Experimental study concerning safety dosage of OK-432 for intrauterine treatment

OBJECTIVE: Clinical intrauterine treatment for fetal cystic hygroma has so far been performed in a few patients; however, it is still difficult to evaluate the results. The aim of this study is to establish the safe dosage of OK-432 in the intrauterine treatment of fetal cystic hygroma. METHODS: OK-432 was injected either subcutaneously behind the neck of the fetuses or into the amniotic cavity through the uterine wall of pregnant Japanese white rabbits at 27 days of gestation. Saline was administered to the controls. The dosage and the site of injection were as follows: group 1, OK-432, 0.01 KE (0.25 KE/kg) in 0.2 mL saline per fetus, subcutis; group 2, OK-432, 0.02 KE (0.5 KE/kg) in 0.2 mL saline per fetus, subcutis; group 3, OK-432, 0.04 KE (1 KE/kg) in 0.2 mL saline per fetus, subcutis; group 4, OK-432, 0.01 KE in 0.2 mL saline per fetus, amniotic cavity; group 5, OK-432, 0.04 KE in 0.2 mL saline per fetus, amniotic cavity; group 6, saline, 0.2 mL per fetus, subcutis; group 7, saline, 0.2 mL per fetus, amniotic cavity. All fetuses were delivered at 29 days of gestation. RESULTS: The mother's rectal temperature was mostly in the normal range throughout the experiment. There was no significant difference between any of the seven groups in fetal body weight. The C reactive protein values of all fetuses were negative. The appearance of the skin of all the fetuses was normal. The histopathological findings of the skin in the OK-432 groups showed a moderate infiltration of monocytes and plasma cells. No pathological changes were observed in the heart, lung, liver or kidneys of any of the fetuses. CONCLUSION: Based on this rabbit experiment, we determined that OK-432 may be safely used at a dose of up to 1 KE/1 kg of fetal body weight as an intrauterine treatment for fetal cystic hygroma.     

Ultrasonography's role in prenatal diagnosis: The nuchal fold

Two cases of nuchal fold abnormalities diagnosed by transvaginal ultrasonography are presented: nuchal edema at 10 weeks (fold = 5.5 mm) of normal size at 13 weeks (fold = 2.6 mm) and cystic hygroma colli associated with omphalocel in a women with a 24-week arrested pregnancy terminated by spontaneous abortion. Genetic tests done in the second case showed a karyotype 45XO. The etiopathogenesis of nuchal skin fold abnormalities--edema and cystic hygroma colli is discussed. The importance of ultrasound screening during pregnancy is emphasized, the detection rate of genetic abnormalities depending on both the quality of the ultrasound equipment and examiners skills. Ultrasound examination at 10 and 20 weeks--ultrasound marker (nuchal fold, LF ratio and DBP/FL ratio)--together with the triple test (AFP, HCG and E3) in the mathematical model used for detecting the risk of genetic defects may represent an alternative to amniocentesis and trophoblast biopsy when this is impracticable or rejected by the patient.     

Surgical management of parapharyngeal cystic hygroma causing sudden airway obstruction

Parapharyngeal cystic hygroma is a rare tumor of the neck. This report describes two cases in which surgical resection was necessary to overcome sudden airway obstruction and details the surgical technique. These cases were considered "near misses" for sudden infant death syndrome (SIDS) and were revealed by computed tomography (CT) and echography to be parapharyngeal cystic hygroma. The location of this malformation could have produced sudden airway collapse and be erroneously diagnosed as SIDS. The postoperative follow-up was satisfactory and no recurrence was detected. We believe CT and echography should be included in the evaluation of such cases.     

Unilocular Cystic Lymphangioma of Thigh—An Extremely Rare Clinical Entity

Majority of cystic lymphangiomas are multilocular and occur in neck and axilla. The cystic lymphangioma is also known as cystic hygroma. Unilocular cystic hygroma of neck and breast have been reported in literature and have been termed hydrocele of neck and breast respectively. However unilocular cystic hygroma of thigh is probably being reported for the first time.     

Report of a case: the anesthetic management for fetal tracheal intubation with intact uteroplacental circulation during cesarean section

We experienced a case of cesarean section in a patient with a fetus having a prenatally diagnosed huge cervical mass, which could cause airway obstruction immediately after delivery. The fetal cervical mass was confirmed at 19 gestational weeks, and amniocentesis was performed at 33 weeks. At 35 weeks, MRI showed the large mass that could disturb the airway patency after birth, and elective cesarean section was scheduled at 37 weeks. In the anesthetic management, anesthesia was maintained with isoflurane in oxygen, and ritodorine and nitroglycerin were used for obtaining uterine relaxation and keeping uteroplacental circulation. Fetal head was exposed from the uterine incision site and tracheal intubation was successfully performed. After confirming the neonatal oxygenation under manual ventilation, the baby was delivered. After delivery, the mass was diagnosed as cystic hygroma and he was maintained under mechanical ventilation in NICU. Five months later subtotal excision of the cervical cystic hygroma and tracheostomy were performed.     

Giant cystic hygroma associated with venous aneurysm.

Complete excision of giant cystic hygroma requires meticulous dissection of the multilocular lymphatic cysts from the adjacent vascular and neural structures. The association of venous aneurysms with cystic hygroma is extremely rare. We report two infants with cystic hygroma in whom preoperative diagnosis of venous aneurysm was helpful in planning complete excision of the lymphatic lesions.     

超声诊断孕早期胎儿颈部水囊状淋巴管瘤

目的探讨孕早期胎儿颈部水囊状淋巴管瘤（NCH）的超声诊断价值。方法收集2005年1月—2011年8月在我院发现的NCH胎儿36胎,超声观察其囊状扩张的部位、大小、内部情况及胎儿有无水肿,头部、胸腹内脏、四肢、脐带有无异常,并进行随访,记录产后新生儿复查情况或染色体、引产的结果。结果 36胎NCH胎儿中,1胎NCH位于腋下,35胎位于颈部。13胎接受染色体检查,其中6胎染色体核型正常;7胎异常染色体核型中,3胎核型为45,XO;1胎为46,XX/45,XO;1胎Bart＇s水肿;1胎21-三体;1胎合并上肢内翻,46,XY。全部36胎中,1胎出生后证实合并尿道下裂;3胎合并全身水肿,中、晚孕胎死宫内;3胎合并心脏畸形,在中、晚孕期接受引产;2胎单房性淋巴水囊瘤自行消失,出生后观察至产后6个月,未见明显结构异常;其余27胎均引产,并经尸体检查证实。结论孕早期超声可在检测胎儿颈部透明层厚度的同时早期诊断NCH,判断有无分隔、合并畸形,对临床医师评估胎儿预后、指导临床处置、适时终止妊娠、实行优生优育有重要临床意义。     

Congenital nephrotic syndrome

Congentital nephrotic syndrome (CNS) is an uncommon disorder. Several different diseases may cause the syndrome. These may be inherited, sporadic, acquired or part of a general malformation syndrome. The problems associated with nephrotic syndrome in early infancy are divided into three parts: diagnosis, treatment and prenatal diagnosis. Accurate diagnosis is essential for the treatment, genetic counselling and prenatal diagnosis. The ultimate curative treatment of CNS is renal transplantation. The supportive treatment before the transplantation is of utmost importance in order to maintain a reasonable clinical condition and prepare the child for the dialysis and renal transplantation. Prenatal diagnosis is possible in some types of CNS by determination of the maternal serum and amniotic fluid alpha-fetoprotein (AFP). Increased AFP indicates fetal proteinuria, and thereby nephrotic syndrome before birth. In some cases with the onset of proteinuria after birth prenatal AFP measurement does not detect the disease.     

Scrotal cystic hygroma in a 13-year-old boy

We describe a patient with cystic hygroma, in a rare location (scrotum). The hygroma was diagnosed incidentally after injury to the scrotum in a 13-year-old boy. The diagnostic methods used, the characteristics of this type of tumor, its treatment and its clinical course are described. We suggest that cystic hygroma be taken into account in the differential diagnosis of other more frequent causes of scrotal masses.     

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)     

Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature

A case of pancreatoblastoma (PB) in a 2 month-old male infant with incomplete Beckwith-Wiedemann syndrome is presented. Clinical examination disclosed left hemihypertrophy, macroglossia, bilateral exophthalmos, and enlargement of the left testis. Imaging with ultrasound and computed tomography scan showed a well-defined, heterogeneous, and grossly cystic mass arising from the head of the pancreas. Serum alpha-fetoprotein (AFP) level was elevated. The tumor was completely resected, and the histological analysis showed PB. The patient's recovery was uneventful, and AFP returned to normal values after surgery. The child has been disease-free for 5 years, and his serum AFP remained within normal values. Six other examples of this association, PB, and Beckwith-Wiedemann syndrome are recorded in the literature. The risk of developing tumor in this syndrome (complete and incomplete form) increases when hemihypertrophy is present, and the need for routine screening examination is warranted. Beckwith-Wiedemann syndrome was suggested to be a favorable biological marker for survival in children who have intraabdominal tumors.     

A case of cystic hygroma of the chest wall

Hygroma is generally regarded as a benign tumor occurring favoritely in the cervical region of a child. Recently we have experienced a case of hygroma which was initially detected as a right chest wall tumor in a 16-year-old man, then was surgically diagnosed as cystic hygroma; chest roentgenography on a check up examination in his senior high school pointed out some anomaly, leading to his hospitalization. On admission, he had no subjective symptom, but chest roentgenography and CT confirmed an abnormal shadow in the right pulmonary area and a tumor with calcification on the right chest wall, respectively, so he was operated on for excisional biopsy. The tumor was extirpated together with the 7th intercostal muscle adhered to the tumor, and postoperative pathohistological examination led to diagnosis as cystic hygroma. Cystic hygroma of the chest wall is so rare that we would like to report this case, together with some addition consideration of related literature.     

Cystic hygroma in Saudi Arabian children

Seven cases of cystic hygroma in Saudi Arabian children were treated in King Khalid University Hospital, Riyadh between 1984 and 1987. One newborn child had bilateral giant cystic hygroma. Investigation included ultrasound and computed tomographic scan. All patients but one underwent single or multiple surgical procedures for excision of the cystic hygroma.     

Spontaneous chylopericardium in an adult due to mediastinal cystic hygroma

Chylopericardium is a rare and benign condition. Apart from common causes like non-surgical trauma, tuberculosis, malignancy, radiation, and postoperative, mediastinal cystic hygroma presenting as chylopericardium is an extremely rare entity. Primary or idiopathic chylopericardium is diagnosed when the precise cause is not known. It is a diagnosis of exclusion. We report a 27-year-old lady with mediastinal cystic hygroma, presenting as spontaneous chylopericardium, who was managed surgically with no recurrence on 18 months follow-up. She was evaluated for complaints of discomfort in the upper abdomen region and breathing difficulty in left lateral position for 4 days, and was found to have a large pericardial effusion with impending tamponade. She underwent pericardiocentesis, and on fluid analysis, it was confirmed as chylous pericardial effusion. She was evaluated thoroughly and was taken up for right video-assisted thoracoscopy. The thoracic duct was clipped and a window was created in the pericardium, the cystic hygroma was excised, and pleurodesis was done. The postoperative period was uneventful. Histopathology of the pericardial window showed chronic inflammatory pathology and cystic lesion was confirmed as a chylous cyst.     

Raised maternal serum alpha-fetoprotein levels not associated with fetal malformations. A case report

A pregnant black woman was found to have extremely high serum alpha-fetoprotein (AFP) concentrations on several occasions between 18 and 28 weeks of gestation; the amniotic fluid total AFP level was borderline at 22 weeks, and the concanavalin-A non-reactive fraction was below normal. At this stage termination of pregnancy was considered, but qualitative acetylcholinesterase electrophoresis was negative and repeated echographic examinations revealed no fetal defect. The pregnancy was allowed to continue, and a normal premature female infant was delivered at 36 weeks. Possible explanations and the potentially serious implications of the results of laboratory tests in a case such as this are discussed.     

Cystic hygroma in the quadriceps muscle: a sanguine diagnosis for knee pain

Reported here is a 28-year-old female who presented with severe right knee pain and swelling nearby the joint. Ultrasonography, magnetic resonance imaging and eventually surgery were performed. She was diagnosed to have a cystic hygroma in the vastus lateralis muscle. This is the first patient of a cystic hygroma in the quadriceps muscle.     

Prenatal diagnosis of cystic fibrosis in South Africa

Prenatal diagnosis of cystic fibrosis (CF) has been made possible by the finding that the activity of various enzymes derived from the microvillar membranes of the fetus is decreased in 2nd trimester amniotic fluid. Gamma-glutamyl transpeptidase, aminopeptidase M and the phenylalanine-inhibitable form of alkaline phosphatase (AP) have been found to be of most diagnostic use in this respect, the odds of the fetus being affected with CF being 28:1 if the AP test is positive. When couples have already had a child with CF, pregnancies are being monitored by these methods at the University of Cape Town.     

Correction of congenital hydrocephalus in utero II: Efficacy of in utero shunting

To study the effect of in utero ventricular decompression of hydrocephalus on brain development and prognosis, and to evaluate the function and possible complications of different shunt designs, we created fetal hydrocephalus in 28 fetal lambs and 17 fetal monkeys by injecting kaolin into the cisterna magna during the third trimester. One fetal lamb had indwelling intracranial subdural and amniotic cavity pressure catheters placed to study serial changes in the relationship of intracranial pressure (ICP) and amniotic fluid pressure (AFP) from the time of injection until term. Twenty hydrocephalic fetal lambs underwent ventricular decompression--ventriculoamniotic (V-A), N = 10; ventriculo-right atrial (V-RA), N = 9; and ventriculo-pleural (V-PL), N = 1--21 to 25 days after the kaolin was injected; seven hydrocephalic fetuses were left unshunted as controls (CON). Eight fetal monkeys underwent V-A decompression 14 to 21 days after kaolin injection; nine were left unshunted as controls. All animals were delivered by cesarean section near term, assessed for viability and their brains examined grossly and microscopically. After kaolin injection in the fetal lamb, ICP (r = 0.94) and ICP minus AFP (r = 0.93) rose in a linear fashion, while AFP showed no trend (r = 0.22). All unshunted newborn lambs and monkeys had split sutures, dilated ventricles, and thinned cortical mantle. Most shunted lambs showed anatomic improvement with decreased head circumference, overriding sutures, normal-sized ventricles, and improved survival. However, histopathology revealed marked white matter destruction. In contrast, most shunted monkeys showed little anatomic improvement. Both shunted and unshunted monkey brains showed a severe inflammatory ventriculitis. In both lambs and monkeys, shunting was associated with a variety of complications including subdural hematoma, subdural hygroma, shunt infection, shunt occlusion, and improper shunt tip placement. In this experimental model, in utero decompression of obstructive hydrocephalus improves overall survival, improves gross ventriculomegaly, does not improve histopathologic brain damage, and is associated with significant complications. The choice of the optimal shunt design and the effect on postnatal neurologic function requires further study. This work emphasizes the need for continuing research in an animal model prior to human application.     

Tumor marker levels in post-chemotherapy cystic masses: clinical implications for patients with germ cell tumors

PURPOSE: Increased tumor markers after induction chemotherapy for patients with germ cell tumor usually represent systemic disease and consequently second line chemotherapy is instituted, while retroperitoneal lymph node dissection (RPLND) is reserved for patients with marker normalization. We report the concentration of alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) in the fluid of post-chemotherapy cystic masses to evaluate this as a potential source for serum marker elevation. MATERIALS AND METHODS: From March 2002 to December 2002, 11 consecutive patients with post-chemotherapy cystic masses underwent RPLND. Following resection, aspirated fluid was analyzed for AFP and HCG. Only 5 post-chemotherapy RPLNDs were performed in patients with increased serum tumor markers, including the 3 patients in our study. Patients with increasing tumor markers and/or multifocal disease with noncystic residual masses after induction chemotherapy underwent salvage chemotherapy despite teratomatous elements in the primary tumor. RESULTS: All 11 patients had teratoma in the orchiectomy specimen and retroperitoneum, including one with malignant transformation. Cystic fluid markers were increased in all patients, 9 of 9 with HCG (range 7.0 to 6,880) and 9 of 11 with AFP (27.5 to 521.2). Two patients with an increased serum AFP before surgery (47.9 and 31.6) had cyst levels of 73.5 and 790.4 respectively. Both serum markers normalized postoperatively. One patient with increased pre-RPLND serum HCG (11.6) had a cyst level of 233. HCG continued to increase postoperatively and the patient died of disease. The remaining 10 patients remain disease free. CONCLUSION: Fluid from cystic teratoma contains variably elevated levels of HCG and AFP in all patients and appears to be independent of serum marker level or pathology. It is possible that a "slow leak" of fluid from cystic teratoma may explain elevated serum markers in selected patients with teratoma and thus may potentially avoid second line chemotherapy.     

Laryngeal cystic hygroma

An unusual case of cervical cystic hygroma with laryngeal extension is reported. A patient with acute upper airway obstruction due to a laryngeal lesion was seen 18 years after the original cervical surgery. Maintenance of an adequate airway has required a partial laryngectomy in addition to repeated endoscopic procedures for removal of recurrent disease. The rationale for this therapy is discussed in light of the current literature.