110
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September 1, 1982 Am. J. Obstet. Gynecol.
in brief
In this case, the patient possibly developed a pelvirectal abscess (above the levator muscle) or a nonhealing, infected sinus tract along a postoperative drain site. Conceivably, the fimbriated end of the right fallopian tube became approximated firmly against the sinus tract opening in the pelvic peritoneum, thereby giving rise to a tubocutaneous fistula. Sinographic studies permitted an accurate diagnosis, and extirpation of the tube and uterus produced a probable cure. REFERENCE
1. Nora, P. F.: Operative Surgery, Principles and Techniques, Philadelphia, 1974, Lea & Febiger, p. 508.
Teratomas: Concordance in mother and fetus Fig. 3. Postoperative sinogram showing ping at the level of the pelvic peritoneum.
fistulous
tract
stop-
The patient and her husband were counseled on the disease process and the various modalities of therapy. They requested sterilization; therefore, hysterectomy and salpingectomy were planned. On December 14, 1981, with the patient under general anesthesia. exploratory laparotomy was carried out. The pelvis contained massive adhesions; there were bilateral ovarian cysts, 3 cm in diameter, and the distal end of the right fallopian tube was densely adherent to the posterior peritoneum on the right side of the midline anterior to the distal segment of the sacrum. This tube was dilated to 1.5 cm in diameter, and after careful dissection, the fimbria was identified. A total abdominal hysterectomy and bilateral salpingooophorectomy were performed. The proximal portion of the fistulous tract was thoroughly curetted with a serrated endocervical curette. A small French catheter was inserted into the tract from above and exited below. The vaginal cuff was closed over a fenestrated “T” tube, and reperitonealization was carried out. The fascia and skin were closed with permanent suture. Two units of blood was transfused to replace the intraoperative loss. The postoperative course was uncomplicated, and she was discharged on the seventh postoperative day. Oral low-dose estrogen replacement therapy was started during the hospitalization. On January 15, 1982, 4 weeks after the operation, sinograptly was performed (Fig. 3). The tract was noted to end at the approximate level where it previously entered the peritoneal cavity. It appears that the healing process has begun in the proximal portion of the listulous tract.
Fist&s are abnormal communications between a hollow organ and the outside or between two hollow organs.’ Most fistulas originate from trauma or from some type of infectious process that disrupts the continuity of the tissues involved. Once a fistula is diagnosed, the basic principle in the treatment is obliteration of the primary opening of the fistulous tract. There is no nonsurgical treatment for fistulas.’
WILLIAM
F.
MASON
BARR,
RAYBURN, JR.,
M.D. M.D.
Obstetric Division, Defiartment of Obstetrics and Gynecology, and Teratology Unit, Department of Pediatrics, The University of Michigan Medical School, Ann Arbor, Michigan SACRAL MASSES of the fetus are increasingly diagnosed antepartum because of the more frequent use of ultrasonography. A sacrococcygeal teratoma and a meningomyelocele should be considered in the differential diagnosis of caudal masses in the fetus. We report here an unusual case of a mother, with an ovarian teratoma, who gave birth to an infant with an antenatally detected sacrococcygeal teratoma. Abnormalities of the amniotic fluid and the concordance for teratomas are discussed.
A 37-year-old, multiparous woman with gestationai-onset diabetes was referred to our hospital at 34 weeks’ gestation because of polyhydramnios and a suspected fetal anomaly. A recent ultrasound examination of the pregnant uterus had revealed a sacral mass on the otherwise normal-appearing fetus. Because of the patient’s age, amniocentesis for genetic indications had been performed at I7 weeks. Alpha-fetoprotein level was normal, and a normal female karyotype was demonstrated. Clinical and ultrasound examinations confirmed the presence of polyhydramnios (probably 4 L or more) and an active fetus with a cystic mass 11 cm in diameter in the sacral region. An amniocentesis yielded a brilliant yellow-orange fluid, with a lecithin/sphingomyelin ratio of 2.91 and an optical density at 450 nm of 0.40 (high zone III on Liley curve). A cesarean section was performed because of the overwhelming polyhydramnios, space-occupying fetal anomaly,
Reprint requests: William Rayburn, Obstetrics and Gynecology, Women’s sity of Michigan, Ann Arbor, Michigan
M.D., Department of Hospital, The Univer48109.
0002-9378/82/170110+03$00.30/0
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@ 1982
Co.
Volume Number
144 1
Communications
Fig. 1. A, Sacrococcygeal teratoma attached croscopic view of maternal ovarian teratoma by mature respiratory epithelium. (Original rococcygeal teratoma illustrating a distended sympathetic ganglion with mature ganglion
in brief
111
to an otherwise normal-appearing term infant. E, Miillustrating mature cartilage next to cystic spaces lined magnification x83.) C, Microscopic view of fetal sacbronchiole adjacent to a mature peripheral nerve and a cells. (Original magnification X 83.)
and evidence of fetal lung maturity. At delivery, the vigorous infant had a 23 by 12 by 9 cm, multilobulated, ulcerated firm mass that extended caudally from the coccyx and distorted the buttocks and perineum (Fig. 1, A). Prior to closure of the abdomen of the mother, a 12 by I5 by 14 cm solid tumor of the left ovary was removed. The right ovary appeared normal. The infant underwent immediate operation for the removal of the sacral mass; two smaller, intrapelvic tumors were dissected from near the rectal wall. The infant tolerated the operation without major morbidity, and both the mother and infant were eventually discharged in satisfactory condition. Histologic examination of the tumor from the baby demonstrated a disorganized variety of tissue types derived from all three germ layers without evidence of a malignancy (Fig. 1, C). Microscopic examination of the ovarian tumor from the mother was similarly consistent with a diagnosis of benign cystic teratoma (Fig. L,B). Ulceration of the surface and escape of fluid from the mass could explain the acute onset of polyhydramnios, the yellow-orange color of the amniotic fluid, and the high optical density at 450 nm. Although there may be an elevation of serum alpha fetoprotein in cases of teratoma, this has usually been associated with malignant yolk-sac elements in the tumor; in our
case, no malignant elements were found. It is likely that the amniotic alpha-fetoprotein level that was normal at 17 weeks’ gestation was measured before surface ulceration occurred. Despite our initial guarded impression of the prognosis for the infant, results following the removal of the entire mass and reconstruction of the perineal region with no apparent residual damage were encouraging. Although unlikely, any recurrence would require excision, plus chemotherapy and radiotherapy if malignancy were present. Several pedigrees have been reported in which two or more family members were affected with teratomas of the same location, either ovarian or sacrococcygeal.‘, * However, we have been unable to find a report of a sacrococcygeal teratoma and an ovarian teratoma in the same kindred. Despite the histologic similarities of these two teratomas, recent evidence indicates that they are of different origins.3 Through the study of chromosome markers and enzyme variants, it has been shown that ovarian teratomas are parthenogenic tumors that arise from a single germ cell after the first meiotic division. In
distinction, extragonadal teratomashave been shownto be of mitotic cell origin. For this reason we think it
112
Communications
unlikely
that
the tumor
September Am. J. Obstet.
in brief
there is an etiologic relationship between of the mother and that of her infant. We
estimate that sacrococcygeal and ovarian teratomas each have an incidence ofabout 1: 20,000. If they are etiologically distinct, concordance of the two types among mother and infant would be expected to occur in 1: 2 X 1 OS mother-infant pairs. Additional reports of a concordance of this type would cast doubt on our contention
that this is an extremely rare coincidence and would to a renewed speculation about an etiologic link.
lead
REFERENCES 1. Ashcraft, K. W., and Holder, T. M.: Hereditary presacral teratoma, J. Pediatr. Surg. 9:691, 1974. 2. Plattner, G., and Oxorn, H.: Familial incidence of ovarian dermoid cysts, Can. Med. Assoc. J. 108:892. 1973. 3. Linder. D.. Hecht, F.. McCaw. B. K.. and Camobell. I. R.: Origin of extragonadal teratimas and endodermal &us tumors, Nature 254:597, 1975.
Gamma globulin therapy during pregnancy in mother with hypogammaglobulinemia CHRISTIAN DIEGO
HAUSSER, BURIOT.
M.D.,
F.R.C.P.(C.)
M.D.
Department (4’ Pediatrics, Sainte-Justine Hospital, Montreal, @uhrc, &nada, and Dppartmrnt of Pediatric Immunology, Hrjpitnl drr Enfants Malades. Paris, France GI.OBULIN injections have been shown to be efficient in preventing infections in patients with hypogatnmagIobulinemia. However, because of side effects or because of the good clinical condition of adult patients, therapy is often temporarily discontinued and is resumed only if infections reappear. Two cases of pregnancy in young women with hypogam-
(;AMMA
maglobulinemia
are presented.
.~lt the age of IO. after recurrent pulmonary, ear, and sinus infections, Patient A. L. was diagnosed as having “common variable hypogammaglobulinemia.” She received intramuscular gamma globulin supplements until age 17. when it was decided to stop therapy because of febrile reactions with hypotension after the injections. At age 21, a pregnancy terminated in a septic abortion at 7 weeks’ gestation. At this time, the immunoglobulin levels were greatly lowered with IgG, 200 mg/dl (normal, 650 to 1,800 mgidl): IgM, 1 I mg/dl (normal, 45 to I.50 mg/dl); IgA, 4 mg/dl (normal, 60 to 350 mg/dl). Titers for polio, diphtheria, tetanus after a complete vaccinanon. and herpes varicella after a severe case of chickenpox at age 7 years were undetectable. B-cell enumeration (surface membrane immunoglobulin was 3% (normal range, IO% 2
Reprint requests: Dr. C. Hausser, Sainte-Justine 3175. (:&e Sainte-Catherine, Montreal, Province Canada H3T lC5. 000"-~l3781~2!1701l2+01~00.10/0
0
1982
The
C.V.
Hospital, of Quebec,
Mosby
Co
1, 1982 Gynecol.
5%). T-cell enumeration (E rosette) and lymphocyte stimulation with phytohemagglutinin, pokeweed mitogen, and concanavalin A were normal. After chronic pulmonary and ear infections, bronchiectasis, and relapsing digestive giardiasis, Patient M. F. was found at age 19 years to have panhypogammaglobulinemia (IgG, 226 mg/dl; IgM, 3 mg/dl; IgA, 27 mg/dl) with normal enumeration of B cells (surface membrane immunoglobulin) and T cells (E rosettes). In vitro lymphocyte stimulation with phytohemagglutinin, pokeweed mitogen, concanavalin A, and allogeneic cells in mixed lymphocyte cultures were normal. Because of the good clinical condition of the patient, no immunoglobulin supplements were prescribed. At age 24 years, she had a normal pregnancy ending in delivery at term of a male newborn infant with no malformations and weighing 2,750 gm. The amniotic fluid was purulent and the newborn infant, with Apgar scores of 5, 5, and 7, quickly showed signs of septicemia. In spite of vigorous antibiotic therapy, he died on the third day of life of generalized infection. The patient was then placed on a regimen of regular gamma globulin therapy of 0.4 ml/kg every 15 days. When her second pregnancy began, this dosage was increased to meet the physiologic gestational rise of the gamma globulin level. During the ninth month of pregnancy, she received intravenous gamma globulin at 0.4 ml/kg every week with an extra injection the day before delivery, to favor transplacental crossing of IgC. At 41 weeks of gestation, she was delivered of a healthy 3,300 gm male infant. The amniotic fluid was aseptic and the placenta was normal. The IgG levels of mother and child were identical (240 mg/dl). The baby was isolated at home, and intramuscular gamma globulin supplementation was without any side effects. At 3 months of a-g&, when the immunologic status of the infant was entirely normal, IgG therapy was discontinued. The mother’s milk contained less than IO mg/dl of IgG and IgA. These
two
case
reports
show
the
importance
of
gamma globulin supplements during the fertile period and pregnancy of hypogammaglobulinemic women in view of the increased risk from infections for the fetus and newborn infant. The physiologic high levels of IgG during late pregnancy are difficult to attain, and intravenous gamtna globulins to the mother at a dosage of 400 mg/kg every 2 to 3 weeks may be needed. The newborn infant, whose only source of IgG is from passive transfer across the placenta, should have a serum IgG level determination closely for signs of
after
delivery
and
be watched
infection. It is not sure if the newborn infant needs injections of gamma globulins,’ but in the absence of a protective range of the mother’s IgG, an increased risk of infection will persist until the newborn infant can synthesize his/her own IgG* and temporary therapy might be indicated.
REFERENCES I. Kobayashi, R. H., Hyman, C. J., and Stiehm, E. R.: Immunologic maturation in an infant born to a mother with agammaglobulinemia, Am. J. Dis. Child. 134:942, 1980. 2. Laursen, H. B., and Christensen, M. F.: Immunoglobulins in normal infant born of severe hypogammaglobulinemic mother, Arch. Dis. Child. 48:646, 1973.