Results of the operative treatment of gastroesophageal reflux in childhood with particular focus on patients with esophageal atresia.

AIM: Although the literature on fundoplications in childhood is relatively extensive, only few reports exist which correlate the rate of complications with the primary disease. Other important questions such as the incidence of postoperative dumping syndrome or Barrett's esophagus in childhood are barely treated. Nor have operative techniques such as single or double-row cuff sutures or the benefit of performing pylorotomy or pyloroplasty in addition to fundoplication been investigated with respect to their recurrence rates. In particular studies on laparoscopic fundoplications tend to be generally confined to a discussion of the feasibility of the endoscopic procedure, the duration of the intervention, the length of the hospital stay and the costs. The study presented here aims to analyze such still unanswered questions listed above for open fundoplication procedures, including an analysis of the authors' own patient population, and to discuss the questions together with the most important reports in the literature. The aim is to create a basis for later studies which will compare conventional and laparoscopic fundoplications. METHODS: In the period between 1993 and 2005, 160 children underwent a fundoplication procedure. From 2003 onwards, fundoplications were carried out laparoscopically. The data of 148 patients were analyzed, some of them on the basis of clinical follow-up and some on the basis of an extensive questionnaire and among others with the help of the parents' support group KEKS. RESULTS: The underlying disease in 87 patients was reflux disease stemming from esophageal atresia, previous diaphragmatic operation in 8 children and mental retardation with swallowing difficulties in 30 patients. Only 23 patients suffered from isolated gastroesophageal reflux disease without an underlying primary disease. Intraoperative complications occurred in 4.6 % of patients with esophageal atresia (EA), while the rate for the remaining collective of patients was 1.6 %. Postoperative complications were observed in 10.3 % of the children with esophageal atresia and in 8.2 % of the other cases. The recurrence rate was 16.1 % in the children with EA and 6.5 % in the other cases. Dysphagia and/or stenosis occurred in 17.2 % and 6.5 % of children, respectively, and dumping syndrome was observed in 18.3 % of the EA group and only in 1.6 % of the comparison group. An evaluation of 79 esophageal biopsies showed no difference between patients with EA and the comparison group concerning the degree of histological changes. When evaluating the suturing technique, it was found that a double-row fundus suture was more effective in preventing reflux recurrence than a single-row suture but also resulted in an increased rate of dysphagia. When considering the not infrequent occurrence of dumping syndrome, it was found that drainage operations such as pyloroplasty or pyloromyotomy are only indicated for reduced gastric motility. The data of the children without EA, who initially served as a comparison group, was analyzed further. Four summaries of the current literature in table form complete this discussion. CONCLUSION: Particularly in children with EA, Nissen fundoplication cannot be considered a procedure with few complications. The problems resulting from disturbed gastric and esophageal motility should not be underestimated nor should the postoperative occurrence of dumping syndrome. Postoperative long-term follow-up until the patients reach adulthood is absolutely necessary to ensure that development of a Barrett's esophagus is not overlooked.

Natural rubber latex sensitisation and allergy in patients with spina bifida, urogenital disorders and oesophageal atresia compared with a normal paediatric population.

BACKGROUND: Natural rubber latex (NRL) contains proteins which, after repeated contact with latex products and an allergic predisposition (atopy), can lead to sensitisation (specific IgE against NRL proteins) or allergy (type 1 allergy with symptoms from urticaria to allergic shock). Spina bifida patients are known to be a high risk group for latex allergy and sensitisation due to numerous operations beginning soon after birth. In the study presented here we compared spina bifida patients with patients who also underwent repeated operations beginning soon after birth (urological malformations) or underwent surgery once in the neonatal period but had numerous anaesthesias because of repeated treatment with a bougie (oesophageal atresia). In this setting the influence of surgery and anaesthesia on NRL-sensitisation was investigated. MATERIALS AND METHODS: We investigated the prevalence of NRL-specific IgE (> 0.35 kU/l, ImmunoCAP system, Pharmacia) in a normal paediatric population (neither atopic nor having undergone surgery) (group I), spina bifida patients (group II), children with urogenital malformations (group III) and children with oesophago-tracheal malformations (group IV). RESULTS: The highest rate of NRL-sensitised patients was found in the spina bifida group (II) (48 %), followed by groups III and IV with 17 % each, compared to 4 % for the control group. NRL-allergic reactions were noted only in the patients with spina bifida and the urological malformation group (18 % in group II, 8 % in Group III). Apart from atopy the number of operations could be identified as a risk factor for the development of NRL-sensitisation and allergy (group II, III). The prevalence of latex allergy was lower after repeated anaesthesia (group IV) than after repeated surgery. CONCLUSIONS: In addition to the known high risk group of spina bifida patients, other patients with congenital malformations and early surgery also have a significant risk for latex sensitisation. When treating patients with malformations requiring repeated surgery, prophylactic measures similar to those for spina bifida patients should be considered.

Primary intervention for posthemorrhagic hydrocephalus in very low birthweight infants by ventriculostomy.

The objective of our study was to determine the efficacy of ventriculostomy as the primary treatment for posthemorrhagic hydrocephalus in premature infants. Within a period of 4 years, 20 very low birthweight (VLBW) infants (birthweight median 1,135 g, range 650-1,470 g) were treated for progressive posthemorrhagic hydrocephalus (PHHC) by right parietal ventriculostomy (Salmon Rickham) at a mean age of 21 days. Serial tapping of the subcutaneous reservoir was performed for temporary drainage until conversion to a permanent ventriculoperitoneal (VP) shunt or spontaneous resolution of hydrocephalus. A total of 1,402 punctures (median 71/infant, range 13-168) was performed. The results showed that only 1/20 patients developed a cerebrospinal fluid (CSF) infection, accounting for a 5% patient-related and 0.07% procedure-related infection rate. Major complications such as skin defects, subdural hygroma, or CSF leaks occurred in three patients (15%). A permanent shunt was needed in 17 patients (85%). We concluded that, as an effective alternative to serial or lumbar puncture, there should be early implantation of ventriculostomy reservoirs for serial taps to control intracranial pressure in PHHC of VLBW infants until a permanent shunt can be placed because of the low incidence of infections and technical complications.

Artificial abdominal hernia for the treatment of hepatomegaly in a neonate with stage 4S neuroblastoma.

Surgical widening of the abdomen by a silastic pouch has been used very rarely in the management of critically ill infants with hepatomegaly due to neuroblastoma stage 4S. A female newborn baby was referred on the second day of life because of local compressive effects of a massive hepatomegaly, which lead to multiorgan failure. An artificial abdominal hernia was created on the third day of life using a silastic pouch. During the operation oxygenation and ventilation improved and urinary output returned. After chemotherapeutic reduction of hepatic metastases and primary tumor the pouch was successfully removed on day 57 without local complications. The child has survived for more than 1 year and is in complete remission. An artificial abdominal hernia should be considered more often in the critically ill neonate with stage 4S neuroblastoma and massive hepatomegaly.

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)

[Treatment of urethral valves with and without valve resection in the 1st year of life]. / Die Urethralklappenbehandlung mit und ohne Klappenresektion im ersten Lebensjahr.

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.

[Long-term results following hepatico-jejunostomy with antireflux valve construction in congenital bile duct dilatation caused by abnormal junction of the choledochus]. / Langzeitresultate nach Hepatiko-Jejunostomie mit antirefluxiver Klappenbildung bei kongenitaler Gallengangsdilatation infolge Choledochusfehlmündung.

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.

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.

[Surgery of the endocrine pancreas]. / Chirurgie des endokrinen Pankreas.

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.

[Sacral teratomas in childhood. An analysis of long-term results in 87 children]. / Steissbeinteratome im Kindesalter. Eine Analyse der Langzeitergebnisse von 87 Kindern.

52 patients were followed up out of a total of 87 children with sacrococcygeal teratomas, within a period of 25 years, from three paediatric surgery hospitals and out of altogether 73 survivors. The results in 35 children where the operation had been performed more than three years ago, were classified as "late results". From this group, 18 children were followed up by clinical examination only, without detecting any dysfunctions. By the additional use of electromanometry of urinary bladder and rectum, however, bladder and rectum dysfunctions were seen in about 40% of 17 further children. These dysfunctions were partly myogenic and partly neurogenic in nature and were directly related to the size, tumour status and intrapelvic extension of the sacrococcygeal teratoma.

[Comparative results of ileal conduit and colonic conduit. Analysis of 50 children with bladder exstrophy]. / Vergleichende Ergebnisse von Ileumkonduit und Kolonkonduit. Analyse von 50 Kindern mit Blasenekstrophie.

During the last 20 years 50 children with exstrophy of the bladder were treated in the Department of Paediatric Surgery of the Children's Hospital of Cologne employing besides other surgical methods such as primary closure, ureterosigmoideostomy, ureterocutaneostomy etc., in 15 cases an ileal conduit and in 12 cases a colonic conduit. These children could be followed up on the average 8.5 or 3 years after the operation, clinically, roentgenologically and in some cases via scintigraphy. Late complications requiring surgical correction, such as stomatostenoses, conduit elongation, stenoses of the ureterointestinal anastomosis, calculus formation in the conduit, or complications like ureteral reflux, recurring infections of the urinary passages with pyelonephritis, occurred only with ileal conduits, whereas no late complications requiring surgery were seen with the colonic conduits. Similar results in respect of late complications were found among the patients in Munich from 1955 to 1983 with 35 exstrophies of the bladder (13 ileal conduits, 1 colonic conduit) in which additionally an adeno-carcinoma was seen after ureterosigmoideostomy with fatal outcome. Hence, we are of the opinion that the method of choice is the preparation of a colonic conduit in patients with exstrophy of the bladder where primary closure is not possible because the bladder lamina is too small or already epithelialised. This approach offers the safest possible long-term protection of the primary normally positioned upper urinary tract.

[Appendicitis in childhood]. / Appendizitis im Kindesalter.

Appendectomy was performed on 1,059 children at the University Clinic of Paediatric Surgery in Mainz from 1. 1. 1975 to 31. 6. 1983. For the retrospective examination of the indication for appendectomy histopathological and intraoperative findings were analysed and evaluated. Histologically five types of appendicitis were differentiated: 1. acute appendicitis (two forms: acute ulcero-phlegmonous appendicitis with or without perforation and acute superficial appendicitis), 2. chronic appendicitis, 3. lymphatic hyperplasia, 4. submucosal fibrosis, 5. rare diseases. In 618 cases (= 58.3%) acute appendicitis was diagnosed histologically. In another 203 cases (= 19.2%) intraoperative findings (e.g. Lymphadenitis mesenterialis, Meckel's diverticulum) were retrospectively collected; they caused symptoms similar to those of appendicitis. However, there remain retrospectively 22% of all appendectomised children with no indication for laparotomy. The statistical analysis of postoperative complications showed a significant dependance from the histopathological findings. The highest rate of complications was seen in cases with perforated (34%) or non-perforated (10%) ulcero-phlegmonous appendicitis. Children with acute superficial appendicitis had a complication-rate of 5%; those with lymphatic hyperplasia and submucosal fibrosis of 6% each. Relaparotomies were almost exclusively necessary in cases with acute appendicitis; septic and pulmonary complications were mostly seen either in infants with malformations or other perinatal risks, or in children with additional severe diseases. Therefore non-acute appendicitis justifies a wide indication for appendectomy because of a low complication-rate; this, however, is not valid for high-risk children (e.g. malformations). In these cases sonography might be useful for preoperative diagnosis.

[Late results following femoral shaft fractures in childhood]. / Spätergebnisse nach Oberschenkelschaftfrakturen im Kindesalter.

The results of 102 children with fractures of the femur shaft treated by osteosynthesis with dynamic compression plates (DCP) in comparison to 87 children which underwent a conservative treatment by extension are presented. Of 329 patients in a 15-years period, 238 children had a follow-up examination with evaluable data and 49 patients were excluded because of a different therapy such as plaster traction, cast, osteosynthesis with screws or nails etc. The children were divided into two groups: series I from 1968 to 1977 and series II from 1978 to 1982. 19.6% of the patients had a residual difference of more than 1 cm in the length of the legs. Operated children were twice as often afflicted as the conservative group. The amount of difference in the length depended on the duration of instability and increased when several repositions were required in the conservative group, or when an operation was carried out secondary to inadequate traction therapy. Axial deviation was found to be much more common in the extended group (16.1%) than in the operated group, in which only 4% axial deviations persisted in series II when DC-plates were used exclusively. Predominant in the conservative group was a persisting antecurvation in 13 of 14 children. Residual rotation malalignment of significant degree was found only in the group treated by extension, while the operated group averosed at minor torsion deformities. Children with closed epiphysis on the date of examination, who had a persisting rotation failure, had been treated nearly exclusively by extension (50% versus 15%).(ABSTRACT TRUNCATED AT 250 WORDS)

[Abnormal choledocho-pancreatico ductal junction causative of cystic-cylindric dilatation of intra- and extrahepatic bile ducts]. / Fehlmündung des Ductus choledochus in den Ductus pancreaticus als Ursache der zystisch-zylindrischen Dilatation der intra- und extrahepatischen Gallenwege.

During the last 5 years there were 6 patients found to have abnormal ductal junction of the choledochus into the pancreas. Clinical signs were: repeating upper abdominal pain, intermittent icterus, signs of pancreatitis and/or palpable tumor. Sonography is the initial examination to show up a dilatation of the intra- and extrahepatic bile ducts. But intraoperative cholangiography alone reveals the true condition of the choledocho-pancreatico ductal junction. Only after this procedure the therapeutic step of a choledocho-jejunostomy should be done.

[Congenital epulis - report of a case of a so-called granular-cell myoblastoma in a newborn (author's transl)]. / Die kongenitale Epulis - ein Beitrag zum sogenannten "Myoblastenmyom des Neugeborenen".

We present a case of multiple congenital epulides in a newborn girl. Though the tumors were not excised completely, we found a complete regression after two months. Considering the different clinical characteristics of granular-cell myoblastomas in newborn children and adults we feel, that these lesions may be different entities with similar histomorphological structure.