Peter W. Callen

Fetal cystic adenomatoid malformation: Prenatal diagnosis and natural history

We studied the natural history and pathophysiology of congenital cystic adenomatoid malformation (CCAM) detected prenatally by ultrasound in twelve fetuses. Two types of fetal CCAM can be distinguished by gross anatomy, ultrasound findings, and prognosis. Microcystic lesions are usually associated with fetal hydrops and have a poor prognosis (five cases with one survivor). Antenatal diagnosis, maternal transport, and immediate thoracotomy after birth allowed the first reported survival of a newborn with a large microcystic CCAM. Macrocystic lesions are not usually associated with hydrops and have a favorable prognosis (five of seven survived). We conclude that fetuses with hydrops are at high risk for fetal or neonatal demise without intervention. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.

FETAL SURGERY FOR CONGENITAL HYDRONEPHROSIS

ALTHOUGH many fetal anatomic abnormalities can now be diagnosed by sonography, only a few will affect prenatal management.1 In the fetus with severe bilateral hydronephrosis secondary to urethral o...

Correction of congenital diaphragmatic hernia in utero, V. Initial clinical experience

Review of our experience with 45 cases of prenatally diagnosed congenital diaphragmatic hernia (CDH) confirms that most fetuses (77%) will not survive despite optimal pre- and postnatal care. Polyhydramnios, associated anomalies, early diagnosis, and a large volume of herniated viscera (including liver) are associated with a particularly dismal prognosis. After extensive experimental work demonstrated the efficacy, feasibility, and safety of repair in utero, we attempted to salvage six highly selected fetuses with severe CDH by open fetal surgery. Five had liver incarcerated in the chest: three died at operation because attempts to reduce the liver compromised umbilical venous return. In one, a Goretex diaphragm was constructed around the liver, but the baby died after birth. The last two fetuses, one with incarcerated liver, were successfully repaired. Both demonstrated rapid growth of the lung in utero, had surprisingly good lung function after birth despite prematurity, had the abdominal patch removed at 2 weeks, and subsequently died of nonpulmonary problems (an unrelated nursery accident in one and intestinal complications in the other). The only maternal complication was amniotic fluid leak and preterm labor. All six women are well and four have had subsequent normal children. From this phase I experience, we conclude that fetal surgery appears safe for the mother and her reproductive potential, that fetal CDH repair is feasible in selected cases, and that the fetal lung responds quickly after decompression. However, fetal repair remains a formidable technical challenge.

Primary fetal hydrothorax : natural history and management

Primary fetal hydrothorax presents a wide spectrum of severity ranging from small, harmless effusions, to life-threatening thoracic compression. To define natural history and management, we reviewed 32 cases seen at two large perinatal centers from 1980 to 1987. Spontaneous resolution of the effusions was seen in three fetuses, all of whom survived. Three fetuses were electively terminated. The overall mortality was 53%. In the 24 untreated fetuses, sex and the presence of polyhydramnios did not influence mortality, but hydrops, gestational age less than 35 weeks at delivery, and bilateral effusions were associated with a poor prognosis. Five fetuses underwent in utero decompression. In four, thoracentesis was performed, with rapid reaccumulation of the effusion. All four died from pulmonary insufficiency. In the fifth fetus, a thoracoamniotic shunt permanently decompressed the effusion, with resolution of the hydrops, and delivery of a normal viable infant. We conclude that (1) primary fetal hydrothorax may resolve or progress to hydrops, necessitating close follow-up with ultrasound; (2) pulmonary hypoplasia as a result of undrained large pleural effusions may result in neonatal mortality; (3) the gestational age at both diagnosis and delivery, the development of hydrops, and bilaterality of effusions are important prognostic predictors; and (4) the fetus with large effusions and hydrops has a poor prognosis, and thoracic decompression with a thoracoamniotic shunt may prove life saving.

Fetal intervention in obstructive uropathy: Prognosticindicators and efficacy of intervention

Management of the fetus with bilateral hydronephrosis is controversial; ability to predict outcome and efficacy of prenatal intervention are unknown. We studied 40 fetuses referred for ultrasonography, examination of fetal urine, and possible therapy. We retrospectively assigned fetuses to a good prognosis group if fetal urine was hypotonic (sodium less than 100 mEq/L, chloride less than 90 mEq/L, osmolarity less than 210 mOsm/L) and there was no ultrasonographic evidence of dysplasia; we assigned fetuses to a poor prognosis group if even one criterion was abnormal. Survival was greater in the good prognosis group than in the poor prognosis group (81% vs 12.5%; 87% vs 30%, excluding abortions) (p less than 0.005). We then attempted to assess the efficacy of prenatal urinary decompression by comparing outcome within the good and poor prognosis groups. Survival with intervention was greater in both the good prognosis group and the poor prognosis group (89% vs 70% and 30% vs 0%). In 6 of the 8 survivors in the good prognosis group, severe oligohydramnios was reversed by decompression. We conclude the fetal urine electrolyte levels and ultrasonographic appear helpful in predicting residual fetal renal function and neonatal outcome and that prenatal decompression may prevent the development of fatal pulmonary hypoplasia.

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.

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...

Severity of intestinal damage in gastroschisis: Correlation with prenatal sonographic findings

From 1982 to 1986, 26 abdominal wall defects were detected prenatally. Seventeen pregnancies culminated in live births with ultrasound correctly distinguishing between omphalocele and gastroschisis in all cases. In the 11 cases of gastroschisis diagnosed before birth, we attempted to correlate the clinical outcome with the size of the abdominal wall defect, sonographic appearance of the eviscerated bowel, and known time of exposure to amniotic fluid (gestational age at birth to gestational age at diagnosis). Neither the time of exposure to amniotic fluid (median duration of 14 weeks) nor the defect size could be correlated with eventual clinical outcome. The presence of small bowel dilatation and mural thickening on prenatal sonography (four patients) had a high correlation with severe intestinal damage and poor clinical outcome. The absence of these two sonographic findings (seven patients) was associated with mild intestinal changes and benign clinical course with no morbidity or mortality. We conclude that obstetric ultrasound cannot only accurately detect the presence and type of abdominal wall defect, but it also gives an indication of the severity of intestinal damage and subsequent clinical course in prenatally diagnosed cases of gastroschisis. Early delivery of the fetus with prenatally diagnosed gastroschisis should no longer be performed to limit exposure to amniotic fluid. Now that reliable sonographic criteria of severe intestinal damage have been defined, the decision to deliver early can be restricted to those fetuses with bowel dilatation and mural thickening.

Comparison of computed tomography, ultrasonography, and gallium-67 scanning in the evaluation of suspected abdominal abscess.

A retrospective study was made of 29 consecutive patients who were evaluated for suspected abdominal abscess by at least two of three imaging modalities: gallium-67 scanning, ultrasonography, and computed tomography. No statistically significant difference in accuracy of the findings could be demonstrated. Consideration of the advantages and disadvantages of each imaging modality will often indicate which to use in an individual case. Findings from the three imaging techniques sometimes provided complementary rather than identical information.

Computed Tomographic Evaluation of Abdominal and Pelvic Abscesses

The CT characteristics of abdominal and pelvic abscesses in 29 patients were analyzed. The pathological development of an abscess as it relates to the CT appearance is discussed. Findings such as low-density areas within a soft-tissue mass or a definable wall or rim, while seen in abscesses, can be seen in other pathological entities as well, such as hematomas, noninfected inflammatory masses, and cystic or necrotic tumors. The most specific CT sign of an abscess was extraluminal gas, seen in 38% of patients.