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Neuroblastoma

Neuroblastoma is the most common solid extracranial malignancy of childhood and the most common malignant tumor in infants. The overall incidence of neuroblastoma is 1 per 100,000 children in the United States, thereby accounting for 7–10% of all malignancies diagnosed in patients younger than 15 years of age. Yet neuroblastoma is responsible for approximately 15% of all childhood cancer deaths. Neuroblastoma is a heterogeneous disease. Tumors…

Renal Tumors

Renal tumors are the second most common abdominal tumor seen in infants and children behind neuroblastoma. They represent a wide spectrum from benign to extremely malignant tumors ( Box 64.1 ). These tumors include Wilms tumor (WT) (also referred to as nephroblastoma or renal embryoma), renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), rhabdoid tumor of the kidney (RTK), congenital mesoblastic nephroma (CMN),…

Principles of Adjuvant Therapy in Childhood Cancer

The significant improvement in cure rates for pediatric malignancies over the past 30 years could not have occurred without the development of multimodality therapy and the cooperative efforts of surgeons, pediatric oncologists, and radiation oncologists. In the 1940s, with the use of extensive surgical resection, only 20% of children with localized solid tumors were cured of their malignancies. However, with the discovery of effective chemotherapeutic agents,…

Differences of Sexual Development

Differences of sexual development (DSD), congenital conditions in which the development of the infant/child’s chromosomal, gonadal, and anatomic sex are atypical, are among the most fascinating conditions confronting the pediatric urologist, gynecologist, and surgeon. Our understanding of these conditions and their causes continues to evolve, but gender assignment and the timing of reconstruction remain controversial. Normal Gender and Sexual Differentiation The most commonly accepted paradigm, described…

Prune Belly Syndrome

Prune belly syndrome (PBS), also named Eagle-Barrett syndrome, is a rare multisystem congenital disorder with a triad presentation of urinary tract dilation, deficient abdominal wall musculature ( Fig. 61.1 ), and bilateral undescended testes. Frohlich (1839) first described the distinct abdominal wall, and then Parker (1895) described the full triad of anomalies. The term prune belly syndrome was first coined by Osler in 1901. There is…

Circumcision

Circumcision (removal of the redundant prepuce) is one of the most frequently performed surgical procedures in the world. There is a wide variability in the rate of circumcision among different populations. A lack of consensus regarding the function of the foreskin and uncertainty regarding the benefits of circumcision has led to controversy regarding the appropriateness of elective circumcision. The most recent policy statement from the American…

Hypospadias

Hypospadias is the second most common congenital abnormality of the genitourinary tract in males after cryptorchidism. It is characterized by a urethral meatus that opens on the ventral surface of the penis proximal to the end of the glans. The meatus may be located anywhere along the length of the penis from the glans to a proximal location as low as the perineum. Ventral curvature of…

Bladder and Cloacal Exstrophy

The exstrophy–epispadias complex (EEC) is a spectrum of embryologic abnormalities. Diagnoses within the EEC range in severity from those involving only one organ to others that are a part of a larger complex of defects. The spectrum includes: ▪ Epispadias—the urethra is a partial or completely open “plate” dorsally on the penis or between the clitoral halves in a girl. ▪ Classic bladder exstrophy (BE)—the urinary…

Posterior Urethral Valves

Posterior urethral valves (PUVs) are the most common cause of lower urinary tract obstruction (LUTO) in boys, with an incidence of 1 in 5000–8000 male births. Although the majority of boys with PUV are diagnosed antenatally, approximately one-third will be diagnosed during childhood or adolescence. Once diagnosed and surgically treated, PUV requires lifelong follow-up since it is the most common cause of LUTO leading to end-stage…

Bladder and Urethra

The bladder and urethra normally function as a coordinated unit to store and discharge urine from the body. Both structural and functional disorders of the bladder or urethra can be responsible for bleeding, incontinence, infection, discomfort, pain, and obstruction that can cause upper tract deterioration to the point of compromising renal function. This chapter focuses on the major diseases and dysfunctional conditions of the bladder and…

Urinary Tract Infections and Vesicoureteral Reflux

Acknowledgments We thank Dr. Andrew J. Kirsch of Georgia Urology for the use of his video on the Deflux injection procedure and Dr. Charles Concodora and Dr. Paul Noh of Cincinnati Children’s Hospital for the use of their video of a robotic-assisted laparoscopic ureteral reimplantation. Urinary Tract Infections Urinary tract infections (UTIs) are a common and significant source of morbidity in children. By age 7, approximately…

Ureteral Obstruction and Malformations

Hydronephrosis and ureteral malformations are among the most common anomalies in the urinary tract in children. Many of these conditions are now detected prenatally. Urinary tract dilation is present in 1 in 100 fetuses, but significant uropathy is found in only 1 in 500 ( Fig. 54.1 ). Open full size image Fig. 54.1 Normal kidneys are typically identifiable by 18 weeks in all fetuses. Dilated…

Developmental and Positional Anomalies of the Kidneys

Anomalies of renal formation and position result in interesting radiographs, but their clinical importance lies in their associated conditions. For example, the multicystic dysplastic kidney (MCDK) often involutes, yet the initial evaluation needs to determine if the contralateral kidney is at risk for vesicoureteral reflux (VUR) or ureteropelvic junction (UPJ) obstruction. Although no therapy is needed for unilateral renal agenesis, the link between a solitary kidney…

The Acute Scrotum

The term acute scrotum is defined as sudden onset scrotal pain with or without swelling and erythema. Early recognition and prompt management are imperative because of the possibility of testicular torsion as the etiology with permanent ischemic damage to the testis. Box 52.1 lists the differential diagnoses for the acute scrotum. Although most conditions are nonemergent, prompt differentiation between testicular torsion and other causes is critical.…

Undescended Testes and Testicular Tumors

Undescended Testes Normal testicular descent relies on a complex interplay of numerous factors. Any deviation from the normal process can result in a cryptorchid or undescended testis (UDT) ( Fig. 51.1 ). UDT is a common abnormality that can have adverse fertility and malignancy implications. Embryology Testicular development and descent depend on a coordinated interaction among endocrine, paracrine, growth, and mechanical factors. Bipotential gonadal tissue located…

Inguinal Hernia

Inguinal hernias have been documented since the dawn of recorded history. The term “hernia” comes from the Greek hernios, meaning offshoot or bud. Because an inguinal hernia is present in many different species of animals, particularly primates, prehistoric humans likely suffered from this condition as well. The Egyptian Ebers papyrus from 1550 bc describes the diagnosis and treatment of an inguinal hernia. Also, mummies (e.g., Ramses…

Umbilical and Other Abdominal Wall Hernias

Umbilical Hernia Umbilical hernia is a common disorder in children and is frequently evaluated and treated by pediatric and general surgeons. Unlike other hernias of childhood, a fascial defect is present at birth but may resolve without the need for an operation. An understanding of the embryology, anatomy, incidence, natural history, and complications is important to any surgeon managing umbilical hernias in children. Anatomy After birth,…

Congenital Abdominal Wall Defects: Gastroschisis and Omphalocele

The two primary congenital abdominal wall defects are omphalocele and gastroschisis. Although often considered together, they are distinct and separate entities in every way from their etiology to management principles. Differences between gastroschisis and omphalocele are illustrated in Figure 48.1 and summarized in Table 48.1 . Open full size image Fig. 48.1 These two photographs nicely depict the differences between an omphalocele and gastroschisis. (A) In…

Splenic Conditions

The essential role of the spleen in the defense against bacterial organisms is well documented. King and Schumacker first described the susceptibility of splenectomized infants to infection in 1952. The immunologic role of the spleen led pediatric surgeons to initiate a nonoperative approach to splenic injuries in children, which has evolved into the preferred method for treating children and also adults. In the modern era, splenic…

Lesions of the Pancreas

The pancreas originates during week 4 of gestation as dual evaginations from the foregut endoderm. The dorsal pancreatic bud gives rise to the body and tail of the pancreas, its minor duct (Santorini) and papilla, and the continuation of the major duct (Wirsung) into the body and tail. The ventral pancreatic bud arises from the biliary diverticulum and swings around the dorsal aspect of the duodenal…