factors, clinical features, and treatment modalities of portal vein thrombosis (PVT) after splenectomy in pediatric hematologic disease. METHODS: Sixty-eight patients who underwent splenectomy for various hematologic diseases were evaluated with regard to age, sex, blood count, and splenic mass. Patients who developed PVT were also reviewed for clinical features, treatment modalities, and outcome. RESULTS: Patients with PVT (n = 4, 5.88%) and without PVT (n = 64, 94.2%) had a mean age and female/male ratio of 13.2 years (range, 10-16 years) and 4:0, and 10.2 years (range, 1-16 years) and 29:35, respectively. Postoperative thrombocyte levels and splenic mass with and without PVT was 804 x 10(3)/mm(3) and 752.5 g, and 465.2 x 10(3)/mm(3) and 441g, respectively. Three patients with PVT presented with abdominal pain, fever, and vomiting. The diagnosis of PVT was made by Doppler ultrasonography in all patients including the asymptomatic case. Protein C, protein S, and antithrombin III levels were mostly decreased and/or normal and di-dimer levels were increased and/or normal after the development of PVT. Antiplatelet (acetylsalicylic acid) and antithrombotic therapy (low molecular weight heparin) were treatment agents. None of the patients needed surgery. During a mean follow-up period of 55.5 months, by Doppler ultrasonography, 1 patient was found to be free of thrombosis, whereas 1 had partial thrombosis. Two patients developed cavernomatous transformation leading to portal hypertension. CONCLUSIONS: Portal vein thrombosis is a rare but significant complication of splenectomy done for hematologic diseases. According to our results, female gender and decreased levels of coagulation inhibitors seem to be risk factors in addition to previously mentioned thrombocytosis and greater splenic mass. Doppler ultrasonography may be performed in all patients after splenectomy to screen PVT. In the presence of well-known risk factors, prophylactic antiplatelet and antithrombotic therapy should be considered after splenectomy.

An unusual penetrating injury in an infant: straight-pin migration from the back to the stomach.

Sharp foreign bodies reach into the abdomen frequently by ingestion and may lead to various complications including perforation. An 11-month-old infant is presented for straight-pin migration into the abdomen with penetration from the back and through the left diaphragm, spleen, and stomach. The infant was treated successfully with a well-organized diagnostic and therapeutic algorithm.

Hepatic lobectomies in children: experience of a center in the light of changing management of malignant liver tumors.

Hepatic resection is the main treatment modality for hepatic tumors in childhood. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood. Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively. Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients. Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7 +/- 0.3 cycles of chemotherapy before the operation. Right lobectomy (n = 12), left lobectomy (n = 5), extended left lobectomy (n = 4), and extended right lobectomy (n = 3) and right lobectomy with enucleation of two masses from left lobe (n = 1) were performed. Intraoperative blood transfusion of 30.7+/-6.0 ml/kg body weight was necessary. Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n = 5), hepatocellular carcinoma (n = 4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n = 1), hemangioma (n = 1), cyst adenoma (n = 1), and metastasis of cellular mesoblastic nephroma (n = 1). Patients were observed in the intensive care unit for 3.4 +/- 0.3 days. Postoperative complications were sepsis (n = 1), disseminated intravascular coagulation (n = 2), fever (n = 3), jaundice (n = 3), intraabdominal abscess (n = 3), ileus (n = 2), and subdiaphragmatic abscess with pleural effusion (n = 1). Hepatic lobectomy is a major operation, which is feasible yielding curative results in children. Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control.

A case of patent ductus venosus with pulmonary arterio-venous fistula as a rare and unique clinical entity.

A 13-year-old boy presenting with digital and lip cyanosis, easy fatigability, and weight gain was diagnosed to have an intrapulmonary arterio-venous fistula. During his routine follow-up examinations, there was fullness on right upper quadrant of his abdomen. Abdominal ultrasonography (USG) showed a mass in portal hilus. We planned abdominal computed (CT) to gain tomography detailed information about the mass. Surprisingly abdominal CT and the color-Doppler sonography showed that the structure mimicking a mass was patent ductus venosus (PDV). We report PDV with intrapulmonary arterio-venous fistula as a unique and rare clinical entity. In addition, we underline the misdiagnosis of portal mass instead of patent ductus venosus. If there is a portal mass in USG or CT, color-Doppler sonography or, if needed, other diagnostic methods should be used to exclude PDV.

Surgical treatment of bronchiectasis in children.

BACKGROUND PURPOSE: Surgical treatment of childhood bronchiectasis has not been discussed extensively because of decline in prevalence and experience with this disease. It remains controversial as to which children would benefit from surgery and surgical points that may affect the outcome. Therefore, a retrospective series was prepared to evaluate the results of surgical treatment of bronchiectasis in children. METHODS: The records of 54 children who underwent surgery for bronchiectasis between 1991 and 2002 were analyzed retrospectively for age; sex; clinical features; radiologic examinations; details of surgery including type of resection, operative morbidity, and mortality; and outcome. RESULTS: Fifty-four patients underwent 58 pulmonary resections during the study period. The mean ages at diagnosis of bronchiectasis and at the time of surgery were 7.80 +/- 3.70 years (range, 1 to 15 years) and 9.25 +/- 3.92 years (range, 1.5 to 17 years), respectively, with a male to female ratio of 5:4. The causes of bronchiectasis were lung infection (n = 39), hereditary and inborn diseases (n = 14), and foreign body aspiration (n = 1). Chest X-rays, bronchography (n = 12) or chest computed tomography (n = 43), and ventilation-perfusion scintigraphy (n = 13) were used, and pulmonary function tests (n = 21) and bronchoscopy (n = 54) were performed. The types of resections were lobectomy (63%), pneumonectomy (18.5%), lobectomy with segmentectomy (11.1%), segmentectomy (3.7%), and bilobectomy (3.7%). Four patients required a second operation. Forty-one patients (76%) had complete resection, and 13 patients (24%) had incomplete resection. Intraoperative and postoperative complications were encountered in 4 (7.4%) and 4 patients (7.4%), respectively. The course after surgery was well in 23 (42.5%), improved in 23 (42.5%), and unchanged or worse in 5 patients (9.4%). The mortality rate was 5.6%. CONCLUSIONS: The decision for bronchiectasis surgery should be made in cooperation with the chest diseases unit. Anatomic localization of the disease should be mapped clearly by radiologic and scintigraphic investigations. The morbidity and mortality rates of bronchiectasis surgery are within acceptable ranges. Most of the children benefit from surgery, especially when total excision is accomplished. Pneumonectomy is well tolerated in children without increase in morbidity and mortality. Therefore, pneumonectomy may be preferred instead of leaving residual disease when bronchiectasis is unilateral.

Feminizing Sertoli cell tumor associated with Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the association of mucocutaneous pigmentation and multiple gastrointestinal hamartomatous polyps and with an increased risk of developing gonadal sex tumors besides other malignancies. We describe a 7 1/2 year-old boy with PJS and bilateral gynecomastia. He has had buccal pigmentation since 1.5 years and had been operated for rectal polyp excision at 3.5 years. On physical examination, his height was at the 90th percentile, and his height age and bone age were 9 and 10 1/2 years, respectively. Increased melanotic buccal pigmentation of the lips and bilateral gynecomastia were noticed. Both of the testes were firm, non-tender and smooth on the surface, and each measured 8 ml. Hormonal measurements were all in the prepubertal range. Testis ultrasonography showed bilateral hyperechogenic areas within the glands. When he was operated for invagination and an ileum segment full of polyps was resected, bilateral testicular biopsies were also performed. Histopathological evaluation of the testes revealed bilateral multicentric benign Sertoli cell tumors. The aromatase inhibitor testolactone was started to slow skeletal maturation. On the basis of this and previous reports, PJS associated with sex-cord tumors is increasingly recognized in males as well as in females.

A huge gastric stromal tumor in a 13-year-old girl.

A 13-year-old girl presenting with severe anemia was diagnosed to have a large gastric tumor protruding toward the antrum with two central ulcerations. Partial gastrectomy including antrectomy and gastroduodenostomy were performed. Histologic and immunohistochemical studies revealed one of the most uncommon gastric tumors in children; a gastrointestinal stromal tumor. Close follow-up of the patient with endoscopy, abdominal ultrasonography and/or computed tomography in three to six month intervals revealed no recurrences or metastasis of the tumor following its complete excision.