Fosfomycin for the initial treatment of acute haematogenous osteomyelitis.

BACKGROUND AND AIMS: At our institution there has been a dichotomous antimicrobial treatment behaviour for acute haematogenous osteomyelitis (AHOM) since 1984. The surgical department favoured fosfomycin as initial choice and the medical department beta lactams. We aimed to compare the performance of both strategies. METHODS: Data from patients discharged with the diagnosis of AHOM between January 1984 and January 1998 were gathered from the charts by means of a questionnaire. Patients receiving fosfomycin treatment (FT) were compared with those receiving fosfomycin plus other antimicrobials (FT+) and those receiving no fosfomycin treatment (NFT). RESULTS: A total of 103 patients aged 0.1-15.5 years (mean 6.5, median 6.9) with AHOM received no surgical treatment initially. In 23 (22.3%) FT was instilled initially, in 47 (45.6%) FT+, and in 33 (32.0%) NFT. The pathogen was established in 30%, 36%, and 42% of FT, FT+, and NFT patients, respectively, Staphylococcus aureus being the predominant isolate. Mean C reactive protein levels and erythrocyte sedimentation rates normalised in all treatment groups after two and four weeks, respectively. The mean duration of intravenous antimicrobial treatment in FT patients was 2.5 weeks, in FT+ patients 3.1 weeks, and in NFT patients 3.8 weeks (p < 0.05), whereas the mean duration of intravenous plus oral treatment was comparable (7.1 v 6.8 v 6.5 weeks). CONCLUSIONS: The leucocyte penetrating fosfomycin performed similarly to extracellular beta lactams in the treatment of AHOM. Intravenous treatment for longer than 2.5 weeks offered no advantage.

Intestinal pseudoobstruction as a paraneoplastic syndrome in ganglioneuroblastoma.

Intestinal pseudoobstruction may be part of a paraneoplastic syndrome. We report a teenage girl with ganglioneuroblastoma who presented with severe constipation. The intestinal pseudoobstruction was presumed to be due to inflammation of the myenteric plexus with destruction of the ganglion cells caused by antineuronal nuclear antibodies (ANNA or Anti-Hu).

Pitfalls in therapy of upside-down stomach.

The upside-down stomach (UDS) is a special form of gastric organoaxial volvulus in a supradiaphragmatic hernial sac. The authors report five cases treated between 1979 and 1998, and seek to point out possible problems and pitfalls in the surgical management of this uncommon anomaly. Retrospective analysis of these cases shows that brachyesophagus as described is not a problem in UDS, as the esophagus is of normal length. The hiatus, on the other hand, is always very large and needs proper narrowing, which may be achieved through a transabdominal approach more easily than through a transthoracic access. A common esophago-aortal hiatus is often present. In conclusion, the authors recommend a hiatal repair and gastropexy, i.e. fundophrenopexy and corpoventropexy along the esophageal axis to prevent recurrent gastric herniation or torsion. Transabdominal access should be chosen since brachyesophagus is not expected. Beware of a common hiatus when preparing the hiatal crura. An antireflux procedure is not necessary as gastro-esophageal reflux usually resolves spontaneously after hiatal repair and gastropexy.

Hypoxia-regulated gene expression in fetal wound regeneration and adult wound repair.

Fetal skin wounds heal scarlessly while adult wounds scar. Fetal wound healing occurs in a physiologically hypoxic environment whereas in adult wound healing, cells have to acutely adapt to hypoxia caused by locally impaired blood supply. We examined the expression of hypoxia-inducible factor 1 (HIF-1), a potent transcriptional regulator of oxygen-dependent genes such as vascular endothelial growth factor (VEGF), and transforming growth factor-beta (TGF-beta), a potentially HIF-1-regulated scarring cytokine, on fetal and adult responses to wounding. Incisional skin wounds were created in four sheep fetuses (twins served as controls) and two ewes at 100 days of gestation (term = 150 days). Fetal and adult wounds as well as non-wounded control tissues were harvested 2 days post-wounding. Intraoperative arterial blood gas analyses and invasive subcutaneous pO2 measurements revealed that the fetuses were indeed hypoxic while the mothers were normoxic. Expression patterns of HIF-1alpha were investigated by Western blot analyses. HIF-1alpha expression in fetal wounds and fetal control skin was similar, whereas HIF-1alpha was only detected in adult wounds but not in adult control skin. Exposure of cultured fetal and adult dermal fibroblasts to hypoxia (1% O2) showed a marked induction of VEGF mRNA. In contrast, exposure of these cell types to hypoxia did not significantly affect TGF-beta1 mRNA expression in comparison to their normoxic controls. The presence of HIF-1alpha in fetal but not in adult normal skin indicates that HIF-1alpha might be involved in fetal skin development. Conversely, the upregulation of HIF-1alpha in adult but not early fetal wound repair might represent a pathway in the pathogenesis of scarring, since several growth factors overexpressed in, and associated, with scarring are hypoxia-inducible. Further studies need to be performed in order to identify hypoxia-regulated HIF-1alpha target genes involved in the pathogenesis of scarring.

An animal model for short-bowel syndrome in piglets to assess the efficiency of bowel-lengthening procedures.

Intestinal lengthening is considered to be one of the most effective surgical interventions to improve the deleterious sequelae of short-bowel syndrome. To assess the efficiency of bowel-lengthening procedures in short-bowel syndrome, an animal model was created in minipigs that show malnutrition, weight loss and small-bowel dilatation. Group 1 consisted of three animals with a 95% distal intestinal resection leaving 15 cm of ileum. Group 2 comprised six animals with a 95% distal intestinal resection leaving only 5 cm of ileum, and group 3 consisted of three animals with a 95% distal intestinal resection leaving 5 cm of ileum and construction of a distal small-bowel stenosis by temporary banding. In group 1 there was a significant increase in length of residual bowel at terminal laparotomy at 10 weeks and a significant difference for protein and potassium. No bowel dilatation occurred and no animal lost weight. Group 2 animals showed a continuous loss of weight after the intestinal resection and significantly different values in animal profile were found at 10 weeks but no significant difference in length or in diameter of the residual bowel. In group 3, all animals demonstrated a significant dilatation of the residual small bowel at 1 week when the stenosis was resected and bowel continuity restored. We conclude that in piglets a 95% distal intestinal resection leaving 5 cm of ileum leads to a short-bowel syndrome with malnutrition and weight loss, but no intestinal dilatation. To provoke an additional intestinal dilatation, creation of a distal stenosis is mandatory. Animals of group 3 represent a suitable model to assess the efficacy of short/bowel lengthening procedures.

Greater omentum flaps and granulocyte transfusions as combined therapy of liver abscess in chronic granulomatous disease.

We report the case of a 17-year-old boy with gp91phax-deficient chronic granulomatous disease who developed a liver abscess due to Staphylococcus aureus. Despite treatment with appropriate antibiotics and gamma interferon for three months as well as incision and drainage, the abscess persisted unchanged in size. After surgical debridement, the abscess cavity was filled with two pedunculated greater omentum flaps as a direct feeder road of granulocytes to the infectious focus. An average of 48.5 x 10(9) granulocytes a day harvested from G-CSF-prestimulated donors were transfused for a total of 8 days without side effects. Ultrasound 3 months later showed no residual abscess. Combination of greater omentum flaps and transfusion of G-CSF-prestimulated granulocytes may be the optimal treatment for liver abscesses refractory to conventional therapy.

Postrenal anuria after appendectomy in childhood.

An 11-year-old boy suffered from macroscopic haematuria and bilateral flank pain a few days after uneventful appendectomy for retrocaecal appendicitis phlegmonosa. Ultrasonography revealed a complete bilateral distal obstruction of the ureters. Renal failure due to postrenal anuria resolved completely after intravenous antibiotics.

In vivo and in vitro characterization of neonatal hyperparathyroidism resulting from a de novo, heterozygous mutation in the Ca2+-sensing receptor gene: normal maternal calcium homeostasis as a cause of secondary hyperparathyroidism in familial benign hypocalciuric hypercalcemia.

We characterized the in vivo, cellular and molecular pathophysiology of a case of neonatal hyperparathyroidism (NHPT) resulting from a de novo, heterozygous missense mutation in the gene for the extracellular Ca2+ (Ca2+(o))-sensing receptor (CaR). The female neonate presented with moderately severe hypercalcemia, markedly undermineralized bones, and multiple metaphyseal fractures. Subtotal parathyroidectomy was performed at 6 wk; hypercalcemia recurred rapidly but the bone disease improved gradually with reversion to an asymptomatic state resembling familial benign hypocalciuric hypercalcemia (FBHH). Dispersed parathyroid cells from the resected tissue showed a set-point (the level of Ca2+(o) half maximally inhibiting PTH secretion) substantially higher than for normal human parathyroid cells (approximately 1.8 vs. approximately 1.0 mM, respectively); a similar increase in set-point was observed in vivo. The proband's CaR gene showed a missense mutation (R185Q) at codon 185, while her normocalcemic parents were homozygous for wild type (WT) CaR sequence. Transient expression of the mutant R185Q CaR in human embryonic kidney (HEK293) cells revealed a substantially attenuated Ca2+(o)-evoked accumulation of total inositol phosphates (IP), while cotransfection of normal and mutant receptors showed an EC50 (the level of Ca2+(o) eliciting a half-maximal increase in IPs) 37% higher than for WT CaR alone (6.3+/-0.4 vs. 4.6+/-0.3 mM Ca2+(o), respectively). Thus this de novo, heterozygous CaR mutation may exert a dominant negative action on the normal CaR, producing NHPT and more severe hypercalcemia than typically seen with FBHH. Moreover, normal maternal calcium homeostasis promoted additional secondary hyperparathyroidism in the fetus, contributing to the severity of the NHPT in this case with FBHH.

Clinical implications of phrenic nerve injury after pediatric cardiac surgery.

Phrenic nerve injury with resulting diaphragm paralysis occurred in 25 (1.5%) of 1,656 cardiac surgical procedures in children during a 10-year period. Phrenic nerve injury was most commonly noted in patients who had undergone previous cardiac surgery (16 of 165, 10%; P < .0001), typically after a previous Blalock-Taussig shunt (10 of 53, 19%; P = .007). Plication of the diaphragm (7 thoracic, 4 abdominal) was performed in 11 patients (44%). Indications for plication were inability to wean from mechanical ventilation (5 patients) and persistent or recurrent respiratory distress (6 patients). The patients who needed diaphragm plication were significantly younger than those who were managed conservatively (median, 11 months [4 days to 23 months] versus 20 months [4 months to 16 years]; P = .01). All patients older than 2 years were extubated within 3 days (mean, 1.5 days) and did not need any surgical intervention. The median follow-up period was 3.2 years, and no patient has had recurrent respiratory problems. There were no deaths as a direct result of phrenic nerve injury. Phrenic nerve injury after cardiac surgery is a serious complication that often leads to respiratory insufficiency in patients under than 2 years of age. For such patients, early diaphragm plication is a simple and effective procedure that prevents the complications of prolonged mechanical ventilation.

Achalasia in childhood and adolescence.

We analysed the charts of paediatric patients suffering from oesophageal disorders in 5 departments of paediatric surgery in Switzerland from 1972-1993 finding only 16 patients with proven achalasia (0.6%). The personal history showed a wide gap of several years from the first symptoms until diagnosis and treatment due to false interpretation of symptoms. Dilatations alone have never been definitely successful. In 16 patients, a Heller's procedure was done, 2 had an additional Thal, 12 a Nissen fundoplication, one a hiatoplasty and one no antireflux procedure. 3 patients needed further postoperative dilatations for several months up to 3 1/2 years. One patient suffering from an AAA syndrome (Achalasia/Alacrimia/ACTH deficiency) did not improve after Heller's procedure with Nissen fundoplication and dilatations. We finally had to resect the oesophagus and replace it by gastric transposition. Two children were lost for long-term follow-up. In 14 patients the follow-up is more than 10 years. 14 patients take normal food and are in the normal percentiles for length and height. Achalasia in childhood shows a wide spectrum of oesophageal dysmotility. In many cases, the propulsive force might be so weak that only a very lose antireflux procedure is tolerated. Occasionally, the whole oesophagus is without appropriate function and has finally to be resected as in our patient suffering from AAA syndrome. This child is now free of clinical pathological symptoms since several years.

Management of petrous bone fractures in children: analysis of 127 cases.

Petrous bone fractures (PBF) in children are relatively frequent. They are mostly diagnosed after collisions and falls. The complications typically associated with PBF were different types of hearing disorders in 69.1% of the patients who had audiometry, liquorrhea in 16.5%, palsy of cranial nerves in 10.8% (facial nerve palsy in 9.4%), bacterial meningitis, stenosis of the external ear canal, and posttraumatic cholesteatoma in 0.7% of the fractures each. Most complications were transient; 8.6% of the patients underwent surgery because of PBF-related complications and 9.4% suffered from severe, irreversible sequelae. Management of PBF in children requires an interdisciplinary approach between pediatric surgeons and pediatric ear, nose, and throat (ENT) specialists. It basically includes daily examination for cranial nerve palsy, liquorrhea, and meningitis during hospitalization as well as routine audiometric examination and antibiotic prophylaxis. Routine vaccination against Streptococcus pneumoniae as a new standard procedure and subtotal petrosectomy after transverse fracture as a new surgical modality are strongly recommended in order to lower the incidence of posttraumatic meningitis. Severe complications such as persistent hearing loss, persistent liquorrhea, cranial nerve palsy, and posttraumatic meningitis require aggressive diagnostic and therapeutic measures in order to minimize further morbidity and irreversible deficits.

Cranioplasties for congenital and acquired skull defects in children--comparison of new concepts with conventional methods.

From 1974 to 1992 fifty-two patients with congenital or acquired skull defects were operated at the Department of Pediatric Surgery of the University Children's Hospital of Zurich. By 1988, in 26 patients conventional methods with PMMA (polymethyl methacrylate) plasties or rib plasties were performed. After 1988, in 26 patients skull reconstruction was done by skull splitting, application of lyophilized bone or cartilage or a combination of both. In the latter period, stabilization and fixation was always provided by biodegradable screws and bands. The results of the different techniques were compared in a retrospective fashion. PMMA plasties provided immediately full stability and good cosmetic results. Another advantage was their availability. In one patient (= 4.8%), a wound infection required the removal of the plasty. In two other patients (9.5%), an increasing mobility of the plasty could be observed during skull growth. Rib plasties were not satisfying. Skull splitting or reconstruction with lyophilized bone or cartilage showed good results with a stable integration within 3-4 months. In one patient (4%), a superficial wound infection occurred, but it did not affect the plasty. From the present study, we conclude that skull splitting or the reconstruction of skull continuity by means of lyophilized bone or cartilage with fixation through biodegradable screws and bands are the methods of first choice in children, because they are fully integrated, avoid foreign material and might have a slightly lower risk of infection. In addition, removal of the implants may be avoided.(ABSTRACT TRUNCATED AT 250 WORDS)

Complications and long-term results of the Nissen fundoplication.

The Nissen fundoplication is the operative therapy of choice in children with symptomatic gastroesophageal reflux. In recent years several reports on specific complications of the Nissen fundoplication have been published. This report summarizes the complications in 45 patients on whom a Nissen fundoplication was performed in our clinic between 1979 and 1989. 18 patients (40%) showed early or late complications. In 10 patients (22%) reoperation was necessary. However, at follow-up with a mean period of 4 1/2 years after surgery, 36 patients (92%) showed good results without any residual symptoms. The Nissen fundoplication had in our hands a rather high rate of complications. However, late results were excellent and our healing rate (92%) was higher than reported in other series with different techniques.

20 years of functional reconstructive surgery in bladder exstrophy--balance 10 years after a preliminary report.

This is an update of a consecutive series of 19 patients with bladder exstrophy presented as a preliminary report 10 years ago with a follow-up of 6 years. The average time of follow-up now is 14 years. In difference to the former report several additional procedures have been performed in most patients. 3 early "good" patients stay "good and continent". Only 3 out of 5 patients categorized as "fair" are now "good and continent" and only one without further intervention. 3 out of 6 patients who were classified as "poor" are now diverted. The overall rate of secondary diversions is 40% including 2 patients with ureterosigmoidostomies. All patients have normal renal function and normal blood pressures. These results show that an early "good" result generally remains "good" but additional surgery may be warranted. The initially "fair" results usually necessitate a secondary procedure, e.g. augmentation cystoplasty or/and bladder neck tightening to show an improvement. Patients with early "poor" results unfortunately remain "poor" and have a great chance to get diverted.

Liaison psychiatry in pediatric surgery--a promising approach.

Liaison psychiatry in pediatric surgery is described and the experiences at the University Children's Hospital in Zurich are reported. The patients seen by the child psychiatrist in surgery are specified and compared with those seen in pediatrics. For the most part the same psychiatric diagnoses were made in both groups with some differences in frequency. It is concluded that liaison psychiatry should be implemented in pediatric surgery, as has long been the case in pediatrics.

[Reconstruction of external genitalia of the girl with adrenogenital syndrome]. / Die Rekonstruktion des äusseren Genitale bei Mädchen mit adrenogenitalem Syndrom.

Congenital adrenal hyperplasia (CAH) is the most common cause for female pseudohermaphroditism. In these patients uterus and Fallopian tubes are present and also in severe cases the clitoris cannot function as penis later in life. Therefore, a neonate with ambiguous genitalia due to CAH should be raised as female. To assess the degree of masculinization, a modification of Prader's classification was used. In 23 girls with ambiguous genitalia reduction clitoroplasty preserving the neurovascular bundle and creating a dorsal preputial skin flap and vaginoplasty with a perineal skin flap were performed. The phallic skin was used to create the labia minora and the anterior vaginal wall. The patients were reevaluated 1 to 14 years after the operation. The cosmetic and functional results have been excellent.

Characteristics and prognosis of extradural hematomas in children.

This is a report on 31 children with operated extradural hematoma. The aim of the study was to elucidate the characteristics of extradural hematoma in childhood and the different clinical pattern and outcome compared to adults. For that reason the charts of the patients seen at the Department of Surgery of the Children's University Hospital Zurich between 1972 and 1990 have been revised. Follow-up criteria were recorded according to the Glasgow Outcome Scale. Most hematomas have been provoked by minor injuries and almost all children presented with at least one typical clinical sign. Associated intracranial injuries have been found in four patients, while two were polytraumatised. 30 of the 31 patients survived. One patient died of malignant brain edema. The mean range of follow-up was seven years. 28 patients had a "good recovery", "moderate disability" and "severe disability" was found in a patient one each. Our results demonstrate that the long-term outcome in children with extradural hematoma is far better than in adults regarding survival as well as quality of life. The causes may be minor traumas with fewer associated lesions and presentation with typical clinical findings and therefore early diagnosis and treatment.

Is intestinal lengthening effective in treating extreme short bowel syndrome?

Our report concerns a child with short bowel syndrome who had 20 cm of small intestine distal to the pylorus (duodenum and jejunum) which remained after subtotal intestinal resection; the resection was necessitated by intrauterine volvulus with intestinal necrosis as a result of gastroschisis. In addition, only 25 cm of the colon remained. Despite continuous enteral nutrition with a semi-elementary diet and conservative therapy, it was not possible to provide within six months at least occasionally half of the caloric intake required on an enteral basis. In such cases treatment is usually discontinued. As final alternative to a small intestine transplantation, we conducted an operation to lengthen the intestine (method according to Bianchi) when the patient was 8 months old. The massively dilatated jejunum section of the intestine was lengthened from 20 cm to 37 cm. Postoperatively enteral caloric intake could be increased from 11 kJ/kg/m to 20 kJ/kg/m. Unfortunately, at 14 months of age, the child died from hepatobiliary complications arising from aggravation of the cholestasis and acidosis. This case shows, nevertheless, that intestinal lengthening had a positive influence on intestinal resorption.