Gastrointestinal duplications

Gastrointestinal duplications are rare congenital lesions that can develop anywhere along the alimentary tract and may present in the newborn period as an abdominal mass. They are differentiated from other intraabdominal cystic lesions by the presence of a normal gastrointestinal mucosal lining. Multiple theories have been proposed to account for these lesions; however, no single theory adequately explains all the known duplications. They are most frequently single, tubular, or cystic and located on the mesenteric side of the native alimentary tract structure. Symptoms often are related to the location of the duplication; oral and esophageal lesions can create respiratory difficulties, whereas lower gastrointestinal lesions may cause nausea, vomiting, bleeding, perforation, or obstruction. Treatment is resection with care taken to protect the common blood supply of the native structures. Occasionally, a partial resection with mucosal excision is required to preserve intestinal mucosa.     

Complications of pyelo-ureteral duplications in children and their surgical treatment. Apropos of 69 cases (78 pathogenic duplications)

The authors report their experience on the surgical treatment of 69 infants and children with pyelo-ureteral duplication. Urinary infection was the main revealing symptom (52 cases). Vesico-ureteral reflux was the major associated anomaly (39 cases) involving most often the lower pole ureter; the surgical correction of the reflux was performed according to the technique of Cohen with a favorable outcome in all cases. Because of poorly functioning and dysplastic upper renal segment, partial upper pole nephrectomy was performed in 8 among 12 cases of associated ectopic ureteral orifice and in 9 among 15 cases of associated ureterocele. A reimplantation of the ureter was made possible in 3 cases of ectopic ureteral orifice and in 5 cases of ureterocele with little corresponding renal lesions. With the experience of one neonate who died from septic shock following partial nephrectomy there were no other post-operative complications in this series.     

Gastrointestinal smooth muscle tumors in children: Report of three cases

We report three rare smooth muscle tumors of the gastrointestinal tract (GIT) occurring in infancy. Two were benign leiomyomata and the other a leiomyosarcoma. The imaging features and presentation of leiomyosarcoma in childhood have not been previously described.     

Blood picture findings in children with Parvovirus B19 infections (fifth disease/erythema infectiosum)]

The human parvovirus B19 provokes erythema infectiosum ("e.i."); moreover there is a wide range of diseases due to parvovirus B19 without exanthema/rash. The erythropoietic blast cells of the bone marrow seems to be the main target cells for this virus. Therefore in cases of prenatal infection the consequences are extremely similar to fetal erythroblastosis ("non-immunological" fetal hydrops). In postnatal life the parvovirus B19 infection causes hyporegenerative phases of the erythropoiesis with anaemia after 3-4 weeks. We studied the white blood cell count (WBC), erythrocytes and thrombocytes in children suffering from (serologically well documented) parvovirus B19 infection with exanthem/"e.i." (group 1; n = 23), without exanthem (group 2; n = 46) and with unknown febrile exanthematous rashes (group 3; n = 76). We did not find any characteristic data in the WBC for a diagnosis of parvovirus B19 infection. However we have for the first time documented a significant thrombocytopenia in "e.i." (group 1) not found in group 2. The thrombocytopenia appears earlier than the anaemia, because the lifespan of thrombocytes is considerably shorter than that of erythrocytes. These data suggest that parvovirus B19 attacks not only "erythropoietic" blast cells but also immature bone marrow cells, which are later responsible for the thrombocytopoiesis.     

Alimentary tract duplications in infants and children.

This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pécs, Hungary, and at the Department of Pediatric Surgery of the Medical Academy of Dresden, Germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 percent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoraco-abdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.     

Gastrointestinal intervention in children

Interventional radiology is a rapidly growing discipline in paediatrics. Many non-vascular interventional techniques may be used in the gastrointestinal tract in children. The technically simpler and more common of these may be adopted by any paediatric radiologist with an interest in interventional radiology. Other rarer and more complex techniques are currently restricted to specialist centres with a higher overall caseload. This review emphasizes the common procedures such as oesophageal dilatation, gastrostomy, insertion of transgastric jejunal feeding tubes and biopsy. Less common salivary, hepatobiliary, pancreatic and intestinal interventions are also described.     

儿童颅内脑膜瘤19例报告

目的 分析总结儿童颅内脑膜瘤的临床特点及治疗效果。方法 经手术和病理证实15岁以下儿童脑膜瘤19例，其中男9例，女10例。年龄6－15岁，平均10.9岁。临床表现以颅内压增高（11例）、颅神经麻痹（7例）和癫痫（6例）为主。CT和MRI检查发现肿瘤钙化4例，占21.5%(4/19)，囊变3例16.5%(3/19)，出血1例5.2%(1/19)。肿瘤位于大脑凸面4例，侧脑室3例，岩斜区3例，侧裂2例，鞍区2例，中颅窝底2例，矢状窦旁、大脑镰及枕大孔各1例。术中见肿瘤与硬脑膜明显粘连5例，贴近硬脑膜但无明显粘连9例，2例侧裂脑膜瘤与硬脑膜完全无关，侧脑室3例起源于脉络丛组织。病理报告纤维型脑膜瘤8例，内皮型脑膜瘤5例，砂粒型脑膜瘤2例，混合型脑膜瘤1例，非典型脑膜瘤1例，未分类2例。结果 肿瘤全切除15例，不全切除4例，无手术死亡。随访9例（6个月－4年），能正常参加学习和劳动者5例，有癫痫发作2例，肿瘤复发2例。结论 儿童颅内脑膜瘤的发病率无女性优势，肿瘤的发生部位与成人不同，肿瘤与硬脑膜关系不密切。手术全切除肿瘤是治疗的关键。     

Gastrointestinal malacoplakia in children

Four children, whose ages ranged from 1 to 13 years, with malacoplakia of the gastrointestinal tract were treated at King Faisal Specialist Hospital between 1979 and 1983. All patients had either a preceding or a coexisting chronic illness. In one patient, malacoplakia was an incidental finding, while the remaining three patients presented with bloody diarrhea, abdominal pain, recurrent fever, and severe malnutrition. Colonoscopy in two patients revealed markedly inflamed and friable mucosa with focal ulceration alternating with patches of normal mucosa and pseudopolyposis. They were treated with antibiotics and cholinergic agonists. Three patients responded favorably, while one patient continued to have extensive active disease. Although the response to therapy is unpredictable, patients may respond if the treatment is continued on a long-term basis.     

Cyclizine-intoxication in six children (author's transl)]

Cyclizine hydrochloride (Marezine, Echnatol) an antihistaminic drug is commonly used for the treatment of motion sickness. During the past two years we observed six cases of accidental moderate intoxication with an average of 200 mg of cyclizine hydrochloride in children between 2 and 4 years of age. The symptoms became obvious about 1 to 2 hours following ingestion. Among the main symptoms our patients revealed were such as excitement, incoordination, ataxia, athetoid movements and severe hallucinationes. The faces were flushed, the pupils dilated, but dryness of the mouth and hoarseness were never found. Convulsions did not occur. The treatment is purely symtomatic, histamine, apomorphine, phenothiazines and analeptics should not be used.     

Rotavirus infections in children (author's transl)]

In 43 out of 90 children suffering from acute gastroenteritis Rotaviruses could be identified in stool-specimens by electronmicroscopy. Symptoms were watery diarrhoea (100%), vomiting (88%), fever (77%), dehydration (47%) and upper respiratory tract infection (21%). In 3 out of 9 patients liver involvement could be documented. In 29 children i.v.-fluid therapy for 1-2 days was necessary. Only in 1 patient severe diarrhoea persisted for 11 days.     

24 cases of human parvovirus B19 infection in children]

From January 1, 1987 through December 31, 1990, twenty-four pediatric patients with human parvovirus B19 (HPV B19) infection were seen. In every case the diagnosis was established by a positive capture immunoassay for IgM antibodies against the HPV B19. Four patients had hematologic manifestations, including one case of transient bone marrow aplasia revealing hereditary spherocytosis, one case of autoimmune hemolytic anemia with beta-thalassemia, and two cases of peripheral thrombocytopenia. Eight patients had skin lesions, with a morbilliform rash in six cases, erythema nodosum in one case, and Gianotti-Crosti syndrome in one case. No patients had erythema infectiosum. Seven patients developed joint manifestations: Henoch-Sch?nlein purpura in two cases, arthralgia in four cases, and polyarticular disease progressing to severe rheumatoid arthritis in a thirteen-year-old girl. Unremarkable symptoms of viral disease were seen in three patients. A five-month-old infant developed severe acute myocarditis. One patient with hepatitis A had acute liver failure. This study confirms the broad spectrum of clinical manifestations of HPV B19 infection. There were a number of unusual findings, including the high rate of joint manifestations (29%) and the severe course of some hematologic and myocardial manifestations. These results raise the question of whether the HPV B19 may be involved in the genesis of chronic juvenile arthritis.     

Gastrointestinal motility disorders in children

The present state of knowledge regarding human gastrointestinal motility is summarized in this article. Areas that are of particular interest in childhood are highlighted. The relationship of this knowledge to disease states is discussed and areas where it might practically be applied indicated.     

Gastrointestinal findings in atopic children

36 children aged 0.13 to 13.05 years with severe manifestations of atopy were studied for circulating parietal cell antibodies (PCA), fasting serum gastrin and ferritin. Gastric acid secretion was measured using a pentagastrin test. In addition, 21 gastric and 28 jejunal biopsies were taken for evaluation of the mucosal morphology. In infants the gastric secretion studies were repeated after a three month hypoallergic diet.Maximal acid output (MAO) was significantly (P<0.01) diminished in atopic infants under one year of age. In this group one girl with transient PCA was achlorhydric and seven others were hypochlorhydric, as compared with controls of the same age. Acid secretion returned to normal in most children while on the elimination diet, and only two remained hypochlorhydric thereafter. The picture was more complex in the older children as some continued to exhibit hyposecretion in their second year of life, while others were even hyperchlorhydric. Epithelial degeneration, found in 13 out of the 21 samples, was the most striking feature in the gastric biopsies. Jejunal biopsies revealed increased eosinophilic infiltration of the lamina propria in 10 out of 28 diagnostic samples; two had slight and another two partial villous atrophy.It is concluded that in atopic children gastric hyposecretion and epithelial degeneration may promote the passage of unhandled food allergens through the jejunal mucosa, predisposing to more severe changes, as seen in cow's milk intolerance. Slight villous atrophy with eosinophilic infiltration and oedema of the lamina propria may cause mild absorption defects, growth and nutritional deficiencies in these children. Atopy should be considered as a possible aetiological factor in prolonged gastrointestinal disorders in infants.     

Infection control in burned children (author's transl)]

Hygienic precautions in hospital have absolute priority for the prevention of infections in patients with burns. For adequate dosage of antibiotics further studies are required. The dosage of many substances has probably been too low. The most promising results might be achieved by methods which increase the defences of the human body itself.     

LH-RH Test in prepuberal children (author's transl)]

The LH-RH test was performed in 62 mainly prepuberal children. A dose of 25 mug/20 kg was given intravenously between 10 and 12a.m. Prepuberal healthy boys between 1 11/12 and 9 7/12 years of age reacted with a fourhold increase of LH. Prepuberal boys with an unilaterally undescended testicle showed no difference in LH response from normal. Even a low increase in LH may be followed by spontaneous puberty in children with pituitary dwarfism. The LH response in children with craniopharyngeoma was heterogeneous and appeared to depend on location of tumor and extent of operation. Birdheaded dwarfism and small stature due to steroid administration and due to unknown etiology showed normal LH responses for age. Cases with anorchia and myotonic dystrophy had an excessive LH increase. The LH response in children with Fanconi's anemia and undescended testicles and in otherwise healthy boys with undescended testicles was normal for age. A case of untreated adrenogenital syndrome without salt loss had a presumably normal increase of LH when related to bone age. Primary and pituitary hypothyroidism was shown to have a higher than normal output of LH. A boy with a tumor the 3rd ventricle hat basal levels of LH that were extremely elevated and associated with precocious puberty and diabetes insipidus. A newborn infant with anencephalus showed no increase of LH and LH-RH injection.     

Chemotherapy of brain-tumors in children (author's transl)]

The poor permeability of the blood/brain barrier for most cytostatic agents makes it difficult to achieve an adaequate drug concentration in brain-tumors. This is the main problem in developing a chemotherapy of brain tumours. On the other hand the therapeutic effect is difficult to evaluate not only because of clinically and hitherto also technically poor accessability of the brain but also because of differences in tumour classification systems, and consequently limited comparability. There is however definite knowledge on the effectiveness of several single drugs as well as strong evidence on the effectiveness of some polychemotherapeutic programs. The measure of success presently is rather prolongation of survival time for months than a change in cure rate. A general recommendation for the cytostatic treatment of brain tumors may not be given in that situation, but within a clinical study the use of cytostatic agents seems promising anyway.