Variations in the bladder exstrophy complex associated with large omphalocele

Two newborns with a large omphalocele associated with the bladder exstrophy complex are presented. In 1 case the omphalocele was combined with a musculoskeletal defect and imperforate anus with an anovulvar fistula. The other patient had a musculoskeletal deformity and a duplicated bladder with exstrophy. Surgical treatment was successful in both cases.     

Glenohumeral deformity secondary to brachial plexus birth palsy

Ninety-four patients who had brachial plexus birth palsy were entered into a prospective study to evaluate the association between persistent palsy, age-related musculoskeletal deformity, and functional limitations. Of these patients, forty-two had either computerized tomography or magnetic resonance imaging to assess the presence and degree of incongruity of the glenohumeral joint, deformity of the humeral head, and hypoplasia of the glenoid as part of the preoperative planning for a reconstructive operation. Functional ability was rated with use of the classification of Mallet, on a scale of 1 to 5. The mean glenoscapular angle (the degree of retroversion of the glenoid) on the affected side was -25.7 degrees compared with -5.5 degrees on the unaffected side. Twenty-six (62 per cent) of the forty-two shoulders had evidence of posterior subluxation of the humeral head, with a mean of only 25 per cent (range, 0 to 50 per cent) of the head being intersected by the scapular line. Progressive deformity was found with increasing age (p < 0.001). The natural history of untreated brachial plexus birth palsy with residual weakness is progressive glenohumeral deformity due to persistent muscle imbalance. The status of the glenohumeral joint must be addressed when the choice between tendon transfer and humeral derotation osteotomy for reconstruction of the shoulder is considered for these patients.     

Use of enzyme replacement therapy for Gaucher disease during pregnancy

OBJECTIVE: To date there has been little published experience with enzyme replacement therapy in pregnant women with symptomatic type I Gaucher disease. STUDY DESIGN: We describe six patients, including three with repeated early pregnancy loss, five of whom successfully carried pregnancies to term; the last pregnancy was terminated because of pulmonary hypertension. RESULTS: All pregnancies were uneventful and five resulted in healthy newborns. CONCLUSION: We concluded that in patients with Gaucher disease of childbearing age,for whom obstetric complications are an important symptom of the disease, pregnancy is not contraindicated (unless there is evidence or suspicion of pulmonary hypertension) and treatment should not be interrupted because the clinical improvement engendered by enzyme replacement therapy is conducive to fewer complications during pregnancy and delivery and post partum.     

Predicting pulmonary hypoplasia: assessment of lung volume or lung function or both?

We present three patients in whom there was an acute presentation of malabsorption in the puerperium and in whom the final diagnosis was celiac sprue. The reason for the dramatic increase in the symptoms after delivery, as well as the absence of symptoms before this, is unclear but may be related to immunologic changes that occur during pregnancy.     

Sub-zonal insemination for extreme male factor infertility

When in-vitro fertilization (IVF) is used for severe male infertility, the zona pellucida constitutes a major barrier to sperm-oocyte interaction, a barrier that may, in principle, be overcome by micro-injecting one or more spermatozoa into the sub-zonal perivitelline space ('sub-zonal insemination' or SZI). We have defined suitable patients for SZI as having 'extreme' male factor in that they have either shown a failure of fertilization in previous IVF cycles or had < 50 000 motile spermatozoa recoverable after semen preparation. (This is distinct from those with only 'severe' male factor in whom sufficient (> 50 000) motile spermatozoa could be recovered from a semen preparation.) A total of 213 SZI cycles were performed at Sydney IVF in the 4 year period September 1988 to September 1992, for extreme male factor patients with previous IVF failures or extremely low sperm numbers for whom SZI was the first option (about two-thirds and one-third of cases respectively). A total of 138 embryo transfers are reported, producing 20 clinical pregnancies after performing SZI on 1899 oocytes. One patient miscarried at 12 weeks gestation and there have been nine normal deliveries (so far) of 10 healthy infants. The first delivery was in February 1990. One pregnancy was achieved in the only patient in whom spermatozoa were obtained by epididymal aspiration, and transfer of three cryopreserved embryos in another patient resulted in a singleton pregnancy. Of the 492 oocytes fertilized, 282 had two pronuclei (57.3%) and normal embryos were transferred in 138/213 (64.8%) treatment cycles, giving an overall pregnancy rate of 14.5% per embryo transfer or 9.4% per cycle.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pregnancy and delivery in the patients with spina bifida--report of 5 cases

BACKGROUND: Because of improvement in the management for life-threatening complications of spina bifida, quality of life in the patient has become better and pregnancy is becoming more common problem in adolescent and adult female patients. In this paper, we reported patients with spina bifida who became pregnant and delivered a baby. METHODS: There were 6 deliveries from 5 patients. Mean patient age at the first pregnancy was 27.6 years (ranged from 26 to 32 years). Four patients had undergone surgical managements before pregnancy, of whom 1 had augmentation cystoplasty. Urological and obstetrical conditions during the pregnancy were analyzed. RESULTS: Upper urinary tract deterioration which was transient, was observed in 3 pregnancies. Serum BUN and creatinine levels remained stable throughout pregnancy in 4 patients. Pyelonephritis complicated 3 of 6 pregnancies. Delivery was vaginal in 4 and by cesarean section in 2. There were 7 obstetrical complications, which consisted of premature labor (2), uterine inertia (2), cephalo pelvic disproportion (2) and hydramnios (1). There were no significant anomalies in the newborns of these patients. CONCLUSION: To achieve successful pregnancy and delivery in the patient with spina bifida, careful urological and obstetrical observation for the potential complications is needed.     

Congenital musculoskeletal malformation in South African Blacks: a study of incidence

Ten thousand South African Black neonates were examined on the day after delivery for assessment of the frequency of congenital malformation of the musculoskeletal system. In 15 neonates it was found that the hip joints could be dislocated by the Ortolani or Barlow techniques. This instability persisted to 1 week of age in only 3 instances. This incidence figure is approximately 10% of the average reported for White's neonates. Talipes equinovarus deformity was found in 35 infants, an incidence of 3,5/1 000, or approximately three times the incidence cited for Whites. Calcaneovalgus deformity, however, was found in 3 infants only-- a much lower incidence than that found among Whites. There was a similar infrequency in the incidence of spina bifida aperta (cystica), which was noted in 7 infants. This figure correlates with reports from North America, but is substantially lower than the cited incidence in Wales and parts of England. Polydactyly, usually consisting of a postaxial pedunculated skin tag only, was the most common deformity found, the incidence being 8,8/1 000.     

Electrodermal activity and obstetric complications in schizophrenia.

The pregnancy and birth records of 79 schizophrenic patients, from whom adult electrodermal data were available, were systematically evaluated for obstetric complications using 34 criteria of nonoptimality in pregnancy, delivery, and postpartum periods. Patients with many obstetric complications had lower levels of electrodermal activity. For frequency of skin conductance responses and spontaneous fluctuations in skin conductance, the association was more evident for women than for men. However, the ratio of stimulus-elicited to spontaneous skin conductance responses showed a reliable association with obstetric complications without any gender differences. The relationship between obstetric complications and electrodermal activity was interpreted in terms of neurodevelopmental insults resulting in structural brain abnormalities interfering with orienting and electrodermal activity. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Daniel C. Darrow

OBJECTIVE: To determine whether magnetic resonance imaging (MRI) in pregnancy would help define a benign pelvic mass, thereby avoiding laparotomy. METHODS: During a 2-3-year period, five pregnant women with adnexal masses suspected to be leiomyomas underwent MRI. RESULTS: Four patients had evidence of leiomyoma and one had a benign cystic teratoma. All avoided laparotomy because of the almost certain radiologic findings of a benign process. In two women, there was an important effect on the pregnancy; one had fetal growth retardation and the other an outlet obstruction precluding vaginal delivery. Four of the patients underwent cesarean delivery. CONCLUSIONS: Magnetic resonance imaging can be used in the differential diagnosis of an adnexal mass in pregnancy. This will enable some pregnant patients to avoid laparotomy and its concomitant risks.     

Headache during pregnancy

A questionnaire was submitted to 430 women 3 days after delivery, asking mainly about features of headache before and during pregnancy, and their possible modification or recurrence; moreover, delivery modalities and the condition of the newborn were evaluated. One-hundred-and-twenty-six (29.3%) were found to be primary headache sufferers (IHS criteria, 1988), 81 of whom had migraine without aura (MO), 12 migraine with aura (MA), and 33 tension-type headache (TH). In all three groups, about 80% showed complete remission or a higher than 50% decrease in the number of attacks. The improvement was more evident after the end of the first trimester; this trend was common to the three primary headaches considered. In our series of primary headaches, there was only one case (MO) which began during pregnancy. In a subgroup of pluripara, headache maintained the improvement presented in the first pregnancy also during the following gravidic periods in about 50% of cases, whereas in the remaining 50% a worsening in parallel with successive pregnancies was found. Primary headaches "per se" do not seem to increase the pregnancy or delivery risks, nor the vitality of the newborn. During pregnancy, drug use was very much reduced and was restricted to a limited number of compounds.     

Introduction to microscopic research of silver halides and related dispersed systems

> Objective: To establish whether uterine artery flow velocity waveforms in the second trimester are associated with adverse pregnancy outcome in women with a poor obstetric history. Methods: We reviewed the obstetric case notes of 50 women with a poor obstetric history in previous pregnancies in whom uterine artery flow velocity waveforms had been obtained at 18 weeks gestation. Results: In this population 40% had an adverse pregnancy outcome (preeclampsia, pregnancy-induced hypertension, preterm delivery, birthweight <10th percentile or perinatal death). Preeclampsia, perinatal death, and preterm delivery were all significantly associated with abnormal uterine artery waveforms at 18 weeks. Conclusions: Assessment of uterine artery flow velocity waveforms at 18 weeks gestation shows promise as a screening test in the high risk obstetric population. The technique requires formal evaluation in a prospective, double blinded study.     

Addison's disease in pregnancy: a report of six cases

OBJECTIVE: To review six cases of pregnancy with Addison's disease at Obstetrics and Gynecology Hospital, Shanghai Medical University from 1949 to 1994. METHOD: This is a clinical retrospective analysis. Five of the 6 patients were treated with hormone replacement therapy, and the remaining one received no hormone treatment due to lack of symptoms during pregnancy and neglect of previous adrenal surgery by obstetricians. RESULTS: The patient received no treatment occurred Addisonian crisis and died soon after delivery. The other five patients had smooth course of delivery and postpartum period. CONCLUSIONS: Hormone replacement therapy is important for patients with Addison's disease in pregnancy especially for acute decompensation of adrenal function. Pregnant women with history of adrenal surgery should be monitored carefully even without any symptoms and hormone treatment is necessary. Vaginal delivery is encouraged unless there is obstetric indication.     

Moyamoya disease and pregnancy: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Many female patients with moyamoya disease are of childbearing years, including those who were diagnosed before entering their childbearing years. However, there have been no extensive reviews of the management of pregnancy and delivery in association with moyamoya disease. The purpose of this report is to describe the case of a patient with moyamoya disease complicated by pregnancy and to review the literature on other such cases. CLINICAL PRESENTATION AND INTERVENTION: We report a 23-year-old primipara with moyamoya disease who delivered uneventfully by cesarean section under spinal anesthesia at 38 weeks of gestation. In the literature, 30 cases were reported of patients who had been diagnosed with moyamoya disease before pregnancy and delivery, and 23 patients who were symptomatic and were diagnosed for the first time with moyamoya disease in association with pregnancy. CONCLUSION: There is no evidence that pregnancy increases the risk of cerebrovascular accident or that bypass surgery decreases its risk. Poor prognosis of the patient or the newborn is mostly caused by cerebral hemorrhage and not by cerebral ischemia. It is important to control blood pressure and especially to avoid toxemia during pregnancy. Either cesarean section or vaginal delivery can be accomplished safely. Any anesthetic method can be used, provided special attention is given to avoiding hypocapnia, hypotension, and hypertension. Oral contraceptives should be avoided.     

Pregnancies complicated by placenta praevia: what is appropriate management?

OBJECTIVE: To review the outcome of pregnancies complicated by placenta praevia over a three-year period (1991-1993) and to describe in detail the antenatal course and the events leading to delivery, assessing retrospectively whether there are clinical features predictive of outcome and whether outpatient management would be reasonable. DESIGN: A retrospective review of the case records of women with a pregnancy complicated by placenta praevia. SETTING: A tertiary referral teaching hospital in Edinburgh. RESULTS: There were 15,930 deliveries in the study period. Fifty-eight women (0.4%) had a placenta praevia in the third trimester, 42 of whom (72%) had at least one episode of bleeding. Overall, 62% of the women had a major praevia with no differences in the grade of praevia between those women who did or did not have bleeding. Both diagnosis and delivery occurred significantly earlier in women with antepartum bleeding than in those without (median gestation at diagnosis 28.6 weeks versus 33.3 weeks (P < 0.01) and at delivery 36.0 weeks versus 37.1 weeks (P = 0.04), respectively). Delivery by emergency caesarean section was more common in women with bleeding (62% versus 38%). An increasing number of bleeding episodes experienced by individuals was not associated with significant differences in outcomes. Rapid emergency delivery for bleeding was necessary for three women, in none of whom could the bleeding have been predicted. CONCLUSIONS: The clinical outcomes of placenta praevia are highly variable and cannot be predicted confidently from antenatal events. Nonetheless, in the majority of cases with or without bleeding and irrespective of the degree of praevia, outpatient management would appear safe and appropriate.     

Individual differences in perceived riskiness of drinking in pregnancy: antecedents and consequences

OBJECTIVE: This study examined whether differences in perceived riskiness of alcohol consumption during pregnancy were related to self-reported alcohol consumption among a community sample of pregnant women. Further, this study examined the impact of prior experiences on risk perceptions, focusing on previous pregnancy experiences and on previous alcohol-related problems. METHOD: The hypothesized relationships among variables were tested simultaneously in a structural equation model. Subjects included 159 pregnant women, all of whom drank regularly before pregnancy recognition, who were recruited from prenatal clinics and through newspaper advertisements. RESULTS: Perceived riskiness of drinking during pregnancy was lower among women who had previously given birth to a healthy child and among women with greater numbers of previous alcohol problems. Prior adverse pregnancy experience did not predict perceived risk. Perceived risk negatively predicted actual alcohol consumption during pregnancy, suggesting that previous healthy pregnancy experiences and alcohol problems increase drinking in pregnancy indirectly, through perceived risk. A direct positive effect from previous alcohol problems to drinking in pregnancy also was observed. CONCLUSIONS: Findings suggest that risk perceptions play a role in drinking behavior among pregnant women and help to illuminate the relationship between parity and alcohol consumption. Interventions designed to reduce drinking among pregnant women, which have generally relied on providing information, may be improved by considering the impact of previous experiences and addressing erroneous beliefs.     

The natural history of hereditary multiple exostoses

We established a database of hereditary multiple exostoses for the state of Washington, on the basis of a retrospective review of the medical records and a clinical evaluation of family members, to determine the prevalence, clinical range of expression, and rate of malignant degeneration. The database comprised forty-six kindreds with 113 affected members; all kindreds had at least one member living in the state of Washington. The over-all prevalence was at least one in 50,000. Approximately 10 per cent of the subjects had no family history of multiple exostoses. With the use of twenty-three pedigrees that demonstrated an adequate multigenerational history for determination of penetrance of the gene, we identified one unaffected individual among twenty-six obligate heterozygotes, a rate of penetrance of 96 per cent. There was no evidence for a substantial reduction of penetrance in female subjects. The median age at the time of the diagnosis in the 113 affected individuals was three years (range, birth to twelve years). In a cohort of eighty-four subjects for whom we had complete information, the clinical range of expression was wide: thirty-three (39 per cent) had an obvious deformity of the forearm, eight (10 per cent) had an inequality in the lengths of the limbs, seven (8 per cent) had an angular deformity of the knee, and two (2 per cent) had a deformity of the ankle. The average number of operations for the patients for whom the operative history was known was two.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment

We reviewed the long-term outcomes of treatment of fibrous dysplasia of the proximal part of the femur in twenty-two patients (twenty-seven femora). There were fifteen male patients and seven female patients. Patients who had monostotic disease had no involvement of the calcar femorale, fewer microfractures, less deformity, and stronger bone that could support internal fixation. Patients who had polyostotic disease had frequent involvement of the calcar femorale; more microfractures; severe deformity, including shepherd's crook deformity; and, in many instances, bone that could not support internal fixation. Twenty-two of the twenty-seven femora had a microfracture at the time of the initial presentation. At least one osteotomy was performed in four femora that had monostotic disease and in nine femora that had polyostotic disease. Curettage and cancellous or cortical bone-grafting did not appear to have any advantage compared with osteotomy alone in the treatment of symptomatic lesions, as all grafts resorbed with persistence of the lesion. At the time of the latest follow-up evaluation, no lesion had been eradicated or had decreased in size. A satisfactory clinical result was achieved in twenty patients (twenty-four femora): nine who had monostotic disease and eleven who had polyostotic disease. Two patients who had polyostotic disease and an endocrinopathy (one of whom had bilateral involvement) had an unsatisfactory result. All three femora in these two patients had a neck-shaft angle of less than 90 degrees at the time of the most recent follow-up evaluation. Varus deformity of the proximal part of the femur is best treated with valgus osteotomy and internal fixation early in the course of the disease. If the calcar of the femoral neck is involved or if the quality of the bone is such that internal fixation is not possible, a medial displacement valgus osteotomy can provide a more mechanically favorable position for healing of the microfracture.     

Identification of hearing loss after age 18 months is not early enough

Choriocarcinoma arising in the placenta, or intraplacental choriocarcinoma, has seldom been reported, particularly in the absence of maternal metastases. Reluctance to diagnose choriocarcinoma in the presence of chorionic villi can delay diagnosis; however, timely diagnosis of choriocarcinoma is prognostically important, both for the mother and infant. We report the clinicopathologic findings in five mothers and infants in whom choriocarcinoma was identified in the placenta. None of the mothers had a history of gestational trophoblastic disease in previous pregnancies. Three placentas were similar with a single small lesion grossly suggesting a small infarct; microscopically these consisted of infarcted areas surrounded by choriocarcinoma. These three mothers were unusual in that none had metastatic choriocarcinoma; two were treated with chemotherapy and remained disease-free; the third was lost to follow-up shortly following delivery. The remaining two mothers had known pulmonary metastases at time of delivery. One of these latter two placentas contained a large marginal lesion microscopically identified as choriocarcinoma. The fifth placenta had rare microscopic foci of choriocarcinoma, and sheets of necrotic choriocarcinoma were identified in "blood clot" submitted with the placenta. In four of the five cases the choriocarcinoma appeared to be arising from otherwise normal chorionic villi, and in no case was there invasion of the villous stroma. All of the infants survived, and none had evidence of choriocarcinoma. These cases support the concept that choriocarcinoma associated with otherwise normal pregnancy arises in the placenta and may be more common than reported.     

The management of diabetes in pregnancy

The aim of the diabetes specialist is to provide a service to the pregnant diabetic woman so that she will present to her obstetrician with such well-controlled plasma glucose levels that her pregnancy will proceed without any diabetes-related problem, and she will be delivered of a normal baby, of normal size, at the normal full-term gestation, by the normal route. There are some problems in achieving this aim. The exact definition of hyperglycaemia in pregnancy is still a matter of dispute. Screening methods to identify the problem differ widely. Many centres have developed joint diabetes/antenatal clinics, but there are practical problems with such an approach. Pre-pregnancy counselling, and discussion of contraceptive measures is an important task for the diabetologist and requires up-to-date knowledge. Control of plasma glucose requires alteration of insulin doses as pregnancy proceeds. Mothers with retinal, renal or cardiac problems will need special care. The medical problems which develop, and the management of blood glucose during labour and delivery, mean that the diabetes team must be very adjacent to the obstetric service, and a centralised approach offers many advantages. The postpartum state, and the long-term outcome for both mother and baby, remain both an interest and a responsibility for the obstetric physician.