Thomas A. Angerpointner – författare

Wir berichten tiber 185 Kinder mit unterschiedlichen Erscheinungen, die her- kommlich als Riesenhamartom, Angiodysplasie, kongenitale Trophoedem, ort- licher Gigantismus (z. B. Macrodaktylie) usw. gekennzeichnet werden. Wir schla- gen vor, aIle diese Erscheinungen als eine Entitat zu verstehen: kongenitale Weich- teildysplasie (congenital soft-tissue dysplasia, CSTD. Nach jtingsten embryologischen und zellbiologischen Untersuchungen tritt die CSTD als Folge embryonaler oder fetaler biosynthetischer Zellfehlregulation auf. Das Konzept einer CSTD-Entitat fiihrt sowohl zu einem gemeinsamen Unter- suchungsprotokoll als auch zu einem gemeinsamen therapeutischen Vorgehen un- ter besonderer Berticksichtigung der "Stabilitat" und der Gutartigkeit des Zu- standes. Die Behandlung sollte auf die funktionelle Verbesserung beschrankt bleiben, anstatt die Korrektur kosmetischer Defekte zu versuchen. References 1. Andre JM (1973) Les dysplasies vasculaires systematisees, vol 1. L'expansion scientifique, Paris 2. Andre JM, Jacquier A, Picard L (1977) La neurofibromatose de Recklinghausen. Pathogenie - Conception actuelle. Ann Chir Thor Cardiovasc 16: 175-185 3.Andrews EJ, Ward BC, Altman NH (1979) Spontaneous animal models of human diseases, vols 1 and 2. Academic, New York 4. Azouz EM (1983) Hematuria, rectal-bleeding and pelvic phleboliths in children with Klippel- Trenaunay syndrome. Pediatr Radiol 13: 82-88 5. Barksy AJ (1967) Macrodactyly. J Bone Joint Surg 49A(7): 1255-1266 6. Berliner DL, Ruhmann AG (1966) Comparison of growth of fibroblasts under the influence of II-beta-hydroxy and ll-keto-corticosteroids. Endocrinology 78: 373 7. Chassaignac C (1959) Hypertrophie congenitale des 2 membres droits. Bull Soc Coo Paris 8.

The clinical application of anorectal manometry was pioneered by pediatric sur- geons. Swenson in 1949 reported that the aganglionic bowel of patients with Hirsch- sprung's disease did not show any propulsive movements. In 1967 Lawson and Nixon and Schnaufer et al. found that the rectosphincteric reflex, which is ob- served in both normal individuals and patients with chronic idiopathic constipa- tion, is absent in patients with Hirschsprung's disease, and suggested anorectal manometry to be useful for the differentiation of Hirschsprung's disease from chronic idiopathic constipation. Studies on the pathophysiology of constipation and incontinence problems have flourished in Japan; the Japanese Research Society for Anorectal Manometry in Childhood was founded in 1975 by Ikeda, Okamoto, Katsumata, and Kasai. The Society has contributed greatly to advances in the clinical application of anorectal manometry. Basic investigations on the function of the anorectum in both normal individuals and patients with constipation or incontinence problems have also been promoted by members of the Society. The technique of anorectal manometry has been refined and standardized.The criteria for manometric diag- nosis of Hirschsprung's disease have been established. And the correlation be- tween postoperative continence in patients with Hirschsprung's disease or ano- rectal malformation and manometric findings has been understood. In this section, works by members of the Society are presented.

In conventional partial resection of parenchymatous organs significant bleeding is one of the main problems, especially in neonates and infants. The patients rapidly lose considerable amounts of their small blood volume. Laser light leads to photo­ thermal effects in tissue and causes coagulation, drying up, carbonization and evaporization, depending on the temperature. The neodymium Y AG laser emits nonvisible light in the near infrared with a wavelength of l. 0611m. This wavelength implies a relatively deep penetration into the tissue. This laser system, properly a coagulation laser, achieves its cutting effect by its high power density [9]. Because of thermal radiation in all directions, both sides of the section plane are coagulated as a positive side effect. Thus, in parenchymatous organs a combination of resection and sealing of the cut vessels and ducts, up to a limited diameter, is obtained. Laser Instruments We use a neodymium-YAG laser mediLas 2 (MBB-Medizintechnik, D-8012 Ottobrunn, Federal Republic of Germany), wavelength l. 0611m, maximal power output around 110 W (Fig. 1). Normally we prefer to work without tissue contact, Fig. l. The Nd-YAG laser system mediLas 2 with maximal power output around 100 W The Neodymium Y AG Laser in Surgery of Parenchymatous Organs 25 Fig. 2. The focusing handpiece with focal distance of 50 mm 1cm Fig. 3.

In conventional partial resection of parenchymatous organs significant bleeding is one of the main problems, especially in neonates and infants. The patients rapidly lose considerable amounts of their small blood volume. Laser light leads to photo­ thermal effects in tissue and causes coagulation, drying up, carbonization and evaporization, depending on the temperature. The neodymium Y AG laser emits nonvisible light in the near infrared with a wavelength of l. 0611m. This wavelength implies a relatively deep penetration into the tissue. This laser system, properly a coagulation laser, achieves its cutting effect by its high power density [9]. Because of thermal radiation in all directions, both sides of the section plane are coagulated as a positive side effect. Thus, in parenchymatous organs a combination of resection and sealing of the cut vessels and ducts, up to a limited diameter, is obtained. Laser Instruments We use a neodymium-YAG laser mediLas 2 (MBB-Medizintechnik, D-8012 Ottobrunn, Federal Republic of Germany), wavelength l. 0611m, maximal power output around 110 W (Fig. 1). Normally we prefer to work without tissue contact, Fig. l. The Nd-YAG laser system mediLas 2 with maximal power output around 100 W The Neodymium Y AG Laser in Surgery of Parenchymatous Organs 25 Fig. 2. The focusing handpiece with focal distance of 50 mm 1cm Fig. 3.

The latest volume in a series for specialists in pediatric surgery.

The latest volume in a series for specialists in pediatric surgery.

Endocrine conditions requiring surgical intervention in the pediatric age­ group are uncommon. When diagnosed, they are the source of great in­ terest and, often, considerable debate. This is understandable, since few centers and even fewer individual surgeons can draw on vast experience of this subject. The great divergence of opinion regarding management is also understandable in that pediatric endocrine lesions often differ considerably from their adult counterparts in histology, natural history and response to treatment. Pediatric endocrine lesions are also, as a rule, less frequently malignant. In addition to the great strides made in surgical and anesthetic technique and operative monitoring, progress in four areas has substantially advanced the management of endocrine dis­ orders in the pediatric age group in the last decade: imaging, pathology, pharmacology and genetics. The new imaging tools, ultrasonography, computed tomography and magnetic resonance imaging, have added great diagnostic possibilities. More recent developments, such as radio nuclide imaging for the adrenal gland and the possibility of using tagged antibodies, promise to expand our imaging horizons even further. In the field of pathology, the develop­ ment of immunocytochemical markers (e. g. , monoclonal antibodies), the refinement in special stains and the continuous perfection of fine needle aspiration biopsies offer great new diagnostic as well as research capabil­ ities. Newer pharmacological agents, such as the alpha and beta blockers, the calcium channel blockers and thyroxine analogs, add a whole new level of safety to the management of the potentially lethal pheochromocytoma.