- Email: [email protected]
Surgical management of extensive congenital hemangiofibrolipoma of the vulva in an infant
260
Communications
in brief
January Am. J. Obstet.
sion of the cervical spinal cord by thickened meninges, and it is the first case of MPS in which a rapid deterioration of spinal cord function occurred during pregnancy. The ease with which successful treatment was accomplished makes this condition an important one to recognize. Paulson and associates2 diagnosed and treated parhymeningitis in a patient who was diagnosed as having Scheie’s syndrome. Sostrin and associates” demonstrated prominent thickening of the dura on transverse section of the cervical spinal cord and dura in a fatal cast: of MPS that also had similar clinical and myelographic findings. This paper has a reference to a similar case reported by Kennedy and associates. Hypertrophy of fibrous tissue, due at least in part to abnormal accumulation of mucopolysaccharides, is characteristic of MPS VI as well as other mucopolysac-charidoses. This chronic abnormality, along with some superimposed acute change, ma)- have been related to one of the several hormonal or enzyme changes which take place in pregnancy. Since neurological deterioration occurred prior to anesthesia and cesarean section, these factors were not causative. The neurological impairment did not clear after termination of the pregnancy but improved remarkably following decompressive laminectotny and opening of the thickened dura. Other patients with MPS VI, as well as patients with other mucopolysaccharidoses, such as Scheie’s disease, who are capable of producing normal offspring, may develop this type of myelopathy during pregnancy. It is important to recognize this complication so it can be treated before permanent impairment results.
Fig. 1. External infant labium
genitals with a capillary majus.
of a W&month-old hemangiofibrolipoma
15, 1980 Gynecol.
Bedouin female of the right
REFERENCES
I. Peterson, D. I., Barchus,
H., Seaich, L., and Kelly. 1‘. E.: Myelopathy associated with Maroteaux-Lamy syndrome, Arch. Neurol. 32:127, 1975. 2. Paulson, G. W., Meagher J. N., and Burkhart, J.: Spinal pachymeningitis secondary to muropolysaccharidosis. J. Neurosurg. 41:618, 1974.
3. Sostrin, E:D., Hasso, A. N., Peterson, D. I.. and Thompson, J. R.: Myelographic new sign, Radiology
features 125:421.
of mucopolysaccharidoses: 1977.
A
Surgical management of extensive congignital hemangiofibrolipoma of the vulva in an infant
Fig. 2. Photomicrograph
MESODERMAL.
pediatric
TUMORS
age group,
of the vulva are rare in the but awareness that they occur is
Reprint requests: Dr. George Box 567. Bcrwick, Pennsylvania ooO?-~378;~~1i020260+02$00.?0/0
C. Tsoutsoplides, 18603. 0
I!Wl
The
R.D.
C. V. Mosby
Co.
#I,
of a benign mesodermal tumor of the right labium majus, in a 3%month-old female Bedouin infant. The section shows slight hyperkeratosis of the epidermis with minimal acanthosis. The tumor occupied all layers of the dermis to the subcutaneous fat. Aggregations of blood vessels of varying sizes separated by bands of fibrous tissue can be seen. Bundles of fat cells lay at random throughout the tumor.
Volume Number
Communications
136
in brief
important from the prognostic and therapeutic standpoints. These lesions may arise from the fatty tissue of the labia majora, the subcutaneous fibrous elements, or the blood vessels. Occasionally these benign tumors may arise from the round ligament at its point of insertion into the labium majus and in such instances must be differentiated from a tense cyst of the canal of Nuck.’ Congenital hemangiofibrolipomas are uncommon on the vulva. Malignant tumors of the vulva are somewhat more common than benign ones, but they are still very unusual in children.
described congenital hemangiofibrolipoma had a satisfactory result.
S. M.. a 3%month-old, well-developed Bedouin female infant, was admitted to the Gynecology Department of ElThowra General Hospital, Beida, Libyan Arab Republic, in February, 1970. A large, bulky mass occupying the right labium majus had been noted at birth. Bleeding from the mass was first observed by the mother when the infant was 4 weeks old. Several more episodes of slight bleeding were observed+
tisteria ~m~io~itis as a cause of fetal distress
but the last one was persistent and the infant was brought our
261
2
hospital.
On admission,
physical
patient ro be a well-nourished
examination
showed
to the
infant, normal in all respects
except for a large, slightly irregular and partially ulcerated mass, occupying the right labium majus, attached to the lower half of the right labium minus and reaching the fourchette. The mass extended to the buttock, groin, and mans pubis but did not involve the prepuce (Fig. 1). The mass was firm and irregular with an ulcerated area occupying the upper third of the tumor, An oozing point from the ulcerated area was controlled adequately with pressure saline dressings. Complete blood count showed a hemoglobin of 12.5 gm/lOO ml, a hematocrit of 38%, and a normal differential cell count and red blood cell morphology. Urinalysis was also normal. Following administration of a light general anesthestic the tumor mass was excised and meticulous hemostasis was accomplished by electrocautery and ligation. The blood loss was
only 8 ml. The histology
report
confirmed
hemangiofibro-
lipoma (Fig. 2). The wound was closed under slight tension, due to the large base of the lesion, and broke down on the fifth postoperative day. The infant was kept in the hospital for 3 weeks during which period of time the wound healed by secondary intention with minimal scar tissue. Oral antibiotics for one week and saline dressings for two weeks were used. The infant was seen six weeks after discharge from the hospital, and the external genitals were noted to be normal with no distortion of the anatomy of the area.
There is great variance of opinion regarding treatment of hemangiomas, mainly those seen in infants, because the lesions almost routinely disappear as the child grows. When ulceration and bleeding occur, as in our case, or when the tumor is growing rapidly with distortion of the surrounding tissues, treatment is a necessity. The more commonly used modes of treatment are by radium plaques (y-radiation) applied directly to the lesinn, x-irradiation, liquid nitrogen, carbon dioxide “snow,” injection of sclerosing solutions into the tumor, and surgical excision.* In our case conservative treatment was not considered because the infant was living in a remote area. Because the tumor was in the area of genitals, radium or x-irradiation treatment also was not considered. Surgical excision of the
of the vulva
REFERENCES 1. Woodruft, sions of 1978.
J. D.: Diagnosis the vulva, Curr.
and management Probl. Obstet.
of benign leGynecol. 1:36,
2. Kaufman, R. H., and Gardner, H. L.: Benign mesodermal tumors, Clin. Obstet. Gynecol. 8~953, 1965.
KEE
SENG
KOH,
F.R.C.S.(C.), TEREY ALAN
B.S.,
M.R.C.O.G.,
F.A.C.O.G. L.
J,
M.B.,
COLE, ORKIN,
M.D. M.D.
Division of Maternal-Fetal Medicine, Dcpariment of Obstetrics and Gywcology, University Southern California School of Medicine, Women’s Hospital, Los Angeles CountylUniversit~ of Southern California Medical Center, Los Angeles, Calzfornza
qf
LISTERIOSIS is usually secondary to low-grade maternal septicemia with transplacental infection of the fetus. Zervoudakis and Cederqvist,’ in a brief communication, discussed the effect of Listeria monocytogenes septicemia during pregnancy on the offspring. The purpose of this report is to document congenital listeriosis as a cause of acute fetal distress demonstrated by ominous fetal heart rate patterns and abnormal fetal acid-base status. The possible pathophysiologic mechanisms are discussed.
CONCEN~TAL
An 18-year-old primigravid patient was admitted at 35% weeks’ gestation in active labor. She gave a 2-day history of pharyngitis and in~uenza-like symptoms. Temperature was 100.6” F. General physical examination was unremarkable. The uterus was nontender and the fundal height was 32 cm. The estimated fetal weight was 2,200 grams and the fetal heart rate (FHR) was 180 bpm. On vaginal examination, the cervix was completely effaced and 2 cm dilated. The membrane was intact and the vertex was at the - 2 station. Laboratory studies showed a white blood cell count of 16,2O~/cu mm with 7f% polymorphonuciear leukocytes, 8% bands, 17% lymphocytes, and 4% monocytes. The hematocrit was 40%. Urinalysis was normal. Amniotomy yielded no amniotic fluid. Based on the above findings, a clinical diagnosis of chorioamnionitis was made and 2 gm of ampicillin was administered intravenously after the blood culture was taken. Direct FHR monitoring was established. A scanty amount of thick, viscous fluid was noted on aspiration through the intrauterine pressure catheter. The FHR recording showed fetal tachycardia (180 bpm), loss of variability, and persistent late decelerations. There was concomitant uterine hyperactivity with contractions of moderately strong intensity (approximately 75 mm Hg) recurring at I- to Zminute intervals Reprint requests: Dr. K. S. Koh, 1240 N. Mission Road, Los Angeles, 0002.9378/80/020261+03$00.30/0
Women’s Hospital Calif. 90033.
5K24,
@ 1980 The C. V. Mosby Co.
Communications
in brief
January Am. J. Obstet.
sion of the cervical spinal cord by thickened meninges, and it is the first case of MPS in which a rapid deterioration of spinal cord function occurred during pregnancy. The ease with which successful treatment was accomplished makes this condition an important one to recognize. Paulson and associates2 diagnosed and treated parhymeningitis in a patient who was diagnosed as having Scheie’s syndrome. Sostrin and associates” demonstrated prominent thickening of the dura on transverse section of the cervical spinal cord and dura in a fatal cast: of MPS that also had similar clinical and myelographic findings. This paper has a reference to a similar case reported by Kennedy and associates. Hypertrophy of fibrous tissue, due at least in part to abnormal accumulation of mucopolysaccharides, is characteristic of MPS VI as well as other mucopolysac-charidoses. This chronic abnormality, along with some superimposed acute change, ma)- have been related to one of the several hormonal or enzyme changes which take place in pregnancy. Since neurological deterioration occurred prior to anesthesia and cesarean section, these factors were not causative. The neurological impairment did not clear after termination of the pregnancy but improved remarkably following decompressive laminectotny and opening of the thickened dura. Other patients with MPS VI, as well as patients with other mucopolysaccharidoses, such as Scheie’s disease, who are capable of producing normal offspring, may develop this type of myelopathy during pregnancy. It is important to recognize this complication so it can be treated before permanent impairment results.
Fig. 1. External infant labium
genitals with a capillary majus.
of a W&month-old hemangiofibrolipoma
15, 1980 Gynecol.
Bedouin female of the right
REFERENCES
I. Peterson, D. I., Barchus,
H., Seaich, L., and Kelly. 1‘. E.: Myelopathy associated with Maroteaux-Lamy syndrome, Arch. Neurol. 32:127, 1975. 2. Paulson, G. W., Meagher J. N., and Burkhart, J.: Spinal pachymeningitis secondary to muropolysaccharidosis. J. Neurosurg. 41:618, 1974.
3. Sostrin, E:D., Hasso, A. N., Peterson, D. I.. and Thompson, J. R.: Myelographic new sign, Radiology
features 125:421.
of mucopolysaccharidoses: 1977.
A
Surgical management of extensive congignital hemangiofibrolipoma of the vulva in an infant
Fig. 2. Photomicrograph
MESODERMAL.
pediatric
TUMORS
age group,
of the vulva are rare in the but awareness that they occur is
Reprint requests: Dr. George Box 567. Bcrwick, Pennsylvania ooO?-~378;~~1i020260+02$00.?0/0
C. Tsoutsoplides, 18603. 0
I!Wl
The
R.D.
C. V. Mosby
Co.
#I,
of a benign mesodermal tumor of the right labium majus, in a 3%month-old female Bedouin infant. The section shows slight hyperkeratosis of the epidermis with minimal acanthosis. The tumor occupied all layers of the dermis to the subcutaneous fat. Aggregations of blood vessels of varying sizes separated by bands of fibrous tissue can be seen. Bundles of fat cells lay at random throughout the tumor.
Volume Number
Communications
136
in brief
important from the prognostic and therapeutic standpoints. These lesions may arise from the fatty tissue of the labia majora, the subcutaneous fibrous elements, or the blood vessels. Occasionally these benign tumors may arise from the round ligament at its point of insertion into the labium majus and in such instances must be differentiated from a tense cyst of the canal of Nuck.’ Congenital hemangiofibrolipomas are uncommon on the vulva. Malignant tumors of the vulva are somewhat more common than benign ones, but they are still very unusual in children.
described congenital hemangiofibrolipoma had a satisfactory result.
S. M.. a 3%month-old, well-developed Bedouin female infant, was admitted to the Gynecology Department of ElThowra General Hospital, Beida, Libyan Arab Republic, in February, 1970. A large, bulky mass occupying the right labium majus had been noted at birth. Bleeding from the mass was first observed by the mother when the infant was 4 weeks old. Several more episodes of slight bleeding were observed+
tisteria ~m~io~itis as a cause of fetal distress
but the last one was persistent and the infant was brought our
261
2
hospital.
On admission,
physical
patient ro be a well-nourished
examination
showed
to the
infant, normal in all respects
except for a large, slightly irregular and partially ulcerated mass, occupying the right labium majus, attached to the lower half of the right labium minus and reaching the fourchette. The mass extended to the buttock, groin, and mans pubis but did not involve the prepuce (Fig. 1). The mass was firm and irregular with an ulcerated area occupying the upper third of the tumor, An oozing point from the ulcerated area was controlled adequately with pressure saline dressings. Complete blood count showed a hemoglobin of 12.5 gm/lOO ml, a hematocrit of 38%, and a normal differential cell count and red blood cell morphology. Urinalysis was also normal. Following administration of a light general anesthestic the tumor mass was excised and meticulous hemostasis was accomplished by electrocautery and ligation. The blood loss was
only 8 ml. The histology
report
confirmed
hemangiofibro-
lipoma (Fig. 2). The wound was closed under slight tension, due to the large base of the lesion, and broke down on the fifth postoperative day. The infant was kept in the hospital for 3 weeks during which period of time the wound healed by secondary intention with minimal scar tissue. Oral antibiotics for one week and saline dressings for two weeks were used. The infant was seen six weeks after discharge from the hospital, and the external genitals were noted to be normal with no distortion of the anatomy of the area.
There is great variance of opinion regarding treatment of hemangiomas, mainly those seen in infants, because the lesions almost routinely disappear as the child grows. When ulceration and bleeding occur, as in our case, or when the tumor is growing rapidly with distortion of the surrounding tissues, treatment is a necessity. The more commonly used modes of treatment are by radium plaques (y-radiation) applied directly to the lesinn, x-irradiation, liquid nitrogen, carbon dioxide “snow,” injection of sclerosing solutions into the tumor, and surgical excision.* In our case conservative treatment was not considered because the infant was living in a remote area. Because the tumor was in the area of genitals, radium or x-irradiation treatment also was not considered. Surgical excision of the
of the vulva
REFERENCES 1. Woodruft, sions of 1978.
J. D.: Diagnosis the vulva, Curr.
and management Probl. Obstet.
of benign leGynecol. 1:36,
2. Kaufman, R. H., and Gardner, H. L.: Benign mesodermal tumors, Clin. Obstet. Gynecol. 8~953, 1965.
KEE
SENG
KOH,
F.R.C.S.(C.), TEREY ALAN
B.S.,
M.R.C.O.G.,
F.A.C.O.G. L.
J,
M.B.,
COLE, ORKIN,
M.D. M.D.
Division of Maternal-Fetal Medicine, Dcpariment of Obstetrics and Gywcology, University Southern California School of Medicine, Women’s Hospital, Los Angeles CountylUniversit~ of Southern California Medical Center, Los Angeles, Calzfornza
qf
LISTERIOSIS is usually secondary to low-grade maternal septicemia with transplacental infection of the fetus. Zervoudakis and Cederqvist,’ in a brief communication, discussed the effect of Listeria monocytogenes septicemia during pregnancy on the offspring. The purpose of this report is to document congenital listeriosis as a cause of acute fetal distress demonstrated by ominous fetal heart rate patterns and abnormal fetal acid-base status. The possible pathophysiologic mechanisms are discussed.
CONCEN~TAL
An 18-year-old primigravid patient was admitted at 35% weeks’ gestation in active labor. She gave a 2-day history of pharyngitis and in~uenza-like symptoms. Temperature was 100.6” F. General physical examination was unremarkable. The uterus was nontender and the fundal height was 32 cm. The estimated fetal weight was 2,200 grams and the fetal heart rate (FHR) was 180 bpm. On vaginal examination, the cervix was completely effaced and 2 cm dilated. The membrane was intact and the vertex was at the - 2 station. Laboratory studies showed a white blood cell count of 16,2O~/cu mm with 7f% polymorphonuciear leukocytes, 8% bands, 17% lymphocytes, and 4% monocytes. The hematocrit was 40%. Urinalysis was normal. Amniotomy yielded no amniotic fluid. Based on the above findings, a clinical diagnosis of chorioamnionitis was made and 2 gm of ampicillin was administered intravenously after the blood culture was taken. Direct FHR monitoring was established. A scanty amount of thick, viscous fluid was noted on aspiration through the intrauterine pressure catheter. The FHR recording showed fetal tachycardia (180 bpm), loss of variability, and persistent late decelerations. There was concomitant uterine hyperactivity with contractions of moderately strong intensity (approximately 75 mm Hg) recurring at I- to Zminute intervals Reprint requests: Dr. K. S. Koh, 1240 N. Mission Road, Los Angeles, 0002.9378/80/020261+03$00.30/0
Women’s Hospital Calif. 90033.
5K24,
@ 1980 The C. V. Mosby Co.