SPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J.R. SiewertSPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J.R. SiewertP. Puri · M. E.H?llwarth (Eds.)

    Pediatric Surgery

    With 589 Color Figures,in 666 separate IllustrationsPrem Puri MS, FRCS, FRCS (Ed), FACS

    Newman Clinical Research Professor,University College,Dublin

    Consultant Paediatric Surgeon and

    Director of Research Children’s Research Centre

    Our Lady’s Hospital for Sick Children

    Crumlin

    Dublin 12, Ireland

    Michael E.H?llwarth MD

    Professor Head

    Department of Paediatric Surgery

    Medical University of Graz

    Auenbruggerplatz

    8036 Graz

    Austria

    ISBN-10 3-540-40738-3

    Springer-Verlag Berlin Heidelberg New York

    ISBN-13 978-3-540-40738-6

    Springer-Verlag Berlin Heidelberg New York

    Library of Congress Control Number: 2004104708

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    Editor: Gabriele Schr?der, Heidelberg, Germany

    Desk Editor: Stephanie Benko, Heidelberg, Germany

    Wissenschaftliche Zeichnungen: Reinhold Henkel, Heidelberg

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    Printed on acid-free paper 213151ML 5 4 3 2 1 0Preface

    During the past two decades major advances in prenatal

    diagnosis, imaging, resuscitation, intensive care, mini-

    mally invasive surgery and operative techniques have

    radically altered the management of infants and chil-

    dren with surgical conditions.There are now several ex-

    cellent paediatric surgery texts available which focus on

    the historical background, embryogenesis, pathophy-

    siology, diagnosis and management of childhood surgi-

    cal disorders. The main aim of this new textbook on

    paediatric surgery was to provide a comprehensive de-

    scription of operative techniques for various conditions

    in children. The book contains contributions by out-

    standing and well-known paediatric surgeons and

    paediatric urologists from five continents.Each contrib-

    utor was selected to provide an authoritative, compre-

    hensive and complete account of their respective topic.

    The text is organised in a systematic manner, providing

    step-by-step, detailed practical advice on the operative

    approach in the management of congenital and ac-

    quired conditions in infants and children. The book is

    intended for trainees in paediatric surgery, established

    paediatric surgeons, paediatric urologists and general

    surgeons with an interest in paediatric surgery. It is our

    sincere hope that the readers will find this volume a use-

    ful reference in the operative management of childhood

    surgical disorders.

    We wish to thank all the contributors most sincerely

    for their outstanding work in producing this innovative

    textbook. We are indebted to Reinhold Henkel for his

    excellent artwork.We wish to express our gratitude to

    Karen Alfred, Louise McCrossan (Dublin) and Gudrun

    Raber (Graz) for their skilful secretarial help. Finally we

    wish to thank the editorial staff of Springer, particular-

    ly Gabriele Schroeder,who has been behind each step of

    this book, from its original concept to its delivery.

    Prem Puri

    Michael H?llwarthSPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J.R. SiewertPART I HEAD and NECK

    Chapter 1 Thyroglossal Duct Cyst . . 3

    Michael E.H?llwarth

    Chapter 2 Branchial Cysts and Sinus . . 7

    Michael E.H?llwarth

    Chapter 3 Cystic Hygroma . . . 13

    Baird M. Smith, Craig T.Albanese

    Chapter 4 Tracheostomy . . . 19

    Thom E. Lobe

    PART II OESOPHAGUS

    Chapter 5 Oesophageal Atresia . . 29

    Michael E.H?llwarth, Paola Zaupa

    Chapter 6 Gastro-oesophageal Reflux

    and Hiatus Hernia . . 49

    Keith E.Georgeson

    Chapter 7 Achalasia . . . 61

    Paul K.H. Tam

    Chapter 8 Colonic Replacement

    of the Oesophagus . . 67

    Alaa Hamza

    Chapter 9 Gastric Transposition

    for Oesophageal Replacement .77

    Lewis Spitz

    PART III CHEST

    Chapter 10 Thoracoscopy . . . 89

    Klaas Bax

    Chapter 11 Repair of Pectus Excavatum . . 97

    Robert C. Shamberger

    Chapter 12 Pulmonary Malformations . . 107

    Brian T. Sweeney,Keith T.Oldham

    Chapter 13 Congenital Diaphragmatic Hernia

    and Eventration . . 115

    Prem Puri

    Contents

    Chapter 14 Extracorporeal Membrane

    Oxygenation . . . 125

    Jason S. Frischer,Charles J.H. Stolar

    PART IV ABDOMEN

    Chapter 15 Hernias – Inguinal,Umbilical,Epigastric, Femoral

    and Hydrocele . . . 139

    Juan A. Tovar

    Chapter 16 Omphalocele . . . 153

    Stig Somme, Jacob C. Langer

    Chapter 17 Gastroschisis . . . 161

    Marshall Z. Schwartz

    Chapter 18 Hypertrophic Pyloric Stenosis . 171

    Takao Fujimoto

    Chapter 19 Gastrostomy . . . 181

    Michael W.L.Gauderer

    Chapter 20 Malrotation . . . 197

    Agostino Pierro, Evelyn GP Ong

    Chapter 21 Duodenal Obstruction . . 203

    Yechiel Sweed

    Chapter 22 Jejuno-ileal Atresia . . 213

    Heinz Rode,Alastair J.W.Millar

    Chapter 23 Meconium Ileus . . 229

    Massimo Rivosecchi

    Chapter 24 Gastrointestinal Duplications . 239

    Mark D. Stringer

    Chapter 25 Short Bowel Syndrome . . 257

    Michael E.H?llwarth

    Chapter 26 Hirschsprung’s Disease . . 275

    Prem Puri

    Chapter 27 Anorectal Anomalies . . 289

    Alberto Pe?a,Marc A. Levitt

    Chapter 28 Intussusception . . 313

    Karl-Ludwig Waag

    Chapter 29 Appendectomy . . . 321

    Vincenzo JasonniContents

    VIII

    Chapter 30 Omphalomesenteric

    Duct Remnants . . . 327

    David Lloyd

    Chapter 31 Ulcerative Colitis . . 333

    Risto J. Rintala

    Chapter 32 Crohn’s Disease . . . 347

    Risto J. Rintala

    PART V LIVER,PANCREAS AND SPLEEN

    Chapter 33 Biliary Atresia . . . 357

    Ryoji Ohi,Masaki Nio

    Chapter 34 Choledochal Cyst . . 371

    Takeshi Miyano,Masahiko Urao,Atsuyuki Yamataka

    Chapter 35 Cholecystectomy . . 387

    Thom E. Lobe

    Chapter 36 Surgery for Persistent

    Hyperinsulinaemic

    Hypoglycaemia of Infancy . . 395

    Lewis Spitz

    Chapter 37 Splenectomy . . . 403

    Peter Borzi

    PART VI SPINA BIFIDA

    AND HYDROCEPHALUS

    Chapter 38 Spina Bifida . . . 413

    Martin T. Corbally

    Chapter 39 Hydrocephalus . . . 419

    Kai Arnell, Leif Olsen,Tomas Wester

    Chapter 40 Dermal Sinus . . . 427

    Andrew B. Pinter

    PART VII TUMOURS

    Chapter 41 Sacrococcygeal Teratoma . . 435

    Kevin C. Pringle

    Chapter 42 Neuroblastoma . . . 443

    Edward Kiely

    Chapter 43 Wilms Tumour . . . 451

    Robert Carachi

    Chapter 44 Liver Tumours ?.?.?.?.?.?.?.?.?.?. 459

    Wendy T. Su,Michael P. La Quaglia

    Chapter 45 Testicular Tumours ?.?.?.?.?.?.?. 477

    Jonathan Ross

    PART VIII UROLOGY

    Chapter 46 Pyeloplasty ?.?.?.?.?.?.?.?.?.?.?. 485

    Boris Chertin, Prem Puri

    Chapter 47 Endoscopic Treatment

    of Vesicoureteral Reflux ?.?.?.?. 493

    Prem Puri

    Chapter 48 Vesicoureteral Reflux –

    Surgical Treatment ?.?.?.?.?.?.?. 499

    Jack S. Elder

    Chapter 49 Ureteric Duplication ?.?.?.?.?.?. 515

    Claude C. Schulman

    Chapter 50 Posterior Urethral Valves ?.?.?.?. 523

    Chester J.Koh,David A.Diamond

    Chapter 51 Hypospadias ?.?.?.?.?.?.?.?.?.?. 529

    Pierre Mouriquand,Pierre-Yves Mure

    Chapter 52 Phimosis and Buried Penis ?.?.?. 543

    Peter Cuckow

    Chapter 53 Orchidopexy ?.?.?.?.?.?.?.?.?.?. 555

    John M.Hutson

    Chapter 54 Variocele ?.?.?.?.?.?.?.?.?.?.?.?. 569

    Michael E.H?llwarth

    Chapter 55 Genitoplasty for Congenital

    Adrenal Hyperplasia ?.?.?.?.?.?. 577

    Amicur Farkas

    Chapter 56 Bladder Exstrophy and Epispadias 589

    Dominic Frimberger,John P.Gearhart

    Chapter 57 Cloacal Exstrophy?.?.?.?.?.?.?.?. 607

    Duncan Wilcox,Manoj Shenoy

    Chapter 58 Augmentation Cystoplasty

    and Appendicovesicostomy

    (Mitrofanoff Principle) ?.?.?.?.?. 613

    Boris Chertin

    Chapter 59 The ACE (Antegrade Continence

    Enema) Procedure ?.?.?.?.?.?.?. 623List of Contributors

    Craig T Albanese MD

    Professor of Surgery

    Chief, Division of Pediatric Surgery

    Stanford University Medical Center

    Palo Alto, Calfornia

    USA

    Kai Arnell MD

    Department of Paediatric Surgery

    University Children’s Hospital

    SE-751 85 Uppsala

    Sweden

    Klass MA Bax MD, PhD, FRCS (Ed)

    Professor of Pediatric Surgery

    Wilhelmina Children’s Hospital

    University Medical Center Utrecht

    PO Box 85090, 3508 AB Utrecht

    The Netherlands

    Peter Borzi MB, BS, FRACS, FRCS

    Paediatric Surgery Paediatric Urology

    Taylor Medical Centre

    40 Annerley Road

    Woolloongabba 4102

    Australia

    Robert Carachi MD, FRCS

    Professor of Paediatric Surgery

    Head of Department

    Department of Surgical Paediatrics

    Royal Hospital for Sick Children

    Yorkhill, Glasgow G2 8SJ

    UK

    Boris Chertin MD

    Consultant Pediatric Urologist

    Department of Urology

    Shaare Zedek Medical Center

    Jerusalem, Israel, 91031

    Martin T Corbally MCh, FRCSI, FRCS

    Consultant Paediatric Surgeon

    Our Lady’s Hospital for Sick Children

    Crumlin

    Dublin 12

    Ireland

    Peter M Cuckow FRCS

    Consultant Paediatric Urologist

    Great Ormond Street Hospital for Sick Children

    30 Guilford Street

    London WC1N 1EH

    UK

    David A Diamond MD

    Associate Professor of Surgery (Urology)

    Children’s Hospital Boston

    and Harvard Medical School

    300 Longwood Avenue, Hunnewell 3

    Boston,MA 02115

    USA

    Jack S Elder MD

    Director

    Division of Pediatric Urology

    Rainbow Babies Children’s Hospital

    11100 Euclid Avenue

    Cleveland, OH 44106

    USA

    Amicur Farkus MD

    Professor and Head

    Department of Urology

    Shaare Zedek Medical Center

    Jerusalem, Israel 91031

    Dominic Frimberger MD

    Johns Hopkins Hospital

    Urology,Marburg 149

    600N Wolfe St

    Baltimore,MD 21287

    USAList of Contributors

    X

    Takao Fujimoto MD, PhD

    Director of Pediatric Surgery

    Imperial Gift Foundation

    The Aiiku Maternal Children’s Medical Centre

    5-6-8 Minami-Azabu,Minato-Ku

    Tokyo 106-8580

    Japan

    Michael W L Gauderer MD, FACS, FAAP

    Professor, Department of Pediatric Surgery

    Children’s Hospital

    Memorial Medical Office Building, Suite 440

    890 West Fans Road

    Greenville, South Carolina 29605-4253

    USA

    John P Gearhart MD

    Professor Director

    Division of Pediatric Urology

    James Buchanan Brady Urological Institute

    Johns Hopkins Hospital

    Baltimore,Maryland

    USA

    Keith E Georgeson MD

    Professor and Director

    Division of Pediatric Surgery

    Children’s Hospital and Alabama

    1600 Seventh Avenue South

    Birmingham,Alabama 35233

    USA

    Alaa F Hamza MD, FRCS

    Consultant Paediatric Surgeon

    45 Ramsis Street

    11341 Heliopolis

    Cairo

    Egypt

    Michael E H?llwarth MD

    Professor Head

    Department of Paediatric Surgery

    Medical University of Graz

    Auenbruggerplatz

    A-8036 Graz

    Austria

    John M Hutson BS,MD(Monash),MD(Melb), FRACS

    Professor Director

    General Surgery

    Royal Children’s Hospital

    Parkville,Victoria 3052

    Australia

    Vincenzo Jasonni MD

    Professor and Director

    Universita degli Studi di Genova

    Largo Gerolamo Gaslini 5

    16147 Genova

    Italy

    Edward Kiely FRCSI, FRCS, FRCPCH

    Consultant Paediatric Surgeon

    234 Great Portland Street

    London W1W 5QT

    UK

    Chester J Koh MD

    Fellow in Pediatric Urology

    Children’s Hospital Boston

    and Harvard Medical School

    300 Longwood Avenue, Hunnewell 3

    Boston,MA 02115

    USA

    Jacob C Langer MD

    Professor, Chief of Paediatric General Surgery

    Hospital for Sick Children

    Rm 1526, 555 University Ave

    Toronto, ON M5G 1X8

    Canada

    Michael P La Quaglia MD

    Department of Surgery

    Memorial Sloan-Kettering Cancer Center

    1275 York Ave.

    New York, NY 10021

    USA

    Marc A Levitt MD

    Assistant Professor of Surgery and Pediatrics

    Schneider Children’s Hospital

    North Shore-Long Island Jewish Health System

    269-01 76th Avenue

    New Hyde Park, NY 11040

    USA

    David A Lloyd Mchir, FRCS, FCS(SA)

    Professor of Paediatric Surgery

    15 Eshe Road North

    Blundellsands

    Liverpool L23 8UE

    UK

    Thom E Lobe MD

    Chairman, Section of Pediatric Surgery

    Blank Childrens Hospital

    Des Moines

    Iowa

    USAList of Contributors

    XI

    Padraig S J Malone MCh, FRCSI, FRCS

    Consultant Paediatric Urologist

    Department of Paediatric Urology

    Southampton University Hospitals NHS Trust

    Tremona Road

    Southampton S016 6YD

    Hampshire, UK

    Alastair J W Millar FRCS(Eng) (Edin), FRACS,DCH

    Consultant Paediatric Surgeon

    Department of Paediatric Surgery

    Birmingham Childres Hospital

    Birmingham

    UK

    Takeshi Miyano MD, PhD, FAAP(Hon), FACS,FAPSA (Hon)

    Professor and Head

    Department of Pediatric Surgery

    Juntendo University School of Medicine

    2-1-1 Hongo, Bunkyo-ku

    Tokyo 113-8421

    Japan

    Pierre Mouriquand MD, FRCS(Eng), FEBU

    Professor, Service d’Urologie Pediatrique

    Hopital Debrousse

    29, rue Soeur Bouvier

    69322 Lyon Cedex 05

    France

    Pierre-Yves Mure

    Service d’Urologie Pediatrique

    Hopital Debrousse

    29, rue Soeur Bouvier

    69322 Lyon Cedes 05

    France

    Masaki Nio MD

    Department of Pediatric Surgery

    Tohoku University School of Medicine

    Sendai, 980

    Japan

    Ryoji Ohi MD

    Professor, Department of Pediatric Surgery

    Tohoku University School of Medicine

    Sendai, 980

    Japan

    Keith Oldham MD

    Division of Pediatric Surgery

    Medical College of Wisconsin

    Children’s Hospital Office Building

    9000 West Wisconsin Av

    Milwaukee,Wisconsin 53201

    USA

    Leif Olsen MD, PhD

    Department of Paediatric Surgery

    University Children’s Hospital

    SE-751 85 Uppsala

    Sweden

    Evelyn G P Ong MBBS, BSc, FRCS (Eng)

    Clinical Research Fellow

    Paediatric Surgery Unit

    Institute of Child Health Great Ormond Street

    Hospital for Children

    30 Guilford Street

    London WC1N 1EH

    UK

    Alberto Pena MD

    Cincinnati Children’s Hospital Medical Center

    Cincinnati

    USA

    Agostino Pierro MD, FRCS (Eng), FRCS (Ed), FAAP

    Professor, Department of Paediatric Surgery

    Institute of Child Health Great Ormond Street

    Hospital for Children

    30 Guilford Street

    London WC1N 1EH

    UK

    Andrew B Pinter

    Professor of Paediatric Surgery

    Department of PaediatricsSurgical Unit

    Jozsef A. u. 7., 7623

    Pecs

    Hungary

    Kevin C Pringle MB, ChB, FRACS

    OG Health of Department

    Capital Coast Health

    Private Bag 8902

    Riddiford Street

    We l l ing ton

    South, New ZealandList of Contributors

    XII

    Prem Puri MS, FRCS, FRCS (Ed), FACS

    Consultant Paediatric Surgeon

    Professor Director of Research

    Children’s Research Centre

    Our Lady’s Hospital for Sick Children

    Crumlin

    Dublin 12, Ireland

    Risto J Rintala MD

    Professor, Department of Paediatric Surgery

    Hospital for Children and Adolescents

    University of Helskinki

    PO Box 281

    Fin-00029 Hus

    Finland

    Massimo Rivosecchi MD

    Professor, Department of Pediatric Surgery

    Bambino Gesu’ Children’s Hospital

    Palidoro

    Rome

    Italy

    Heinz Rode Mmed(Chir), FC(SA), FRCSEd

    Professor of Paediatric Surgery

    Red Cross Children’s Hospital

    Rondebosch 7700

    South Africa

    Jonathan Ross MD

    Head, Section of Pediatric Urology

    Glickman Urological Institute

    Cleveland Clinic Children’s Hospital

    9500 Euclid Avenue

    Cleveland, OH 44195

    USA

    Claude C Schulman MD, PhD

    Professor

    Hospital Erasure

    Route de Lennik 808

    1070 Bruxelles

    Belgium

    Marshall Z Schwartz MD

    St. Christopher’s Hospital for Children

    Department of Surgery

    Erie Avenue at Front Street

    Philadelphia, PA 19134

    USA

    Robert C Shamberger MD

    Department of Surgery

    Children’s Hospital Boston

    300 Longwood Avenue

    Boston,Massachusetts 02115

    USA

    Manoj Shenoy FRCS

    Consultant Paediatric Urologist

    City Hospital

    Nottingham

    UK

    Baird M Smith MD

    Assistant Professor of Surgery

    Division of Pediatric Surgery

    Stanford University

    Palo Alto, California

    USA

    Stig Somme MD

    Research Fellow

    Department of Surgery

    Hospital for Sick Children

    555 University Avenue

    Toronto, ON M5G 1X8

    Canada

    Lewis Spitz MB, ChB, PhD,MD(Hon), FRCS(Edin),FRCS(Eng)

    Nuffield Professor of Paediatric Surgery

    Institute of Child Health

    30 Guilford Street

    London WC1N 1EH

    UK

    Charles J H Stolar MD

    Children’s Hospital of New York

    3959 Broadway, 202N

    New York, NY 10032

    USA

    Mark D Stringer BSc,MS, FRCS FRCS(Paed), FRCP,FRCPCH

    Consultant Paediatric Surgeon

    Children’s Liver GI Unit

    Gledhow Wing

    St James’s University Hospital

    Leeds LS9 7TF

    UKList of Contributors

    XIII

    Wendy T Su MD

    Department of Surgery

    Memorial Sloan-Kettering Cancer Center

    1275 York Av.

    New York, NY 10021

    USA

    Yechiel Sweed MD

    Head, Paediatric Surgery

    Western Galilee Hospital

    Nahariya

    Israel 2122100

    Brian T Sweeney MD

    Pediatric Surgery Fellow

    Division of Pediatric Surgery

    Medical College of Wisconsin

    9000 W.Wisconsin Ave.

    Milwaukee,WI 53226

    USA

    Paul Tam MD FRCS

    Professor, Division of Paediatric Surgery

    University of Hong Kong

    Medical Centre

    Queen Mary’s Hospital

    Pokfulam Road

    Hong Kong

    Juan A Tovar MD

    Professor, Department of Pediatric

    Surgery Hospital Universitario “La Paz”

    Paseo de la Castellana 261

    28046 Madrid

    Spain

    Masahiko Urao MD, PhD

    Department of Pediatric Surgery

    Juntendo University, School of Medicine

    2-1-1 Hongo, Gunkyo-ku

    Tokyo 113-8421

    Japan

    Karl-Ludwig Waag MD

    Professor, Department of Paediatric Surgery

    MannheimHeidelberg

    Im Neuenheimer Feld 110

    D-69120 Heidelberg

    Germany

    Tomas Wester MD, PhD

    Department of Paediatric Surgery

    University Children’s Hospital

    SE-751 85 Uppsala

    Sweden

    Duncan Wilcox MD, FRCS (Paed)

    Associate Professor

    Department of Urology

    University of Texas

    South Western Medical Center

    Dallas, Texas

    USA

    Atsuyuki Yamataka MD

    Department of Pediatric Surgery

    Juntendo University School of Medicine

    2-1-1 Hongo, Bunkyo-ku

    Tokyo 113-8421

    Japan

    Paola Zaupa MD

    Department of Paediatric Surgery

    Medical University Graz

    Auenbruggerplatz 34

    A-8036 Graz

    AustriaPart I

    Head and NeckSPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J.R. SiewertCHAPTER 1

    INTRODUCTION

    Thyroglossal Duct Cyst

    The median cervical cyst is a remnant of the thyro-

    glossus duct, which runs from the pyramidal lobe of

    the thyroid gland to the foramen caecum in the dor-

    sal part of the tongue. Embryologically, the thyroid

    diverticulum develops in a caudal direction from the

    foramen caecum after formation of the tongue. The

    thyroid gland descends to the neck in the same peri-

    od of gestation as the hyoid bone develops from the

    second branchial arch. The thyroglossal duct may

    pass in front, behind or through the body of the

    hyoid bone in the middle of the neck, and islands of

    thyroid tissue may be found scattered along the tract.

    At no time during embryogenesis does the thyroglos-

    sal duct contact the body surface; the original cysts

    thus never open to the skin.A fistula can only devel-

    op secondarily, e.g., following spontaneous perfora-

    tion or surgical incision of an infected cyst.

    Thyroglossal cysts are the most common tumours

    of the anterior cervical region. They are usually lo-

    cated in the midline at the level of or somewhat be-

    low the hyoid bone. Due to the connection with the

    foramen caecum of the tongue, the lesion typically

    moves upwards with swallowing like the thyroid

    gland, and, different from the latter, also with tongue

    protrusion. In contrast, dermoid cysts or lymph

    nodes do not change their position with either act.

    Ultrasound examination may be helpful, in the first

    instance to ascertain the presence of a normally situ-

    ated normally sized thyroid gland as well as to con-

    firm the cystic nature of the mass under considera-

    tion. In cases of a suppurative infection, incision and

    drainage in combination with antibiotics is the ap-

    propriate treatment followed by excision once the

    acute inflammation has settled.

    Michael E.H?llwarthMichael E.H?llwarth

    4

    1

    Following induction of general anaesthesia with en-

    dotracheal intubation, the neck is hyperextended by

    placing a sandbag or towel roll beneath the shoul-

    ders.A horizontal skin incision is made over the cyst.

    In case of a fistula, the cutaneous orifice is circum-

    cised in a horizontally oriented elliptical fashion.

    Subcutaneous tissue,platysma and cervical fascia are

    divided exposing the capsule of the cyst. In cases

    with previous history of inflammation, these layers

    may be fibrosed and lack a clear demarcation against

    each other as well as against the cyst wall. The cyst is

    carefully separated from the surrounding tissue by

    blunt and sharp dissection.

    Figure 1.1

    The duct is attached to the cyst running in a cephalad

    direction between the sternohyoid muscles to the

    body of the hyoid bone. It is usually not possible to

    recognize whether the duct perforates the hyoid

    body or passes across its anterior or posterior sur-

    face. The central part of the hyoid bone is freed from

    the muscles attached to its upper and lower margin.

    The thyrohyoid membrane is carefully dissected off

    the posterior aspect with scissors.

    Figure 1.2

    The exposed hyoid bone is then stabilized with

    strong Kocher forceps on one side, clearly lateral to

    the median line, and the central segment is excised

    with strong Mayo scissors.

    Figure 1.3

    If the duct is extending beyond on the posterior as-

    pect of the hyoid bone, it is followed cephalad and

    divided close to the base of the tongue with a 50 ab-

    sorbable transfixation ligature. If the floor of the

    mouth is entered accidentally, the mucosa of the

    tongue is closed with interrupted plain absorbable

    sutures.Often, however, no duct structures are found

    behind the hyoid bone, in which case some of the

    midline connective tissue is excised in the cranial di-

    rection to make sure that no duct epithelium is left

    behind.

    The lateral segments of the hyoid bone are left

    separated, but the anterior neck muscles are approx-

    imated in the midline with absorbable 40 sutures.

    Platysma and subcutaneous fat are closed with ab-

    sorbable 50 sutures, and the skin is closed either

    with interrupted subcuticular absorbable 60 stitch-

    es or with a continuous subcuticular nonabsorbable

    40 suture, which can be removed 3–4 days later. A

    drain is usually not necessary, except in cases requir-

    ing extensive dissection as may occur after a previ-

    ously infected cyst or a recurrent cyst.

    Figure 1.4Chapter 1 Thyroglossal Duct Cyst

    5

    Figure 1.1 Figure 1.2

    Figure 1.3 Figure 1.4Michael E.H?llwarth

    6

    CONCLUSION 1

    Complete excision of the thyroglossal cyst consists of

    removal of the cyst, the entire tract and the midpor-

    tion of the hyoid bone through which the tract pass-

    es. If this principle is followed, recurrence is extreme-

    ly unlikely.While the procedure is easily performed

    in native tissue, dissection is much more difficult in a

    previously infected cyst. Therefore, postponement of

    the surgical procedure is not to be recommended

    once the diagnosis has been made.

    SELECTED BIBLIOGRAPHY

    Horisawa M,Niiomi N, Ito T (1991) Anatomical reconstruction

    of the thyroglossal duct. J Pediatr Surg 26 : 766–769

    Smith CD (1998) Cysts and sinuses of the neck. In: O’Neill JA,Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG (eds) Pe-

    diatric surgery.Mosby, St Louis, pp 757–772

    Telander RL, Deane S (1977) Thyroglossal and branchial cleft

    cysts and sinuses. Surg Clin North Am 57 : 779–791

    Waldhausen JHT, Tapper D (2000) Head and neck sinuses and

    masses. In: Ashcraft KW (ed) Pediatric surgery. WB

    Saunders, Philadelphia, pp 987–999CHAPTER 2

    INTRODUCTION

    Branchial Cysts and Sinus

    During the fourth to eighth week of gestation, four

    pairs of branchial arches and their intervening clefts

    and pouches are formed. Congenital branchial cysts

    and sinus are remnants of these embryonic struc-

    tures that have failed to regress completely. Treat-

    ment of branchial remnants requires knowledge of

    the related embryology. The first arch, cleft and

    pouch form the mandible, the maxillary process of

    the upper jaw, the external ear, parts of the Eusta-

    chian tube, and the tympanic cavity. Anomalies of

    the first branchial pouch are rare. Sinuses typically

    have their external orifice inferior to the ramus of the

    mandible. They may traverse the parotid gland, and

    run in close vicinity to the facial nerve in the external

    auditory canal. Cysts are located anterior or posteri-

    or to the ear or in the submandibular region. They

    have to be distinguished from the preauricular cysts

    and sinuses,which are ectodermal remnants from an

    aberrant development of the auditory tubercles, tend

    to be bilateral, and are localized anterior to the tragus

    of the ear. Sinuses are blind, ending in close vicinity

    of the external auditory meatus.

    The most common branchial cysts and sinus de-

    rive from the second branchial pouch, which forms

    the tonsillar fossa and the palatine tonsils. The exter-

    nal orifice of the sinus can be located anywhere along

    the middle- to lower-third of the anterior border of

    the sternocleidomastoid muscle. The sinus pene-

    trates the platysma and runs parallel to the common

    carotid artery, crosses through its bifurcation and

    most commonly exits internally in the posterior ton-

    sillar fossa. A complete sinus may discharge clear

    saliva. A cyst, as a remnant of the second branchial

    pouch, presents as a soft mass deep to the upper-

    third of the sternocleidomastoid muscle. The depth

    distinguishes it from cystic hygromas, which are lo-

    cated in the subcutaneous plane.

    The third arch forms the inferior parathyroid

    glands and the thymus, while the fourth arch mi-

    grates less far down and develops into the superior

    parathyroid glands. Sinuses of the third arch open

    externally in the same region as those of the second

    one, but run upwards behind the carotid artery to the

    piriform fossa. Cystic remnants may compress the

    trachea and cause stridor. Sinuses and cysts of the

    fourth branchial arch and cleft are extremely rare.

    Both, third and fourth arch remnants most common-

    ly present as inflammatory lateral neck masses,more

    often on the left side. The cyst may evoke a false im-

    pression of acute thyroiditis. Computed tomography

    (CT) of the neck helps to identify the origin of such

    lesions. In an acute suppurative phase, external pres-

    sure onto the mass may result in laryngoscopically

    visible evacuation of pus into the piriform fossa.

    Cystic remnants present commonly in adoles-

    cence and adulthood, whereas sinuses and fistulas

    are usually seen in infancy and early childhood. In

    principle, clinical manifestation – no matter at what

    age – should be taken as an indication for elective

    excision before complications – mainly of an inflam-

    matory nature – supervene.

    Michael E.H?llwarthMichael E.H?llwarth

    8

    The patient is placed in a supine position. Following

    induction of general anaesthesia with endotracheal

    intubation, the head is turned to the side.A sandbag

    is placed underneath the shoulders to expose the af-

    fected side. Instillation of Methylene blue into the or-

    ifice aids identification of the sinus during dissec-

    tion. Some surgeons introduce a lacrymal duct probe

    into the orifice to guide dissection of the tract.

    2

    Figure 2.1

    In case of branchial cyst the incision is made over the

    cyst along the Langer’s lines. An elliptical incision is

    made around the sinus. A traction suture is applied

    to it just underneath the skin for manipulation dur-

    ing further dissection.

    Figure 2.2Chapter 2 Branchial Cysts and Sinus

    9

    Figure 2.1

    Hypoglossal nerve

    Carotid bifurcation

    Figure 2.2Michael E.H?llwarth

    10

    Figure 2.3

    Subcutaneous tissue and platysma are divided until

    the sinus tract is reached, which is easily palpable

    when the traction suture is gently tensed. Mobiliza-

    tion of the sinus continues in cephalad direction as

    far as possible with gentle traction. The operation

    can usually be done through a single elliptical inci-

    sion by keeping traction on the sinus tract and by the

    anaesthetist placing a gloved finger to push the ton-

    sillar fossa downwards. Dissection then continues

    through the carotid bifurcation to the tonsillar fossa.

    Close contact with the sinus is obligatory to avoid

    any injury to the arteries or the hypoglossal nerve.

    Close to the tonsillar fossa, the sinus is ligated with a

    50 absorbable transfixation suture and divided.

    Figure 2.5

    For the first branchial pouch remnants, the opening

    of the fistula is circumcised with an elliptical skin in-

    cision. Careful dissection liberates the subcutaneous

    segment of the embryological remnant,which is now

    transfixed with a stay suture.This is used for traction

    on the duct, which facilitates its identification on

    subsequent dissection into the depth towards the au-

    ditory canal.Because of intimate contact with the pa-

    rotid gland and potentially in the immediate vicinity

    of the fascial nerve, dissection must stay close to the

    tract, and – exclusively bipolar – electrocoagulation

    must be used sparingly. A neurosurgical nerve stim-

    ulator may be employed to identify and preserve fine

    nerve fibres. The sinus is transected and ligated with

    an absorbable 50 stitch close to the auditory canal.

    The subcutaneous tissue is approximated using 50

    absorbable sutures, followed by interrupted subcut-

    icular absorbable 60 sutures.

    Figure 2.4

    In adolescents a second transverse (stepladder) inci-

    sion,made approximately 4–5 cm above the first,may

    be necessary to completely excise the sinus tract.

    Both incisions are closed with absorbable interrupt-

    ed fine subcutaneous (50) and subcuticular (60) su-

    tures.

    2Chapter 2 Branchial Cysts and Sinus

    11

    Figure 2.3

    Facial nerve

    Figure 2.5

    Figure 2.4Michael E.H?llwarth

    12

    CONCLUSION

    Recurrences are most likely due to proliferation of

    residual epithelium from cysts or sinuses. The surgi-

    cal procedure should thus be performed electively

    soon after diagnosis. Infected cysts and sinuses are

    treated with antibiotics until the inflammatory signs

    subside, unless abscess formation mandates incision

    and drainage. Repeated infections render identifica-

    tion of the tissue layers much more difficult. Surgery

    after infections of remnants of the first branchial

    pouch carries an increased risk of facial nerve injury.

    In order to avoid damage to vital vascular and nerve

    structures it is important to confine dissection close

    to the sinus tract.

    SELECTED BIBLIOGRAPHY

    Deane SA, Telander RL (1978) Surgery for thyroglossal duct

    and branchial cleft anomalies.Am J Surg 136 : 348–353

    Smith CD (1998) Cysts and sinuses of the neck. In: O’Neill JA,Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG (eds) Pe-

    diatric surgery.Mosby, St Louis, pp 757–772

    Waldhausen JH, Tapper D (2000) Head and neck sinuses and

    masses. In: Ashcraft (ed) Pediatric surgery.WB Saunders,Philadelphia, pp 787–799

    2CHAPTER 3

    INTRODUCTION

    Cystic Hygroma

    Lymphangiomas are benign masses with multinodu-

    lar cysts of different sizes and contents. Microcysts

    are less than 1 cm in diameter; macrocysts are great-

    er than 1 cm in diameter and tend to be less invasive,less numerous, and less difficult to remove. Both mi-

    crocysts and macrocysts may contain blood andor

    lymph, a consequence of similar lymphatic and vas-

    cular embryology. In general, microcysts are more

    likely to contain blood and macrocysts more likely to

    contain lymph.Macrocysts that contain lymph are al-

    so called cystic hygromas and they are subsumed in

    the general category of lymphatic malformations.

    The risks of expectant management include infec-

    tion, progressive growth and disfigurement, exten-

    sion into previously uninvolved areas, dysphagia, air-

    way compromise, and erosion into vascular struc-

    tures.Asymptomatic cysts in the premature or small-

    for-dates child may await growth and development of

    the infant. For the majority of patients there is no

    need to defer excision.

    The determination of a lymphangioma’s size and

    character is based on location, clinical examination

    and investigation. Some regions tend to have typical

    lesions: for example, reddish lesions in the base of the

    tongue are typically microcystic with a significant

    vascular component; soft boggy masses in the super-

    ficial neck or axilla – sometimes with a bluish hue –

    are often macrocysts with lymph. The best investi-

    gations to determine cyst contents is either a T2-

    weighted gadolinium-enhanced magnetic resonance

    imaging (MRI) or needle aspiration of the dominant

    cyst. Lymph is straw-coloured; thin bloody fluid may

    occur when a lymphatic cyst is enlarged by a rup-

    tured blood vessel.Abundant dark or red blood indi-

    cates a significant vascular component. Viscid yel-

    low-clear fluid from an intra-oral lesion may signal a

    ranula, deriving from salivary tissue. Depth of inva-

    sion and an estimate of the structures involved is best

    determined by MRI scanning. Rarely, a neck lesion

    may extend to the anterior mediastinum and com-

    press the trachea. Spontaneous enlargement may oc-

    cur following an upper respiratory tract infection;

    spontaneous regression is rare although sometimes

    follows local infection

    Baird M. Smith, Craig T. AlbaneseBaird M. Smith, Craig T. Albanese

    14

    General anaesthesia is used and blood made avail-

    able if the lesion appears vascular on pre-operative

    screening. If lesions are close to important motor

    nerves, one may use a nerve stimulator and interdict

    use of musculoskeletal blocking agents.

    Pre-operative planning will usually demonstrate a

    safe plane of attack and may set expectations with re-

    gard to a complete excision or a debulking operation.

    Loupe magnification is often helpful, as is a bipolar

    cautery when working close to nerves or vital struc-

    tures. Microvascular lesions tend to infiltrate tissue

    planes, are more likely to bleed and have a high rate

    of recurrence. Macrocystic lesions tend to spread

    along fascial planes and around neurovascular struc-

    tures. Intra-operative rupture decreases the likeli-

    hood of complete resection,which averages 50%.Any

    residual cystic tissue will increase the likelihood of

    recurrence. Because this is not a malignant lesion, it

    is seldom necessary to sacrifice essential local struc-

    tures. It is commonly necessary to place a closed suc-

    tion drain, particularly when the lesion is incom-

    pletely excised. For the most common (cervical) le-

    sions, a transverse skin crease incision extending the

    length of the mass is placed in Langer’s lines.A first-

    generation cephalosporin is used peri-operatively.

    If the lymphangioma demonstrates dermal infiltra-

    tion, an ellipse of skin is removed. Otherwise, gener-

    ous sub-platysmal skin flaps are raised. The external

    jugular vein and ansa cervicalis are not considered

    essential and may be sacrificed.

    3

    Figure 3.1 Figure 3.2

    Dissection of cervical lesions begins at the superior

    margin of the mass, near the ramus of the mandible.

    Upward reflection of the facial artery and vein allow

    the precise visualization necessary to preserve the

    marginal branch of the facial nerve. Bipolar cautery

    may be used and optical magnification is often help-

    ful.

    The dissection proceeds medially, lifting the cyst

    from the surrounding alveolar tissue.

    It may be necessary to divide the middle thyroid

    vein and artery as the carotid sheath is approached.

    Deep dissection frequently involves the contents of

    the carotid sheath and sometimes the following

    nerves: vagus, spinal accessory, hypoglossal, sympa-

    thetic trunk, phrenic and the brachial plexus.

    Figure 3.3 Figure 3.4Chapter 3 Cystic Hygroma

    15

    Figure 3.1 Figure 3.2

    Mandibular branch

    of the facial nerve

    Facial artery and vein

    Figure 3.3

    Vagus nerve

    Figure 3.4Baird M. Smith, Craig T. Albanese

    16

    Figure 3.5

    Care is taken to preserve the hypoglossal nerve as it

    passes through the bifurcation of the carotid artery.

    The mass must then be freed from the hyoid bone

    and submandibular gland. It is rarely necessary to re-

    move the submandibular gland en bloc with the

    mass, sacrificing the facial artery. The mass may be

    adherent to the brachial plexus in the floor of the an-

    terior triangle or the spinal accessory nerve as it

    courses through the posterior triangle. Extension of

    the lymphangioma under the clavicle may lead to

    axillary or mediastinal involvement (requiring ster-

    notomy if the lesion proceeds deeply). Combined

    masses may be delivered either above or below the

    clavicle.

    Figure 3.6

    The platysma is re-approximated with fine absorb-

    able sutures and the skin closed with subcuticular

    sutures of similar material. Closed suction drainage

    is used for most lesions.

    3Chapter 3 Cystic Hygroma

    17

    Figure 3.5

    Figure 3.6Baird M. Smith, Craig T. Albanese

    18

    CONCLUSION

    Feeding resumes when the infant is awake and alert.

    Extensive intra-oral dissection may temporarily im-

    pair swallowing and delay the onset of oral feeds.

    Drain removal may take days or weeks and is dictat-

    ed by the daily drainage volume. Antibiotics are ad-

    ministered daily from 1 to 3 days.

    In cases of partial resection, recurrence typically

    occurs within a year of surgery. Lymph leaks and

    nerve injuries are minimized by the use of bipolar di-

    athermy.Rarely, lymph leaks may require re-explora-

    tion when drains are inadequate or removed early.

    Excision is the current gold-standard therapy.

    There are several reports of successful use of scleros-

    ing agents such as OK-432 or bleomycin in lymph-

    angiomas. This appears to be effective mainly in

    macrocystic lesions.

    An exciting advance in the management of fetuses

    with a high probability of upper-airway obstruction

    at birth due to a giant cervical lymphangioma, is the

    development of the ex utero intrapartum treatment

    (EXIT).

    SELECTED BIBLIOGRAPHY

    Banieghbal B, Davies MR (2003) Guidelines for the successful

    treatment of lymphangioma with OK-432. Eur J Paediatr

    Surg 13 : 103–107

    Bouchard S, Johnson MP, Flake AW, Howell LJ, Myers LB, Ad-

    zick NS, Crombleholme TM (2002) The EXIT procedure:

    experience and outcome in 31 cases. J Pediatr Surg

    37 : 418–426

    Charabi B, Bretlau P, Bille M, Holmelund M(2000) Cystic hy-

    groma of the head and neck – long-term follow up of 44

    cases.Acta Otolaryngol Suppl 543 : 248–250

    Hirose S, Farmer DL, Lee H, Nobuhara KK, Harrison MR

    (2004) The exutero intrapartum treatment procedure:

    Looking back at the EXIT. J Pediatr Surg 39: 375–380

    Schuster T, Grantzow R, Nicolai T (2003) Lymphangioma coli:

    a new classification contributing to prognosis. Eur J Paedi-

    atr Surg 13 : 97–102

    3CHAPTER 4

    INTRODUCTION

    Tracheostomy

    The indications for tracheostomy in infants and chil-

    dren fall into five main categories: airway immatur-

    ity, obstructing congenital anomalies, acquired ob-

    structions, tumours and trauma.

    The immature airway manifests itself as laryn-

    gomalacia, tracheomalacia or a combination of the

    two conditions. These infants present with inspirato-

    ry stridor, and some degree of nasal flaring and chest

    retractions. Other related conditions are congenital

    vocal chord paralysis, which is usually due to a cen-

    tral nervous system deficit, phrenic nerve injury,which may be associated with a difficult delivery, and

    recurrent laryngeal nerve injury, which may occur

    after ligation of a patent ductus arteriosus.

    Some patients with choanal atresia and Pierre

    Robin syndrome or other craniofacial abnormalities

    may be candidates for tracheostomy.

    Patients with a congenitally stenotic airway or tra-

    cheal agenesis are special cases. In the case of agene-

    sis, an emergency tracheostomy may be necessary

    where the trachea reestablishes distally.

    There are several acquired conditions that require

    tracheostomy.Among them are infection, neuromus-

    cular failure, chronic aspiration and subglottic steno-

    sis.Chronic respiratory failure, sleep apnea or neuro-

    motor problems resulting in poor airway mainte-

    nance also require tracheostomy. Long-term respira-

    tory support after major surgery, repair of laryngo-

    tracheo-oesophageal cleft or major trauma may ne-

    cessitate tracheostomy.

    Occasionally the management of a tumour such as

    a cervical teratoma or sarcoma in infancy will man-

    date a tracheostomy. More likely, a hemangioma or

    lymphangioma will compromise the airway to the

    extent that a more stable airway is needed.

    Tracheostomy in infants and children routinely is

    performed under general anaesthesia with the pa-

    tient intubated unless the patient’s condition is so

    unstable that the patient cannot tolerate the neces-

    sary drugs.

    Thom E. LobeThom E. Lobe

    20

    The patient is placed supine on the operating table

    toward the head of the table so that the surgeon can

    access the patient’s neck easily, but not so far down

    on the table that the anaesthesiologist cannot reach

    the patient to manipulate the endotracheal tube

    when required. The anaesthesiologist or anaesthetist

    must be able to maintain control of the airway while

    the surgeon is exposing and manipulating the tra-

    chea. The neck should be extended sufficiently to al-

    low complete access to the neck. Sometimes,on chub-

    by infants, it is still difficult to see the entire neck, de-

    spite the best attempts.A roll should be placed under

    the infant’s shoulders to facilitate proper positioning.

    The endotracheal tube should be secured so that the

    anaesthesiologist can easily remove the tube at the

    appropriate time.This means that any tape should be

    loosened before hand. If there is a feeding tube in

    place, it should be removed so that it does not inter-

    fere with endotracheal tube manipulation.When the

    infant is properly positioned and monitored, the en-

    tire neck from the lower lip to below the nipples

    should be prepped with a suitable surgical prep and

    draped. The superior most surgical drape should al-

    low easy access to the patient by the anaesthesiolo-

    gist.

    4

    Figure 4.1

    Incision is made in the lower neck crease, about the

    width of one finger above the jugular notch.A trans-

    verse incision is preferable. If the incision is too low

    you will end up in the mediastinum and the cannula

    will end up too low in the trachea.We first score the

    skin with a scalpel, then use a needle-point electro-

    cautery device to deepen the incision, taking care not

    to burn the skin.

    Figure 4.2Chapter 4 Tracheostomy

    21

    Figure 4.1

    Figure 4.2Thom E. Lobe

    22

    Figure 4.3

    This incision is extended through the subcutaneous

    fascia and platysma muscle,which is quite thin in the

    small infant. It is helpful to insert two right-angled

    retractors in the corners of this incision to better ex-

    pose the operative site.

    Next, we use two atraumatic forceps to grasp the

    cervical fascia on either side of the midline and open

    it vertically in the midline.We extend this incision in-

    feriorly to the jugular notch and superiorly to the

    thyroid gland.

    The strap muscles, immediately beneath the ante-

    rior cervical fascia similarly are separated in the

    midline. Usually, there are few to no blood vessels in

    the dissection thus far.Occasionally, you will encoun-

    ter a few small vessels that cross the midline. These

    should be cauterized and divided as they are encoun-

    tered.

    Once these muscles are separated, we place the

    two retractors deep to the muscle edges and gently

    retract laterally to better expose the trachea below.

    Sometimes it is necessary to free the muscle edges

    sufficiently to allow room for the blade of the retrac-

    tor to gain a secure purchase.

    The trachea should be visualized easily. If not,then palpation in the wound with manipulation of

    the endotracheal tube by the anaesthesiologist will

    help locate the trachea.

    Figure 4.4

    The proposed tracheostomy cannula should be se-

    lected, opened and its outer diameter visually

    checked against the exposed trachea to judge the cor-

    rectness of its size. If it seems that the initial selection

    was incorrect, then a tracheostomy cannula of a more

    appropriate size should be selected.

    The pre-tracheal fascia should be scored with the

    cautery to coagulate any tiny vessels on the surface of

    the trachea in the midline.Again, the blades of the re-

    tractors should be deep in the wound on either side

    of the trachea for optimal exposure.

    A suture of 40 monofilament nonabsorbable su-

    ture or its equivalent is placed on either side of the

    midline scored anterior trachea. Each suture incor-

    porates one or two tracheal rings. These sutures are

    not tied onto the tracheal wall, but can be tied at their

    ends and should be left 6–8 cm in length.At the end

    of the case, these sutures will be taped securely to the

    anterior chest wall and will be used to locate the tra-

    cheal incision in the event of a post-operative emer-

    gency in which the newly placed tracheostomy can-

    nula dislodges.These sutures also can be used to hold

    open the edges of the tracheal incision for ease of

    placement of the tracheostomy cannula at operation.

    4Chapter 4 Tracheostomy

    23

    Figure 4.3

    Figure 4.4Thom E. Lobe

    24

    Figure 4.5

    The surgeon should request that the endotracheal

    tube be loosened and prepared for removal. Using a

    number 11 blade, a vertical incision is made through

    the tracheal wall along the score mark. Two or three

    tracheal rings should be divided. Usually these are

    rings 2, 3 and 4. Rarely, it is necessary to divide the

    isthmus of the thyroid gland for proper tracheosto-

    my positioning. A transverse tracheal incision or re-

    moval of a tracheal ring is likely to result in a tracheal

    deformity and thus should be avoided.

    Suction should be available in case blood or secre-

    tions interfere with the surgeon’s view of the tracheal

    lumen. The tip of the cannula to be inserted should

    be lubricated with a water-soluble surgical lubricant

    and positioned over the incision, poised for insertion

    when the endotracheal tube is withdrawn.

    The surgeon then requests the anaesthesiologist

    to withdraw the endotracheal tube sufficiently to

    clear the lumen so that the tracheostomy cannula can

    be inserted and directed caudally toward the carina.

    One way to avoid misplacement is to insert a suc-

    tion catheter through the lumen, beyond the tip of

    the cannula. The suction catheter then can be insert-

    ed into the tracheal lumen first and serve as a guide

    over which the cannula can be passed.This technique

    also is useful should the cannula become dislodged

    after the procedure.

    If, for any reason, the tracheostomy cannula does

    not fit easily into the trachea, it should be removed

    and the endotracheal tube should be advanced be-

    yond the tracheal incision so that ventilation will not

    be compromised. This might occur if the diameter of

    the tracheal lumen has been over estimated and the

    previously selected tracheostomy cannula is too

    large to fit into the trachea. In that case, a smaller

    cannula should be selected.

    Figure 4.6

    As soon as the cannula is in place, the obturator or

    suction catheter should be removed and the an-

    aesthesiologist should disconnect the ventilator hose

    from the endotracheal tube and connect it to the

    tracheostomy cannula. Once that is done, the an-

    aesthesiologist should administer several deep

    breaths to the patient to confirm that the cannula is

    in the proper place and that the infant can be venti-

    lated satisfactorily. If it appears that, although the

    cannula width is appropriate, the cannula is too long

    and its tip rests on the carina, then several pieces of

    gauze can be used to build up the gap between the

    neck and the tracheostomy collar, thus backing the

    tip of the cannula away from the carina. Once ade-

    quate ventilation is confirmed, then the endotracheal

    tube can be removed completely.

    Once the cannula is connected to the ventilator,the cervical wings of the body of the cannula need to

    be secured to the patient.We don’t rely on a tie placed

    around the neck, but accomplish this with the aid of

    sutures.

    For each wing, a suture of 30 silk or its equivalent

    is passed through the skin of the neck, then through

    the upper edge of the wing of the cannula (midway

    between the midline and the end of the wing),through the lower edge of the wing, then again

    through the skin. When this suture is tied, the skin

    will be drawn over the wing and usually will cover it.

    After you have placed these sutures, both wings will

    be securely fixed to the skin of the neck.

    The two ties that were placed in the anterior tra-

    cheal wall are now taped securely to the anterior

    chest wall in such a fashion that their ends are easily

    accessible in case they are needed in an emergency to

    reinsert the cannula.

    Finally, the umbilical tape or tie that usually comes

    with the cannula is passed through the holes in the

    end of the wings and tied around the neck to further

    secure the cannula. This should be tied in back of the

    neck. A simple gauze dressing with some antibiotic

    ointment is placed underneath the wings of the can-

    nula over the cervical incision to complete the proce-

    dure.

    We send our infants to the intensive care unit after

    a fresh tracheostomy in case of emergency.

    4Chapter 4 Tracheostomy

    25

    Figure 4.5

    Figure 4.6Thom E. Lobe

    26

    CONCLUSION

    Tracheostomy is a simple technical procedure to per-

    form, but it can be one of the more difficult proce-

    dures in paediatrics. The cannula should be selected

    carefully to make certain that it is not too long after

    the roll (used to extend the neck) is removed and the

    patient is repositioned. Occasionally, it is necessary

    to order a special tracheostomy cannula. Such is the

    case for a short,wide trachea.

    The most common problems occur post-opera-

    tively when the cannula becomes occluded or, worse

    yet, dislodged. This is why we secure the sutures to

    the chest wall, to make certain that if the cannula be-

    comes dislodged it will be as easy to re-insert it or a

    new cannula into the tracheal lumen.

    We change the cannula 10 days after the surgery,before the patient is discharged from the hospital, to

    make certain that the cannula can be changed easily

    and to minimize the risk of cannula-related prob-

    lems after discharge.

    These patients need to be followed closely as they

    grow to assure the optimal cannula size and to deter-

    mine whether the tracheostomy still is necessary.

    Decannulation, when possible, is done in the hos-

    pital, usually after flexible or rigid bronchoscopy to

    assess the adequacy of the tracheal lumen and the

    presence of obstructing granulation tissue or mala-

    cia.

    SELECTED BIBLIOGRAPHY

    Bach JR, Zhitnikov S (1998) The management of neuromuscu-

    lar ventilatory failure. Semin Pediatr Neurol 5 : 92–105

    Carr MM, Poje CP,Kingston L,Kielma D,Heard C (2001) Com-

    plications in pediatric tracheostomies. Laryngoscope 111 :

    1925–1928

    Estournet-Mathiaud B (2001) Tracheostomy in chronic lung

    disease: care and follow-up. Pediatr Pulmonol 23 : 135–136

    Kenigsberg K (1994) Tracheostomy in infants. Semin Thorac

    Cardiovasc Surg 6 : 196–199

    Kremer B, Botos-Kremer AI, Eckel HE, Schlondorff G (2002)

    Indications, complications, and surgical techniques for

    pediatric tracheostomies – an update. J Pediatr Surg 37 :

    1556–1562

    4Part II

    OesophagusSPRINGER SURGERY ATLAS SERIES Series Editors: J. S. P. Lumley · J.R. SiewertCHAPTER 5

    INTRODUCTION

    Oesophageal Atresia

    Oesophageal atresia is defined as an interruption in

    the continuity of the oesophagus with or without fis-

    tula to the trachea. The anomaly results from an in-

    sult occurring within the fourth week of gestation,during which separation of trachea and oesophagus

    by folding of the primitive foregut normally takes

    place. Familial cases affecting siblings or offspring

    suggest genetic factors. Most cases, however, occur

    sporadically without evidence for either hereditary

    or specific environmental teratogenic causes. The in-

    cidence approximates to 1:4,500 live births with a

    slight male preponderance (59%).Associated malfor-

    mations are obvious or easily detected in 40–60% of

    cases, and may be found in up to 80% by meticulous

    search for structural and numerical anomalies in the

    skeletal system. At least 18 different syndromes have

    been reported in association with oesophageal atre-

    sia. The best known is probably the VATER or

    VACTERL association of anomalies (Vertebral-Anal-

    Cardiac-Tracheal-Esophageal-Renal-Limb).

    The earliest symptom of oesophageal atresia is a

    polyhydramnios in the second half of pregnancy.

    Polyhydramnios is an unspecific manifestation of

    swallowing disorders or of disturbance of fluid pas-

    sage through the uppermost part of the intestinal

    tract of the fetus.Prenatal ultrasound may further re-

    veal forward and backward shifting of fluid in the

    upper pouch, and in cases without a lower fistula, a

    paucity of fluid in the stomach and small intestine.

    Postnatal presentation is characterized by drooling

    of saliva and cyanotic attacks. If passage of 12 F feed-

    ing tube into the stomach is not possible, oesopha-

    geal atresia is almost certain. Immediate oro- or na-

    so-oesophageal insertion of a Replogle tube as soon

    as the diagnosis is established is mandatory for con-

    tinuous or intermittent aspiration of saliva in order

    to prevent aspiration. The baby should be nursed

    propped up in order to prevent aspiration of gastric

    contents in to the tracheobronchial tree.

    Prior to surgery, the type of atresia should be de-

    termined. Air below the diaphragm on a plain X-ray

    film including neck, chest and abdomen provides ev-

    idence of a commonly seen lower tracheo-oesopha-

    geal fistula. In most of these cases (type 3bC or

    3cD), a primary anastomosis between the oesopha-

    geal segments is possible. In contrast, a gasless abdo-

    men indicates that a pure oesophageal atresia with-

    out lower fistula is present, and a long distance

    between the segments is to be expected (type 1–, 2A

    or 3aB). A Replogle tube maximally advanced into

    the upper pouch helps to estimate its approximate

    length.

    Additional malformations are looked for. Every

    neonate is checked for visible anomalies such as anal

    atresia or limb malformations. The thoraco-abdomi-

    nal radiography may reveal duodenal or lower intes-

    tinal atresia, a diaphragmatic hernia andor skeletal

    anomalies. Ribs and vertebrae must be counted and

    carefully examined for deformations. Usage of con-

    trast medium is rarely indicated. Cardiologic assess-

    ment, including echocardiography, forms part of rou-

    tine pre-operative workup in order to recognize asso-

    ciated congenital cardiac abnormalities, which may

    influence anaesthetic management, and the presence

    of right-sided aortic arch, which is of importance for

    the surgeon.Abdominal ultrasound searching for uri-

    nary tract anomalies is performed routinely.

    The baby is nursed in the intensive care unit

    (ICU). Immediate surgery is rarely required, so that

    all above-mentioned investigations can be per-

    formed step by step. Intubation and ventilation is on-

    ly necessary in cases of respiratory distress, severe

    pneumonia or severe associated malformations de-

    manding respirator therapy. The endotracheal tube

    should be positioned beyond a distal tracheo-oe-

    sophageal fistula to avoid insufflation of gas into the

    stomach inducing a risk of rupture, especially if a

    high gastrointestinal atresia is associated.

    Michael E.H?llwarth, Paola ZaupaMichael E.H?llwarth, Paola Zaupa

    30

    5

    Figure 5.1a–e

    Classifications usually take their orientation on con-

    currence and type of tracheo-oesophageal fistula.

    The commonly used systems are those described by

    Vogt (numbers ± lower case letters) and Gross (capi-

    tal letters). The most frequent type of oesophageal

    atresia (3b according to Vogt,C by Gross) affects over

    85% of the patients and consists of a blind-ending

    upper pouch with a fistula between trachea and low-

    er oesophagus. Vogt’s extremely rare type 1, charac-

    terized by a more or less total lack of the oesophagus

    is not included in Gross’ classification.Type 2A (7%)

    corresponds to pure atresia without a fistula.The dis-

    tance between the two segments is usually too long –

    the same as in type 3aB (2%) – with a fistula to the

    upper oesophageal pouch. The patients with type

    3cD oesophageal atresia (3%) have an upper and a

    lower pouch fistula. Some authors classify an isolated

    tracheo-oesophageal fistula without atresia – H-type

    fistula – as type 4E (3%), although it belongs to a dif-

    ferent spectrum because the oesophagus is patent. In

    Gross’ classification, congenital oesophageal stenosis

    constitutes type F.Chapter 5 Oesophageal Atresia

    31

    Figure 5.1a–e

    2A 3aB 3bC

    3cD 4EMichael E.H?llwarth, Paola Zaupa

    32

    5

    Figure 5.2

    Surgical repair is performed under general anaesthe-

    sia with endotracheal intubation. The endotracheal

    tube is advanced close to the tracheal bifurcation,and the infant is ventilated manually with rather low

    inspiration pressures and small tidal volumes. These

    measures serve to avoid overinflation of the stomach

    as well as to stabilize the trachea throughout the

    intervention. The Replogle tube is initially kept in

    place to easily identify the upper pouch intra-opera-

    tively. Broad-spectrum antibiotic prophylaxis is ad-

    ministered on induction. We routinely start with a

    tracheo-bronchoscopy using a rigid 3.5 mm endo-

    scope. Trachea and main bronchi are briefly inspect-

    ed, and the fistula to the oesophagus is localized,which is usually approximately 5–7mm above the ca-

    rina. Exceptionally, it may be found at the carina or

    even in the right main bronchus, indicating a short

    lower segment, and most likely with a long oesopha-

    geal gap. The next step is to look for an upper fistula.

    The dorsal – membranous – region of the tracheal

    wall is inspected carefully up to the cricoid cartilage.

    Small upper fistulas are easily missed. To avoid this

    pitfall, irregularities of the dorsal wall are gently

    probed with the tip of a 3F ureteric catheter passed

    through the bronchoscope. If a fistula is present, the

    ureteric catheter will glide into it.

    Figure 5.3

    The standard approach for repair of an oesophageal

    atresia is a right latero-dorsal thoracotomy. If a right

    aortic arch is diagnosed pre-operatively, a left-sided

    thoracotomy is recommended.However, if an unsus-

    pected right descending aorta is encountered during

    s ......