Choledochal cysts: classification and cholangiographic appearance.
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Child↗
Biomedical subjects
M Gharib
Publications and source records attributed to M Gharib.
Biliary atresia--a 25-year survey.
In the past 25 years, from 1963 to 1988, 90 children from the Department of Pediatric Surgery of the Pediatric Hospital in Cologne, Germany were treated for biliary atresia. Of these, 47 had purely extrahepatic bile duct lesions, 21 had purely intrahepatic bile duct lesions, and 22 had both extrahepatic and intrahepatic bile duct lesions. Forty-five of the children underwent a drainage operation, whereas the remaining 45 children underwent no surgery at all or simply a diagnostic laparotomy. Until 1966 hepato-jejunostomy with implantation of artificial bile ducts was conducted in 12 cases. Later, cholecystoduodenostomy was performed 4 times and hepatoporto-jejunostomy according to Kasai-Kimura 29 times. The latter was performed either without and enterostomy (n = 16) or with an enterostomy in the respective intestinal loop (n = 13). 27 patients survived (30%). If only the children with intrahepatic bile duct hypoplasia are considered, the survival rate was 12 out of 15 patients (80%). Eight children (27.5%) of the 29 with hepatoporto-jejunostomy are still alive today. This survival rate, compared with the survival rate of the total, is comparably large with 27.7%. Current data from the 23 surviving patients was retrospectively gathered in our hospital or was collected from outside the establishment. It was analyzed with regard to prognosis and long-term results. Only in 5 of the 29 cases of children with hepatoporto-jejunostomy could a lasting postoperative biliary flow be achieved. Only one of the children can be classified as completely healthy in regard to his liver.(ABSTRACT TRUNCATED AT 250 WORDS)
Anastomosis, Surgical↗
[Treatment of urethral valves with and without valve resection in the 1st year of life].
25 infants of a total of 72 child patients, who were treated between 1967 and 1987 in the Department of Paediatric Surgery of the Paediatric Hospital of the City of Cologne and were suffering from congenital urethral valves, had been transferred to the hospital for in-patient treatment within their first year of life. In accordance with a change in the treatment concept the patient material was divided into two groups: 1. Treated children up to 1978 These patients were subjected to a prolonged transurethral bouginage treatment of the urethra. Valve resection was performed subsequently at an average age of 13 months only. 2. Treated children after 1978 In these children valve resection was performed as soon as possible after birth. These two patient groups were compared with each other. It was found that both the survival times and the long-term renal function results of the children treated after 1978 had markedly improved compared with those treated before 1978, thanks to the modern treatment concept. That is true both for the incidence of secondary correction operations at the upper part of the urinary tract and for the frequency of nephrectomy.
Cystoscopy↗
[Tracheal instability in tracheo-esophageal abnormalities].
The clinical pattern of signs and symptoms of respiratory complications due to flaccid trachea has been analysed in 83 children treated in our hospital between 1983 and 1988 for tracheo-oesophageal malformations. These signs and symptoms are classified according to endoscopic findings, and are thus arranged according to various degrees of severity. Of the surviving children who were followed up and who were suffering from oesophageal atresia Vogt III B, only 5 of 57 were without a pointer towards flaccid trachea, whereas in oesophageal atresia Vogt II there were two of three. The two children with an isolated tracheo-oesophageal fistula showed abnormal findings both clinically and via endoscopy. 16 of the 20 children with very severely pronounced flaccid trachea--defined by the occurrence of life-threating apnoeas and an endoscopically identifiable tracheal collapse of more than two-thirds of the lumen--were subjected to surgery via aortosternopexy. A marked and identifiable improvement was obtained in 15 cases. Complications caused by surgery consisted of temporary phrenicus lesions in two cases.
Aorta, Thoracic↗
Confocal laser tomographic analysis of the retina in eyes with macular hole formation and other focal macular diseases.
To study the retinal surface in the human eye in normal and diseased states we used laser scanning tomography. The confocal arrangement of the laser tomographic scanner permits examination of retinal topography in the axis perpendicular to the retinal surface. The eyes examined with the laser tomographic scanner included normal eyes, eyes with macular holes, impending macular holes, radiation retinopathy, macular edema, photocoagulation scars, subfoveal scars, and serous detachment of the fovea associated with subretinal neovascularization. The laser tomographic scanner is a new method that allows measurements of the topography of the internal limiting membrane in the macular area and may improve our understanding of the pathophysiologic characteristics and treatment of a variety of disorders of the macula.
Adult↗
Bladder shrinkage as a complication of long-term supravesical urinary diversion in children with solitary kidneys.
Failures in the treatment of terminal ureteral stenosis are not seldom burdened by considerable bladder shrinkage, particularly in infants. Congenital anomalies of the urinary tract such as bilaterally ectopic ureteral ostia can also lead to bladder shrinkage, even in newborns. Using examples from our patient series, we discuss problems of contracted bladder in children and describe a method of continuous bladder distention by means of catheters with different balloon volumes and a simultaneous, intermittent, hydrostatic bladder dilatation. This method enables reintegration of a bladder which has been excluded from the urinary drainage system for a long period of time into the urinary tract, even in complicated cases, thus avoiding a permanent supravesical urinary diversion.
Child↗
[Long-term results following hepatico-jejunostomy with antireflux valve construction in congenital bile duct dilatation caused by abnormal junction of the choledochus].
Cystic-cylindrical dilatation of the intrahepatic and extrahepatic bile ducts occurs rarely in childhood. Aetiologically, congenital pancreaticobiliary junction anomalies play a decisive part. Accurate preoperative diagnosis is by no means an easy matter. The best possible diagnostic approach to clarify the pathologico-anatomic conditions consists in sonography coupled with on-target partial intraoperative cholangiography presenting the preduodenal section of choledochus and pancreatic duct. Between 1979 and 1987 surgery was performed at the Paediatric Surgical Department of the Municipal Paediatric Hospital of Cologne on 18 patients suffering from intrahepatic and extrahepatic cysticocylindrical dilatation of the bile ducts due to confirmed ectopic pancreaticobiliary junction anomaly. This study does not include all other types of choledochus cysts or biliary duct dilatations without proven pancreaticobiliary junction anomaly. The treatment of choice is the resection of the dilated extrahepatic biliary ducts followed by hepatico-jejunostomy using the Roux-en-Y technique. To avoid cholangitis due to reflux of intestinal contents via the shunted jejunum loop, we are constructing a two-stage anti-refluxive muscular mucosa valve in the shunted loop; this practice has been followed by us since 1983. Permanent postoperative freedom from cholangitis in 10 children after construction of the valve prompts us to recommend this procedure also in other types of bile duct surgery, especially in the treatment of atresias.
Anastomosis, Roux-en-Y↗
[Emergency separation of a neonatal xypho-omphalopagus twin].
We report on the emergency separation of xyphophagus-omphalophagus conjoined twins. The twins were delivered by Caesarean section in the 35th week of pregnancy. They were conjoined between the omphalus and xyphoid process and had a fusioned common liver. All other organs including the peritoneal cavities were separate. One of the twins showed normal development, whereas the other was multifocally deformed with microcephalus, complex vitium cordis, microintestine and malformation of the extremities. Due to the extremely poor general condition of the deformed twin immediate emergency separation was mandatory even before completing the diagnosis of circulatory condition and liver formation. The deformed twin died during the operation and the fully developed twin on the 7th day after the operation after cerebral haemorrhage with haemorrhagic diathesis. The report gives a detailed description of the specific foetal circulatory condition and the problem of a postoperatively occurring partial necrosis of the liver.
Cardiovascular Abnormalities↗
Imaging of uretero-pelvic junction obstruction with stimulated diuresis. With consideration of the reliability of ultrasonography.
Eighty-nine kidneys of 81 infants and children with uretero-pelvic junction obstruction were investigated using ultrasonography (US), diuretic urography (DUR) and diuretic renography (DREN). Results of the US were false-negative in 14%. This can be avoided by ensuring that the patients get an amount of fluid appropriate to their age before the examination or by performing diuretic ultrasonography with frusemide. False-positive results were found with US in 15%, but obstruction was excluded by using the DUR and/or the DREN. During post-operative follow up the DUS is useful and after 6 months the clearance with 123-iodine hippurate combined with the DREN should be performed. The performance of the Whitaker-test is unnecessary.
Child↗
[Clinical aspects, classification and prognosis of 7 cases of pediatric fibromatosis].
Seven cases of fibromatoses in infancy and childhood serve as examples to demonstrate this group of rare tumours, taking especially the aggressive forms of juvenile fibromatoses into account. There is a tendency to locally invasive destructive growth without metastasising at any time and a markedly high relapse rate after surgery--a tendency that showed up clearly in our patients, too. Only one child out of four having infantile (desmoid-type) fibromatosis of different localisation did not show any recurrence. The article reports, among other cases, on a desmoid of the stomach and oesophagus in a 15-year-old girl, not described in the literature so far, and on a congenital fibromatosis of the pancreas. Several classifications had to be performed to properly grade the individual cases (1, 6), since no generally valid systematic description of these tumours exists.
Abdominal Neoplasms↗
[Surgery of the endocrine pancreas].
After classification of the endocrine tumours of the pancreas our group of patients during the last 17 years is demonstrated including patients with nesidioblastosis in every case except one with islet cell carcinoma. Beside typical anamnesis and symptoms, diagnosis was confirmed mainly by the glucose/insulin-ratio, the extremely low fasting blood glucose and the increased need of glucose supply for normalisation. Medical therapy with somatostatin and/or diazoxide was not successful in any case for a longer time, although in persistent cases nesidioblastosis became treatable postoperatively with diazoxide. Usually, a so-called 7/8-resection was performed. Typical complications from surgical management of pancreatectomy did not occur none of the children died. As for long-term results, no patient suffered from insulin dependency or insufficiency of the exocrine pancreas; reoperation was also never necessary. The established neurological damage of children who had been operated on late, could not be repaired. Despite the heterogeneous forms of the samples, histochemical investigation established nesidioblastosis in every pancreas specimen. An explanation of the histological findings would be a failure of the B-cell-function as pathogenetic cause of nesidioblastosis.
Adenoma, Islet Cell↗
[Cecil-Duplay/Cecil-Leveuf urethral reconstruction in severe hypospadias].
A total of 199 boys with severe hypospadias were subjected to surgery during 1981 to 1984 at the Department of Surgery of the Red Cross Paediatric Hospital in Siegen and from 1971 to 1984 at the Department of Surgery of the Municipal Paediatric Hospital in Cologne. Plastic reconstruction of the urethra was performed according to a multiple-stage technique after Cecil-Duplay/Cecil-Leveuf. It is evident that good results can be obtained in such severe forms of hypospadias by means of differentiated multistep surgery. A fistula rate of 14% is an essential improvement over the results previously obtained by the urethral reconstruction method after Denis-Browne.
Child↗
[Bridging extensive esophageal atresias by a peritoneal transplant].
A method is presented that represents a modification of Rehbein's perlon filament technique for treating extensive oesophageal atresias. This new approach enables, in case of congenital oesophageal defects exceeding three vertebral bodies in length, to eventually perform end-to-end anastomosis of the patient's oesophagus. For this purpose, the distance between the mobilised oesophageal segments that are interconnected by means of a centrally positioned perlon filament, is additionally bridged over with a peritoneal cylinder in the sense of an autotransplant. The advantages of the method are the creation of a wide preformed channel for subsequent bougineages, the protective effect exercised on the mediastinum to prevent possible inflammatory conditions, and especially the continuous traction exercised on both oesophageal pouches by the shrinking of the transplant resulting in a ring-shaped stenosis that is easy to resect and can be bypassed via end-to-end anastomosis. We treated according to this method from 1982 onwards seven children with congenital oesophageal atresias extending over the length of three to seven vertebral bodies; four of these patients now present with patent oesophageal passage.
Esophageal Atresia↗
[Indications for splenectomy in children and adults].
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Adult↗
[Stomach duplication].
The eighty-four cases of stomach duplication known to date from world literature are presented including one case of the authors' own experience. The most varied theories of pathogenesis are discussed, as well as the very different clinical course the disease may take, and the diagnostic difficulties presented by X-ray examination. Finally, attention is drawn to the current use of the scintigraphy, which has broadened the diagnostic possibilities.
Humans↗
[Surgical treatment of vesico-ureteral reflux (VUR) in double ureter].
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Child↗
[Traumatic subluxation of the head of the radius (author's transl)].
Radial-head subluxations were noted in 194 patients in the course of two years. It was more frequent in girls than boys (57.7% and 42.3%). The highest incidence was between the first and fourth year of life, with a peak in the first and second years. It no longer occurs after the seventh year. In 82.5% of cases the history was typical: a sudden longitudinal pull of the affected arm. In the remaining 17.5% no exact event could be ascertained. A questionnaire sent to the attending doctors provided replies in 82 cases (42.3%). Reposition by pull and pressure on the head of the radius, supination and acute flexing achieved repositioning in 92.7%, while in 7.3% repeat repositioning was necessary on the same or the next day. After successful repositioning complete absence of symptoms occurred in 98.8%. Incidence of recurrence was 30.5% during a follow-up period of up to a year and a half. The diagnosis had at first been had at first been missed 9.8% of cases.
Age Factors↗
[Complete and incomplete urethra duplications (author's transl)].
With the help of four cases of urethra duplications, a report is presented on the occurrence, symptomatology, diagnostics, and therapy of the clinical picture. The developmental anatomy is dealt with briefly.
Child↗