A 6-week-old boy has a 6-day history of vomiting, intermittent fussiness, periods of inconsolability, and a 0.3-kg weight loss. On examination his anterior fontanelle is flattened and his mucous membranes are dry. Abdominal examination is unremarkable. Laboratory data are as follows: Na, 130; Ch, 87; CO2, 30. An ultrasound of the abdomen reveals a 3.5 mm × 12 mm pylorus. The next most appropriate step in management of this child would be:
B. Esopahgeal pH monitoring
C. Initiate proton pump inhibitor therapy
D. Order an immediate small bowel follow-through study
E. Begin IV fluid resuscitation
The age of this child’s presentation should exclude diagonoses of duodenal atresia (which typically present earlier) and the clinical presentation, as well as age, may exclude later presenting pathologies, such as intussusception. GERD may be suspected. However, in light of weight loss, dehydration, and electrolyte derangement, other diagnosis must be excluded. This child presents with metabolic alkalosis and subclinical findings on ultrasound of pyloric stenosis (typically 4 mm × 14 mm pylorus effectively clenches the diagnosis). The first goal should be correction of the electrolytes and close monitoring.
A 32-week gestational male presents with persistent cough when feeding and spits up after every feed. An abdominal radiograph reveals gasless stomach. Tracheoesophageal fistula is suspected. Appropriate workup should include:
A. Ultrasound of the back
B. Ultrasound of the kidneys
TEF can be seen in the VACTERL association and additional anomalies should be ruled out.
Vertebral anomalies: Defects of the spinal column usually consist of hypoplastic vertebrae or hemivertebra. 70% of patients with VACTERL association will have vertebral anomalies.
Imperforate Anus: Anal atresia or imperforate anus is seen in about 55% of patients with VACTERL association.
Cardiac defects: Up to 75% of patients with VACTERL association have been reported to have congenital heart disease. The most common heart defects seen with VACTERL association are ventricular septal defect, atrial septal defects, and tetralogy of Fallot.
Tracheo-Esophageal fistula: EA with TEF is seen in about 70% of patients with VACTERL association, although it can frequently occur as an isolated defect.
Renal anomalies: Renal defects are seen in approximately 50% of patients with VACTERL association. In addition, up to 35% of patients with VACTERL association have a single umbilical artery. These defects can be severe with incomplete formation of one or both kidneys or urologic abnormalities such as obstruction of outflow of urine from the kidneys or severe reflux of urine into the kidneys from the bladder. These problems can cause kidney failure early in life and may require kidney transplant. Many of these problems can be corrected surgically before any damage can occur.
Limb anomalies: Limb defects occur in up to 70% of babies with VACTERL association and include a displaced or hypoplastic thumb, polydactyly, syndactyly, and forearm defects such as radial aplasia.
A two-day-old 34-week gestational female with Down syndrome presents with feeding intolerance and forceful bilious vomiting. A ‘babygram’ demonstrates two large gas-filled structures in the upper abdomen (presumed stomach and duodenum). What is the most appropriate surgical option?
Approximately 20% to 40% of all infants with duodenal atresia have Down syndrome and approximately 8% all infants with Down syndrome have duodenal atresia. Forceful bilious vomiting is commonly seen and the diagnosis of duodenal atresia is usually confirmed by radiography. A duodenodeuodenostomy is the surgical Rx of choice in these cases because it provides physiologic continuity of the GI tract.
What is the most common childhood malgnancy?
The most common solid tumor in children is neuroblastoma. However, the most common overall malignancy is leukemia (40% of all childhood malignancies).
A 4-day-old 28-week gestational male develops increasing abdominal distention and feeding intolerance. His nurse notes scant blood with the patient’s last bowel movement. An abdominal radiograph reveals portal venous gas and pneumatosis intestinalis. An orogastric tube is placed and IV antibiotic therapy is initiated. All of the following are indications for immediate operation except:
A. Worsening pneumatosis intestinalis and portal venous gas
C. Abdominal wall erythema
D. Positive paracentesis cultures
Initial therapy for NEC is medical; however, the pathologic process may progress to frank peritonitis and systemic sepsis. Close monitoring of children with NEC is required.
You see a 4-year-old boy regarding an abdominal mass. His parents have noted that he has been increasingly lethargic over the past several weeks. They think he is pale, and he has recently started to take long afternoon naps. His appetite has been poor, and he has lost 4 pounds in the last 2 months.
Upon physical examination, the child is thin and pale. His lungs are clear with equal breath sounds. His heart tones are normal. His abdomen is quite full. You feel a large mass under the left costal margin that is firm and immobile.
The most likely diagnosis is:
The most common solid abdominal tumors of childhood are Wilm tumor and neuroblastoma. Patients with Wilm tumor are typically asymptomatic and present when they are found to have a large abdominal mass on routine examination. Children with neuroblastoma, on the other hand, typically present with a variety of constitutional symptoms.
Which of the following factors influence this patient’s prognosis?
The age of the patient at the time of diagnosis has a profound influence on outcome for neuroblastoma. Survival rates for children with neuroblastoma under 1 year of age are between 70% and 90%, whereas survival rates for children older than 1 year are 20% to 30%. Much of this discrepancy is explained by the unusual maturation seen in neuroblastoma in infancy.
Which of the following are associated with an increased incidence of neuroblastoma?
A. Maternal phenytoin use
B. Fetal alcohol syndrome
D. Beckwith-Weidermann syndrome
Hirschsprung disease is a disorder of ganglion cell migration in the large intestine. These ganglion cells are thought to be neural crest in origin. Beckwith-Weidermann syndrome results in an increased incidence of a number of abdominal tumors, including Wilm tumor, neuroblastoma, hepatoblastoma, and adrenocortical carcinoma. Fetal alcohol syndrome and maternal Dilantin use have been found to correlate with neuroblastoma by unknown mechanisms.
Which of the following should be included in the initial evaluation of patients with neuroblastoma?
Bone marrow biopsy should be obtained in order to rule out any metastatic disease in the case of neuroblastoma.
If bone marrow did not reveal small round blue cells consistent with neuroblastoma. Which of the following is the most appropriate Rx for this 4-year-old boy?
A. Fine-needle aspiration cytology under local anesthesia
B. Wide resection of the mass with resection of all contiguous structures
C. Laparotomy and tumor biopsy followed by chemotherapy
D. Limited resection with preservation of all vital structures followed by chemotherapy
E. Chemotherapy without biopsy
Neuroblastoma is commonly metastatic to the bone marrow. In fact, the diagnosis can often be made by means of bone marrow examination without biopsy of the primary tumor. In this particular case, the best procedure would be small laparotomy with tumor biopsy for histologic and cytogenetic analysis. This information would then be used to guide most appropriate course of chemotherapy.