Continuous double U-stitch gastrostomy in children.

BACKGROUND: In children, a gastrostomy button was placed as the initial feeding tube, using laparoscopy and a modified surgical technique. The aim of this study was to test the hypothesis that a new surgical procedure developed at our institution would result in fewer postoperative complications. PATIENTS AND METHODS: Sixty-two consecutive children with nutritional problems underwent a video-assisted gastrostomy operation (VAG). The technique requires the use of a 2 or 3 mm laparoscope optic and a 5 mm trocar placed at the exit site chosen for the gastrostomy. A continuous double U-stitch absorbable suture created a purse string suture around the gastrostoma on the stomach and fixated the stomach to the abdominal wall. For comparison, we used a control group of 68 children with nutritional problems operated on with our previously published VAG technique. After surgery, the children were followed up at one and six months and all complications were documented according to a protocol. RESULTS: The two groups of children were comparable with regard to their demographic data. There were no serious intra-operative or postoperative intra-abdominal complications requiring reoperation. There was a significantly lower incidence of the minor complication of granuloma around the gastrostoma in the study group compared with the control group. CONCLUSION: This variation of the surgical technique is simple and effective. It allows primary placement of a gastrostomy button that is functionally and cosmetically comparable to a gastrostomy tube surgically placed by other methods. In this study, the patients had fewer postoperative problems than the control group.

Interventional treatment of children with portal hypertension secondary to portal vein occlusion.

PURPOSE: To present methods and results of interventional treatment of children with portal hypertension (PH) secondary to portal vein occlusion (PVO). MATERIAL AND METHODS: Five children, four boys and one girl, 8 - 14 years old, with symptomatic PH secondary to PVO were treated. All children had one or more episodes of bleeding from oesophageal varices, enlarged spleen and thrombocytopenia. Partial embolisation of the spleen was performed in four children. Attempts to recanalize the occluded part of the portal vein were done in all children using transjugular (n = 4), transhepatic (n = 4) and transsplenic (n = 3) approaches. RESULTS: All procedures were carried out without serious complications and were followed by normalisation of the platelet count, decrease in splenic size and disappearance of bleeding. Recanalisation of the occluded portal vein with a stent was possible in one child and partial stent recanalisation was possible in another child. Transjugular intrahepatic portosystemic shunt (TIPS) with partly extrahepatic, intraperitoneal route was created in one patient. All children were scheduled for follow-up. During the observation time of 22 months (5 - 46 months), additional balloon dilation and placement of new stents were necessary in two children. CONCLUSION: Interventional procedures are valuable in the treatment of children with symptoms secondary to PVO. Treatment should be customized and scheduled follow-ups of the patients are necessary.

Embolization of hepatic hemangiomas in infants.

A small number of cavernous liver hemangiomas in infants cause serious symptoms, requiring active treatment. We report two newborns with giant liver hemangiomas, treated by intra-arterial embolization. The babies presented at 2 - 8 days after birth with tachypnoea and cardiac dilation. A giant liver hemangioma located in the right liver lobe in one infant and in the left liver lobe in the other was found at ultrasonography and computed tomography. Dilated liver veins indicated abnormal shunting of the blood through the hemangiomas. Because of progress of symptoms superselective embolization of the arteries feeding the hemangiomas and arising from the celiac trunk was performed with a mixture of Lipoidol and Histoacryl. A decrease of tachypnoea and of heart volume was noted after embolization. In one infant surgery was necessary due to gastrointestinal bleeding. The intra-arterial embolization is a valuable method for the treatment of newborns with symptomatic cavernous liver hemangiomas.

Stenting of the biliary tract in children.

We report on the technique and results of percutaneous transhepatic biliary drainage (PTBD) in children with obstructive jaundice. Three patients aged 8 - 15 years were treated, two of them for a benign and one for a malignant stricture. Endoscopic treatment was not possible and all the PTBD procedures were done under general anaesthesia. One of the children was treated with external-internal drainage, and the two others by insertion of a plastic endoprosthesis. There were no immediate complications. The PTBD had a good palliative effect in two cases, and in one case surgical treatment was necessary. We conclude that PTBD is a safe modality and that it can be used in children for the relief of obstructive jaundice.

Cytogenetics of hepatoblastoma: further characterization of 1q rearrangements by fluorescence in situ hybridization: an international collaborative study.

BACKGROUND: Hepatoblastoma (HBT) is the most common hepatic neoplasm in children. This notwithstanding, little is known about pathogenetic factors, such as genetic abnormalities, of importance for the development and progression of this tumor type. To date, only 33 cytogenetically abnormal HBT have been published, and trisomies for chromosomes 2 and 20 have been shown to be the most frequent aberrations. Recently, unbalanced translocations involving proximal 1q have been described in several HBT, suggesting that a pathogenetically important gene maps to 1q. PROCEDURE: Six primary and one recurrent HBT were cytogenetically analyzed after short-term tissue culture. In addition, fluorescence in situ hybridization (FISH) studies, using locus-specific probes, were performed on three of these pediatric HBT as well as on one previously reported adult HBT. RESULTS: Total or partial trisomy 8, gain of chromosome 20, and structural rearrangements of chromosome 1 were detected in three HBT, and overrepresentation of chromosome 2 material was found in two HBT. The adjacent chromosome bands 1q12 and 1q21 were involved in three translocations, t(1;2), t(1;4), and t(1;11), which were all unbalanced and resulted in gain of 1q material. The previously reported adult HBT displayed 1q deletions with breakpoints at 1q12-21. FISH analyses of the 1q rearrangements revealed that all breakpoints were within the heterochromatic region. CONCLUSIONS: These findings provide further support for the importance of trisomies 2, 8, and 20 and rearrangements of 1q in the development of HBT. Furthermore, the consistent localization of breakpoints within the heterochromatic segment of chromosome 1 suggests that the important pathogenetic consequence of 1q abnormalities is the resulting genomic imbalance rather than a specific gene rearrangement.

Granulocytic sarcomas in body cavities in childhood acute myeloid leukemias with 11q23/MLL rearrangements.

Three childhood acute monoblastic leukemias (AML M5) with granulocytic sarcomas (GSs) are described. All displayed 11q23/MLL abnormalities, t(9;11)(p22;q23) in two cases and t(11;17)(q23;q21) in one case, constituting around 20% of all 11q23-positive AML cytogenetically investigated in our department. Two of the patients had GS in multiple locations, and all three had abdominal GS. In two of them, t(9;11)-positive GS was diagnosed prior to the diagnosis of AML. Fourteen (1.9%) of 752 published AML cases with 11q23 aberrations have had GS, either as a presenting feature or during disease progression. The incidence of GS has varied significantly (P < 0.05) between children (3.8%) and adults (0.8%). The most common AML subtype has been AML M5 ( approximately 75%) and the most frequent GS sites have been the skin, abdomen, orbit, and thorax. Considering the possibility of underreporting of GS in published cases and the relatively high frequency in our own series, we believe that 11q23/MLL rearrangements may predispose to GS development. Although extramedullary infiltrates in the skin are known to be frequent in cases of AML M5, which is often associated with 11q23 aberrations, the present findings indicate that GS in the abdomen, orbit, and thorax may also be common, especially in pediatric AML. Thus, the possibility of 11q23/MLL-positive GS should be suspected when tumors of uncertain derivation occur in these sites. Finally, the identification of 11q23/MLL abnormalities in GSs in two patients without overt AML underscores the importance of using cytogenetic and molecular genetic investigations as a diagnostic approach in the evaluation of tumorous lesions of unknown origin. Genes Chromosomes Cancer 27:136-142, 2000.

Routine laparoscopy for nonpalpable testes?

There are still no accepted criteria for the selection of patients with nonpalpable testes for laparoscopy versus a primary surgical exploration. We here report our experience using routine laparoscopy in such patients. The aim was to determine whether laparoscopy should be the first operative intervention or follow an inguinal exploration. Included in the study were 61 boys with 69 nonpalpable testes. Thirty-three testes were found in the abdomen, and 36 testes were extra-abdominal or nonexistent. If an exploration of the inguinal region had been the initial surgical intervention, six testes would have been found, making laparoscopy unnecessary. On the other hand, in the search for 63 missing testes, laparoscopy saved the patients from laparotomy or an extensive inguinal exploration. We conclude that an accurate knowledge of testis, vas, and vessel location gained by laparoscopy facilitates the selection of an appropriate surgical strategy, saving at least 51% of patients from laparotomy or an extensive inguinal exploration.

Cytogenetic aberrations in Ewing sarcoma: are secondary changes associated with clinical outcome?

BACKGROUND: Ewing sarcoma is associated with a nonrandom pattern of primary and secondary chromosomal aberrations. Whereas the finding of rearrangements of chromosome 22, usually in the form of a balanced translocation t(11;22)(q24;q12), is important diagnostically, nothing is known about the potential prognostic impact of the secondary chromosomal aberrations. PROCEDURE: During a 1 3-year-period, short-term cultured tumor samples from 21 children and young adults with Ewing sarcoma were cytogenetically analyzed successfully. RESULTS: Clonal chromosome aberrations were detected in 18 patients, 17 of whom had the characteristic t(11;22)(q24;q12) or variants thereof. The most frequent secondary change was +8, followed by +12, +2, +5, +9, +15, and gain of material from the long and short arms of chromosome 1. The only recurrent secondary change that was restricted to tumors from the ten patients that were dead at latest follow-up was gain of 1q material. Furthermore, all three patients with tumors with chromosome numbers over 50 had died, and the only patient with a tumor karyotype lacking chromosome 22 rearrangement was alive without evidence of disease. CONCLUSIONS: These data and previously published results indicate that the karyotypic pattern not only may be of diagnostic significance but also may be important prognostically.

Bilateral Wilms' tumor.

During a 16-year period, 49 children were treated for Wilms' tumor (WT); 7 were bilateral, 5 synchronous and 2 metachronous. The age at primary diagnosis was 6 months to 5 years (mean 2.4 years). All but 1 child received preoperative chemotherapy with tumor reduction. Unilateral nephrectomy was performed in 2 metachronous cases. In 3 synchronous WTs, the tumor was enucleated in 5 kidneys and a heminephrectomy was performed in 1 kidney with a double pelvis. Two children were not operated upon. At relapse in the contralateral kidney the tumor was enucleated. Three patients died of WT, 1 is alive with disease, and 3 are without evidence of disease. Renal salvage procedures were technically feasible without complications and are advocated to preserve renal function.

Complex karyotype in a childhood adrenocortical carcinoma.

Cytogenetic analysis of short-term cultured cells from an 11-cm adrenocortical carcinoma in a 3.5-year-old girl revealed the karyotype 46,XX,inv(9)(p11q12)c/[2]/56-57,XX,+2,+4,+5,+7,+8,inv(9)c,+10,+add (13)(p11), +14,+15,+19,+20,+20,+mar[cp19]. To our knowledge, this is the first description of an abnormal karyotype in a pediatric adrenocortical tumor. Inasmuch as the distinction between benign and malignant adrenocortical tumors is often difficult to make from clinical and histopathologic data alone, the present findings suggest that cytogenetic analysis may be a valuable adjunct in the differential diagnosis.

Wilms' tumour in infancy.

During a 15-y period, 48 children were treated for Wilms' tumour (WT). Seven of them were < 1 y of age at diagnosis. One child presented with non-traumatic haematuria, but in all the other children WT was revealed as a palpable abdominal mass at routine examination or investigation due to another disease. The four children under 6 months of age at diagnosis were primarily operated upon; the others received preoperative chemotherapy. Two children had chromosomal aberrations in the WT tumour specimen. The follow-up revealed that postoperatively six children are healthy with no evidence of relapse from WT, but one child had a contralateral relapse successfully enucleated. The clinical behaviour and management of WT in infants differ compared with that in older children. The diagnosis may be uncertain and it can be difficult to distinguish between malignant and non-malignant lesions. It is essential to realize the possibility of WT, even in children < 1 y of age.

Trisomy 3 as the sole karyotypic change in a pediatric immature teratoma.

Cytogenetic analysis of short-term cultured cells from an immature ovarian teratoma of a 9-year-old girl disclosed an extra copy of chromosome 3 as the sole clonal abnormality. The fact that trisomy 3 was previously reported as the only karyotypic change in two ovarian germ-cell tumors--one teratoma NOS and one immature teratoma--suggests that gain of chromosome 3 constitutes an early and pathogenetically important change in a subset of female germ-cell tumors.

Chromosomal aberrations in a consecutive series of childhood rhabdomyosarcoma.

BACKGROUND AND PROCEDURE: During a 13-year period, 22 children were treated for rhabdomyosarcoma (RMS). In 18 of these patients chromosome analysis was attempted on material from tumor biopsies, fine needle aspiration biopsies and/or bone marrow samples. RESULTS: Clonal chromosome aberrations were detected in 14 cases; 7 of 9 embryonal RMS, 6 of 8 alveolar RMS and in the single case of pleomorphic RMS cytogenetic failures were more frequent in fine needle aspiration biopsies than in tumor biopsies. The characteristic t(2;13) translocation was seen in 2 alveolar RMS but not in any of the other subtypes. In 3 of the embryonal RMS hyperdiploid or hypertetraploid karyotypes with few or no structural rearrangements were seen. In all 3 cases the clinical course was relatively benign, suggesting that certain karyotypic patterns in RMS may be of prognostic significance. CONCLUSIONS: Our results add to the evidence that cytogenetic analysis should be an integral part of the diagnostic examinations of children with RMS.

Chromosomal aberrations in Wilms' tumour.

In 26 consecutive patients operated for Wilms' tumour samples from the tumour were genetically analyzed. Clonal acquired chromosome aberrations were found in 13 patients and a constitutional trisomy 18 as the sole change in 1. The chromosome number was altered in 13 patients. Numerical changes occurred in 16 patients and breakpoint of chromosome 1 in 6 patients. There was no structural alteration of chromosome 11. The observed cytogenetic heterogeneity illustrates the complexity of genetic changes involved in the genesis and progression of Wilms' tumour. To further elucidate the phenotypic impact of chromosomal aberrations the correlation to histology and the clinical course will be important.

Membranous duodenal stenosis.

The experience of our 16 patients treated for membranous duodenal stenosis is reported. Their treatment and course was analysed in a retrospective study. Eight patients were operated on within the first 16 days of life and in the remaining group surgery was performed at 1 month to 4 y of age. The presenting symptom leading to diagnosis was, in all but one case, non-bile-stained vomiting. Associated malformations were found in all but four patients, mostly morbus Down. The operative procedure performed was a partial excision of the duodenal membrane and a duodenoplasty in 10 patients, a duodenojejunostomy in 5 patients, and a duodenoplasty only in 1 patient. The postoperative course was without lethal complications. One late stricture in an anastomosis occurred. We conclude that in its presentation, duodenal stenosis differs from duodenal atresia, and can often be misinterpreted, resulting in a late diagnosis, and should be reported as a separate entity.

Efficacy of bleomycin treatment for symptomatic hemangiomas in children.

Five children aged 5-19 years had pain in massive, inoperable hemangiomas. They were treated with intralesional injections of 2 mg bleomycin as a 0.4 mg/ml solution in the painful area. The injections were repeated after 4 -6 weeks for a total of 6 -10 times. All children were relieved of pain, and the swelling was reduced in all cases. There were no complications or side effects. Bleomycin therapy of painful, massive hemangiomas can be recommended in older children.

Salvage treatment of relapsing Wilms' tumour by autologous bone marrow transplantation.

Despite the remarkable success in treatment of Wilms' tumour there is still a small group who will suffer from relapse. The optimal therapy for relapsing Wilms tumour has not been determined. We have treated four children with high-dose chemotherapy (HDC) followed by stem-cell support known as autologous bone marrow transplantation (ABMT). The patients were two girls and two boys aged 1-9 years. The histology was favourable in 3 cases and unfavourable, clear cell sarcoma, in 1. The relapses were pulmonary in 2 cases and skeletal in 2 cases. There was no detectable disease prior to ABMT. After ABMT 2 children got further relapses and died. On the other hand 2 children had no further relapse and are alive and well 1.5-2.5 years later. Treatment with HDC and ABMT is complicated and expensive but may be of benefit and should be added to the treatment options in children with otherwise poor prognosis of Wilms' tumour.

Spontaneous regression of intraspinal neuroblastoma.

In two infants aged 5 days and 2 months paresis of the legs due to spinal cord compression by a dumbbell neuroblastoma suddenly appeared. Immediate surgical resection of only the paraspinal tumor mass was performed. Without any further treatment, prompt spontaneous regression of the intraspinal component occurred, and the paresis disappeared within 2 weeks in both infants. We advocate surgical excision of the paraspinal tumor mass as the sole treatment in infants with symptomatic dumbbell neuroblastoma.

Hepatic veno-occlusive disease in Wilms' tumor.

Invasive surgical procedures will be avoided by recognition of veno-occlusive disease (VOD) as a clinical syndrome which sometimes leads to serious complications in children receiving Actinomycin D for Wilms' tumor. In order to document the unusually frequent occurrence of VOD far beyond the observations of others, a prospective study was undertaken in 13 consecutive Actinomycin D-treated children. There were 9 children experiencing 27 events of mostly mild VOD. Six of them were below 3 years of age having in 5 cases a unilateral kidney tumor on the right side. The criteria used for VOD were painful hepatomegaly and abdominal distension accompanied by weight gain, ascites, hemoglobin and platelet drop, with or without elevated bilirubin level found in all patients developing VOD. Supportive management of these patients should attempt to preserve respiratory and renal function, generally resulting in a favorable outcome.

National reevaluation of staging in Wilms tumor.

In order to evaluate if incorrect staging of Wilms tumor resulted in inadequate treatment a retrospective reevaluation was performed. During 1982-1990 153 patients were treated in Sweden. The review revealed that 6 cases were not Wilms tumor and 25 cases had incomplete information. The remaining material consisted of 122 cases. The survey of the charges revealed that the initial distribution was stage I 58, stage II 17, stage III 21, stage IV 15 and stage V 11. The stage was changed in 12 cases, mainly in the initial stage I. The causes for changing of the stage were thick needle puncture preoperatively in 4, capsule histologically not intact 4, tumor not radically excised 2, tumor rupture peroperatively 1 and lymph node malignancy 1. The staging procedure cannot be safely performed by a single doctor. It is dependent on cooperation in a pediatric oncologic team including the pediatric surgeon.