Endocrine Surgery in Children

 

Description:

 

This book provides in-depth practical advice on how to manage children with endocrine conditions that may benefit from surgery. It is more detailed than general pediatric surgery texts and more surgically oriented than endocrinology texts. The first section is devoted to the thyroid and parathyroid, with detailed discussion of thyroid nodules, thyroid cancer, hyperthyroidism, hyperparathyroidism, and multiple endocrine neoplasia. The second section on the pancreas focuses on nesidioblastosis, islet cell transplantation, the surgical treatment of diabetes, and surgical complications of diabetes. Adrenal disorders are then discussed, followed by a section on the evaluation and management of ovarian and testicular torsion and tumors. The closing section addresses miscellaneous topics such as gynecomastia in boys and growth restriction surgery. This book will serve as an invaluable reference for all practitioners and trainees who care for children with endocrine problems for which surgery is considered.

Table of Contents:

Foreword

Preface

Acknowledgements

Contents

Contributors

Thyroid

1 Thyroid Gland Embryology, Anatomy, and Physiology

Thyroid Gland—Embryology, Anatomy, and Physiology

Embryology of the Thyroid Gland

Embryological Anomalies of the Thyroid with Clinical Significance

Embryology of the Recurrent Laryngeal Nerve

Anatomy of the Thyroid Gland

Gross Anatomy

Histology of the Thyroid Gland

Thyroid Physiology

Thyroid Hormone Synthesis

Circulating Thyroid Hormones

Cellular Effects of Thyroid Hormones

Control of Thyroid Function by TSH

Calcitonin

References

2 Hyperthyroidism

Pathophysiology

Aetiology and Incidence

Evaluation

History

Physical Examination

Laboratory Tests

Other Investigations

Management

Medical Management

Radioactive Iodine Treatment

Surgical Management of Hyperthyroidism

Indications for Operative Management of Hyperthyroidism

Preoperative Preparation

Perioperative Anaesthesia Considerations

Choice of Operation for Hyperthyroidism

Postoperative Care

Results of Thyroidectomy for Hyperthyroidism

References

3 Thyroid Nodules in Children

Incidence and Epidemiology

Etiology

Evaluation of Thyroid Nodules

History and Physical Examination of the Child with a Thyroid Nodule

Laboratory Tests to Evaluate Thyroid Nodules

Imaging in the Evaluation of Thyroid Nodules

Fine Needle Aspiration of Thyroid Nodules

References

4 Thyroid Cancer in Children

Incidence

Epidemiology

Etiology

Risk of Thyroid Cancer After Radiation Exposure

Risk of Thyroid Cancer in Syndromes

Pathology

Papillary Thyroid Cancer

Follicular Thyroid Cancer

Other Thyroid Cancers

Clinical Presentations of Thyroid Cancer

Evaluation

Treatment

Surgical Treatment

Postoperative Staging

Postoperative Radioactive Iodine Treatment

Outcomes/Complications

References

Parathyroid

5 Parathyroid Gland Embryology, Anatomy and Physiology

Parathyroid Embryology

Parathyroid Anatomy

Macroscopic Appearance of Parathyroid Glands

Relationship of Parathyroid Glands to the Recurrent Laryngeal Nerves

Blood Supply of Parathyroid Glands

Ectopic Parathyroid Glands

Histology

Parathyroid Physiology

Parathyroid Hormone

References

6 Parathyroid Surgery in Children

Incidence/Epidemiology

Etiology

Pathology

Clinical Presentation

History and Symptoms

Physical Examination

Blood Tests

Imaging

Indications for Operation

Management

Preoperative Management

Hypercalcemic Crisis

Operative Management

Postoperative Management

Complications

Long-Term Outcomes and Follow-up

References

Adrenal

7 Adrenal Gland Embryology, Anatomy, and Physiology

Embryology

Adrenal Cortex Histology and Physiology

Adrenal Medulla Histology and Physiology

Adrenal Anatomy

Relationship to Retroperitoneal Structures

Venous Anatomy

Arterial Anatomy

Lymphatic Anatomy

Anatomic Basis of Surgical Approaches to the Adrenal

References

8 Pheochromocytoma

Epidemiology

Aetiology and Molecular Genetics

Von Hippel–Lindau Disease (VHL)

Multiple Endocrine Neoplasia Type 2A and 2B (MEN 2A and 2B)

Neurofibromatosis Type 1 (Von Recklinghausen Disease)

Hereditary Paraganglioma and Pheochromocytoma Syndromes

Other Genetic Disorders

Pathology

Clinical Presentation

Evaluation

History

Physical Examination

Investigations

Differential Diagnosis

Imaging and Localisation

Computerised Tomography (CT) and Magnetic Resonance Imaging (MRI)

Other Imaging and Investigations

Management

Preoperative Management

Surgery

Adrenalectomy for PHEO

Surgery for Thoracic and Abdominal PGL

Surgery for Malignant PHEO

Preoperative Considerations

Intraoperative Considerations

Operative Technique

Right Adrenalectomy

Left Adrenalectomy

Bilateral Laparoscopic Adrenalectomy

Open Adrenalectomy

Post-operative Care

Complications

Intraoperative Complications

Early Post-operative Complications

Late Complications

Follow-Up and Prognosis

Other Treatment for Malignant Disease

Radioactive 131I-MIBG

Radioactive Somatostatin Analogues

Chemotherapy, Radiotherapy and Novel Treatments

References

9 Neuroblastoma

History

Clinical Features

Investigation

Treatment History

Staging

Pathology

Molecular Pathology

Risk-Based Therapy

Predicting the Risk of Operation

Predicting the Risk of Disease

Treatment Strategies

Localised Disease—No Image Defined Risk Factors (INSS Stage 1 and 2—INRG Stage L1)

Localised Disease with Risk Factors (INSS Stage 3 INRG Stage L2)

Metastatic Disease in Patient Over 18 Months of Age (INSS Stage 4 INRG Stage M)

Metastatic ‘Special’ in a Patient Under 18 Months of Age (INSS Stage 4S, INRG Stage MS)

Radiation Therapy

Screening

References

10 The Evaluation and Management of Adrenal Masses and Adrenocortical Tumors (Act)

Physiology of Adrenocortical Tumors

Etiology of Adrenocortical Tumors

Epidemiology of Adrenocortical Tumors in Children

Pathology of Adrenocortical Tumors in Children

Gross Features of Adrenocortical Tumors in Children

Microscopic Features of Adrenocortical Tumors in Children

Adrenocortical Adenoma or Carcinoma?

Evaluation of Adrenal Masses

Symptoms and Signs of Adrenal Masses

Laboratory Investigation of Adrenal Masses

Imaging Evaluation of Adrenal Masses

Differential Diagnosis of Adrenal Masses

Neuroblastoma

Pheochromocytoma

Adrenal Hemorrhage

Renal Lesions

ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH)

Primary Pigmented Nodular Adrenocortical Disease (PPNAD)

Surgery for Adrenocortical Tumors

Retroperitoneal Lymph Node Dissection (RPLND)

Minimally Invasive Surgery for Adrenocortical Tumors in Children

Staging of Adrenocortical Tumors

Adjuvant Therapy

Mitotane

Chemotherapy

Radiotherapy

Future Therapeutic Options

Outcomes

Future Considerations

References

Pancreas

11 Pancreas Embryology, Anatomy, and Physiology

Basic Anatomy

Physiology

Imaging

Plain Abdominal Radiographs

Abdominal Ultrasonography (US)

Abdominal Computed Tomography (CT) Scans

Endoscopic Retrograde Cholangiopancreatography (ERCP)

Magnetic Resonance Cholangiopancreatography (MRCP)

Other Imaging Modalities for the Endocrine Pancreas

Basic Pancreatic Embryology

Dorsal and Ventral Pancreatic Bud Development

Initiation of the Pancreas with Endodermal Patterning

Pancreatic Mesenchyme

Key Signaling Molecules

TGF-β Signaling

Notch Signaling

Hedgehog Signaling

Wnt’s

Endothelial Cells and Oxygen Tension

Endothelial Cells

Extracellular Matrix

Glucagon

Transcription Factors

PTf1a

Ngn3

Rfx6

Nkx2.2

MafA/B

Sox9

References

12 Congenital Hyperinsulinism

Clinical Presentation and Diagnosis

Pathophysiology of CHI

Medical Management

Histological Subtypes of Phhi

Differential of Focal and Diffuse

Surgery for CHI

Surgery for Diffuse CHI

Open Near-Total Pancreatectomy

Laparoscopic Near-Total Pancreatectomy

Surgery for Focal CHI

Focal Lesion in Head or Neck of Pancreas

Focal Lesion in Body or Tail of Pancreas

Summary

References

13 Pancreatic Neuroendocrine Tumours

Aetiology

Pathology and Classification

Clinical Presentation

Functioning PaNETs

Gastrinomas

Insulinomas

Other Functioning PNETs

Non-functioning PaNETs

Diagnosis

Biochemical Tests

Imaging

Management

Management of Functioning PaNETs

Surgical Management

Non-operative Management

Management of Non-functioning PaNETs

Outcomes and Prognosis

Acknowledgements

References

14 Islet Cell Transplantation

Islet Transplantation: A Historical Perspective

Donor Selection and Donor Availability

Human Islet Isolation

Pancreas Digestion

Density Gradient Purification

Islet Culture

The Islet Transplant Procedure

Pre-transplant Preparation

The Transplant Procedure

Post-transplant Care

Complications

Procedure-Related Complications

Immunosuppression-Related Complications

Clinical Islet Transplantation Outcomes

Indications and Patient Selection for Islet Transplantation

Pediatric Islet Allotransplantation—A Real Possibility?

Concluding Remarks

References

15 Diabetes in the Pediatric Surgical Patient

Diagnosis

Pathophysiology and Classification

Normal Glucose Homeostasis

Type 1 Diabetes

Type 2 Diabetes

Other Forms of Diabetes

Treatment of Type 1 Diabetes

Insulin Preparations and Regimens

Insulin Pumps

Blood Glucose Monitoring

Treatment of Type 2 Diabetes

Lifestyle Modification

Metformin

Sulfonylureas and Meglitinides

Thiazolidinediones

Insulin in Type 2 Diabetes

Complications of Diabetes

Diabetic Ketoacidosis (DKA)

Hyperosmolar Hyperglycemic Syndrome (HHS)

Hypoglycemia

Risk of Infection

Microvascular and Macrovascular Complications

Preoperative Evaluation

General Principles of Perioperative Management

Diabetes Management in the Fasting Patient

Intravenous Insulin Infusions

Preparation for Surgery

Perioperative Glycemic Control: Pathophysiology and Goals of Treatment

Stress and Glycemic Control

Adverse Effects of Perioperative Glucose Abnormalities

Goals for Perioperative Glycemic Control

Intraoperative Management

Postoperative Management

References

Bariatric Surgery

16 Assessing and Selecting Patients for Bariatric Surgery

Pediatric Obesity and Consequences

Treatment Approaches

Bariatric Surgery

Preoperative Assessment

Patient Selection

Reproductive Health Concerns

Conclusions

References

17 Bariatric Surgery in Children

Etiology of Obesity

Genetic Predisposition

Endocrinopathies

Environmental Factors

Psychological Factors

Management

Education

Diet

Psychological Input

Medication

Surgery

Preoperative Assessment

Surgical Options

Laparoscopic Gastric Band

Laparoscopic Sleeve Gastrectomy

Laparoscopic Gastric Bypass

Duodenal Switch

Postoperative Complications

Operative Complications

Nutritional Deficiencies

Outcomes

Conclusions

References

Further Reading

Ovary, Testicles, and Fertility

18 Pathogenesis and Treatment of Disorders of Sexual Development

Developmental and Molecular Events Contributing to Normal Mammalian Sexual Differentiation

Pathophysiology of Disorders of Sexual Differentiation (DSD)

46, XX DSD, Overandrogenization of the Genetic Female

46, XY DSD, Undervirilization of the Genetic Male

Testosterone Deficiency

Androgen Insensitivity

5α-Reductase Deficiency

Abnormalities of Gonadal Development and Differentiation

46, XY Pure Gonadal Dysgenesis DSD

Mixed Gonadal Dysgenesis (MGD) DSD

Ovotesticular DSD

Diagnosis

46, XX DSD Overandrogenization of the Genetic Female

46, XY DSD, Undervirilization of the Genetic Male

Testosterone Deficiency

Androgen Insensitivity

5α-Reductase Deficiency

Abnormalities of Gonadal Development and Differentiation

46, XY Pure Gonadal Dysgenesis

Mixed Gonadal Dysgenesis (MGD)

Ovotesticular DSD (True Hermaphroditism)

Medical Management

46, XX DSD, Overandrogenization of the Genetic Female

46, XY DSD, Undervirilization of the Genetic Male

Abnormalities of Gonadal Development and Differentiation

Surgical Treatment of Urogenital Sinus Anomalies and Disorders of Sexual Differentiation

Preoperative Evaluation of Anatomy

Preoperative Preparation

Surgical Reconstruction

Panendoscopy

Reconstruction for Female Gender Assignment

Combined Clitoroplasty, Labioplasty, and Vaginoplasty

Clitoroplasty

Labioplasty

Vaginoplasty

Flap Vaginoplasty for Low Confluence UGS

Vaginoplasty Using Urogenital Mobilization

Total Urogenital Sinus Mobilization

Partial Urogenital Sinus Mobilization

Splitting the Urogenital Sinus

Pull-Through Vaginoplasty for Mid Level Vaginal Confluence

Postoperative Care of the Female Patient

Reconstruction for Male Gender Assignment

Penoscrotal Transposition

Removal of Müllerian Duct Remnants and Creation of a Neoseminal Vesicle

Penile Agenesis

Vaginal Agenesis

Surgical Treatment of Vaginal Agenesis

References

19 Testis Embryology, Anatomy and Physiology

Testicular Embryology

Early Sexual Development

Transabdominal Testicular Migration

Inguinal-Scrotal Testicular Migration

Relationship Between Gubernaculum and Mammary Bud

Patho-Embryology of Abnormal Testicular Descent

Anatomy of the Testis

Physiology of the Testis

References

20 Surgical Considerations of Testicular Maldescent

Normal Postnatal Development of the Testis

Postnatal Development in Cryptorchidism

Timing of Orchidopexy

Acquired Cryptorchidism

Indications for Treatment

Surgical Management

Inguinal Orchidopexy

Laparoscopic Orchidopexy

Scrotal Orchidopexy

Outcomes of Orchidopexy

Short-Term Outcomes

Long-Term Outcomes

References

21 Testicular Torsion

Aetiology

Anatomy

Genetic Factors

Precipitating Factors

Incidence

Pathophysiology

Mechanisms of Tissue Damage

Ischemia

Reperfusion Injury

The Contralateral Testis

Reduced Perfusion

AutoImmune Damage

Congenital Dysplasia

Reduced Germ Cell Mass

Clinical Presentation

Differential Diagnosis

Torsion of Hydatid of Morgagni (Torsion of a Testicular Appendage)

Epididymo-Orchitis

Idiopathic Scrotal Edema

Other Causes

Investigation

Management

Outcome

Viability and Testicular Atrophy

Fertility and Semen Quality

Endocrine Function

Neonatal Torsion

References

22 Testicular Tumors in Children

Epidemiology

Risk Factors and Associations

Cryptorchidism

Disorders of Sexual Differentiation

Environmental Factors

Microlithiasis

Clinical Presentation and Evaluation

History and Physical Examination

Imaging and Other Diagnostics

Surgical Approach

Radical Orchiectomy

Testis-Sparing Surgery

Staging

Types

Germ Cell Tumors

Teratoma

Epidermoid Cysts

Yolk Sac Tumors

Gonadal Stromal Tumors

Sertoli Cell Tumor

Leydig Cell Tumor

Juvenile Granulosa Cell Tumor

Gonadoblastoma

Others

Leukemia

Hyperplastic Adrenal Rests

Post-surgical Management and Prognosis

Conclusion

References

23 Ovarian Embryology, Anatomy, and Physiology Including Normal Menstrual Physiology

Embryology of the Ovary and Ovum

Embryology of the Ovary

Embryology of the Ovum

Anatomy of the Ovary and Adnexa

Physiology of the Ovary

Menarche

The Menstrual Cycle

Abnormal Menses

Amenorrhea and Oligomenorrhea

Menorrhagia and Metrorrhagia

References

24 Ovarian Tumours in Children

Fetal and Infant Ovarian Cysts

Aetiology and Incidence

Pathology

Presentation and Differential Diagnosis

Management

Post-operative Complications and Outcomes

Ovarian Cysts in Children

Aetiology and Incidence

Presentation and Management

Outcome and Follow-Up

Ovarian Cysts in Adolescents

Aetiology and Incidence

Presentation and Management

Outcome

Ovarian Tumours

Incidence and Classification

Pathology of Benign Ovarian Tumours

Pathology of Malignant Ovarian Tumours

Presentation of Ovarian Tumours

Investigations of Ovarian Tumours

Surgery for Ovarian Tumours

Post-operative Course and Outcomes

Acknowledgements

References

25 Ovarian Torsion

Pathophysiology

Epidemiology

Etiology

Anatomic Factors in Ovarian Torsion

Hormonal Factors in Ovarian Torsion

Adnexal Abnormalities

Clinical Manifestations

Investigations

Imaging

Treatment

Outcomes

References

26 Fertility Preservation

History of Procedures to Preserve Fertility

Indications and Patient Selection for Fertility Preservation

Fertility Preservation Procedures in Males

Harvesting

Storage

Reimplantation

Fertility Preservation Procedures in Females

Harvesting

Storage

Reimplantation

Complications

Follow-Up

Outcomes of Fertility Preservation Procedures

Outcomes of Fertility Preservation Procedures in Males

Outcomes of Fertility Preservation Procedures in Females

Future Prospects

References

Breast

27 Breast Embryology, Anatomy, and Physiology

Embryology

Normal Development

Congenital Anomalies

Anatomy

Gross Structure

Blood Supply

Lymph Nodes and Nerves

Microscopic Anatomy

Breast Development and Physiology

References

28 Paediatric and Adolescent Breast Disorders

Premature Thelarche

Congenital Anomalies

Amastia and Hypomastia

Polymastia and Polythelia

Nipple Anomalies

Developmental Anomalies

Neonatal Breast Hypertrophy

Breast Asymmetry

Macromastia and Breast Hypertrophy

Tubular Breast Deformity

Gynaecomastia

Breast Infection

Mastitis

Nipple Discharge

Galactorrhoea

Bloody Discharge

Nipple Adenoma

Prepubertal Breast Masses

Haemangioma

Lymphangioma

Lipoma

Galactocele

Juvenile Papillomatosis

Breast Masses in Adolescents

Fibroadenoma

Juvenile (Giant) Fibroadenoma

Phyllodes Tumours (Cystosarcoma Phyllodes)

Hamartoma

Malignant Primary Breast Tumours

Breast Cancer Risk

Breast Carcinoma

Juvenile Secretory Carcinoma

Ductal Carcinoma

Rhabdomyosarcoma

Malignant Secondary Breast Tumours

References

29 Gynecomastia

Definition

Incidence

Aetiology

Physiological

Pathological

Pharmacological

Idiopathic/Pseudo/Obesity

Familial

Differential Diagnosis

Pathogenesis

Histopathology

Diagnosis

Classification

Treatment

Neonatal

First Decade

Second Decade

Pharmacotherapy

Surgery

Liposuction Alone

Excisional Surgery

Nodular Excision

Concentric Ring Approach for Larger Excision

Psychosocial Considerations

References

Miscellaneous

30 Multiple Endocrine Neoplasia Type 1 and Type 4

Multiple Endocrine Neoplasia Type 1 (MEN1)

Clinical Features

Diagnosis and Treatments of MEN1-Associated Tumours

Parathyroid Tumours

Pancreatic Islet Neuroendocrine Tumours (Nets)

Anterior Pituitary NETs

Adrenal Cortical Tumours

Thymic, Bronchopulmonary and Gastric Nets

Treatment of NETs in Patients with MEN1 Versus NETs in Sporadic Patients

Molecular Genetics of MEN1

Multiple Endocrine Neoplasia Type 4

Clinical Features of the MEN4 Syndrome

Diagnosis and Treatments of MEN4-Associated Tumours

Parathyroid Tumours

Anterior Pituitary NETs

Molecular Genetics of MEN4

Conclusions

References

31 Multiple Endocrine Neoplasia Type 2

Multiple Endocrine Neoplasia 2A

Multiple Endocrine Neoplasia 2B

Familial Medullary Thyroid Cancer

Genetics

Genotype–Phenotype Correlations

Genetic Screening

Diagnosis and Management

Medullary Thyroid Cancer

Pheochromocytoma

Primary Hyperparathyroidism

Hirschsprung’s Disease

Cutaneous Lichen Amyloidosis

References

Pituitary

32 Pituitary Gland Embryology, Anatomy and Physiology

Anatomy of the Pituitary Gland

Anterior Pituitary

Posterior Pituitary

Blood Supply of the Pituitary

Embryology of the Pituitary

Physiology of the Pituitary

Growth Hormone (GH)

Prolactin (PRL)

Adrenocorticotropic Hormone (ACTH)

Melanocyte-Stimulating Hormone (MSH)

Thyroid-Stimulating Hormone (TSH)

Gonadotrophins—Follicle-Stimulating Hormone (FSH) and Luteinising Hormone (LH)

References

33 Surgical Considerations of the Pituitary

Pituitary Lesions

Preoperative Evaluation

Surgical Goals

History of Surgical Treatment of Sellar Tumors

Surgical Approaches to the Pituitary

Transsphenoidal Approaches

Complications

References

34 Erratum to: Ovarian Tumours in Children

Erratum to:Chapter 24 in: D.J. Ledbetter and P.R.V. Johnson (eds.), Endocrine Surgery in Childre

Index

An aparitie	16 Aug 2017
Autor	Daniel J. Ledbetter, Paul R.V. Johnson
Dimensiuni	178 x 254 mm
Editura	Springer
Format	Paperback
ISBN	9783662542545
Limba	Engleza
Nr pag	462