[Liver injuries in children. Experience in surgical and non-surgical treatment].
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Biomedical subjects
Publications and source records attributed to P Debeugny.
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22 cases of blunt pancreatic trauma are reported (17 boys, 5 girls) for the period 1968-1986; 5 of them were received later after the accident. Early diagnosis was not made in eight polytrauma and suspected in 9 abdominal trauma; CT scanner confirmed it at earlier period (1 case); the lesion was revealed later by complication (pseudo-cyst) in 5 cases. The operative treatment (20 times for 22 children) included 15 earlier laparotomies, 1 secondary laparotomy (drain 8, suture 3, partial pancreatectomy 3, duodeno-pancreatectomy 1). 5 late interventions were performed for pseudo-cysts (4 parietal drain; 1 Roux-internal derivation). The results comprise 1 death, 7 good results and 14 cases with different complications but secondary available results.
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42 observations of imperforate anus with recurrent recto-urethral or rectovaginal fistula are summarized. The results are divided in early good results: 32 cases and poor results 10, all re-operated one or more times; at long term, were obtained 39 good, 2 poor results and one with short follow-up. Two factors may determine the recurrence of fistula: earlier post-surgical infection, and technique of rectal dissection.
A non-selective series of 48 neo-nates was treated during a period of 8 years (1978-1985), with follow-up more than four months for recent observations. Surgical treatment comported Gross-technique in 32 cases; immediate total-closure in 7 cases and progressive reintegration (Schuster) in 10 cases. Long-term parenteral nutrition was performed in 38 neo-nates. The etiology of different deaths (toxic++-infection, mechanical obstruction) is discussed. The percentage of survivors was progressively raised: 70% since 3 years, and better in the last 11 cases, all with good result.
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Report of corticoadrenaloma in three children (1 malignant and 2 benign tumors). These 3 cases are analysed in comparison with the 72 cases found in the medical literature. From the whole series, it can be concluded that these tumors are rare, and occur predominantly in females and in very young children. Clinically, the secreting forms with rich and variable symptomatology contrast with the tumoral forms usually asymptomatic. The diagnosis between benign and malignant tumors remains difficult in cytopathological studies. In benign form, the treatment is always surgical and is successful. In malignant form, surgical treatment must be completed by chemotherapy, but the prognosis is definitively better if the tumor could be entirely removed.
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The authors present diagnosis particularities in 32 cases of hypertrophic pyloric stenosis, from 1980 to 1984, whom 21 cases since 1982 (25 boys, 7 girls). The diagnosis of HPS was confirmed by sonographic and roentgen explorations, isolated or associated: ultrasound alone 3 cases, ultrasound and roentgen combined 29 cases either roentgen first (22) or ultrasound first (7). Fiability was made for 22 cases; two times, error came from false interpretation; eight times were "false negative" results, corrected by roentgenogram, and corresponding with a voluminous pylorus (3) or little, sclerosis pyloric lesions (5). Amelioration of results give, from the last year, right diagnosis in 85 per cent, about 13 cases.
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The observation of a thirteen-year-old boy who presented with motor developmental delay, muscular weakness and a waddling gait is reported. Moderately increased muscular enzyme activities and electromyographic findings suggested muscular disease. Histological and ultrastructural examinations of muscle biopsy specimens showed both hypotrophia of type 1 fibers and characteristic distribution of the nuclei within the sarcomere in a chainlike pattern in the center of the fiber surrounded by a space devoid of myofibrils. A few aspects of myophagia were found explaining why muscular enzyme activities were increased. The mother was clinically normal with slightly increased muscular enzyme activities and normal muscle biopsy specimens. The maternal grandmother had delayed gait abnormalities with normal muscular enzyme activities. Approximately one-hundred cases such as ours, isolated within a family, have been published. The course and the mode of inheritance suggest heterogeneity.
This report describes the symptoms, clinical course and radiological features in three cases of idiopathic carpotarsal osteolysis. Before signs of extensive osteolysis were noted, one of our patients showed flattening, loss of harmonious curvature and minimal osteoporosis of carpal and tarsal bones, all early radiological signs which have not been described previously. We discuss the findings, classification and our ideas as to the cause of the disorder.
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Comparative investigation of 92 cases of V.A.T.E.R. syndrome (4 personal cases) and 62 cases of caudal regression (Duhamel syndrome) (2 personal cases) are performed. There is much analogy between these two entities. Initial impairment would be an early dysfunction of mesoderm setting up located on esophagus in V.A.T.E.R. syndrome and on kidneys in Duhamel syndrome. Etiopathogenic factors remain unknown. Genetic counseling is good. Detection of only one mesodermal malformation leads to inquire other unnoticed anomalies (kidneys, heart, spine, alimentary duct).