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Biomedical subjects

S Holland-Cunz

Publications and source records attributed to S Holland-Cunz.

11 recordsLinked to original sources

Acute testicular torsion in children: the role of sonography in the diagnostic workup.

Acute testicular torsion in children is an emergency and has to be diagnosed urgently. Doppler sonography is increasingly used in imaging the acute scrotum. Nevertheless, in uncertain cases, surgical exploration is required. In this study, we attempted to define the role of Doppler sonography in the diagnostic workup of the acutely painful scrotum. All patients admitted between 1999 and 2005 with acute scrotal pain were included. After clinical assessment, patients were imaged by Doppler sonography with a ''high-end'' instrument. In cases of absent arterial perfusion of the testis in Doppler sonography, surgical exploration was carried out. Patients with unaffected perfusion were followed clinically by ultrasound for up to 2 years. Sixty-one infants and children aged 1 day to 17 years (median: 7.9 years) were included. In 14 cases, sonography demonstrated absent central perfusion, with abnormal parenchymal echogenicity in six. Absence of venous blood flow together with reduction of central arterial perfusion was found in one infant. In these 15 patients, surgical exploration confirmed testicular torsion. Among the other 46 patients, we found four cases with increased testicular perfusion and 27 with increased perfusion of the epididymis. In one infant, a testicular tumour was found sonographically, and orchiectomy confirmed diagnosis of a teratoma. Follow-up examinations of the conservatively treated patients showed good clinical outcome with physiologic central perfusion as well as normal echogenic pattern of both testes. No case of testicular torsion was missed. By means of Doppler sonography, an unequivocal statement regarding testicular perfusion was possible in all cases. The initial Doppler diagnosis was confirmed by operative evaluation and follow-up ultrasound. Testicular torsion can therefore be excluded by correctly performed ultrasound with modern equipment.

Acute Disease↗

[Computer-assisted operational planning for pediatric abdominal surgery. 3D-visualized MRI with volume rendering].

Exact surgical planning is necessary for complex operations of pathological changes in anatomical structures of the pediatric abdomen. 3D visualization and computer-assisted operational planning based on CT data are being increasingly used for difficult operations in adults. To minimize radiation exposure and for better soft tissue contrast, sonography and MRI are the preferred diagnostic methods in pediatric patients. Because of manifold difficulties 3D visualization of these MRI data has not been realized so far, even though the field of embryonal malformations and tumors could benefit from this.A newly developed and modified raycasting-based powerful 3D volume rendering software (VG Studio Max 1.2) for the planning of pediatric abdominal surgery is presented. With the help of specifically developed algorithms, a useful surgical planning system is demonstrated. Thanks to the easy handling and high-quality visualization with enormous gain of information, the presented system is now an established part of routine surgical planning.

Abdomen↗

Feminising genitoplasty: one-stage genital reconstruction in congenital adrenal hyperplasia: 30 years' experience.

The study objective is to evaluate the results of our surgical technique for children with congenital adrenal hyperplasia and ambiguous genitalia at the University Hospital of Heidelberg, Department of Paediatric Surgery. The records of 19 patients with congenital adrenal hyperplasia treated between 1972 and 2004 were reviewed with respect to age at surgery, operative procedures and outcome. We describe the recession clitoroplasty technique currently used in our hospital and highlight the importance of short and long-term follow-up results with respect to appearance, position and size of the clitoris and quality of the vagina. One-stage recession clitoroplasty and vaginoplasty gives very satisfactory cosmetic and functional results, with few complications and a reduced need for secondary surgical interventions. The results of this study support the assumption that total correction can be achieved through a single-stage operation, performed in infancy.

Adrenal Hyperplasia, Congenital↗

[Fetal surgery].

Fetal surgery is a newly evolving multidisciplinary medical field, being performed in specialized centers. It deals with the treatment of selected fetuses with congenital anomalies that cause high morbidity and mortality. The aim of the surgical treatment is to avoid the development of progressive and eventually irreversible damage at birth. Examples of entities treated are obstructive uropathy leading to renal insufficiency, lung hypoplasia in severe congenital diaphragmatic hernia, severe congenital cystic adenomatoid malformation of the lung, and sacrococcygeal teratoma. This review describes principles of fetal surgery, physiopathologic processes of some of the anomalies treated in this way, and diagnostic and therapeutic approaches. Recently published results are presented and discussed.

Coccyx↗

Three-dimensional co-culture model of enterocytes and primary enteric neuronal tissue.

AIM: The aim of this study was to establish a three-dimensional model of the innervated mucosal barrier using a co-culture of an enterocyte cell line and enteric glial and nerve cells. Such a model might form the basis for further studies of interactions between the single compartments of the bowel wall, as well as of extrinsic influences on intestinal development and plasticity. METHODS: Isolated and dissociated myenteric plexus was resuspended in either collagen or extracellular matrix (ECM) solutions. After incubation at 37 degrees C the solution gelled and formed stable plugs where neurons and glial cells reaggregated to form secondary neuronal networks. HT-29-enterocytes were seeded on top of the gels either immediately (collagen, ECM), or after adding a thin layer of collagen II (ECM). RESULTS: While the neuronal tissue formed complex networks within the gel, the enterocytes on top of the gels grew differently depending on the substrate and innervation. So enterocytes on ECM gels did not grow to confluence, while on collagen gels or on ECM plus collagen larger patches and increasing confluence could be observed. In general HT-29 grew better on innervated gels than on gels with no neuronal tissue. CONCLUSIONS: With the presented model of different compartments of the bowel wall, various parameters of intercellular dependencies and influences can be observed in vitro. Moreover, the first results are also steps towards developing an innervated gut wall in vitro which might be able to restore functional capacity in infants with short bowel syndrome or other disorders that severely impair bowel function.

Animals↗

Protective fibrin-sealed plication of the small bowel in recurrent laparotomy.

PURPOSE: Adhesions after recurrent abdominal operations remain extremely common and are sources of severe morbidity. Fibrin-glued plication of the small gut in a meander-like formation is supposed to guarantee a decreased risk of intestinal obstruction postoperatively. This retrospective study analyses the clinical outcome after recurrent laparotomy in children treated with bowel plication by fibrin sealant. METHODS: The surgical technique of performing the fibrin-glued plication is rather simple and quick: after taking off all adhesions two to four loops of the small gut are positioned so that they lie side by side. Beginning proximal fibrin [Tissucol fibrin sealant (Baxter)] is applied between the loops; approximately 20-30 s are needed to keep the loops in position until the fibrin dries. This manoeuvre is continued until all of the small gut is fixed in one block. The gut is brought back into the abdominal cavity without loosening the loops. This fixed formation by sero-serosal adhesions or mesenterial plications is supposed to guarantee postoperative free passage. The charts of 60 children who had undergone a fibrin plication of the small bowel between 1991 and 1999 were evaluated. Additionally, questionnaires were sent to all patients, and they were invited for an examination. RESULTS: Sixty patients (38 boys and 22 girls) received a fibrin sealant plication because of recurrent laparotomies with heavily serosal defects or recurrent ileus because of adhesions. The youngest baby was 10 days. Since 23 patients were premature the oldest patient was 11 years old. There were no intraoperative complications attributed to the method. In the postoperative period 7/60 (12%) patients had a recurrent ileus or subileus, leading in three (5%) patients to an early relaparotomy. CONCLUSION: The fibrin-glued plication of the small bowel decreases the risk of recurrent ileus or subileus considering the high figures in the literature concerning this issue. The time-saving method is very simple and easily feasible. No side effects after the treatment with fibrin glue were observed.

Child↗

Long-term results of Rehbein's procedure: a retrospective study in German-speaking countries.

AIM: In contrast to other countries, no collective study of Rehbein's procedure in German-speaking nations has been performed. Therefore, our intention was, analogously to Goto and Ikeda's (10) Japanese study in 1984, Kleinhaus's (13) study on Swenson's procedure in 1979, Bourdelat's (2) French-Canadian investigation into Duhamel's technique in 1997 and Martuciello's (11) and Teitelbaum's (16) follow-up in the year 2000, to perform a follow-up study of Rehbein's technique of deep anterior resection. METHODS: The data of 200 patients from 22 German-speaking centers in Switzerland, Austria and Germany were collected. These data were gathered by questionnaire and the children were followed up in the individual participating hospitals for at least 3.5 years after the procedure. The procedure was performed between 1993 and 1997, over a 5-year period. The questionnaire contained 74 items including anamnestic data, diagnostic postoperative treatment and reoperations. RESULTS: Concerning the incidence of anastomotic leaks and resolving anastomotic strictures there was no significant difference between the results in our series and those of the collective analyses made by Hofmann von Kap-herr (7), Holschneider (9) and Sherman (18). In 6.6 % of the 191 patients an anastomotic leak and in 9.9 % a rectal stricture, which had to be dilated, was observed. Concerning late complications, 22.8 % of the children suffered from constipation, 4.3 % from encopresis, 10.6 % from enterocolitis and only 0.5 % from enuresis. The frequency of constipation diminishes over the years. A comparison of the different large series in the literature clearly shows that the incidence of constipation is higher after Rehbein's procedure and the frequency of urinary incontinence and encopresis higher following Swenson's, Soave's and Duhamel's techniques. The incidence of enterocolitis is less after Rehbein's procedure than after Swenson's, Soave's and Duhamel's techniques. CONCLUSIONS: The different results in the literature are due to the individual experience of the author, the very different follow-up methods and the date of follow-up. Therefore, the different results are hard to compare with our study. Nevertheless, Rehbein's anterior resection still could be presented as an adequate and important method to treat Hirschsprung's disease.

Austria↗

How to approach the ENS: various ways to analyse motility disorders in situ and in vitro.

Motility disorders of the human intestine are so variable that they cannot be diagnosed by just one technique. Their aetiology is obviously so varied that they have to be approached with a broad range of technical methods. These reach from the simple haematoxylin-stained section to the isolation of stem or precursor cells. In this study, various methods to investigate the enteric nervous system and its surrounding tissue are demonstrated. While sections from paraffin-embedded material or cryostat sections provide only a two-dimensional perspective of the ENS, the whole-mount method yields three-dimensional perspectives of large areas of the gut wall. The three-dimensional impression can even be enhanced by electron microscopy of the isolated ENS. Dynamical aspects of ENS development can be tackled by in vitro studies. The myenteric plexus can be isolated and cultivated under the influence of the microenvironment (protein extracts). Although the postnatal myenteric plexus is not fully developed, the choice of embryological neuronal cells seems to be more effective for certain approaches. They can be isolated from the embryonic mouse gut and cultivated under the influence of various factors. This method seems to us a valuable tool for the investigation of the aetiology of motility disorders, although only a "complete" approach which considers all available methods will yield at the end a clear understanding which might lead to new therapeutical concepts.

Animals↗

What do knockout models teach us about the enteric nervous system?

Since the first histological studies, enormous strides have been made in understanding the genetics and cell biology of enteric nervous system (ENS) formation. Several mitogenic and trophic factors have been implicated in the process of neural cell proliferation and differentiation. A number of natural (piebald-lethal mice [s l], lethal spotting mice [ls], spotting lethal rats [sl]) or target (Gfralpha1-deficient mice, ret.k - mice, and NT-4 knockout mice) mutations have been reported to produce developmental defects in neural crest cell migration, differentiation or survival. Study of these mutations continues to provide new insights into this complex system. In the present investigation, we showed that a lack of basic fibroblast growth factor (FGF) or growth hormone (GH) leads to morphological abnormalities of the enteric nervous system. Because knockouts, neither of FGF nor of GH, produce enteric nervous system defects substantial enough to compromise the ability of the gut to support life, we postulate that FGF and GH affect only a relatively small subset of neurons and/or that compensatory effects of other growth factors might occur.

Animals↗

Molecular genetics of colorectal motility disorders.

In the last years, several genes have been identified which are involved in the development and differentiation of the enteric nervous system (ENS). Among the congenital intestinal innervation disorders described (aganglionosis, hypoganglionosis, heterotopic ganglia, intestinal neuronal dysplasia), up to now Hirschsprung's disease (HSCR) has been linked to mutational defects in these genes. GDNF and its co-receptor RET are the genes with the most mitogene potency on precursor cells of the ENS. The endothelin system (EDNRB/EDN3) also plays a key role in the development of the ENS by preventing its premature differentiation. Our own studies could show that, whereas a homozygous mutation of EDNRB causes long-segment HSCR, a heterozygous EDNRB deficiency leads to alterations of the ENS resembling the histopathology observed in intestinal neuronal dysplasia. Modern molecular genetic technologies combined with a subtle phenotypic assessment of the ENS will allow investigators to identify other genes within the complex signalling cascade required for the formation of the ENS. The recognition that intestinal innervation disorders are, at least in part, a multigenetic disease should provide support for consequent genetic screening in these patients.

Animals↗

[Indications and outcome of anal sphincter myectomy in childhood].

Anal sphinctermyectomy according to Lynn is an established therapy for chronic constipation in children. However, no consensus on indications exists and, according to the literature, results are contradictory. In order to clarify this problem, 33 children were examined and divided into two groups: I (n = 12) chronic constipation, and II (n = 21) dysganglionosis. All children were treated conservatively for at least a 6-month period and showed elevated anal resting tone in manometry. The operative results were assessed by anorectal manometry and a physical examination; subjective opinion was evaluated using a questionnaire. Anorectal manometry showed a marked decrease of anal resting tone of 30%, correlating to subjective relief of the symptoms.

Adolescent↗