Alfred A. de Lorimier

Correction of congenital hydronephrosis in utero II. Decompression reverses the effects of obstruction on the fetal lung and urinary tract

Urethral obstruction and urachal ligation at 93-107 days gestation produced severe hydronephrosis, hydroureter, megacystis, and urinary ascites as well as significant pulmonary hypoplasia in 17 fetal lambs. Obstructions in 9 fetuses subsequently were relieved in utero by suprapubic cutaneous cystostomy. At birth, all 4 liveborn obstructed lambs had respiratory insufficiency, and only 1 survived. Four others were stillborn. The lungs were significantly hypoplastic by weight and volume (p less than 0.025). All 8 had advanced megacystis, hydroureter, and hydronephrosis but no cystic or dysplastic renal changes. In contrast, 7 liveborn lambs diverted in utero had far less respiratory difficulty and all survived (p = 0.002). Two were stillborn. The lung weight was significantly increased (p less than 0.05). All lambs undergoing in utero decompression showed significant resolution of the severe urinary tract dilatation seen in the obstructed lambs. In utero decompression of the obstructed fetal urinary tract allows the abnormally small lungs to grow and develop and hydronephrosis to resolve.

Management of the fetus with congenital hydronephrosis

Twenty-six fetuses with dilated urinary tracts were studied with serial sonograms. Eight fetuses with unilateral hydronephrosis were followed without intervention; all are well after postnatal surgical correction. Three cases of bilateral hydronephrosis resolved spontaneously before birth. Eight fetuses with bilateral hydronephrosis had evidence of poor function: Three were not treated and died shortly after birth with small lungs and dysplastic kidneys; three others had diagnostic intervention that demonstrated irreversible disease and allowed termination of the pregnancy; two had obstruction successfully corrected in utero, but renal damage proved irreversible and precluded survival at birth. Seven fetuses with bilateral hydronephrosis and equivocal function underwent early decompression. Four were delivered early and corrected ex utero; 1 has renal failure and the other 3 are well. Three had obstruction relieved in utero by a catheter shunt placed percutaneously; 1 had multiple anomalies and died; the other 2 are well. Serial sonographic observation improves perinatal management of the fetus with a dilated urinary tract. The need for diagnostic or therapeutic intervention depends on the type and severity of obstruction and the time in gestation when it is discovered. Most fetuses do not require treatment before birth; a few may benefit from early decompression in or ex utero.

Sclerotherapy for venous malformations

Of the congenital vascular abnormalities, venous malformations receive little attention and essentially no discussion of treatment. The author describes a 30-year experience with sclerotherapy, which was used for 34 venous malformations. In some cases, these lesions are localized and can be excised, but all the patients in this series had such extensive involvement of adjacent organ systems that no other treatment than sclerotherapy was tenable. Five patients had Klippel-Trenaunay Syndrome, five had head and neck involvement, two had involvement of the entire left side and the remainder had other areas affected. Sodium morrhuate, ethanolamine, sotradecol, and absolute ethyl alcohol were the sclerosing agents used. A butterfly needle was inserted into an anomalous vein, and a three-way stopcock connected to saline and the sclerosing solution was used to ensure intraluminal injection. When rapid runoff into normal venous tributaries could be a concern, a venogram on the operating table preceded injection of the sclerosing solution. Small lesions required only one treatment; widespread bulky lesions required more than 30 injections. The volume of sclerosing solution varied from 5 to 90 mL per injection course. Because of pain, general anesthesia and an overnight hospital stay were necessary. Patients with pharyngeal and/or laryngeal involvement required preliminary tracheostomy or endotracheal ventilatory support for 3 days. Complications included skin necrosis, transient nerve palsy, hemoglobinuria, and one case of anaphylaxis. Repeated aggressive treatment was required for the very large malformations because recanalization occurred. All the patients have been very satisfied with the results.

Correction of congenital hydronephrosis in utero I. The model: Fetal urethral obstruction produces hydronephrosis and pulmonary hypoplasia in fetal lambs†

In an attempt to simulate congenital hydronephrosis secondary to urethral obstruction, we evaluated the consequences of obstructing the urethra in fetal lambs by several techniques. When the urethra was ligated before 108 days gestation, the bladder decompressed through the urachus and the upper-urinary tract developed normally. Ligation of both the urethra and urachus produced gross bilateral hydronephrosis, hydroureter, and megacystis, as well as severe pulmonary hypoplasia, and was associated with a high perinatal mortality. Intermittent urethral obstruction produced with a balloon constrictor or by diverting urine externally and pressure-limited obstruction produced with a Pudenz valve both proved unsatisfactory for producing congenital hydronephrosis. Finally, ligation of the urachus and progressive gradual obstruction of the urethra with an ameroid constrictor produced hydronephrosis and pulmonary hypoplasia. This model simulates urethral obstruction in the human fetus and produces a clinical and pathologic picture similar to that seen in infants born with congenital hydronephrosis.

Desmoid tumors: Local control and patterns of relapse following radiation therapy☆☆☆

Desmoid tumors are benign neoplasms, arising from musculoaponeurotic tissues, which tend to be locally infiltrative, resulting in a high rate of local recurrence following surgical resection. Nineteen patients with desmoid tumors underwent radiation therapy at the University of California, San Francisco, between 1970 and 1980. Fifteen patients were referred with local recurrence following one or more surgical resections. Three patients were referred for initial radiation therapy with unresectable tumors, and one patient received planned postoperative irradiation following subtotal tumor resection. At the time of treatment, 8 patients had nonresectable disease measuring greater than 10 cm. Five patients had residual tumor masses measuring 4 to 6 cm, and six had only microscopic disease following resection. The majority of patients were treated to a tumor dose of 50-55 Gy at 1.6 to 1.8 Gy per fraction. With a median follow-up of 8 years, 13 patients remained free of recurrent disease following radiation therapy. The 5 year relapse free survival was 72% with 10 patients continuing to be free of disease 5 to 11 years following therapy. Local control was not related to the amount of disease present at the time of treatment. Of the 6 patients who developed recurrent disease, only 1 patient had a true in-field recurrence. Four patients recurred at the margin of the radiation field 1 to 5 years following therapy. Of these four patients, 3 were successfully salvaged while 1 died as a result of tumor extension into a major vessel. One patient with an extensive mesenteric mass did not respond to therapy and died 1 month post irradiation. The patient with the in-field recurrence and 1 patient with a marginal recurrence were successfully treated with combination chemotherapy. Moderate dose radiation therapy to desmoid tumors can result in lasting local control when surgical resection is not possible. Post operative radiation can improve the rate of local control for patients with a high risk of recurrence. As desmoid tumors tend to be locally infiltrative, fields must be very generous to prevent marginal recurrence. Systemic chemotherapy offers an alternative to ablative surgery in the event of local failure following radiation therapy.

Correction of congenital diaphragmatic hernia in utero. III. Development of a successful surgical technique using abdominoplasty to avoid compromise of umbilical blood flow

Infants born with congenital diaphragmatic hernia (CDH) die because their lungs are hypoplastic. In the fetal lamb, the lung made hypoplastic by compression with an intrathoracic balloon can, if decompressed in utero, grow and develop enough to permit survival at term. To study the efficacy and feasibility of in utero repair of CDH, we created diaphragmatic hernias in fetal lambs at approximately 100 days gestation and corrected them surgically at approximately 120 days. Repair of the diaphragm with closure of the abdomen resulted in postoperative fetal death in six lambs. Acute studies demonstrated that increased intraabdominal pressure compromises blood flow in the umbilical vein and produces severe fetal distress. When a silastic patch was used to enlarge the abdomen after reduction of the viscera and repair of the diaphragm, six of nine lambs were viable after term delivery. In sacrificed lambs, the lungs were well expanded, mature histologically, and greatly increased in size. Correction of CDH in utero appears physiologically sound and technically feasible.

Prognosis of posterior urethral valves presenting at birth

Urethral obstruction due to posterior urethral valves (PUV) is a more lethal condition in the newborn than is commonly recognized. To study its prognosis when the diagnosis of PUV is made shortly after birth, cases of PUV presenting in the first week of life were reviewed. Eleven cases were seen at the University of California, San Francisco from 1974 to 1982. Five babies died, three within two hours of birth of respiratory insufficiency secondary to severe pulmonary hypoplasia, and two within three weeks with rapidly progressive renal failure. Of the six survivors, four exhibited respiratory difficulty (prolonged ventilatory support required in two, and bilateral pneumothoraces in two), three developed renal failure despite early urinary diversion, and one had renal tubular dysfunction. Only two infants had normal renal function after undergoing a successful series of reconstructions. Prune-belly syndrome and limb deformities were present in two surviving and two fatal cases. Although most cases of congenital hydronephrosis can be successfully treated after birth, those presenting in the newborn period represent a subset with high morbidity and mortality. With advances in prenatal diagnosis of congenital hydronephrosis, salvage of severe cases detected early in gestation may require more aggressive management in the prenatal period.

Fetal treatment 1982.

Perinatal obstetricians, surgeons, ultrasonographers, pediatricians, bioethicists, and physiologists from centers active in fetal treatment (13 centers in 5 countries) gathered at Santa Ynez Valley...

Alcohol, wine, and health

Abstract Background: For the past 20 years numerous epidemiological studies have correlated the consumption of alcohol and a variety of disease states: overall mortality, arteriosclerotic vascular diseases, hypertension, cancers, peptic ulcer, respiratory infections, gall stones, kidney stones, age-related macular degeneration, bone density, and cognitive function. Methods: A review of these articles reveals that each of these studies has compared the outcome of individuals at various levels of alcohol consumption with that of abstainers. Results: Each analysis has identified a U-shaped or J-shaped curve of reduced relative risk for a given disease state compared with abstainers. A clear definition of consumption in moderation becomes evident: for men it should not exceed 2 to 4 drinks per day, and for women it should not exceed 1 to 2 drinks per day. Conclusions: Alcohol by itself has favorable effects on the level of high-density lipoprotein cholesterol, and inhibition of platelet aggregation. Wine, particularly red wine, has high levels of phenolic compounds that favorably influence multiple biochemical systems, such as increased high-density lipoprotein cholesterol, antioxidant activity, decreased platelet aggregation and endothelial adhesion, suppression of cancer cell growth, and promotion of nitric oxide production.

Reconstructive surgery for obstructing lesions of the intrathoracic trachea in infants and small children

This is a report of ten infants and small children with congenital obstructive lesions of the distal trachea and main bronchi. Four were successfully resected. One with a distal segment stenosis required tracheal resection at age 6 wk, another with stenosis of the distal half of the trachea at age 18 mo, and 2 (1 with distal stenosis and 1 with tracheal hamartoma) at age 4 yr. All 4 are presently free of symptoms and their anastomoses have grown without stricture. A child with coil-spring mucosal stenosis of the left main bronchus developed an excellent airway following bronchoscopic removal of the folds, and a baby with tracheomalacia was successfully treated with a rib splint on a segment of distal tracheomalacia, but she died later of associated cyanotic congenital heart disease. Four babies died with airway obstruction in the newborn period. Two with critical distal stenoses died before tracheal reconstruction could be performed. Two died following emergency resections in which all of the congenital stenosis could not be removed. In both, stenotic trachea remained despite operation. All of these infants had complete cartilage rings the entire length of the trachea. Congenital lesions of the distal trachea may become suddenly life-threatening at birth or during the onset of a respiratory infection. An abrupt or insidious onset of airway symptoms requires an expeditious diagnostic evaluation to define the tracheobronchial anatomy, and the operating team has to be prepared for emergency tracheal reconstruction.