Anorectal Malformation Classification

This multiauthor book is an update on the science and surgery of malformations of the rectum and anus. It carries on Douglas Stephens' book "Congenital Malformations of the Rectum, Anus, and Genito-uri-nary Tracts" published in 1963. This first book, which deals exclusively with malformations of the lower end of the digestive and urogenital tracts, was based on fundamental studies on paediatric pathology, surgery and surgical anatomy performed at the Department of Surgical Research of the Royal Children's Hospital, Melbourne, Victoria, Australia. Until today these studies have represented the embryological and path-oanatomical basis of our knowledge in the diagnosis and treatment of anorectal malformations (ARM). In 1971 Douglas Stephens and Durham Smith published the first update of their book, called "Ano-Rectal Malformations in Children". It became the standard work for ARM for the following 17 years. In 1984 an international workshop took place at the Wingspread Convention Center, Wisconsin, USA, hosted by the Department of Surgery, Chicago Children's Memorial Hospital, where Douglas Stephens worked at that time. The chief objects of that meeting were an update of the approximately 170 years of experience with modern treatment of ARM and to set standards for the classification and treatment of this malformation. At the end of the conference the so-called Wing-spread classification was settled, technical details for abdominal, sacral, and perineal approaches were proposed and the great variety of ARM listed again. The results of the Wingspread meeting were finally published by Stephens and Smith in 1988 with support of the March of Dimes Birth Defects Foundation at Alan R. Liss, New York [1]. The Wingspread classification of ARM divided ARM into high, intermediate, and low types and correlated the individual underlying pathoanatomy with the appropriate surgical procedures. This meant, roughly speaking, that a perineal approach should be performed for low-type, a sacral approach for intermediate-type, and an abdomino-sacro-perineal pullthrough for high-type malformations. These Wingspread considerations continue to have great influence on the diagnosis and therapy of ARM.

As time went on, however, new aspects were developed, particularly concerning the surgical therapy of children with imperforate anus. Special merit should be given to Alberto Peña, Cincinnati, USA, who described the sacral approach as the method of choice for almost all types of imperforate anus. Peña and de Vries described in 1982 the important details of the posterior sagittal anorectoplasty, which became the classic approach for the treatment of ARM in the subsequent years [2, 3]. This more simplified concept was based on the observation that the anatomical structures described by anatomists could hardly be identified during the operation. The different structures of the levator muscle, the puborectalis sling and the three slings of the external anal sphincter muscle could frequently only be realised as a muscle complex. According to the large experience of Alberto Peña with thousands of patients operated by himself in his former centre for ARM, the Jewish Hospital in Long Island, New York, USA, and throughout the whole world, a therapeutic concept based on anatomical observations seemed to be less important to him than a classification based on clinical experience.

Therefore, in 1990 Peña published an "Atlas of Surgical Management of Anorectal Malformations", describing in detail his new procedure, and in 1995 a clinical classification of ARM according to the type of the associated fistula. By closely comparing both proposals, the Wingspread classification and Peña's suggestions, it became clear that there was no real contradiction between them. Perineal and vestibular fistulas could be regarded as low malformations, bulbar fistulas, imperforate anus without a fistula and some of the vestibular fistulas may be regarded as intermediate-type anomalies, and prostatic and bladderneck fistulas are considered as high-type imperforate anus. However, it became evident that a new conference, 21 years after the Wingspread meeting, would help to clarify these problems. Therefore, an International Conference for the Development of Standards for the Treatment of Anorectal Malformations was organized at Krickenbeck Castle near Cologne, Germany (17-20 May 2005). This workshop brought together 26 international authorities on congenital malforma tions of the organs of the pelvis and perineum. Recent advances in aetiology and genetics, diagnosis, early and late management and methods of improvement of urorectal continence were reviewed. In addition, the participants developed a new international classification for ARM and a new grouping for follow-up assessment and standard surgical procedures. The principle idea of the Krickenbeck workshop and the subsequent international conference on 21 May 2005 in Cologne was to enhance the current fundamental concepts in the diagnosis and treatment of ARM, to update the recent knowledge on this not infrequent congenital malformation and to prepare this new update of Stephens and Smith's book from 1988 (Fig. 1).

The editors would like to thank Mrs. Gabriele Schröder and Mrs. Stephanie Benko, Springer International Publishers, for their interest and agreement to publish this book. We would also like to thank Mr. Janis Biermann, The March of Dimes Birth Defects Foundation and Alan R Liss, New York for giving us back all rights for publishing, tables, figures and chapters of the previous edition. We are especially pleased and honoured, that the former editors F. Douglas Stephens and E. Durham Smith attended the Krickenbeck Conference and helped with their advice and contributions to continue with their work. Special thanks go to Alberto Peña, who contributed tremendously to this book with many chapters written together with his associate Dr. Marc Levitt. Professor Peña's influence has changed fundamentally the concept of the former edition as he has changed the concept for the diagnosis and treatment of ARM. The Krickenbeck conference and this book are now building up a bridge between the important and still valid pathoanatomical considerations published by Stephens and the large clinical experience described by Peña. The anatomical aspects are supported by an unpublished series of autopsies performed by F. Douglas Stephens in children with imperforate anus who died from other reasons. His findings are presented in this book on a CD with a special index (Chap. 6). They confirm the clinical observations of Peña in a magnificent way. However, they also point out the necessity for an accurate anatomical knowledge of the individual deformity. The new classifications proposed at the Krickenbeck Conference are part of Chaps. 8 and 25. They have also been published by Holschneider et al. as a preliminary report [4]. The authors would like to thank Professor Jay Grosfield for his help for the quick and uncomplicated acceptance of this report.

We would also like to thank all of the co-authors who have contributed their time and effort to the research with or without the support of their parent universities, institutions, or hospitals; none will receive royalties on the sale of this book. Thanks are due to their supporting institutions, the names of which appear in the list of contributors.

Members of many disciplines in hospitals and universities have played important roles in the elucidation of the occult structural anomalies and the overall management of afflicted babies. In this context we are especially grateful to Professor J. Koepke, Head of the Anatomical Institute of the University of Cologne, Professor W. Lierse, former Head of the Institute for Neuroanatomy and Anatomy of the University of Hamburg, and Professor W. Meier-Ruge, Basel, Switzerland, for their support and advice in solving anatomical and pathological questions dealing with the pathophysiology of ARM.

Many other co-workers like physicians, nurses, radiologists, ancillary artists, photographers and hardworking secretaries in many countries have contributed their knowledge and expertise generously to the research, diagnosis, and management of ARM and the manuscript of this book. Mrs. Elisabeth Herschel at the Children's Hospital of the City of Cologne, Germany, and Mrs Shirley D'Cruz at the Royal Children's Hospital in Melbourne, Victoria, Australia, had exceptionally onerous work keeping track of correspondence and manuscripts and retyping, and we thank them for work well done.

Last but not least we would like to thank Dr. Winfried and Danielle Hartwick, Meerbusch, Germany and the Foerderverein Blankenheimer Dorf, Blankenheim, Germany for supported the idea of the Krickenbeck Conference financially. We are grateful to Mr. Thomas Gemein for good cooperation with the Verein der Freunde and Förderer des Kinderkrankenhauses Amsterdamer Strasse, Köln and the WestLB Akademie Schloss Krickenbeck, and Mrs. Svitlana Görden, Düsseldorf/Germany for the organisation of the Krickenbeck Conference.

All of the authors would like to thank all the parents' associations for children with ARM for their confidence and support of our daily work. We are especially grateful for the contribution of their experience and data to this book.

Alexander M. Holschneider, Köln John M. Hutson, Parkville April 2006

Puborectalis

Fig. 1 Group photograph of the participants at the International Conference for the Development of Standards for the Classification and Treatment of Anorectal Malformations, Krickenbeck, Germany, 17-20 May 2005. Upper row (left to right): Naomi Iwai, Kyoto, Japan; Guiseppe Martuciello, Pavia, Italy; Dieter Kluth, Hamburg, Germany; Uomas Boemers, Cologne, Germany; Keith Georgeson, Birmingham, Alabama; Alberto Peña, Cincinnati, USA; Alexander Holschneider, Cologne Germany; Devendra Gupta, New Delhi, India. Middle row(left to right): Sudipta Sen, Vellore, India; Middle row right: V.Sripathi, Chennai, India; Sabine Grasshoff, Germany; Feilim

Fig. 1 Group photograph of the participants at the International Conference for the Development of Standards for the Classification and Treatment of Anorectal Malformations, Krickenbeck, Germany, 17-20 May 2005. Upper row (left to right): Naomi Iwai, Kyoto, Japan; Guiseppe Martuciello, Pavia, Italy; Dieter Kluth, Hamburg, Germany; Uomas Boemers, Cologne, Germany; Keith Georgeson, Birmingham, Alabama; Alberto Peña, Cincinnati, USA; Alexander Holschneider, Cologne Germany; Devendra Gupta, New Delhi, India. Middle row(left to right): Sudipta Sen, Vellore, India; Middle row right: V.Sripathi, Chennai, India; Sabine Grasshoff, Germany; Feilim

Murphy, Dublin, Ireland. Lower row (left to right): Reinhold Engelskirchen, Düsseldorf, Germany; Risto Rintala, Helsinki, Finnland; Benno Ure, Hannover, Germany; Samuael Moore, Stellenbosch, South Africa; Michael Davies, Cape Town, South Africa; Arnold Coran, AnnArbor Michigan, USA; Durham Smith, Victoria, Australia; Douglas Stephens, Toorak, Australia; John Hutson, Melbourne, Australia; Subir Chatterchee, Calcutta, India; Jay Grosfeld, Indianapolis, USA; Yunus Sölet, Istanbul, Turkey; Elhamy Bekhit, Parkville, Australia. Photograph taken with permission from Holschneider et al. [4]

References

1. Stephens FD, Smith ED (1988) Anorectal Malformations in Children: Update 1988. Alan R. Liss, New York, and March of Dimes Birth Defects Foundation

2. DeVries P, Peña A (1982) Posterior Sagittal Anorectoplasty. J Pediatr Surg 17:638-643

3. Peña A, DeVries PA (1982) Posterior Sagittal Anorectoplasty: Important Technical Considerations and New Applications. J Pediatr Surg 17:796-811

4. Holschneider A, Hutson J, Peña A, Bekhit E, et al (2005) Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg 40:1521-1526

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