91 HEART TRANSPLANTATION
Daniel R. Meldrum M.D., Azad Raiesdana M.D., Jeffrey A. Breall M.D., John W. Brown M.D.
1. Who performed the first experimental heart-lung transplant?
Show answer
Alexis Carrel, a French-born American surgeon, developed the vascular techniques required for heart-lung transplantation and performed the first experimental heart-lung transplant in 1907. He transplanted the lungs, heart, aorta, and vena cava of a 1-week-old cat into the neck of a large adult cat. For devising the technique of vascular anastomosis and other outstanding accomplishments, Carrel received the Nobel Prize in 1912 (the first Nobel Prize awarded to a scientist working in an American laboratory).
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KIDNEY AND PANCREAS TRANSPLANTATION
CONTROVERSIES
13. Is HLA (human leukocyte) matching still important?
Show answer
It is somewhat important. Historically, HLA matching was an important consideration when matching cadaver kidneys to recipients. With today’s improved immunosuppressive agents, many transplant surgeons believe that HLA matching is no longer critical. Six antigen match kidneys are still shared nationally and do enjoy some improvement in long-term graft survival. Donor organ quality remains the primary determinant in how well the transplanted organ functions. For example, a poorly matched living-donor kidney will still usually outlast a well-matched cadaveric kidney.
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Kidney & Pancreas Transplantation
90 KIDNEY AND PANCREAS TRANSPLANTATION
Thomas E. Bak M.D., Michael E. Wachs M.D., Igal Kam M.D.
1. What are the most common indications for kidney transplantation?
Show answer
End-stage renal disease (ESRD) caused by hypertension, diabetes, glomerulonephritis, and polycystic kidney disease.
Liver Transplantation. Controversies
CONTROVERSIES
12. Should liver transplants be performed in individuals with alcoholic liver disease?
Show answer
Transplant centers have strict criteria that alcohol-induced liver transplant recipients must undergo extensive psychological testing and abstain from alcohol before being placed on the waiting list. The recidivism rate (i.e., transplant patients who start drinking again) remains low. Financially, the cost is comparable, if not lower, than continued medical management of end-stage liver disease. We currently do provide care for other self-inflicted medical problems, such as cigarette smokers. The public must realize that people are not being pulled off bar stools and taken to the hospital for their transplant.
Liver Transplantation
89 LIVER TRANSPLANTATION
Thomas E. Bak M.D., Michael E. Wachs M.D., Igal Kam M.D.
1. When and where was the first liver transplant performed?
Show answer
Dr. Thomas Starzl performed the first operation on March 1, 1963, at the University of Colorado in Denver.
2. Is liver transplantation considered a safe and effective operation?
Show answer
Yes. Although still a major operation with significant risks, patient and graft survival have continuously improved. One-year survival should be well over 90% in major centers.
3. What are the most common indications for liver transplantation in the United States?
Congenital Cysts & Sinuses Of The Neck
88 CONGENITAL CYSTS AND SINUSES OF THE NECK
Frederick M. Karrer M.D., Denis D. Bensard M.D.
1. What are branchial cleft anomalies?
Show answer
Cysts, sinuses, and fistulas that result from incomplete obliteration of the first, second, or third branchial clefts, and are present in early fetal development.
Abdominal Tumors. Controversy
CONTROVERSY
6. Should patients with hepatoblastoma receive preoperative chemotherapy to shrink the tumors?
Abdominal Tumors
87 ABDOMINAL TUMORS
Frederick M. Karrer M.D., Denis D. Bensard M.D.
1. What are the most common malignant solid abdominal tumors in children?
Show answer
Neuroblastomas, Wilms’ tumors, and hepatoblastomas, in that order. Neuroblastomas are derived from neural crest tissue; in the abdomen, they originate from the adrenal glands and paraspinal sympathetic ganglia. Wilms’ tumor (nephroblastoma) derives from the kidney, and hepatoblastomas originate in the liver.
2. Is it tough to differentiate Wilms’ tumor from neuroblastomas clinically?
Show answer
Yes. Both tumors present as an asymptomatic abdominal mass. The differences are summarized in Table 87-1. In addition, because neuroblastomas produce hormones, affected children may exhibit flushing, hypertension (catecholamine release), watery diarrhea, periorbital ecchymosis, and abnormal ocular movements.
Table 87-1. DIFFERENTATION BETWEEN WILMS’ TUMOR AND NEUROLASTOMA
|
Wilms’ Tumor
|
Neuroblastoma
|
|
|
Age at presentation
|
3-4 yr
|
1-2 yr
|
|
Extend across midline
|
Rare
|
Common
|
|
Surface on palpation
|
Smooth
|
Knobby
|
|
X-ray calcifications
|
No
|
Yes
|
3. How are Wilms’ tumors and neuroblastomas treated?
Table 87-2. TREATMENT OF WILMS’ TUMOR AND NEUROBLASTOMA
|
Wilms’ Tumor
|
Neuroblastoma
|
|
|
Primary surgical excision
|
Important (likely)
|
Important (less likely)
|
|
Chemotherapy
|
Enormous impact
|
Less responsive
|
4. What are the major prognostic factors in neuroblastomas and Wilms’ tumor?
In neuroblastomas, age at presentation is the major prognostic factor. Children younger than 1 year have an overall survival rate > 70%, whereas the survival rate for children older than 1 year is < 35%. Shimada proposed a prognostic classification based on evaluation of histologic parameters (tumor differentiation, mitosis-karyorrhexis index [MKI]) as well as age. Aneuploid tumors, tumors with low MKI, and tumors with < 10 copies of the n-myc gene also have better outcomes.
Age is also important in children with Wilms’ tumors, but the prognosis is better because the tumors are more readily excised and much more sensitive to chemotherapy.
5. What are the differences between hepatoblastomas and hepatocellular carcinomas? How are the tumors treated?
Hepatoblastomas usually occur in infants and young children, whereas hepatocellular carcinoma usually occurs in children older than 10 years. Hepatocellular carcinoma usually is associated with cirrhosis and hepatitis B and is histologically identical to the adult form. Surgical resection is the primary therapy for both tumors. Hepatoblastomas often have a good response to adjunctive chemotherapy, whereas hepatocellular carcinoma rarely responds to chemotherapy.
Congenital Diaphragmatic Hernia
86 CONGENITAL DIAPHRAGMATIC HERNIA
Denis D. Bensard M.D., Richard J. Hendrickson M.D.
1. What is the most common type of congenital diaphragmatic hernia (CDH)?
Show answer
Congenital abnormalities of the diaphragm include a posterolateral defect (Bochdalek hernia), an anteromedial defect (Morgagni hernia), or the eventration (central weakening) of the diaphragm. The Bochdalek hernia is the most common variant and generally occurs on the left (80%). Approximately 20% occur on the right, and < 1% are bilateral.
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Tracheoesophageal Malformations
85 TRACHEOESOPHAGEAL MALFORMATIONS
Denis D. Bensard M.D., David A. Partrick M.D.
1. What are tracheoesophageal fistula (TEF) and esophageal atresia (EA)?
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The trachea and esophagus appear as a ventral diverticulum arising from the primitive foregut during the third week of gestation. The trachea and esophagus undergo separation by the ingrowth of ectodermal ridges during the fourth week of gestation. Failure of separation results in anomalous connection of the trachea to the esophagus (i.e., TEF) with or without incomplete formation of the esophagus (i.e., EA).
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Imperforate Anus
84 IMPERFORATE ANUS
Frederick M. Karrer M.D., Denis D. Bensard M.D.
1. What is imperforate anus?
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It is a congenital defect in which the opening of the anus is absent or misplaced, usually fistulizing anteriorly to the perineum or genitourinary (GU) tract. Anorectal malformations range from slight anterior malpositioning of the anus to complex cloacal deformities. Children with anorectal malformations commonly have other congenital anomalies, such as the VACTERL association.
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Intestinal Obstruction Of Neonates & Infants
83 INTESTINAL OBSTRUCTION OF NEONATES AND INFANTS
Richard J. Hendrickson M.D., Denis D. Bensard M.D.
1. What signs or symptoms suggest intestinal obstruction in the neonate?
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Signs and symptoms vary according to the level of obstruction. Proximal intestinal obstruction leads to the early onset of bilious emesis, generally with minimal abdominal distention. In contrast, neonates with distal intestinal obstruction present after the first day of life with bilious vomiting and pronounced abdominal distention. Bilious emesis should always be interrogated further in infants and children.
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Hypertrophic Pyloric Stenosis
82 HYPERTROPHIC PYLORIC STENOSIS
Denis D. Bensard M.D.
1. What is pyloric stenosis?
Show answer
Hypertrophic pyloric stenosis (HPS) is idiopathic thickening and elongation of the pylorus that produces gastric outlet obstruction. HPS is the most common surgical cause of nonbilious vomiting in infants. Offspring of an affected parent have an increased incidence of HPS (10%); the highest rate (20%) occurs in boys born to affected mothers.
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Dissecting Aortic Aneurysm. Controversies
CONTROVERSIES
13. Which is preferred: surgical or medical management of descending dissections?
Show answer
* Initial surgical managementApproximately 25% of patients initially treated medically need an operation eventually.
* Operative mortality is much lower today (20%) than in the past.
* Medical management has the same in-hospital mortality (20%).
* Initial medical managementThis avoids unnecessary operation and its attendant cost and complication rate.
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Dissecting Aortic Aneurysm
81 DISSECTING AORTIC ANEURYSM
Laurence H. Brinckerhoff M.D., David N. Campbell M.D.
1. Why is the term dissecting aortic aneurysm really incorrect?
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The correct term should be dissecting aortic hematoma because the lesion is not an aneurysm. Blood dissects between the middle and outer layers of the media and adventitia of the aorta (specifically, there does not need to be an intimal tear, although there usually is).
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Abernathy’s Surgical Secrets, Updated Edition (Book w / Student Consult)
Author / s: Harken Alden H., Abernathy Charles, Moore Ernest Eugene
Year: 2004
Pages: 473
Publishers: Elsevier Mosby; 5th Bk & Acc edition
ISBN: 0323034160
