Manual of Neonatal Surgical Intensive Care

By PMPH USA, Ltd.

The third edition of the Manual of Surgical Neonatal Intensive Care addresses the interdisciplinary area of the perioperative management of newborns with surgical conditions. The manual is a collaborative effort between the surgical staff at Children’s Hospital and the medical staff at Boston Children’s Hospital, Beth Israel Deaconess Medical Center, Brigham and Women’s Hospital, and hospitals further afield. Its intended audience is surgeons, neonatologists, pediatricians, neonatal nurse practitioners, neonatal nurses, critical care transport clinicians, and all other healthcare providers trained to render pre- or postoperative services or counseling in the care of newborns.

The authors intend for the manual to be useful to both medical and surgical clinicians. Where appropriate, chapters follow a standard order: embryology; prenatal diagnosis (treatment); postnatal presentation; postnatal diagnosis; differential diagnosis; preoperative management; implications for anesthesia; surgical management; postoperative management, complications and other outcomes. Illustrated in black and white with tables, drawings, and photographs, the text includes hands-on guidelines to the nonoperative management of newborns with surgical conditions.

• Language: English
• Publisher: PMPH USA, Ltd.
• Release date: Jun 1, 2016
• ISBN: 9781607959403

Book preview

Manual of

Neonatal

Surgical

Intensive Care

Third Edition

Manual of

Neonatal Surgical

Intensive Care

Third Edition

Anne R. Hansen, MD, MPH

Associate in Medicine

Medical Director, Neonatal Intensive Care Unit

Boston Children’s Hospital

Associate Professor of Pediatrics

Harvard Medical School

Boston, Massachusetts

Mark Puder, MD, PhD

Associate in Surgery Boston Children’s Hospital

Professor of Surgery

Harvard Medical School

Boston, Massachusetts

2016

PEOPLE’S MEDICAL PUBLISHING HOUSE—USA

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PMPH-USA

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© 2016 Anne R. Hansen and Mark Puder

All rights reserved. Without limiting the rights under copyright reserved above, no part of this publication may be reproduced, stored in or introduced into a retrieval system, or transmitted, in any form or by any means (electronic, mechanical, photocopying, recording, or otherwise), without the prior written permission of the publisher.

16 17 18 19/KING/9 8 7 6 5 4 3 2 1

ISBN-13 978-1-60795-194-0

ISBN-10 1-60795-194-0

eISBN-13 978-1-60795-940-3

Printed in the United States of America by King Printing Company.

Editor: Linda Mehta; Copyeditor/Typesetter: diacriTech; Cover designer: Mary McKeon

Library of Congress Cataloging-in-Publication Data

Names: Hansen, Anne R., editor. | Puder, Mark, editor.

Title: Manual of neonatal surgical intensive care / [edited by] Anne Hansen, Mark Puder.

Other titles: Neonatal surgical intensive care

Description: Third edition. | Shelton, Connecticut : People's Medical Publishing House-USA, 2016. | Includes bibliographical references and index.

Identifiers: LCCN 2015043653 (print) | LCCN 2015043994 (ebook) | ISBN 9781607951940 | ISBN 1607951940 | ISBN 9781607959403 (e-ISBN)| ISBN 9781607959403 ()

Subjects: | MESH: Intensive Care, Neonatal-Handbooks. | Infant, Newborn-Handbooks. | Infant, Newborn, Diseases—surgery—Handbooks.| Surgical Procedures, Operative—methods—Handbooks.

Classification: LCC RJ253 (print) | LCC RJ253 (ebook) | NLM WS 39 | DDC 618.92/01—dc23

LC record available at http://lccn.loc.gov/2015043653

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Notice: The authors and publisher have made every effort to ensure that the patient care recommended herein, including choice of drugs and drug dosages, is in accord with the accepted standard and practice at the time of publication. However, since research and regulation constantly change clinical standards, the reader is urged to check the product information sheet included in the package of each drug, which includes recommended doses, warnings, and contraindications. This is particularly important with new or infrequently used drugs. Any treatment regimen, particularly one involving medication, involves inherent risk that must be weighed on a case-by-case basis against the benefits anticipated. The reader is cautioned that the purpose of this book is to inform and enlighten; the information contained herein is not intended as, and should not be employed as, a substitute for individual diagnosis and treatment.

Table of Contents

Cover

Title page

CONTRIBUTORS

PREFACE

1 General Considerations

1.1 Medical Considerations

1.2 Surgical Considerations

1.3 Respiratory Management

1.4 Differential Diagnoses According to Presenting Symptoms

1.5 Vascular Access

2 The Fetus

3 Otolaryngology, Head and Neck

3.1 Laryngeal and Tracheal Anomalies

3.2 Nasal Anomalies

4 Cleft Lip/Palate and Robin Sequence

5 Cardiovascular Disorders

5.1 Patent Ductus Arteriosus

5.2 Vascular Rings

5.3 Vascular Anomalies

6 Respiratory Disorders

6.1 Esophageal Atresia and Tracheoesophageal Fistula

6.2 Congenital Diaphragmatic Hernia and Diaphragmatic Eventration

6.3 Pneumothorax and Air Leak

6.4 Chylothorax

6.5 Thoracic Mass Lesions

6.6 Extracorporeal Life Support

7 Gastrointestinal Disorders

7.1 Gastroschisis

7.2 Omphalocele

7.3 Necrotizing Enterocolitis

7.4 Obstruction

7.5 Gastrointestinal Bleeding

7.6 Intestinal Failure and Short-Bowel Syndrome

7.7 Inguinal Hernia

7.8 Umbilical Hernia

7.9 Feeding Tubes

7.10 Gastroesophageal Reflux

7.11 Ostomy Diversions and Management

8 Genitourinary Disorders

8.1 Obstruction of the Urinary Tract, Including Hydronephrosis

8.2 Renal Venous Thrombosis

8.3 Multicystic Dysplastic Kidney

8.4 Cystic Diseases of the Kidney Including Polycystic Kidney Disease

8.5 Bladder Exstrophy

8.6 Cloacal Exstrophy

8.7 Urolithiasis in Neonates

8.8 Dialysis/Catheters

8.9 Neurogenic Bladder Dysfunction in the Newborn

8.10 Testicular Torsion

8.11 Circumcision

8.12 Ambiguous Genitalia

9 Neurological Disorders

9.1 Neonatal Hydrocephalus

9.2 Myelomeningocele

10 Retinopathy of Prematurity

11 Intravenous Extravasation Injuries

12 Orthopedic Considerations in the Surgical Neonate

13 Genetic Considerations

14 Anesthesiology

14.1 Anesthesia for Neonatal Surgical Emergencies

14.2 Pain Assessment and Management

15 Health Maintenance/ Discharge Planning

INDEX

CONTRIBUTORS

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Pankaj B. Agrawal, MD, MMSC

Medical Director, Gene Discovery Core

Manton Center for Orphan Disease Research

Boston Children’s Hospital

Assistant Professor of Pediatrics

Harvard Medical School

Boston, Massachusetts

Theresa M. Andrews, RN, CCRN

Staff Nurse, Neonatal Intensive Care Unit

Boston Children’s Hospital

Boston, Massachusetts

Stuart B. Bauer, MD

Professor of Surgery (Urology)

Harvard Medical School

Senior Associate, Department of Urology

Boston Children’s Hospital

Boston, Massachusetts

Mandy Brown Belfort, MD, MPH

Assistant in Medicine

Division of Newborn Medicine

Boston Children’s Hospital

Assistant Professor in Pediatrics

Harvard Medical School

Boston, Massachusetts

Joseph G. Borer, MBBS, FRACP

Co-Director, Neurourology and Urodynamics

Boston Children’s Hospital

Associate Professor of Surgery (Urology)

Harvard Medical School

Boston, Massachusetts

Roland Brusseau, MD

Associate in Perioperative Anesthesia

Boston Children’s Hospital

Instructor in Anesthesia

Harvard Medical School

Boston, Massachusetts

Yee-Ming Chan, MD, PhD

Division of Endocrinology

Department of Medicine

Boston Children’s Hospital

Instructor in Pediatrics

Harvard Medical School

Boston, Massachusetts

Celeste J. Chandonnet, RN, BSN, CCRN

Infection Prevention Nurse

Neonatal Intensive Care Unit

Boston Children’s Hospital

Boston, Massachusetts

Bartley G. Cilento, Jr., MD, MPH, FAAP, FACS

Assistant Professor of Surgery (Urology)

Boston Children’s Hospital

Harvard Medical School

Boston, Massachusetts

David A. Diamond, MD

Professor of Surgery (Urology)

Harvard Medical School

Urologist-in-Chief

Boston Children’s Hospital

Boston, Massachusetts

Christopher Duggan, MD, MPH

Professor, Department of Nutrition

Professor, Department of Global Health and Population

Harvard School of Public Health

Director, Center for Nutrition

Medical Director, Center for Advanced Intestinal Failure

Boston Children’s Hospital

Associate Professor of Pediatrics

Harvard Medical School

Boston, Massachusetts

Debora Duro, MD, MS

Program Director, Pediatric Gastroenterology, Hepatology and Nutrition

Broward Health Medical Center - Chris Evert Children’s Hospital

Fort Lauderdale, Florida

John B. Emans, MD

Director, Spinal Surgery Division

Orthopedic Surgeon

Boston Children’s Hospital

Professor in Orthopedic Surgery

Harvard Medical School

Boston, Massachusetts

Neil R. Feins, MD, FAAP, FACS

Senior Surgeon, Department of Surgery

Boston Children’s Hospital

Professor of Surgery

Harvard Medical School

Boston, Massachusetts

Gillian L. Fell, MD, PhD

Department of Surgery

The Vascular Biology Program

Boston Children’s Hospital

Harvard Medical School

Boston, Massachusetts

Steven J. Fishman, MD

Professor of Surgery

Harvard Medical School

Co-Director, Vascular Anomalies Center

Boston Children’s Hospital

Boston, Massachusetts

Francis Flynn-Thompson, MD

Surgical Director, Ventricular Assist Device Program

Surgical Director, Lung Transplant and Heart Transplant Programs

Associate in Cardiac Surgery

Boston Children’s Hospital

Assistant Professor of Surgery

Harvard Medical School

Boston, Massachusetts

Terri Gorman, MD

Clinical Instructor, Pediatrics

Harvard Medical School

Medical Director, Neonatal Intensive Care Unit

St. Elizabeth’s Medical Center

Brighton, Massachusetts

Michael P. Glotzbecker

Assistant Professor of Orthopedic Surgery

Harvard Medical School

Orthopedic Surgeon

Boston Children’s Hospital

Boston, Massachusetts

Christine Greco, MD

Director, Acute Pain Services

Boston Children’s Hospital

Assistant Professor in Anesthesia

Harvard Medical School

Boston, Massachusetts

Arin K. Greene, MD, MMSc

Department of Plastic and Oral Surgery

Boston Children’s Hospital

Associate Professor of Surgery

Harvard Medical School

Boston, Massachusetts

Thomas E. Hamilton, MD

Assistant Program Director, Pediatric Surgery Fellowship Program

Attending Surgeon, Esophageal Atresia Treatment Program

Boston Children’s Hospital

Assistant Professor of Surgery

Harvard Medical School

Boston, Massachusetts

Anne R. Hansen, MD, MPH

Associate in Medicine

Medical Director, Neonatal Intensive Care Unit

Boston Children’s Hospital

Division of Newborn Medicine

Associate Professor of Pediatrics

Harvard Medical School

Boston, Massachusetts

John T. Herrin, MBBS, FRACP

Attending Nephrologist (Emeritus)

Senior Physician (Emeritus)

Division of Nephrology

Boston Children’s Hospital

Boston, Massachusetts

Monica E. Kleinman, MD

Clinical Director, Medical/Surgical Intensive Care Unit

Medical Director, Transport Program

Senior Associate in Clinical Care Medicine

Boston Children’s Hospital

Associate Professor of Anesthesia

Harvard Medical School

Boston, Massachusetts

Anjuli Koka, MD

Department of Anesthesiology, Perioperative and Pain Medicine

Boston Children’s Hospital

Boston, Massachusetts

Babu Koka, MD

Clinical Director, Anesthesia Services

Senior Associate in Perioperative Anesthesia

Boston Children’s Hospital

Assistant Professor of Anesthesia

Harvard Medical School

Boston, Massachusetts

Michelle LaBrecque, MSN, RN

Clinical Nurse Specialist

Neonatal Intensive Care Unit

Boston Children’s Hospital

Boston, Massachusetts

Richard S. Lee, MD

Assistant Professor of Surgery (Urology)

Harvard Medical School

Department of Urology

Boston Children’s Hospital

Boston, Massachusetts

Kristen T. Leeman, MD

Physician in Medicine

Boston Children’s Hospital

Instructor in Pediatrics

Harvard Medical School

Boston, Massachusetts

Craig Lillehei, MD

Senior Associate in Surgery

Transplant Surgeon

Boston Children’s Hospital

Assistant Professor in Surgery

Harvard Medical School

Boston, Massachusetts

Dorothy M. MacDonald, BSN, RN

Nursing Coordinator of Cleft Lip/Palate Program

Boston Children’s Hospital

Boston, Massachusetts

Joseph R. Madsen, MD, FACS, FAAP

Director, Epilepsy Surgery

Associate in Neurosurgery

Boston Children’s Hospital

Associate Professor of Neurosurgery

Harvard Medical School

Boston, Massachusetts

Michael A. Manfredi, MD

Instructor of Pediatrics

Harvard Medical School

Co-Director, Esophageal Atresia Treatment Program

Boston Children’s Hospital

Boston, Massachusetts

Karen McAlmon, MD

Medical Director, Special Care Nursery

Winchester Hospital

Winchester, Massachusetts

Director, Boston Children’s Hospital Neonatal Network

Instructor in Pediatrics

Harvard Medical School

Boston, Massachusetts

Jessica L. Miller, PharmD, BCPS

Critical Care Pharmacist

Boston Children’s Hospital

Boston, Massachusetts

Biren P. Modi, MD

Instructor in Surgery

Harvard Medical School

Associate Surgical Director

Center for Advanced Intestinal Rehabilitation (CIAR)

Assistant in Surgery

Boston Children’s Hospital

Boston, Massachusetts

John B. Mulliken, MD

Director, Craniofacial Center

Department of Plastic and Oral Surgery

Boston Children’s Hospital

Professor of Surgery

Harvard Medical School

Boston, Massachusetts

Leena Nahata, MD

Assistant in Medicine

Boston Children’s Hospital

Instructor of Pediatrics

Harvard Medical School

Boston, Massachusetts

Prathima Nandivada, MD, MS

Research Fellow and Resident Physician

Vascular Biology Program and Department of Surgery

Boston Children’s Hospital

Boston, Massachusetts

Samuel Nurko, MD, MPH

Associate Professor of Pediatrics

Harvard Medical School

Center for Motility and Functional Gastrointestinal Disorders

Boston Children’s Hospital

Boston, Massachusetts

Laurie A. Ohlms, MD, FACS

Associate in Otolaryngology

Boston Children’s Hospital

Assistant Professor

Department of Otology and Laryngology

Harvard Medical School

Boston, Massachusetts

Deirdre O’Reilly, MD,MPH, FAAP

Instructor in Pediatrics

Harvard Medical School

Division of Newborn Medicine

Boston Children’s Hospital

Boston, Massachusetts

Konstantinos Papadakis, MD

Instructor in Surgery

Boston Children’s Hospital

Harvard Medical School

Boston, Massachusetts

Mark Puder, MD, PhD

Associate in Surgery

Boston Children’s Hospital

Professor of Surgery

Harvard Medical School

Boston, Massachusetts

DeWayne M. Pursley, MD, MPH

Chief of Neonatology

Director, Klarman Family Neonatal Intensive Care Unit

Beth Israel Deaconess Medical Center

Division of Newborn Medicine

Boston Children’s Hospital

Boston, Massachusetts

Sandy Quigley, MSN, CWOCN, CPNP

Boston Children’s Hospital

Boston, Massachusetts

Lawrence Rhein, MD

Director, Center for Healthy Infant Lung Development

Boston Children’s Hospital

Assistant Professor of Pediatrics

Harvard Medical School

Boston, Massachusetts

Steven Alan Ringer, MD, PhD, FAACS

Chief, Newborn Medicine

Brigham and Women’s Hospital

Boston Children’s Hospital

Associate Professor, Harvard Medical School

Boston, Massachusetts

Cristhiane S. Santos, MS, RD

Clinical Dietitian

Broward Health Medical Center

Fort Lauderdale, Florida

Anthony John Schaeffer, MD, MPH

Department of Pediatric Urology

Primary Children’s Hospital

University of Utah Health Care

Salt Lake City, Utah

Charles F. Simmons, Jr., MD

Professor and Chairman, Department of Pediatrics

Chair of Neonatology

Cedars-Sinai Medical Center

Los Angeles, California

C. Jason Smithers, MD

Instructor in Surgery

Harvard Medical School

Assistant in Surgery

Boston Children’s Hospital

Boston, Massachusetts

Janet Soul, MD, CM, FRCPC

Director, Fetal-Neonatology Neurology Program

Boston Children’s Hospital

Boston, Massachusetts

Jane E. Stewart, MD

Associate Director, Neonatal Intensive Care Unit

Director of Infant Follow-up Program

Medical Director, Newborn Hearing Screening Program

Beth Israel Deaconess Medical Center

Boston Children’s Hospital

Assistant Professor, Pediatrics

Harvard Medical School

Boston, Massachusetts

Kevin A. Sztam, MD, MPH

Center for Nutrition

Division of Gastroenterology, Hepatology, and Nutrition

Boston Children’s Hospital

Harvard Medical School

Boston, Massachusetts

Deborah K. VanderVeen, MD

Associate Professor, Department of Ophthalmology

Boston Children’s Hospital

Harvard Medical School

Boston, Massachusetts

Linda Van Marter, MD, MPH

Neonatologist

Brigham and Women’s Hospital

Boston Children’s Hospital

Associate Professor of Pediatrics

Harvard Medical School

Boston, Massachusetts

Benjamin C. Warf, MD

Hydrocephalus and Spina Bifida Chair

Director of Neonatal and Congenital Anomalies Neurosurgery

Boston Children’s Hospital

Associate Professor of Neurosurgery

Harvard Medical School

Boston, Massachusetts

Jay M. Wilson, MD

Co-Director ECMO Program

Director, Surgical Critical Care

Senior Associate in Surgery

Associate in Critical Care Medicine

Boston Children’s Hospital

Associate Professor, Harvard Medical School

Boston, Massachusetts

Jill M. Zalieckas, MD, MPH

Assistant in Surgery

Assistant in Critical Care Medicine

Boston Children’s Hospital

Boston, Massachusetts

PREFACE

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The third edition of the Manual of Surgical Neonatal Intensive Care addresses the interdisciplinary area of the perioperative management of newborns with surgical conditions. These infants generally spend less than a day in the operating room, but require days, weeks, or even months of complex pre- and postoperative care that spans medical and surgical areas of expertise. Though many textbooks and manuals address the strictly medical or surgical management of newborns, relatively little has been written about issues that cross medical and surgical specialties. This tends to be working knowledge that is gained by experience. We hope the information in this manual will be useful to both medical and surgical clinicians.

This work is a collaborative effort between the surgical staff at Children’s Hospital and the medical staff at Boston Children’s Hospital, Beth Israel Deaconess Medical Center, Brigham and Women’s Hospital, and hospitals further afield. Its intended audience is surgeons, neonatologists, pediatricians, neonatal nurse practitioners, neonatal nurses, transport clinicians, and any other health care providers expected to render pre- or postoperative care or counseling for newborns with surgical conditions.

Children’s Hospital cares for newborns with surgical conditions in both its neonatal and pediatric intensive care units. We receive infants who require surgery and complex postoperative surgical care from local newborn nurseries, our associated special care nurseries, Beth Israel Deaconess Medical Center, and Brigham and Women’s Hospital, as well as from other referring hospitals across the country and around the world. The contributing authors reflect this web of community and tertiary hospitals.

Where appropriate, chapters follow a standard order: embryology; prenatal diagnosis (treatment); postnatal presentation; postnatal diagnosis; differential diagnosis; preoperative management; implications for anesthesia, surgical management, postoperative management, and complications, and other outcomes. In this third edition, we have added new chapters including Extracorporeal Life Support and Ambiguous Genitalia—We have updated all existing chapters and added many illustrations to clarify the written descriptions.

We would like to thank all of our contributing authors as well as our chiefs, Drs. Kourembanas and Shamberger, for their support. We would also like to thank the nurse practitioners and nurses, fellows and residents, respiratory therapists, nutritionists, and—most importantly—the babies and their families for all that they have taught us.

Anne R. Hansen, MD, MPH

Mark Puder, MD, PhD

  1    General Considerations

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Part 1.1    Medical Considerations

Part 1.2    Surgical Considerations

Part 1.3    Respiratory Management

Part 1.4    Differential Diagnoses According to Presenting Symptoms

Part 1.5    Vascular Access

  1.1    Medical Considerations

---

Anne R. Hansen, MD, MPH and Mandy Brown Belfort, MD, MPH

Full-term human gestation is 37-42 weeks. Currently, the borderline of viability is approximately 23-24 weeks of gestation.

Definitions

Gestational age: Time from last menstrual period (LMP) to birth

Chronologic age: Age since birth

Post menstrual age (PMA) = gestational age + chronologic age

Corrected age: Age after due date

For example, an infant born 15 weeks ago after 27 weeks of gestation has a gestational age of 27 weeks, a chronologic age of 15 weeks, a PMA of 42 weeks and a corrected age of 2 weeks.

High-Risk Infants 

Prematurity

Infants born at <37 completed weeks of gestation are considered premature. Preterm delivery can be either induced or spontaneous, starting as contractions or premature rupture of membranes. Induced deliveries, whether vaginal or cesarean section, can be for either maternal etiologies (e.g., progressive pregnancy-induced hypertension or cervical incompetence) or fetal etiologies (e.g., distress, infection, poor growth, or oligohydramnios).

The etiology of spontaneous preterm labor is infection in some cases, but often it is not known. Risk factors include low socioeconomic status, black race, younger (<16) or older (>35 years) maternal age, maternal illness (acute or chronic), multiple gestations, and previous preterm delivery.

Anticipated Complications

Risk of complications is highest at lower gestational ages, including the following:

Neurological/Sensory: Intraventricular hemorrhage (IVH), posthemorrhagic hydrocephalus (PHH), periventricular leukomalacia (PVL), retinopathy of prematurity (ROP), hearing impairment

Respiratory: Surfactant deficiency/hyaline membrane disease/respiratory distress syndrome, pneumothorax and other air-leak conditions, pulmonary interstitial emphysema, immature control of breathing or apnea/bradycardia of prematurity, chronic lung disease/bronchopulmonary dysplasia (CLD/BPD)

Cardiac: Patent ductus arteriosus (PDA), hypotension (secondary to intravascular volume depletion, poor myocardial function and vascular tone, and/or component of adrenal insufficiency)

Renal/fluid and electrolyte balance: Initially, low glomerular filtration rate (GFR); poor concentrating ability with wasting of free water, electrolytes, and bicarbonate; immature skin and large insensible losses, result is need for close monitoring of fluids and electrolytes, large total fluid requirement, high level of electrolyte supplementation, sometimes HCO3 therapy, longer half-life for many medications

Gastrointestinal (GI): Suck and swallow dysco ordination with requirement for gavage feedings until suck reflex matures at approximately 34-36 weeks PMA, feeding intolerance, necrotizing enterocolitis (NEC), spontaneous intestinal perforation (increased risk with indomethacin and hydrocortisone treatment), immature hepatic function combined with relative polycythemia resulting in increased risk of hyperbilirubinemia

Hematologic: Exaggerated and delayed physiologic anemia, anemia due to phlebotomy losses

Temperature regulation: Tendency toward hypothermia and temperature instability, with resulting need for monitoring, external heat source, generally warming lights or incubator (isolette)

Small for Gestational Age/Intrauterine Growth Restriction

Although the terms small for gestational age (SGA) and intrauterine growth restriction (IUGR) are often used interchangeably, they have two distinct meanings. Fetuses are SGA if they are >2 SDs below the mean or <10% for gestational age (for growth charts, see Table 1.1-3 on page 13). Fetuses are IUGR if they do not reach their growth potential. A constitutionally small infant who grows steadily along the 5% for gestational age is SGA but not IUGR. A fetus that started growing at the 90% and then drops to the 20% due to maternal hypertension is IUGR but not SGA. Poor growth that starts early in gestation tends to result in symmetric IUGR, in which weight, length, and head circumference (HC) are proportionately small. Poor growth that starts later in gestation generally results in asymmetric IUGR, in which the weight is affected most profoundly, the length less so, and the HC is relatively spared. SGA/IUGR status can result from maternal, placental, or fetal factors including the following:

Maternal: Older maternal age (>40 years), small constitutional size, race, high altitude, medications and/or drugs, malnutrition, chronic disease, any maternal condition resulting in decreased placental blood and oxygen flow (e.g., cardiac disease including chronic or gestational hypertension, advanced diabetes, renal disease, hemoglobinopathies including sickle cell, pulmonary disease, collagen vascular disease, antiphospholipid antibodies), uterine anomalies

Placental: Insufficiency resulting from abruption, abnormal implantation, maternal vascular disease (e.g., infarction), multiple gestations

Fetal: Familial and/or constitutional, chromosomal abnormalities and/or genetic syndromes, congenital infection (especially rubella and cytomegalovirus), multiple gestations, some postterm infants

Anticipated Complications

The complications depend on the etiology but can include any of the following: Fetal distress, perinatal depression, meconium aspiration, hypoxia, hypothermia, hypoglycemia, polycythemia, hyponatremia, hypocalcemia, pulmonary hemorrhage, and persistent pulmonary hypertension. Thrombocytopenia and leukopenia may occur, particularly with maternal preeclampsia as the cause for fetal growth restriction.

Large for Gestational Age

Infants are generally considered to be large for gestational age (LGA) if they are >2 SDs above the mean or >90% for their gestational age at birth (Table 1.1-3). LGA status can result from maternal or fetal factors including the following:

Maternal: Large constitutional size, obesity, inadequately controlled gestational or preexisting diabetes

Fetal: Familial and/or constitutional factors, some postterm infants, Beckwith-Wiedemann syndrome, hydrops fetalis

Anticipated Complications

The complications depend on the etiology but can include any of the following: Increased rate of cesarean delivery, birth injury (e.g., brachial plexus injury), hypoglycemia, polycythemia, and delayed pulmonary maturity.

Fluid, Electrolytes, and Nutrition

Daily fluid and electrolyte requirements depend on gestational and postmen-strual age (Table 1.1-1). After an initially low GFR in the first few days of birth, renal perfusion improves and total fluid and electrolyte requirements tend to increase. A large daily fluid volume is required by infants who have immature renal function and/or increased insensible losses due to poor skin integrity, need to remain under an open warmer (vs. an incubator), or require phototherapy. Infants typically need 3-5 mEq/kg/d of sodium, 2-3 mEq/kg/d of potassium, and 200-500 mg/kg/d of calcium gluconate; however, this varies considerably, especially for infants receiving diuretic therapy. Infants fed donor human milk often require supplementation with sodium chloride.

The initial goal is to achieve a mild degree of dehydration in infants with respiratory disease to minimize risk of pulmonary edema and subsequent CLD. For preterm infants, relative dehydration also decreases the risk of persistent PDA and IVH.

 Table 1.1-1   Approximate Total Fluids (mL/kg/d) Required by Birth Weight and Age

In the first week of life, normally term infants lose approximately 5%-10% of birth weight and preterm infants may lose up to 15% of birth weight. Infants should regain their birth weight by 2 weeks of age.

Once infants are beyond the acute phase of their illness, recuperating and growing on full enteral feeds, they most typically receive total fluids of 150 mL/kg/d. Some infants with CLD or congenital heart disease require fluid restriction to avoid pulmonary edema. Other infants with high caloric needs require additional fluids or high caloric density milk to achieve optimal growth and nutrition.

Intravenous Nutrition and Hydration

If an infant cannot be started on enteral feedings, nutrition and hydration need to be maintained intravenously. Intravenous (IV) fluid with glucose and electrolytes (as opposed to parenteral nutrition [PN]) is administered if it is anticipated that the infant will be receiving enteral feedings within 3 (preterm) to 5 (term) days.

Glucose concentration is determined to avoid hypoglycemia or hyperglycemia. IV glucose should initially provide 4-6 mg/kg/min and then be adjusted to keep the patient euglycemic. For infants, 10% glucose (D10W) is the typical initial IV fluid, but infants requiring more than approximately 150 mL/kg/d may need 5% glucose (D5W) to avoid hyperglycemia. Note that D5W is hypo-osmolar at 252 mOsm/L compared with 308 mOsm/L for normal saline and should not be run for more than 24 hours without carefully weighing the risks against the benefits.

IVF with an osmolarity of <200 mOsm/L carry an unacceptable risk of hypo-osmolar red blood cell lysis.

Electrolyte supplementation is adjusted based on measurement of serum electrolytes. Choice and concentration of electrolytes vary with the degree of prematurity and chronologic age of the infant, as well as with the total fluid volume being given and ongoing losses. D10W with 2-3 mEq NaCl/100 mL and 1-2 mEq KCl/100 mL is a typical maintenance fluid to run at 100-150 mL/kg/d. If an infant is hypocalcemic, calcium should be added to the IV fluid, especially if the infant is symptomatic (e.g., hypotensive). A typical concentration of calcium gluconate is 100-300 g/100 mL in order to provide calcium in the range of 200-500 mg/kg/d. IV fluids containing calcium should be administered only through a well-functioning IV line, preferably central, to avoid the severe damage caused by infiltration of calcium.

Parenteral Nutrition

If it is anticipated that the infant will require IV fluid for longer than three (for preterm infants) to five (for term infants) days, PN should be started as soon as possible to minimize energy and protein deficits. Initiation of PN may be delayed for infants whose glucose and electrolyte requirements are too unstable to permit prediction of fluid and electrolyte needs when ordered up to 24 hours in advance. Ordering PN requires a systematic approach, with daily advancement and adjustment. We prepare a standard starter PN in the pharmacy to give to infants <1500 g on the day of admission. It contains 10% dextrose and 40 g/L of amino acids, without electrolytes or micronutrients. We run it at 50 mL/kg/d to provide 2 g/kg/day of amino acids, with flexibility to adjust for the specific glucose and electrolyte needs of each infant with the remainder of the total fluids.

Fat

For most infants, we use 20% intralipid (IL). Start with IL calculation to know how much volume to subtract from total fluids to determine volume of non-IL PN. Start at 1 g/kg/d, advance by 1 g/kg/d until reaching 3 g/kg/d. Note that for infants at risk for the development of intestinal failure (e.g., expected to be nothing by mouth [NPO] or receive minimal enteral nutrition for at least 3 weeks), we restrict IL to 1 g/kg/day as long as energy needs for growth can be met through carbohydrate and protein provision. If weight gain is not adequate, consider risks and benefits of increasing fat intake, particularly for very low birth weight (VLBW) infants in whom early growth failure is associated with later neurodevelopmental impairments.

For infants with established PN-associated liver disease (direct bilirubin >2 for two weeks in a row with no other etiology of cholestasis), we use Omegaven 10% fish oil emulsion. Omegaven is not yet approved by the FDA for marketing in the United States, so providers must obtain permission from the FDA to use it. http://www.fda.gov/Drugs/DevelopmentApprovalProcess/HowDrugsareDevelopedandApproved/ApprovalApplications/InvestigationalNewDrugINDApplication/ucm368740.htm

Hyperbilirubinemia

In the setting of hyperbilirubinemia, historically clinicians have limited lipids to 1 g/kg/d because of the risk of lipids displacing bilirubin from albumin. More recent research¹ has diminished this theoretical concern, such that the benefits of providing adequate intake of calories and fat to these nutritionally vulnerable infants is generally judged to outweigh the risk of bilirubin crossing the blood-brain barrier and causing kernicterus.

Lipid Calculation 

To calculate daily lipids:

fig1.1a

*The mL/d of IL must be subtracted from the total daily fluid volume to determine the volume of non-IL PN.

Monitoring Serum Triglycerides (TG)

Serum TG should remain <250 mg/dL. Measure serum TG within 24 hours of starting the infusion, after dose advancement, and at least once weekly once goal IL provision is achieved. If TG > 250 mg/dL and specimen was drawn while IL was infusing, hold IL for 4 hours and repeat TG. If TF < 250 after holding the IL for 4 hours, the IL may be resumed at the same total daily dose and infused over 20 hours. If TG persistently > 250 mg/dL despite IL infusion being held for 4-24 hours, discuss alternative schedule (e.g., 1-3 times per week infusion) with nutrition service.

Glucose

Start at 4-6 mg/kg/min. Minimum of 4-5 mg/kg/min is required to provide adequate glucose to meet basal metabolic requirements.

Advance glucose concentrations daily as tolerated to provide goal energy needs while maintaining serum glucose in the normal range. If advancing or weaning off IV fluid, do not change glucose provision by >2 mg/kg/min in a day to avoid hyperglycemia or hypoglycemia.

Via peripheral IV, best to give 10% or less to avoid sclerosis of vein. Can give up to 12.5% if necessary to maintain serum glucose in normal range, but consider final osmolality of the solution.

Insulin therapy may be necessary to allow a patient to tolerate the administration of adequate glucose for growth without hyperglycemia.

Glucose Calculation

To calculate glucose administration in mg/kg/min:

(Glucose infusion rate) x (% glucose)

(144)

Example: Patient on 150 mL/kg/d of D125 W:

fig1.1b

Protein

VLBW infants require high-dose amino acid administration to minimize deficits. Starting dose is 2 g/kg/d for all infants. For infants <1500 g, after 24 hours advance to goal dose of 4 g/kg/day; for infants >1500 g, advance to goal dose of 3 g/kg/day. If BUN >50, decrease amino acid dose by 1 g/kg/day and repeat BUN every 24-48 hours until <50, then may readvance amino acid dose.

Protein Calculation

To calculate daily protein:

fig1.1c

Total and Ideal Distribution of Daily Calories

Like enteral energy requirements, parenteral caloric intake should be titrated to achieve optimal weight gain (Table 1.1-3) and somatic growth. Because of the increased efficiency with which parenterally administered calories are absorbed, as well as the risk of PN-associated liver disease with high IV fat intake, parenteral energy intake generally should not exceed 80-100 kcal/kg/d. Infants who are appropriate for gestational age (AGA) and >28 weeks of gestation typically need 80-90 kcal/kg/d, whereas infants who are SGA or <28 weeks often need 90-100 kcal/kg/d to support optimal weight gain. As a general guide, calories should be distributed as follows: Fat, 30%-55%; carbohydrates (glucose), 35%-65%; protein, 7%-15%. However the optimal caloric intake and macronutrient balance for each individual infant is that which supports optimal weight gain.

If the patient is receiving PN peripherally, there is a risk of vein sclerosis, precipitation, or excessive osmolality; therefore, the final osmolality of the solution should be <900 mOsm, and the following concentrations should not be exceeded:

Elemental calcium: 30 mg (=1.5 mEq)/100 mL

Potassium: 4 mEq/100 mL

Dextrose: 10% with <3% amino acids; 12.5% with <2% amino acids

Monitoring

Along with monitoring weight gain and somatic growth, infants receiving PN should be monitored biochemically. Weekly, measure serum electrolytes, blood glucose, total and direct bilirubin, alanine transaminase (ALT), alkaline phosphatase, TG, albumin, prealbumin, calcium, phosphorous, and magnesium. In addition, for infants receiving PN for >1 month, measure zinc, copper, selenium, aluminum, carnitine, and iron (if not recently transfused).

Enteral Nutrition and Hydration

If the infant has no contraindications to enteral feeding (e.g., respiratory distress, hypotension, presser use, hemodynamically significant PDA, umbilical artery catheter, recent hypoxic or ischemic exposure, ileus or bowel obstruction), the infant may be started on enteral feedings. The suck reflex matures at 34-36 weeks of gestation; therefore, below this gestational age, infants will initially need to receive enteral feedings via a gavage tube. Premature infants have immature GI tracts and should not immediately receive their full daily volume enterally or milk that has been calorically enhanced. Signs of feeding intolerance include increased volume of gastric aspirates, emesis, abdominal distention, and heme-positive stool (see Chapter 7, Gastrointestinal Disorders).

Enteral Feeding

Guidelines for initial and target rates and volume increases for enteral feeding are provided in Table 1.1-2.

Transition from IV to Enteral Nutrition

Fat and protein during transition from IV to enteral nutrition:

Fat: To prioritize caloric intake, continue to supply the full 3 g/kg/d of fat parenterally in addition to enteral fat intake. This approach offsets the lower caloric density of unfortified enteral feedings compared with parenteral and is our typical approach. Some centers prioritize maintaining the correct ratio of fat to other nutrients over caloric intake and therefore decrease IV lipid provision while advancing enteral feeds. Either approach is acceptable.

Protein: IV + PO = 3-5 g/kg/d total.

 Table 1.1-2   Enteral Feeding Guidelines for Newborn Infants

aConsider ≥150 based on nutritional needs (macronutrient, calorie), infant hunger, weight gain, ability to tolerate additional fluid volume versus preferable to increase caloric density.

Advancement of Caloric Density

Human milk and term formula contain 20 kcal/oz, which may not be adequate to support nutrient and energy needs for sick or preterm infants. Once the infant is tolerating 100 mL/kg/day of enteral feedings, the caloric density of milk can be increased directly to 24 kcal/oz, with additional increases by 2 kcal/oz every 24-48 hours, as tolerated, until optimal growth is achieved.

Recommendations:

For infants fed formula, first add 4-6 kcal/oz as concentrated formula by adding extra powder or using a ready-to-feed liquid formula.

For full-term infants fed expressed breast milk, add first 4 kcal/oz as formula powder. For preterm infants (<1500 g), add 4 kcal/oz as human milk fortifier (HMF).

Next we add Beneprotein 1/8 teaspoon per 25 mL, which provides approximately 1 kcal/oz. The target for daily total protein is 4-4.5 g/kg/day.

Next give 3-4 kcal/oz as medium-chain triglycerides (MCT) for preterm infants or canola oil for full-term infants.

For additional calories, consult with nutrition service and consider adding Duocal.

Calcium and phosphorous (PO4) should be checked 1-2 weeks after concentrated formula or HMF is added. If no hypercalcemia, continue extra calories and protein based on growth pattern, consider decreasing if growth is excessive.

Alkaline phosphatase should be checked every 1-2 weeks. If bone density concerns (e.g., osteopenia, fractures), check PTH and 25-OH Vit. D and supplement as indicated.

Calories for Optimal Weight Gain

Infants being fed enterally typically need between 110 and 130 kcal/kg/d, provided in volume of 130-160 mL/kg/d to achieve optimal weight gain, which should be assessed by plotting infant on a standard growth chart over time (Table 1.1-3). Infants with CLD or other conditions that increase metabolic demand can require up to 150 kcal/kg/d for optimal weight gain.

Assessment of Fetal Growth Status and F’ostnatal Growth Monitoring

For preterm infants, fetal growth status (SGA, AGA, LGA) can be determined by plotting the weight, length, and HC on an intrauterine growth chart (e.g. Olsen or Fenton, see Table 1.1-3).

Measurements should be plotted weekly, with the goal to achieve a growth rate similar to normal intrauterine growth.

For full-term infants, fetal growth status can also be determined using an intrauterine growth chart; the WHO growth standards should be used to monitor postnatal growth.

Typical weight gain is at least 20-30 g/d.

Typical linear growth is 0.8-1.1 cm/wk.

Typical head growth is

0.5-1 cm/wk until term.

0.5 cm/wk from term to 3 months.

0.25 cm/wk from 3 to 6 months of age.

All infants should ideally be given breast milk. Preterm infants fed breast milk require supplementation to meet their calorie and nutrient needs. If the mother’s own milk is not available or not adequate, for infants <1500 g we use pasteurized donor human milk, which we obtain from a Human Milk Banking Association of North America (HMBANA) certified milk bank. For larger infants we supplement with infant formula. Infants born at <34 weeks of gestation should be started on preterm infant formula rather than formula made for term infants. Infants born at 35-36 weeks of gestation should be started on a transitional formula. Infants with milk protein allergy may be fed breast milk provided the mother removes cow’s milk from her diet, with guidance from the nutrition service to ensure adequate calcium intake during lactation.

 Table 1.1-3   Growth Charts for Full-Term and Preterm Infants Breast Milk and Formula

Source: Adapted from Olsen 2010: https://www.nursing.upenn.edu/live/tags/infant%20development; Fenton 2013: https://live-ucalgary.ucalgary.ca/resource/preterm-growth-chart/preterm-growth-chart; WHO Growth Standards: http://www.cdc.gov/growthcharts/who_charts.htm

For infants with dietary restrictions, there are many specialized formulas.

Alimentum and Nutramigen are semi-elemental hypoallergenic formulas with hydrolyzed casein as the protein source, not amino acids. Pregestimil is another semi-elemental formula, but requires a special pharmacy order for parents to obtain it outside of the hospital setting. These formulas can all be used for infants with milk to moderate protein allergy without fat malabsorption.

The most elemental formulas are EleCare and neocate. Both have amino acids as protein source. Babies with severe protein allergies or multiple food allergies should be given EleCare or neocate. Babies with fat malabsorption short bowel syndrome will be able to absorb the most nutrients from EleCare or neocate.

Supplemental Vitamins and Minerals

Vitamin D

Because the vitamin D content of breast milk is quite low, infants who receive primarily breast milk should be supplemented with vitamin D, 400 IU/d. Infants fed formula should also receive supplementation if the formula contains <400 IU/d. Infants taking >1 L/d of formula generally do not require supplementation.

Supplemental Iron Preterm and iron deficient infants should be started on supplemental iron (Fe) once they are tolerating full volume feedings (of 24 kcal/oz milk for preterms). A total of 4 mg/kg/d elemental Fe should be provided routinely to prematurely born infants. If the baby is <1 kg and/or the hematocrit/ reticulocyte count is unusually low, or if erythropoietin is being used, a total of 6 mg/kg/d Fe can be given.

A volume of 150 mL/kg/d of formula made for preterm infants generally provides 2 mg/kg/d Fe; therefore, an additional 2 mg/kg/d of supplemental Fe should be given.

Breast milk has less Fe than formula, although it is thought to be more bio-available. Infants receiving breast milk should be given 4 mg/kg/d supplemental Fe. HMFs generally contain some Fe and should therefore be subtracted from the total goal to determine the supplemental Fe dose.

Considerations at Discharge

Patients should be discharged home on calorically enhanced milk if required to support optimal growth. Preterm infants fed formula are often discharged on a transitional formula. After discharge, preterm infants should be reassessed by their pediatrician and/or by the nutrition service for ongoing adjustment of caloric intake and supplementation.

Reference

1. Rubin M, Naor N, Sirota L, et al. Are bilirubin and plasma lipid profiles of premature infants dependent on the lipid emulsion infused? J Pediatr Gastroenterol Nutr. 1995;21:25-30.

Recommended Reading

Cloherty JP, Eichenwald EC, Hansen AR, Stark AR. Manual of Neonatal Care. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2012.

  1.2    Surgical Considerations

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Prathima Nandivada, MD and Mark Puder, MD, PhD

The newborn is not simply a small child. Preoperative and postoperative care of newborns require attention to age-specific details, with collaboration between medical and surgical specialists.

Stabilization and Transport of the Newborn for Surgery

Newborns with problems that require surgery are frequently referred from community hospitals to tertiary care centers. It is important to facilitate a smooth exchange of information between the referring center and the accepting center, including the surgeon and other staff.

Referring Center

The referring center should

Provide details of the infant’s specific problem(s), birth weight, gestational age, vascular access, medications, and intravenous (IV) infusions.

Copy appropriate records (including infant’s and mother’s chart) and radiographs to accompany the patient.

Initiate stabilization and management.

Avoid nonessential procedures or tests that may delay transfer.

Refer to material in this chapter as well as to the detailed recommendations in subsequent chapters for specific diagnoses.

Accepting Center

The accepting center should

Provide instructions regarding any specific management recommendations before and during transport.

Inform the receiving medical providers (neonatologists and surgeons) of the infant’s expected problems, condition, vascular access, and specific needs.

Inform all other relevant staff who will be participating in the infant’s care (e.g., the, radiologist, anesthesiologist, and other relevant specialties).

Preoperative Preparation of the Newborn

Confirm blood type and cross match.

Complete consent forms for surgery and anesthesia.

Infants with possible cardiac anomalies should have an electrocardiogram, a chest radiograph, and four limb pressures to facilitate a cardiology evaluation.

Newborns must receive 1 mg vitamin K intramuscularly to prevent hemorrhagic disease. Infants <1 kg should receive only 0.5 mg. This is sometimes overlooked during a difficult delivery or resuscitation and can lead to disastrous bleeding complications.

Diseases Requiring Surgery in the Newborn Period

Please also see chapters on specific diseases for full discussion.

Tracheoesophageal Fistula and Esophageal Atresia

The infant’s head should be kept up at a 45° angle. A sump nasogastric tube should be passed through the nose or mouth and be kept on continuous suction to keep the upper pouch empty.

Bag and mask ventilation is to be avoided if there is a distal tracheoesophageal fistula to prevent gastric distention and further respiratory impairment or gastric perforation.

Avoid abdominal palpation as this can cause reflux of gastric contents into the lungs via a distal fistula.

Look for vertebral, anal, cardiac, tracheal, esophageal, renal, and limb anomalies. Although it is not necessary to perform before transport, a preoperative echocardiogram is required for all of these patients to determine whether there is a right or left aortic arch. This will determine the side of thoracotomy for repair.

Abdominal distention is a surgical emergency. This can cause cardiac arrest due to pulmonary compression and reflux into the trachea with acute life-threatening pneumonitis.

A large-bore (14 French [14Fr]) angiocatheter should be taped to the infant’s bed for rapid gastric decompression in case life-threatening distention suddenly develops.

Intestinal Obstruction

The diagnosis and management of intestinal obstruction in infants is different from that in older children. The common causes of obstruction are classified as proximal (high) or distal (low) and include intestinal atresias, Hirschsprung’s disease, meconium ileus, and malrotation.

Bilious emesis in an infant denotes midgut volvulus until proven otherwise. This is a surgical emergency.

All infants require an adequate IV and nasogastric tube when intestinal obstruction is suspected.

Infants with suspected intestinal obstruction should receive an abdominal X-ray.

Newborn plain radiographs cannot distinguish large from small bowel obstruction because haustral markings are not yet detectable. However, the extent of bowel dilation can suggest a proximal (paucity of bowel gas) or distal (many loops of dilated bowel) obstruction.

Only filling the colon with a contrast agent will determine whether dilated zX-ray, one may proceed with an upper gastrointestinal (GI) series. If a distal obstruction is suspected based on X-ray, a contrast enema study may be indicated.

If meconium ileus is a suspected etiology based on X-ray (presence of calcium deposits is suggestive), a Gastrografin enema can be both diagnostic and therapeutic. Of note, Gastrografin is hyperosmolar and can cause rapid loss of fluid into the GI tract. This may lead to dehydration and shock. Infants should always have an IV placed and be adequately hydrated prior to a Gastrografin study.

For other intestinal obstruction, Cysto-Conray (othalamate meglumine) and in some cases barium is used.

Intestinal Atresia

Intestinal atresia occurs in decreasing order of frequency as follows: Ileum, duodenum, jejunum, colon, and pylorus. A careful antenatal and perinatal history may help to localize the site of atresia. Prenatal ultrasound (U/S) diagnosis of a dilated stomach and/or duodenum may be indicative of duodenal atresia. Abdominal distention is seen in most newborns with bowel atresia, although it may be minimal or absent with more proximal atresias. Vomiting usually occurs within the first 48 hours of life. Emesis is bilious except in pyloric atresia.

Plain radiographs of the abdomen should be obtained in all cases. The double bubble of duodenal atresia is pathognomonic, and no contrast study is indicated. Multiple loops of dilated bowel suggest a distal atresia, necessitating a contrast study, as earlier.

A contrast enema is helpful to identify microcolon, which is highly reliable in diagnosing a distal small bowel obstruction or to confirm patency of the colon.

Up to one-third of children with duodenal atresia have trisomy 21. These children may have complex cardiac anomalies.

All infants with duodenal atresia require a cardiology evaluation prior to surgery.

Hirschsprung's Disease

Hirschsprung’s disease is also called congenital aganglionic megacolon. It is a frequent cause of neonatal intestinal obstruction. In this disease, there is an absence of ganglion cells that results in a functional obstruction due to ineffective conduction of peristalsis. The aganglionic segment may be limited to the rectosigmoid or extend proximally to involve the entire colon and even up to the stomach. Symptoms are nonspecific and include failure to pass meconium in the first 48 hours of life, episodic abdominal distention, constipation, obstipation, or diarrhea. The diagnosis is often suspected in a neonate with evidence of distal obstruction on abdominal plain film. Additional evaluation may include a contrast enema. Classically, this shows a transition zone at the narrowed rectum with a dilated colon proximally; however, the finding is often absent in infants. If the contrast enema is normal and there is a high suspicion for Hirschsprung’s disease, obtain a plain radiograph of the abdomen on the following day. Retained contrast in the colon on this follow-up film is highly suspicious for Hirschsprung’s disease.

A diagnosis of Hirschsprung’s disease is confirmed by suction mucosal rectal biopsy or full-thickness rectal biopsy showing an absence of ganglion cells and hypertrophied nerves in the myenteric plexus of the muscularis layer. There is also increased acetylcholinesterase in the aganglionic rectum.

Initial management of Hirschsprung’s disease is with saline rectal irrigations every six hours. As long as the infant passes stool with irrigations and the abdomen decompresses appropriately, the infant may be fed ad lib.

Neonatal primary perineal pull through, with or without laparoscopic assistance, may be performed once the infant is stabilized.

Colostomy can usually be avoided, unless the patient is unable to pass stool with irrigations and severe and/or recurrent enterocolitis occurs.

Colostomy may be indicated for enterocolitis or the inability to obtain adequate decompression with irrigation. Long-segment Hirschsprung’s disease may also require stoma formation.

The sphincter is also aganglionic. Some children develop functional obstruction as a result. Botox injections within the sphincter can be performed at the time of initial surgery and/or postoperatively to manage high anal tone.

Meconium Ileus

Meconium ileus accounts for almost one-third of all obstructions in the small intestine of newborns. It occurs in about 15% of infants with cystic fibrosis. However, 90% of patients with meconium ileus have cystic fibrosis. The incidence of cystic fibrosis in the United States is 1 in 3000 live births. Males and females are equally affected. It is extremely rare in non-Caucasian populations.

The diagnosis of meconium ileus is suspected in the infant who develops generalized abdominal distention, bilious vomiting, and failure to pass meconium in the first 24-48 hours. There is a history of polyhydramnios in 20% of cases. The presence of a family history of cystic fibrosis should be determined.

The meconium may be palpable as a doughy substance in the dilated loops of distended bowel. The anus and rectum are typically narrow.

Plain radiographs of the abdomen show bowel loops of variable sizes with a soap-bubble appearance of the bowel contents. Calcifications usually indicate meconium peritonitis, resulting from an intrauterine intestinal perforation. Microcolon is a highly reliable finding for distal bowel obstruction that may be a functional stenosis from inspissated meconium or atresia due to intrauterine volvulus. A contrast enema demonstrates the microcolon with inspissated meconium proximally. A contrast enema is contraindicated if the plain radiograph shows calcification.

The initial treatment is nonsurgical and begins with a Gastrografin enema. Under fluoroscopic control, a 50% solution of Gastrografin and water is infused into the rectum and colon through a catheter. This will usually result in a rapid passage of semiliquid meconium that continues for the next 24-48 hours. Follow-up radiographs of kidney, ureter, and bladder (KUBs) at 12 and 24 hours should be obtained to evaluate the progress. Multiple Gastrografin enemas are frequently required. Mucomyst (N- acetylcysteine) can also be used as an enema or by mouth or nasogastric tube to assist in cleaning out the thick meconium (dilute 20% solution to 5% by adding sterile water).

Surgery is indicated for meconium ileus if the Gastrografin enema fails to relieve the obstruction, there are calcifications in the abdominal cavity, the infant appears too ill to delay operation, or the diagnosis of meconium ileus is in doubt.

Infants diagnosed with meconium ileus require a sweat test to pursue the diagnosis of cystic fibrosis. This test is usually not practical prior to surgery because the child must weigh at least 2 kg and be older than 72 hours. A minimum of 100 mg of sweat is collected, and a concentration of sodium and chloride above 60 mEq/L is diagnostic. DNA analysis of a buccal smear can detect cystic fibrosis with 80%-90% sensitivity, because it looks for the most common genetic mutations. Furthermore, DNA sequencing of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) can be performed to identify the specific mutation affecting the patient, however this is not necessary for diagnosis. Infants with cystic fibrosis should receive pancreatic enzymes when enteral feedings are begun.

Midgut Malrotation and Volvulus

Midgut malrotation and volvulus are very common causes of intestinal obstruction in infants and must be considered in every infant with bilious emesis.

Clinical findings: The presentation can range from asymptomatic to acutely ill. More than 50% present in the first month of life, 30% in the first week. Ninety-five percent have vomiting that becomes bilious. Bloody emesis suggests bowel necrosis. Twenty-eight percent have bloody stools. Plain radiographs are most commonly normal, but they may show either a gasless abdomen, dilated intestine suggesting small bowel obstruction, or a duodenal obstruction with a double bubble. Unless immediate surgery is necessary, the diagnosis should always be confirmed with an upper GI study to determine the position of the duodenal-jejunal junction.

Midgut volvulus is one of the most serious emergencies seen in the newborn period. Delay in diagnosis can result in loss of the entire midgut and may be fatal. Sudden onset of bilious emesis is the primary presenting sign. Abdominal distention is common, but it may be absent. Abdominal tenderness varies. Rectal examination is usually guaiac positive. Definitive diagnosis requires a contrast study. An upper GI is the preferred study. With shock or a clear indication for exploration, no studies are necessary, and the infant should be brought directly to the operating room (OR). If studies are obtained, they should be done expeditiously because a few hours may be the difference between a totally reversible condition and loss of the entire midgut and possibly death.

The treatment is always surgical. A nasogastric tube must be placed, IV hydration started, and the infant transported immediately to the OR. The surgeon decompresses the volvulus by rotating the bowel around its pedicle in a counterclockwise manner. Adhesions are lysed, and the small bowel is placed in the right lower quadrant (RLQ) and the cecum and colon into the left lower quadrant. An appendectomy is performed. Recurrent volvulus occurs in up to 8% of cases.

Omphalocele and Gastroschisis

Infants with exposed bowel are at high risk of hypothermia. This is a complication that should be anticipated and avoided by paying close attention to thermoregulation.

The sac (omphalocele) or exposed intestines (gastroschisis or omphalocele) is immediately covered with an occlusive dressing. The most desirable dressing is to place the entire lowerhalf of the infant’s body into a Lahey bag (obtained from the OR) to prevent evaporative losses. Coverage with a clear wrap (e.g., Saran Wrap) allows inspection of the bowel to monitor for ischemia. Gauze should generally be avoided because it tends to stick to the bowel even if moistened.

To prevent angulation of the bowel and ischemia, the infant should be placed on his or her side with the bowel supported by towels.

To prevent further GI distention and aspiration of gastric contents, a nasogastric tube should be placed to continuous suction.

IV hydration is essential.

Systemic IV antibiotics (ampicillin and gentamicin) are given to protect the contaminated amnion and viscera. Infection is a devastating problem, especially if a mesh closure is necessary.

Associated anomalies in infants with omphalocele are common and may include the following types: Cardiac (a preoperative echocardiogram is necessary to assess for a cardiac disease or cardiovascular dysfunction), renal (postoperative renal U/S), chromosomal (trisomy 13, 18, 21), Beckwith-Wiedemann syndrome (large tongue, gigantism, hypoglycemia), and rectal (imperforate anus). Gastroschisis is associated with intestinal atresia.

Immediate surgery is performed with either primary closure of the defect or placement of a Silastic silo for gradual reduction. Silo reduction is usually accomplished within two weeks.

If the defect is too large for closure or if there are severe associated abnormalities, omphaloceles may be allowed to epithelialize. Topical agents such as silver nitrate or silver sulfadiazine are applied. Epithelialization takes several weeks and leaves a hernia defect that is repaired at a later date. Tissue expanders can also be used to provide laxity for subsequent abdominal wall closure.

Necrotizing Enterocolitis

Necrotizing enterocolitis usually occurs in premature and/or low-birth-weight infants; 10% of cases are in term infants. Clinical presentation is nonspecific, and radiography is important for diagnosis and to follow the progression of disease. Bowel dilation is the earliest and most common sign. Intramural gas (pneumatosis) confirms the diagnosis. The amount of gas is not related to the severity of disease. Resolution of the gaseous distention is not necessarily related to improvement. Portal venous gas is usually associated with severe disease. Free air is diagnostic of intestinal perforation.

Evaluation and Treatment

Nothing by mouth (NPO)

Nasogastric suction

Broad spectrum antibiotics

KUB and lateral decubitus radiographs, intitially every 6-8 hours

Serial complete blood count, platelet count, blood pH, and electrolytes

Surgical Indications

Pneumoperitoneum is an absolute indication for surgery and is best seen on a lateral decubitus radiograph.

Relative Indications

Abdominal wall cellulitis

RLQ mass

Fixed loop of bowel

Failure to respond to medical therapy

Persistent thrombocytopenia, acidosis, or hemodynamic instability

Once surgical indications emerge, the infant must be promptly taken to the OR. The critically ill infant with extremely low-birth weight (i.e., <1000 g) may undergo bedside peritoneal drainage as a temporizing measure or, in some cases, as definitive treatment.

Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia requires specialized medical and surgical care. Despite intensive therapy including extracorporeal membrane oxygenation, mortality continues to be high.

Transport to Treatment Center

Respiratory: Intubation is required in infants with respiratory distress. The peak inspiratory pressure should be just enough to move the chest, in general <30 cm H2O. Avoid muscle relaxation if possible. If the infant is able to oxygenate and ventilate sufficiently, intubation is not necessary.

A functional nasogastric tube for suction is essential to prevent gaseous distention of the intestinal contents in the chest.

Lines: Only peripheral IV lines are necessary for transfer. Umbilical lines and arterial lines can be placed after arrival at the receiving hospital. After transfer, one arterial line (preferably preductal) should be placed, with pulse oximetry in the posductal position.

General Perioperative Fluid Management 

NPO Orders

Infants may be given clear liquids containing glucose up to four hours prior to surgery. Breast milk is considered a clear fluid unless the infant has received a bowel preparation.

IV Fluids

An infant should not remain without fluid intake for longer than six hours. If surgery is delayed, IV fluids should be started preoperatively.

Patients with fever, vomiting, diarrhea, or undergoing bowel preparation should have IV infusions started the night prior to surgery.

Bowel Preparation for GI Surgery

Elective bowel surgery is often managed with preoperative mechanical bowel preparation (Golytely) followed by oral antibiotics of erythromycin base 50 mg/kg/d plus oral neomycin 50 mg/kg/d divided every 2-3 hours for 3 doses on the day prior to surgery. Preoperative IV antibiotics are given 30 minutes prior to incision time (on call to OR).

Golytely is an isotonic solution of polyethylene glycol and electrolytes. The polyethylene glycol has a high-molecular weight and is not absorbed in the GI tract. This is used for colon cleaning preoperatively and must be given before midnight. If the effluent is not clear after 4-6 hours, the dose may be repeated once. Infants under 10 kg should receive maintenance IV fluids during the bowel preparation. Golytely dose: PO/PG 12.5 mL/kg/h x 4 hours. When giving PG, it should be through an enteral infusion pump.

Deficit Therapy

Gastric losses: D5W 1/2 NS + 20-40 mEq KCl/L to replace measured losses

Distal GI losses: 5% dextrose with Ringer’s lactate (D5RL) to replace measured losses

Third-space losses: D5RL

Body Fluid Compositions

Table 1.2-1 provides guidelines regarding the typical composition of body fluids in infants. If more precision is necessary, the actual fluid(s) may be sent for electrolyte analysis.

 Table 1.2-1   Guidelines Regarding Composition of Body Fluids in Infants