Congenital cystic adenomatoid malformation detected by prenatal sonography; report of 2 cases

We report 2 cases of congenital cystic adenomatoid malformation (CCAM) detected by prenatal sonography. The first CCAM was diagnosed by fetal sonography in a female fetus at 30 weeks' gestation. The infant was born at 37 weeks' gestation, with a body weight of 2,770 g. After birth, chest computed tomography (CT) showed a multicystic mass in the middle lobe of the lung. She remained asymptomatic until age 21 months, when she suffered pneumonia. Two months later, middle lobectomy was performed. The second CCAM was diagnosed by fetal sonography in a female fetus at 25 weeks' gestation. She was born at 39 weeks' gestation, with a body weight of 3,292 g. Four days after birth, CCAM type II was diagnosed by chest CT. The infant was asymptomatic, and left lower lobectomy was performed 11 months after birth.     

Development of the cavernous sinus in the fetal period: a morphological study

The development and morphological structure of the lateral sellar compartment (LSC), an interdural space containing the cavernous sinus (CS), cranial nerves, and internal carotid artery (ICA), was investigated by histological examination of sections of the LSC and cerebral venograms from human fetal specimens. Twenty-eight LSC coronal sections were obtained from 14 fetuses of 13-32 weeks' gestation. Venograms of 11 other fetuses of 13-32 weeks' gestation were studied to observe changes in venous drainage. The CS appeared as a collection of small venous canals with an endothelial layer. These venous canals gradually became much larger through expansion and unification. The CS and basilar venous plexus were demonstrated as a faint cluster of small vessels on venograms obtained after 13 weeks' gestation. The dura mater increased in thickness and collagen fiber networks developed around all the components in the LSC after 23 weeks' gestation. The LSC lateral wall could not be histologically differentiated as separate multiple layers. Branching and joining of the cranial nerve fascicles were completed with the envelopment of collagen fibers after 23 weeks' gestation. The ICA at 13-15 weeks' gestation ran straight within the LSC, becoming tortuous before birth. CS formation occurs through the development of venous canals without smooth muscle layers, followed by web-formation by collagen fibers in the mesenchymal interstices. LSC formation, including the dense dura mater and an internal structure like that seen in the adult, is largely completed before birth.     

Diagnosis and management of fetal intrapericardial Morgagni diaphragmatic hernia with massive pericardial effussion

Herniation of the liver into the fluid-filled pericardial sac resulting in a thoracic mass is a particularly rare form of Morgagni hernia (congenital diaphragmatic hernia of Morgagni). We report an early antenatal diagnosis of congenital diaphragmatic hernia of Morgagni with pericardium effussion at 21 weeks' gestation. Two pericardiocentesis were performed at 21 and 22 4/7 weeks' gestation because of recurrence of pericardial effussion. Regular ultrasound assessments showed progressive herniation of the liver to practically fill the right hemithorax. An ex utero intrapartum treatment procedure was performed at 37 weeks' gestation to rescue maximum intrathoracic space for ventilation of the remaining functional lung tissue and to establish an airway for postnatal support. After birth, the patient successfully underwent early correction of the hernia. Postoperative course was uneventful, and the newborn girl was discharged 18 days later without complications and is currently doing well.     

Anaesthesia for phaeochromocytoma and sickle cell disease in pregnancy

A 33-year-old woman (G(1)P(0)) presented to a maternity hospital at 36 weeks' gestation. She suffered from sickle cell disease with three acute crises in the previous five months of her pregnancy. She also had a phaeochromocytoma with inadequately controlled hypertension. This report describes the multi-disciplinary work-up and peri-operative management necessary to optimise her medical condition before caesarean section at 39 weeks' gestation and subsequent removal of a malignant phaeochromocytoma.     

Gastric emptying is delayed at 8-12 weeks' gestation

Gastric emptying was studied indirectly by paracetamol absorptionin 20 patients at 87–12 weeks' gestation and also in 20non-pregnant controls. Subjects received paracetamol 1.5 g ina tablet with 50 ml of water and remained semi-recumbent for2 h while venous blood samples were obtained at 15-min intervals.The maximum concentration of paracetamol was significantly lowerand the time to maximum concentration significantly greaterin pregnant patients. In addition, the areas under the time-concentrationcurves at 60 and 120 min were significantly smaller in the pregnantgroup. These changes are indicative of a delay in gastric emptyingat 8–12 weeks' gestation.     

妊娠合并嗜铬细胞瘤的诊断与处理

目的 探讨妊娠合并嗜铬细胞瘤的诊断与处理方法.方法 妊娠合并嗜铬细胞瘤病例3例.年龄分别为41、28、32岁,均为妊娠期间出现高血压,并伴有头痛、心悸、多汗等症状,分别于妊娠27周+1,12周+4,14周+3就诊.24 h尿儿茶酚胺明显升高;肿瘤位于右肾上腺2例,双侧肾上腺1例,肿瘤直径4.5～10.0 cm,术前诊断"妊娠合并嗜铬细胞瘤".结果 例1于孕32周行全麻下剖宫产术,顺利分娩一健康女婴,同时行后腹腔镜下右肾上腺肿瘤切除术,切除7.0 cm肿瘤1枚.例2及例3均选择流产.例2行后腹腔镜下右肾上腺肿瘤切除术,切除右肾上腺4.5 cm肿瘤1枚.例3拒绝进一步治疗.例1及例2术后病理均证实为嗜铬细胞瘤.结论 对于妊娠早期出现高血压、典型发作性高血压或伴有头痛、心悸、大汗三联征,分娩后高血压仍未恢复正常的孕妇,应考虑嗜铬细胞瘤;及时诊断可显著降低母亲及胎儿死亡率;处理上应根据妊娠所处的时期及嗜铬细胞瘤的功能状态选择恰当的方式.妊娠中期是较理想的手术治疗时机.腹腔镜手术治疗妊娠嗜铬细胞瘤安全、有效.     

Scanning electron microscope study of the development of the human respiratory acinus.

Plastic corrosion casts were made of lungs from fetuses aged from 19 weeks' gestation to term and of lungs from a child and two adults to study the development of the respiratory acinus. To achieve reliable infusion of the most peripheral airspaces a high viscosity plastic was used that is not known to have been employed previously for corrosion casting of lungs. The casts were examined in the scanning electron microscope and showed the increase both in number and in length of the airways distal to the terminal bronchiole and also the change in shape and complexity of the most peripheral airspaces as the lung matures. The terminal airspaces change from short, simple, tubular endings at 19 weeks' gestation to short, shallow saccules from around 30 weeks' gestation to full term and contrast with the deep cup shaped alveoli in the adult. Measurements of the size of the terminal airspace at various stages of development are presented. This new approach, allowing three dimensional study of the peripheral airspaces of the developing lung, will be useful for investigating the lung pathology of neonates.     

Anesthetic management for neonates with congenital cystic adenomatoid malformation

We experienced two cases of congenital cystic adenomatoid malformation (CCAM) diagnosed by prenatal ultrasonography. The first case with type I CCAM underwent the resection of the right upper lobe 8 days after birth. Postoperative course was uneventful. The second case with type III CCAM developed non-immune hydrops, severe mediastinal shift, and polyhydroamnios at 29 weeks' gestation. At 30 weeks' gestation, cesarean section was performed. Immediately after birth, the resection of lung tumor for the neonate was performed. However, the neonate died on the 1st postoperative day.     

Haemoperitoneum due to placenta percreta. A case report

A case of haemoperitoneum due to placenta percreta occurring at approximately 22 weeks' gestation is presented. The diagnosis and treatment are discussed.     

妊娠合并嗜铬细胞瘤的诊断与处理

目的 探讨妊娠合并嗜铬细胞瘤的诊断与处理方法.方法 妊娠合并嗜铬细胞瘤病例3例.年龄分别为41、28、32岁,均为妊娠期间出现高血压,并伴有头痛、心悸、多汗等症状,分别于妊娠27周+1,12周+4,14周+3就诊.24 h尿儿茶酚胺明显升高;肿瘤位于右肾上腺2例,双侧肾上腺1例,肿瘤直径4.5～10.0 cm,术前诊断"妊娠合并嗜铬细胞瘤".结果 例1于孕32周行全麻下剖宫产术,顺利分娩一健康女婴,同时行后腹腔镜下右肾上腺肿瘤切除术,切除7.0 cm肿瘤1枚.例2及例3均选择流产.例2行后腹腔镜下右肾上腺肿瘤切除术,切除右肾上腺4.5 cm肿瘤1枚.例3拒绝进一步治疗.例1及例2术后病理均证实为嗜铬细胞瘤.结论 对于妊娠早期出现高血压、典型发作性高血压或伴有头痛、心悸、大汗三联征,分娩后高血压仍未恢复正常的孕妇,应考虑嗜铬细胞瘤;及时诊断可显著降低母亲及胎儿死亡率;处理上应根据妊娠所处的时期及嗜铬细胞瘤的功能状态选择恰当的方式.妊娠中期是较理想的手术治疗时机.腹腔镜手术治疗妊娠嗜铬细胞瘤安全、有效.     

妊娠合并嗜铬细胞瘤的诊断与处理

目的 探讨妊娠合并嗜铬细胞瘤的诊断与处理方法.方法 妊娠合并嗜铬细胞瘤病例3例.年龄分别为41、28、32岁,均为妊娠期间出现高血压,并伴有头痛、心悸、多汗等症状,分别于妊娠27周+1,12周+4,14周+3就诊.24 h尿儿茶酚胺明显升高;肿瘤位于右肾上腺2例,双侧肾上腺1例,肿瘤直径4.5～10.0 cm,术前诊断"妊娠合并嗜铬细胞瘤".结果 例1于孕32周行全麻下剖宫产术,顺利分娩一健康女婴,同时行后腹腔镜下右肾上腺肿瘤切除术,切除7.0 cm肿瘤1枚.例2及例3均选择流产.例2行后腹腔镜下右肾上腺肿瘤切除术,切除右肾上腺4.5 cm肿瘤1枚.例3拒绝进一步治疗.例1及例2术后病理均证实为嗜铬细胞瘤.结论 对于妊娠早期出现高血压、典型发作性高血压或伴有头痛、心悸、大汗三联征,分娩后高血压仍未恢复正常的孕妇,应考虑嗜铬细胞瘤;及时诊断可显著降低母亲及胎儿死亡率;处理上应根据妊娠所处的时期及嗜铬细胞瘤的功能状态选择恰当的方式.妊娠中期是较理想的手术治疗时机.腹腔镜手术治疗妊娠嗜铬细胞瘤安全、有效.

Abstract：

Objective To discuss the diagnosis and management of pheochromocytoma in pregnancy. Methods Three cases of pheochromocytoma in pregnancy were managed from 2007- 2009.Case 1, a 41-year-old woman presented with paroxysmal hypertension during 27+1 weeks' gestation.The accompanied symptoms included dizziness, palpitation and sweating. Both B ultrasound and MRI revealed a right adrenal tumor about 7 cm in diameter. The 24-hour catecholamine was higher than normal. A diagnosis of right adrenal pheochromocytoma was made. Case 2, a 28-year-old woman presented with hypertension during 12+4 weeks' gestation. B ultrasound showed a right adrenal tumor about 5 cm in diameter. The 24-hour catecholamine was higher than normal. Case 3, a 32-year-old woman presented with hypertension during 14+3 weeks' gestation. B ultrasound revealed bilateral adrenal tumors. The 24-hour catecholamine was higher than normal. The diagnosis of bilateral adrenal pheochromocytomas was made. Results The first patient delivered a healthy female infant through cesarean section at 32 weeks' gestation. Laparoscopic resection of right adrenal pheochromocytoma was successfully performed at the same time. Both the second and third patients had elective abortion for fear of fetotoxicity. Case 2 accepted laparoscopic resection of right adrenal pheochromocytoma after 4 weeks' medical preparation. Case 3 rejected surgery. The histopathological findings proved pheochromocytoma in case 1 and case 2. Conclusions Pheochromocytoma should be included in the defferential diagnosis in hypertension occurring during pregnancy especially in the following situations:hypertension during early pregnancy; paroxymal hypertension accompanied by headache, palpitation and sweating; hypertension does not relieve after delivery. Early diagnosis and management with medical treatment followed by surgical removal usually results in good maternal and fetal outcomes. The second trimester is good timing of surgical management. Laparoscopic removal of pheochromocytoma is safe in pregnancy.     

Conservative management of severe proteinuric hypertension before 28 weeks' gestation

Forty-five patients with severe proteinuric hypertension who presented before 28 weeks' gestation were managed conservatively by bed rest, antihypertensive treatment, betamethasone administration after 26 weeks' gestation, and intensive fetal and maternal monitoring. Eleven patients presented before 24 weeks and their babies all died; 34 patients presented at or after 24 weeks and 13 of their babies survived (38%). The indications for delivery were intra-uterine death (13), fetal distress (9), deterioration in the mother's condition (17), and maternal complications--pulmonary oedema in 3 cases and pleural effusion in 1. One patient went into spontaneous labour and one was induced at 34 weeks. At postpartum follow-up examination all the mothers in the group that had presented before 24 weeks were found to have underlying diseases, compared with 42% of those who had presented between 24 and 27 weeks. The low incidence of maternal complications and the relatively good survival rate of 38% indicate that there is a place for conservative management in patients with severe proteinuric hypertension presenting at 24 weeks or later. Termination of the pregnancy should, however, be seriously considered in those patients presenting before 24 weeks' gestation.     

Anesthetic management of a pregnant patient with congenitally corrected transposition of the great arteries for emergency cesarean delivery

A 30-year-old woman at 35 weeks' gestation with congenitally corrected transposition of the great arteries (CCTGA), whose associated anomalies were VSD, ASD and pulmonary stenosis, underwent emergency cesarean delivery under general anesthesia. She had hypoxemia due to right-to-left shunt. We discussed with obstetricians and pediatric cardiologists about her at 25 weeks' gestation. Discussed points included: 1) delivery after 30 weeks' gestation, 2) her cardiac function and anomalies, 3) cesarean delivery under general anesthesia in case of emergency, 4) management of a sleeping baby after cesarean delivery by pediatricians. Cyanosis and heart failure were noted worsening during pregnancy. General anesthesia was induced by midazolam and fentanyl following cannulation of radial artery and right internal jugular vein for blood pressure and CVP monitoring. Anesthesia was maintained with sevoflurane and fentnyl. Dopamine and dobutamine were used to support blood pressure and cardiac function. After delivery, because of decreased CVP, lactated Ringer's solution was administrated. After operation, the patient was hemodynamically stable and trachea was extubated. Anesthetic management of the patient depends on the anatomical defects of CCTGA and associated conditions. Anesthesiologist should obtain information about a pregnant woman with CCTGA from cardiologists and obstetricians in early gestation to manage patient successfully.     

Cervical cystic hygroma in the fetus: clinical spectrum and outcome

Cervical cystic hygroma is thought by most pediatric surgeons to be an isolated, usually resectable lesion with an excellent prognosis. However, prenatal sonography has revealed a high "hidden mortality" among fetuses with this condition, and most perinatologists consider it to be uniformly fatal. In an attempt to resolve these two differing perspectives, we analyzed 29 cases seen at two centers over 4 years. Of 27 fetuses diagnosed before 30 weeks' gestation, only one survived. Twenty-five of the 27 were aborted; severe hydrops was present in 21 of these 25. Two of the 27, both with stigmata of Noonan's syndrome, underwent spontaneous regression during the second trimester: one died at 2 weeks of age, and the other survived. Successful karyotypes were obtained on 17 fetuses: nine were normal, seven were 45X, and one was trisomy 21. Fetuses with abnormal karyotypes had a lower incidence of polyhydramnios (0% v 67%), additional anomalies (12% v 67%), and consanguinity or a history of abnormal pregnancies (0% v 89%). Two fetuses were diagnosed after 30 weeks' gestation. Neither had hydrops, polyhydramnios, associated anomalies, or an abnormal karyotype. One had a completely normal sonogram at 17 weeks' gestation. Both were operated on within the first 4 days of life; one did well without complications, and the other required a permanent tracheostomy because of extensive hypopharyngeal involvement. A cystic hygroma presenting in the fetus has a different natural history and prognosis from one presenting postnatally. The vast majority of fetal cases are diagnosed before 30 weeks' gestation, and present with hydrops or diffuse lymphangiomatosis. The dismal outlook in this group justifies elective termination in most cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cesarean delivery in a parturient with syringomyelia and worsening neurological symptoms

A parturient presented at 35 weeks' gestation with worsening neurological symptoms caused by syringomyelia. She underwent urgent cesarean delivery. The etiology and anesthetic considerations for an obstetrical patient with syringomyelia are discussed.     

Congenital infantile fibrosarcoma in a very low-birth-weight infant

We describe a prenatally diagnosed case with congenital infantile fibrosarcoma, which is a rare malignant tumor, of which there have been only 14 cases reported in the English literature. A giant mass on the left thigh was detected at 24 weeks' gestation by prenatal ultrasonography. Because of its rapid growth and nonreassuring fetal status in utero, the baby was delivered by cesarean delivery at 27 weeks' gestation. The case was complicated by hemorrhagic anemia, coagulopathy, and cardiac failure, and a tumor resection was performed on day 3. As of age 2.5 years, he was healthy without evidence of recurrence. Because some fetal cases with congenital infantile fibrosarcoma have a poor prognosis, termination of pregnancy at an appropriate time and postnatal early treatment are crucial for successful treatment.     

Two autopsy cases of prune belly syndrome in stillborn and its pathogenesis

Two autopsy cases of prune belly syndrome are reported. In Japan, 111 cases have been reported. Abdominal muscle deficiency or hypoplasia, urinary tract abnormalities and cryptorchidism are usually recognized. Case 1 was a female stillborn at 33 weeks' gestation and case 2 was a male stillborn at 21 weeks' gestation. Both cases had anomalies of genitourinary and digestive system. There was anatomic obstruction of external urethral orifice in case 1 and overriding urethra in case 2 which meant functional obstruction. In relation to the pathogenesis, we propose the important role of the urethral obstruction, whatever anatomic or functional. Further examination of the lower urinary tract including serial section analysis might be necessary to identify the pathogenesis of prune belly syndrome.     

Prenatal karyotype and ultrasound discordance in intersex conditions

An infant born at 38 weeks' gestation with ambiguous genitalia had a prenatal 45X karyotype but an enlarged phallus on an ultrasound scan at 31 weeks' gestation. The newborn examination demonstrated penoscrotal hypospadias with chordee and two gonads palpable in the scrotum with a right hydrocele. Ultrasound showed a saccular structure containing debris behind the bladder. The postnatal karyotype was revealed to be 45X/46XY, with a pseudodicentric Y chromosome. Cystoscopy/genitography identified a uterus and a right fallopian tube, which were removed along with a dysgenetic right gonad. Biopsy of the descended left gonad revealed rare germ cells. The final diagnosis was 45X/46XY male pseudohermaphroditism with testicular dysgenesis. One should be aware of possible chromosomal mosaicism and combine the prenatal karyotype with the ultrasound genital findings to formulate an intersex differential diagnosis.     

Cesarean section in a pregnant patient with placenta percreta involving the urinary bladder

Placenta percreta involving adjacent structures is serious complication of pregnancy with a high mortality rate. A 32-year-old woman, gravida 4, para 3, who had previously undergone a cesarean section, was admitted to our hospital at 31 weeks' gestation for placenta previa. At 33 weeks' gestation, the diagnosis of placenta percreta with involvement of the urinary bladder was made by ultrasonography and magnetic resonance imaging. At 34 weeks' gestation, an elective cesarean section was scheduled. Anesthesia was maintained with sevoflurane in oxygen before delivery, and with nitrous oxide in oxygen, fentanyl and midazolam after delivery. During the operation, attempts to remove the placenta resulted in massive hemorrhage. Blood loss for the procedure was 13,800 g. Because of the extreme hemorrhage, we encountered hemorrhagic shock and postoperative complications despite the preoperative preparation. In case of placenta percreta, it is essential to prepare adequate volume of blood for transfusion at the start of surgery and secure large bore intravenous lines. A rapid transfusion device may be recommended. Regarding the anesthetic management, general anesthesia is preferable in consideration of the risk of hemorrhagic shock and the length of operation time. Furthermore, we need team approach and preoperative management to prevent the uncontrolled hemorrhage in such a severe case.     

Management and outcome of pregnancy complicated by severe pre-eclampsia of early onset

Fifty-six patients with severe gestational proteinuric hypertension presenting at less than 32 weeks' gestation with a live fetus were studied to determine complications, possible treatment and outcome. Sixteen patients (28%) were delivered within 72 hours of presentation for maternal or fetal reasons. In 10 patients the blood pressure settled and they received no antihypertensive therapy but 33 patients received oral antihypertensive therapy (mainly monohydralazine and oxprenolol) in an attempt to delay delivery. The mean presentation to delivery interval in these patients was 11.4 +/- 9.1 days with a range of 1 day - 7 weeks, but 17 patients (51.5%) were delivered within 1 week of presentation. The final 13 patients in the series developed serious medical or obstetric complications during the attempt to delay delivery. The caesarean section rate was 75%. There was a 24.5% overall perinatal mortality, and 19% of infants were small for gestational age. Nine infants were delivered before 28 weeks' gestation with only 1 survivor. Antihypertensive therapy allowed delivery to be delayed to after 28 weeks in 11 patients with only 1 perinatal death. The fetal survival was 100% in patients presenting after 30 weeks' gestation. The unpredictability of the course of disease leads to difficulty in deciding the most appropriate management.