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Arachnoid cyst as a cause of the amenorrhea-galactorrhea syndrome
Communications in brief
Volume 149 Number 1
87
Transient fetal anuria of unknown etiology: A case report
disseminated staphylococcal infection was confirmed. The anuria and the relatively rapid return of renal function were felt to be compatible with acute tubular necrosis.
Robert L. Goldenberg, M.D., Richard O. Davis, M.D., and Cynthia G. Brumfield, M.D.
This case, in which the fetus and then newborn infant had an obvious but temporary anuria, is not typical of the cases of urinary tract malfunction previously described. In fact, we are aware of no other case in which anuria was diagnosed prior to delivery, confirmed after delivery, and followed by a spontaneous return of normal function. Apparently, with oligohydramnios and fetal anuria, in the presence of ultrasonographically demonstrated fetal kidneys, renal function may resume. This patient had nothing in her history to suggest an etiology for acute anuria. Nevertheless, for several reasons, we believe that the renal malfunction was not of long-standing duration. First, amniotic fluid was noted to be normal in volume at 14 weeks' gestational age. Second, the fetus did not have the syndrome of pulmonary hypoplasia and joint deformities usually associated with long-standing severe oligohydramnios. Third, the time course of acute tubular necrosis is usually about 2 or 3 weeks' duration. Finally, amniotic fluid is, for the most part, composed of fetal urine and is produced at a rate of about 600 ml/24 hours. 2 To compensate for this rapid production of amniotic fluid, the fetus swallows and/or inhales fluid at about the same rate. Since the total volume of amniotic fluid at this gestational age should not exceed 1 L, in the presence of an acute loss of renal function, the fetus should be able to swallow and inhale all of the amniotic fluid in 2 days. It is possible, therefore, that there was an acute cessation of renal function only days prior to our patient's hospital admission. In summary, we believe this to be the first case report of a fetus in which temporary renal malfunction occurred in utero, was documented, and resolved spontaneously after birth. The etiology of the acute renal failure remains obscure.
Division of Maternal-Fetal Medicine, the Department of Obstetrics and Gynecology, The University of Alabama in Birmingham, Birmingham, Alabama
The association between fetal urinary tract malfunction, oligohydramnios, pulmonary hypoplasia, and early neonatal death is well established. l Transient in utero renal failure with postnatal recovery as documented in this report has not been described previously. A 28-year-old primigravid woman undergoing an apparently uncomplicated pregnancy noted decreased fetal movement at 33 weeks' gestational age. On admission to her local hospital, a contraction stress test showed non repetitive late decelerations and was nonreactive. An ultrasound examination showed severe oligohydramnios. Earlier in the pregnancy, an ultrasound examination performed at 14 weeks' gestational age showed a normal amount of amniotic fluid. On transfer to our institution, the suspicious nonreactive fetal heart monitor tracing was confirmed. Ultrasound examination revealed oligohydramnios, a biparietal diameter of 7.6 em, and an estimated weight of 1800 gm. One and possibly both kidneys were noted to be present. The next morning the mother was given 20 mg of furosemide intravenously. Filling of the fetal bladder was not observed over 4 hours. The examination, repeated the next day, yielded the same result. That afternoon, electronic monitoring revealed spontaneous contractions with repeated late fetal heart rate decelerations. Since the cervix was closed, rupture of the membranes and internal monitoring were not feasible. After extensive discussions with the parents, a low-transverse cesarean section was performed, and a 1860 gm, meconium-stained, male infant, with Apgar scores of 9 and 9, was delivered. There was no amniotic fluid. The placenta weighed 680 gm and was normal in appearance. The infant had a normal physical examination and had no evidence of respiratory distress. An ultrasound examination showed normal-sized kidneys bilaterally. The infant had no urine output, and within 24 hours the serum creatinine level rose from 0.8 to 2.1 mg/dl. A renal scan performed at 24 hours of age showed minimal renal function with no radioactivity demonstrable over the bladder. Because of rising serum creatinine levels and continued anuria, peritoneal dialysis was initiated. The infant remained anuric until the fifth postnatal day, when 2 ml of urine was produced. Over the next 4 days, the volume of urine gradually increased in quantity to 30 ml in 24 hours. The serum creatinine level fell to 0.8 mg/dl. Dialysis was discontinued and the serum creatinine level remained stable. Unfortunately, the infant developed staphylococcal peritonitis and, despite vigorous antibiotic therapy, died at 3 weeks of age. At autopsy, the infant had normal kidneys, ureters, bladder, and renal arteries and veins. The Reprint requests: Robert L. Goldenberg, M.D., Department of Obstetrics and Gynecology, University of Alabama in Birmingham, School of Medicine, University Station, Birmingham, AL 35294.
REFERENCES 1. Keirse, M. j., and Meermon, R. H.: Antenatal diagnosis of Potter Syndrome, Clin. Obstet. Gynecol. 52:645, 1978. 2. Wallenberg, H. S. C.: The amniotic fluid, water and electrolyte hemostasis, j. Perinat. Med. 5: 193, 1977.
Arachnoid cyst as a cause of the amenorrhea-galactorrhea syndrome Steve N. London, M.D., and David L. Olive, M.D. Division of Reproductive Endocrinology and Infertility, Duke University Medical Center, Durham, North Carolina
The amenorrhea-galactorrhea syndrome with concomitant hyperprolactinemia is most commonly the Reprint requests: David L. Olive, M.D., Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, NC 27710.
88
Communications in brief
May I, 1984
Am.
result of a prolactin-secreting pituitary adenoma. However, a wide variety of other intracranial, spaceoccupying lesions have occasionally been implicated in the pathogenesis of this syndrome. Described here is a patient developing the amenorrhea-galactorrhea syndrome in association with an arachnoid cyst, the first such case to appear in the literature. A 24-year-old, black woman, gravida 1, para 1, abortus 0, living children 1, was referred for reevaluation of headaches, galactorrhea, and amenorrhea secondary to the "empty sella" syndrome. The patient had a normal menstrual history until she became pregnant in 1978. Her pregnancy and delivery were uneventful. She then used oral contraceptives for 1 year and discontinued them in late 1979 to become pregnant. Six months later she presented to her local physician complaining of infertility, amenorrhea, galactorrhea, and frontal headaches. The serum prolactin level was 130 ng/ml; visual field examination showed bilateral field cuts identical to old choroidal scars from congenital toxoplasmosis. Thin-section tomography of the sella turcica showed an enlarged pituitary fossa with no evidence of erosion. A computerized tomographic scan of the pituitary was compatible with an empty sella. In 1981, she began complaining of increasing frequency and severity of frontal headaches. Repeat determination of the serum prolactin level and computerized tomographic scan of the pituitary were unchanged. In March of 1982, the patient was referred to Duke University Medical Center for reevaluation. At this time, the patient complained of amenorrhea, galactorrhea, and severe frontal headaches. Endocrine evaluation revealed a prolactin level of 112 ng/ml, a thyroidstimulating hormone level of 2.4 ILU Iml, a follicle-stimulating hormone level of 7.2 mIU/ml, a thyroxine level by radioimmunoassay of 7.8 ng/ml, a triiodothyronine resin uptake of 34.8%, and a free thyroxine index of 2.7. A repeat computerized tomographic scan with the use of a fourthgeneration scanner revealed a large sellar and suprasellar water density with mildly enhancing margins. This finding was consistent with a cystic pituitary adenoma, craniopharyngioma, or other suprasellar cystic mass. A metrizamide cisternogram demonstrated that the previously described water density did not communicate with the cerebrospinal fluid. These findings were most consistent with an arachnoid cyst. In April of 1982, the patient underwent right subfrontal craniotomy and decompression of an arachnoid cyst. Postoperatively, the patient developed transient diabetes insipidus that spontaneously resolved on the fifth postoperative day, and recovery was otherwise uneventful. She is currently receiving no medications and is cycling regularly at follow-up 1 year after operation. The most recent serum prolactin level was 37 ng/ml. A computerized tomographic scan performed 6 months after decompression was normal. Arachnoid cys~s account for approximately I % of all intracranial space-occupying lesions, but intrasellar localization is considered rare. The etiology of such cysts is unknown. One theory states that suprasellar cysts are acquired lesions. According to this theory, adhesions that form between the pia mater and arachnoid create loculations with one-way valves. These loculations then slowly enlarge with cerebrospinal fluid. Arachnoidocele development would presumably augment susceptibility to fusion and cyst formation. The "empty sella" syndrome, a frequent cause of the
J. Dbstet. Gynecol.
amenorrhea-galactorrhea syndrome, is in essence an intrasellar arachnoidocele. The "empty sella" and the intrasellar arachnoid cyst may represent two stages of the same anatomic-clinical entity. First, a defective sellar diaphragm allows passage of the arachnoid membrane into the sella. Finally, fusion of the pia and arachnoid at the sellar inlet results in arachnoid cyst formation. ! The "empty sella" syndrome, when associated with amenorrhea-galactorrhea syndrome, can produce prolactin values> 100 ng/ml.2 A similar value was observed with this patient. An arachnoid cyst may be difficult to distinguish from an "empty sella." This differentiation is important as arachnoid cysts may produce intracranial bleeding, elevated intracranial pressure, and focal neurological signs. Frontal headaches and visual disturbances are common. Expansion can occur rapidly; thus, arachnoid cysts require careful and frequent evaluation. Surgical intervention is warranted only with visual disturbances, hypopituitarism, or an enlarging lesion. The effects of pregnancy, clinical sex steroids, and bromocriptine on the arachnoid cyst are unknown at this time. In summary, this case is the first to demonstrate an association between the arachnoid cyst and the amenorrhea-galactorrhea syndrome. It further supports the theory that arachnoid cysts may arise from a prior "empty sella." Finally, it illustrates that conditions other than prolactin-secreting adenomas can produce markedly elevated prolactin levels as well as abnormal sellar radiographs. REFERENCES 1. Benedetti, A., Carbonin, C., and Colombo, F.: Possible aetiopathogenetic correlation between primary empty sella and arachnoid cyst, Acta Neurochir. 38:269, 1977. 2. Haney, A. F., Kramer, R. S., Wiebe, R. H., and Hammond, C. B.: Hypothalamic-pituitary function and radiographic evaluation of women with hyperprolactinemia and an "empty" sella turcica, AM. J. OBSTET. GYNECOL. 134:917, 1979.
Hemoglobin A 1c levels and variant hemoglobins Patrick Merrill and Suzanne Trupin, M.D. University of Illinois Medical School and School Medicine, Urbana, Illinois
of Clinical
Since the development of the hemoglobin Ale assay, this test has become a routinely used analysis for documentation of long-term blood glucose control in diabetic patients.! Recently a patient of ours presented Reprint requests: Dr. Suzanne Trupin, Department of Obstetrics and Gynecology, University of Illinois, School of Clinical Medicine, Urbana, IL 61801.
Volume 149 Number 1
87
Transient fetal anuria of unknown etiology: A case report
disseminated staphylococcal infection was confirmed. The anuria and the relatively rapid return of renal function were felt to be compatible with acute tubular necrosis.
Robert L. Goldenberg, M.D., Richard O. Davis, M.D., and Cynthia G. Brumfield, M.D.
This case, in which the fetus and then newborn infant had an obvious but temporary anuria, is not typical of the cases of urinary tract malfunction previously described. In fact, we are aware of no other case in which anuria was diagnosed prior to delivery, confirmed after delivery, and followed by a spontaneous return of normal function. Apparently, with oligohydramnios and fetal anuria, in the presence of ultrasonographically demonstrated fetal kidneys, renal function may resume. This patient had nothing in her history to suggest an etiology for acute anuria. Nevertheless, for several reasons, we believe that the renal malfunction was not of long-standing duration. First, amniotic fluid was noted to be normal in volume at 14 weeks' gestational age. Second, the fetus did not have the syndrome of pulmonary hypoplasia and joint deformities usually associated with long-standing severe oligohydramnios. Third, the time course of acute tubular necrosis is usually about 2 or 3 weeks' duration. Finally, amniotic fluid is, for the most part, composed of fetal urine and is produced at a rate of about 600 ml/24 hours. 2 To compensate for this rapid production of amniotic fluid, the fetus swallows and/or inhales fluid at about the same rate. Since the total volume of amniotic fluid at this gestational age should not exceed 1 L, in the presence of an acute loss of renal function, the fetus should be able to swallow and inhale all of the amniotic fluid in 2 days. It is possible, therefore, that there was an acute cessation of renal function only days prior to our patient's hospital admission. In summary, we believe this to be the first case report of a fetus in which temporary renal malfunction occurred in utero, was documented, and resolved spontaneously after birth. The etiology of the acute renal failure remains obscure.
Division of Maternal-Fetal Medicine, the Department of Obstetrics and Gynecology, The University of Alabama in Birmingham, Birmingham, Alabama
The association between fetal urinary tract malfunction, oligohydramnios, pulmonary hypoplasia, and early neonatal death is well established. l Transient in utero renal failure with postnatal recovery as documented in this report has not been described previously. A 28-year-old primigravid woman undergoing an apparently uncomplicated pregnancy noted decreased fetal movement at 33 weeks' gestational age. On admission to her local hospital, a contraction stress test showed non repetitive late decelerations and was nonreactive. An ultrasound examination showed severe oligohydramnios. Earlier in the pregnancy, an ultrasound examination performed at 14 weeks' gestational age showed a normal amount of amniotic fluid. On transfer to our institution, the suspicious nonreactive fetal heart monitor tracing was confirmed. Ultrasound examination revealed oligohydramnios, a biparietal diameter of 7.6 em, and an estimated weight of 1800 gm. One and possibly both kidneys were noted to be present. The next morning the mother was given 20 mg of furosemide intravenously. Filling of the fetal bladder was not observed over 4 hours. The examination, repeated the next day, yielded the same result. That afternoon, electronic monitoring revealed spontaneous contractions with repeated late fetal heart rate decelerations. Since the cervix was closed, rupture of the membranes and internal monitoring were not feasible. After extensive discussions with the parents, a low-transverse cesarean section was performed, and a 1860 gm, meconium-stained, male infant, with Apgar scores of 9 and 9, was delivered. There was no amniotic fluid. The placenta weighed 680 gm and was normal in appearance. The infant had a normal physical examination and had no evidence of respiratory distress. An ultrasound examination showed normal-sized kidneys bilaterally. The infant had no urine output, and within 24 hours the serum creatinine level rose from 0.8 to 2.1 mg/dl. A renal scan performed at 24 hours of age showed minimal renal function with no radioactivity demonstrable over the bladder. Because of rising serum creatinine levels and continued anuria, peritoneal dialysis was initiated. The infant remained anuric until the fifth postnatal day, when 2 ml of urine was produced. Over the next 4 days, the volume of urine gradually increased in quantity to 30 ml in 24 hours. The serum creatinine level fell to 0.8 mg/dl. Dialysis was discontinued and the serum creatinine level remained stable. Unfortunately, the infant developed staphylococcal peritonitis and, despite vigorous antibiotic therapy, died at 3 weeks of age. At autopsy, the infant had normal kidneys, ureters, bladder, and renal arteries and veins. The Reprint requests: Robert L. Goldenberg, M.D., Department of Obstetrics and Gynecology, University of Alabama in Birmingham, School of Medicine, University Station, Birmingham, AL 35294.
REFERENCES 1. Keirse, M. j., and Meermon, R. H.: Antenatal diagnosis of Potter Syndrome, Clin. Obstet. Gynecol. 52:645, 1978. 2. Wallenberg, H. S. C.: The amniotic fluid, water and electrolyte hemostasis, j. Perinat. Med. 5: 193, 1977.
Arachnoid cyst as a cause of the amenorrhea-galactorrhea syndrome Steve N. London, M.D., and David L. Olive, M.D. Division of Reproductive Endocrinology and Infertility, Duke University Medical Center, Durham, North Carolina
The amenorrhea-galactorrhea syndrome with concomitant hyperprolactinemia is most commonly the Reprint requests: David L. Olive, M.D., Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, NC 27710.
88
Communications in brief
May I, 1984
Am.
result of a prolactin-secreting pituitary adenoma. However, a wide variety of other intracranial, spaceoccupying lesions have occasionally been implicated in the pathogenesis of this syndrome. Described here is a patient developing the amenorrhea-galactorrhea syndrome in association with an arachnoid cyst, the first such case to appear in the literature. A 24-year-old, black woman, gravida 1, para 1, abortus 0, living children 1, was referred for reevaluation of headaches, galactorrhea, and amenorrhea secondary to the "empty sella" syndrome. The patient had a normal menstrual history until she became pregnant in 1978. Her pregnancy and delivery were uneventful. She then used oral contraceptives for 1 year and discontinued them in late 1979 to become pregnant. Six months later she presented to her local physician complaining of infertility, amenorrhea, galactorrhea, and frontal headaches. The serum prolactin level was 130 ng/ml; visual field examination showed bilateral field cuts identical to old choroidal scars from congenital toxoplasmosis. Thin-section tomography of the sella turcica showed an enlarged pituitary fossa with no evidence of erosion. A computerized tomographic scan of the pituitary was compatible with an empty sella. In 1981, she began complaining of increasing frequency and severity of frontal headaches. Repeat determination of the serum prolactin level and computerized tomographic scan of the pituitary were unchanged. In March of 1982, the patient was referred to Duke University Medical Center for reevaluation. At this time, the patient complained of amenorrhea, galactorrhea, and severe frontal headaches. Endocrine evaluation revealed a prolactin level of 112 ng/ml, a thyroidstimulating hormone level of 2.4 ILU Iml, a follicle-stimulating hormone level of 7.2 mIU/ml, a thyroxine level by radioimmunoassay of 7.8 ng/ml, a triiodothyronine resin uptake of 34.8%, and a free thyroxine index of 2.7. A repeat computerized tomographic scan with the use of a fourthgeneration scanner revealed a large sellar and suprasellar water density with mildly enhancing margins. This finding was consistent with a cystic pituitary adenoma, craniopharyngioma, or other suprasellar cystic mass. A metrizamide cisternogram demonstrated that the previously described water density did not communicate with the cerebrospinal fluid. These findings were most consistent with an arachnoid cyst. In April of 1982, the patient underwent right subfrontal craniotomy and decompression of an arachnoid cyst. Postoperatively, the patient developed transient diabetes insipidus that spontaneously resolved on the fifth postoperative day, and recovery was otherwise uneventful. She is currently receiving no medications and is cycling regularly at follow-up 1 year after operation. The most recent serum prolactin level was 37 ng/ml. A computerized tomographic scan performed 6 months after decompression was normal. Arachnoid cys~s account for approximately I % of all intracranial space-occupying lesions, but intrasellar localization is considered rare. The etiology of such cysts is unknown. One theory states that suprasellar cysts are acquired lesions. According to this theory, adhesions that form between the pia mater and arachnoid create loculations with one-way valves. These loculations then slowly enlarge with cerebrospinal fluid. Arachnoidocele development would presumably augment susceptibility to fusion and cyst formation. The "empty sella" syndrome, a frequent cause of the
J. Dbstet. Gynecol.
amenorrhea-galactorrhea syndrome, is in essence an intrasellar arachnoidocele. The "empty sella" and the intrasellar arachnoid cyst may represent two stages of the same anatomic-clinical entity. First, a defective sellar diaphragm allows passage of the arachnoid membrane into the sella. Finally, fusion of the pia and arachnoid at the sellar inlet results in arachnoid cyst formation. ! The "empty sella" syndrome, when associated with amenorrhea-galactorrhea syndrome, can produce prolactin values> 100 ng/ml.2 A similar value was observed with this patient. An arachnoid cyst may be difficult to distinguish from an "empty sella." This differentiation is important as arachnoid cysts may produce intracranial bleeding, elevated intracranial pressure, and focal neurological signs. Frontal headaches and visual disturbances are common. Expansion can occur rapidly; thus, arachnoid cysts require careful and frequent evaluation. Surgical intervention is warranted only with visual disturbances, hypopituitarism, or an enlarging lesion. The effects of pregnancy, clinical sex steroids, and bromocriptine on the arachnoid cyst are unknown at this time. In summary, this case is the first to demonstrate an association between the arachnoid cyst and the amenorrhea-galactorrhea syndrome. It further supports the theory that arachnoid cysts may arise from a prior "empty sella." Finally, it illustrates that conditions other than prolactin-secreting adenomas can produce markedly elevated prolactin levels as well as abnormal sellar radiographs. REFERENCES 1. Benedetti, A., Carbonin, C., and Colombo, F.: Possible aetiopathogenetic correlation between primary empty sella and arachnoid cyst, Acta Neurochir. 38:269, 1977. 2. Haney, A. F., Kramer, R. S., Wiebe, R. H., and Hammond, C. B.: Hypothalamic-pituitary function and radiographic evaluation of women with hyperprolactinemia and an "empty" sella turcica, AM. J. OBSTET. GYNECOL. 134:917, 1979.
Hemoglobin A 1c levels and variant hemoglobins Patrick Merrill and Suzanne Trupin, M.D. University of Illinois Medical School and School Medicine, Urbana, Illinois
of Clinical
Since the development of the hemoglobin Ale assay, this test has become a routinely used analysis for documentation of long-term blood glucose control in diabetic patients.! Recently a patient of ours presented Reprint requests: Dr. Suzanne Trupin, Department of Obstetrics and Gynecology, University of Illinois, School of Clinical Medicine, Urbana, IL 61801.