Fetus in fetu: a fetiform teratoma

Examination of a retroperitoneal fetus in fetu, diagnosed preoperatively, revealed previously unreported histologic findings in the vascular pedicle and membranous capsule that indicated that these structures are not "umbilical cord" or "amnion." These findings include nervous innervation of both structures and a well-defined lamina elastica interna and vaso vasorum in the artery of the vascular pedicle. Thus, strong support is provided for the theory that many examples of fetus in fetu are remarkably complex, well differentiated, highly organized teratomas. Additional arguments that favor the identity of fetus in fetu and teratoma are presented.     

Primitive Neuroectodermal Tumor: A Newly Recognized Cause of Early Fetal Death

Reviews in the literature of fetal and neonatal neoplasms deal only with congenital tumors, that is, tumors presenting at or shortly after birth. Virtually nothing is known about neoplasms occurring in the embryonal period or early fetal life. We report five small macerated fetuses with a very primitive embryonal tumor of neuroectodermal origin. The tumor characteristically showed massive vascular permeation. Expulsion of the fetus occurred during the fourth or fifth month of pregnancy. It is suggested that this primitive neuroectodermal tumor is a “new” cause of spontaneous late abortion.     

Locking and Reverse Molding of the Fetal Skull

In 1972 Emery described a condition that he called “locking and reverse moulding of the fetal skull.” Crossing and fixation (locking) of the fetal cranial bones prevented normal skull molding, thus giving rise to pressure on the basal structures of the brain. We describe 7 such cases and compared them with the 9 reported by Emery. Multiple hemorrhages were found, frequently at the base of the brain. All pregnancies had gone near or to term. Most mothers were primiparous and labor was often complicated. In several of our cases there was clinical evidence for cephalopelvic disproportion. The babies had normal birth weights and lengths and died during labor or within 48 h after birth. At autopsy, in a number of instances, other traumatic lesions were found. Clinical history and autopsy findings point to an acute condition, and in most cases there were no other findings acceptable as “cause of death.”     

Multiple fetuses in fetu:imaging findings

Fetus in fetu is an extremely rare developmental abnormality secondary to abnormal embryogenesis in a diamniotic monochorionic pregnancy. It occurs when a vertebrate fetus is enclosed within the abdomen of a normally developing fetus. This report describes the prospective diagnosis of fetus in fetu by findings on a plain radiograph and CT scan. At surgery, two fetus in fetu were discovered.     

Normal studies are essential for objective medical evaluations of children who may have been sexually abused

The findings of carefully conducted research studies of non-abused children should be used in medical evaluations for suspected sexual abuse if they are to be legally defensible. These studies have shown that a “wide” hymenal opening and a “narrow” rim of hymen should not be used as markers of abuse.

Conclusion: The study by Myhre and associates is another addition to a growing collection of good science in a field of medicine where objectivity is essential.     

ACARDIAC TWIN FETUS WITH SEVERE HYDROPS FETALIS AND BILATERAL TALIPES VARUS DEFORMITY

Twin reversed-arterial-perfusion syndrome (TRAPS) is a rare complication of monochorionic twin pregnancies. TRAPS is characterized by the hemodynamic dependence of a “recipient” twin from a “pump” twin. The “recipient” twin exhibits lethal abnormalities, such as acardia and acephaly. Circulatory failure of the normal twin derives from the existence of arterio-arterial and veno-venous anastomoses within the placenta that allow retrograde perfusion of the acardiac twin by blood coming from the normal twin. Acardiac twinning is the most extreme manifestation of the twin-twin transfusion syndrome. This occurs in 1 in 100 monozygotic twin pregnancies and 1 in 35,000 births. We report a case of diamnionic monochorionic female twins in which the acardiac twin demonstrated severe hydrops fetalis and bilateral talipes varus deformity. Cesarean section was performed on a 27-year-old hypertensive gravida 2, para 1 mother for fetal indications at 32 6/7 weeks gestation. The acardiac fetus had a two-vessel umbilical cord measuring 43.5 cm in length and 0.8 cm in diameter. The proximal end inserted into the root of the normal twin's umbilical cord in an acute angle forming a “v” close to the placental disc. Structures rostral to the thorax were absent except for a round mass of flesh with three small buds in place of the head and neck, and bilateral upper extremities. Only the kidneys, right adrenal, small and large intestine, and rudimentary urinary bladder were present. Both feet demonstrated talipes varus deformity. The fetus was severely hydropic. The subcutaneous fat measured 4.5 cm in maximum thickness. The normal twin had a protracted course complicated with respiratory distress syndrome, moderate secundum atrial septal defect with left to right shunt, and thrombocytopenia of prematurity. The baby was eventually discharged after approximately 1 month. At the time of this report, 5 months postpartum, the neonate is growing and developing normally. To our knowledge, this is the first report of severe hydrops fetalis and talipes varus deformity in an acardiac twin.     

Attitudes and feelings towards menstruation and womanhood in girls at menarche

Aim: To elucidate early adolescent girls' attitudes, thoughts and feelings towards menstruation and their bodies. Methods: 309 12-y-old girls answered questionnaires. One part of the questionnaire dealt with thoughts and feelings towards menstruation. The other part dealt with thoughts and feelings towards menstruation and sex and ability to communicate on aspects of womanhood. Results: Postmenarcheal girls were less positive towards menstruation than premenarcheal girls (p=1×10^-6). Many girls (43%) did not reaffirm the statement “I like my body” and almost one quarter stated being teased for their appearance. Many of the girls claimed that they had been called “cunt” (38%) or “whore” (46%). If called “cunt” or “whore”, 17% stated that they felt alone, 76% felt anger and 50% were offended. Mothers were those with whom girls could most easily “chat” about their period. Sixty-seven per cent received information about menstruation from school nurses.

Conclusion: Wanting to be an adult and liking that their body develops seem to be associated with a more positive feeling towards menstruation. Furthermore, mothers' timing and ability to communicate attitudes towards menstruation and the body are as important as those in a girl's immediate environment.     

Fetus in fetu associated with an undescended testis

Fetus in fetu is an extremely uncommon cause of an abdominal mass in the neonate; fewer than 30 generally accepted cases are recorded in the literature. We report a case of intraabdominal fetus in fetu, with a unique location within an undescended left testicle. Chromosomal studies of cells from the fetus in fetu and the surviving infant revealed identical 46,XY karyotypes.     

Association of a fetus in fetu and two teratomas: US and MRI

We report a case of an abdominal mass in a newborn girl containing a fetus in fetu and two teratomas. Obstetrical sonography revealed the abnormality at 28 weeks of gestation. Post-natal US examination suggested the diagnosis of a fetus in fetu upon the finding of a vertebral column and fetal skeletal bones. US also showed two other rounded masses connected to the main lesion by vascular bundles. Preoperative MRI examination supplied further information regarding tissue composition and vascularisation of the mass lesion. The diagnosis was confirmed by pathological examination. Aetiological factors and radiological diagnosis of this rare tumour are reviewed and discussed. Received: 29 January 1996 Accepted: 2 February 1996     

Syndromal Associations of Common Origin of the Carotid Arteries

The term “common origin of the carotid arteries” (COCA) has been proposed to replace the older terms “origin of the left carotid artery from the innominate stem” and “bicarotid trunk with anomalous right or left subclavian artery.” These anatomic patterns are usually reported to occur in about 11% of whites and 20-25% of blacks and have been reported to have increased frequency in patients with esophageal atresia-tracheoesophageal fistula, DiGeorge anomaly, and anomalous origin of the left coronary artery from the pulmonary artery. COCA is a significant, if not invariant, feature of the great arteries in the condition usually called in the more recent literature “anomalous origin of the innominate artery,” the most frequent cause of symptomatic tracheal compression by anomalous systemic arteries. Analysis of associations of COCA with various other congenital cardiovascular lesions showed, in addition, significant association with congenital polyvalvular disease, truncus arteriosus, aorticopulmonary window, trisomy 13, 18, and 21 syndromes, acrocephalosyndactyly (especially Apert syndrome), tetralogy of Fallot not associated with DiGeorge anomaly, and clinical Noonan phenotype. Pentalogy of Cantrell was associated with no increase in incidence of COCA.     

THE SPECTRUM OF TYPE III LISSENCEPHALY: A CLINICOPATHOLOGICAL UPDATE

A third type of lissencephaly that does not fulfil diagnostic criteria of type I (“classical”) and type II (“cobblestone”) lissencephaly was described by our group as a new entity identified as OMIM 601160. This lethal familial syndrome comprises micrencephaly/lissencephaly and a spectrum of abnormalities lined to a severe fetal akinesia deformation sequence. Neuropathological findings suggest severe neurodegeneration leading to a marked neuronal dropout of the entire central nervous system and atrophy. Similar neuropathological findings have been described in the Neu-Laxova syndrome (NLS), an apparently different lethal malformation syndrome. Neuropathological similarities between OMIM 601160 and NLS raise the question of clinicopathological variability and genetic heterogeneity of type III lissencephaly. To answer this question, we compared our clinicopathological findings in a series of fetuses with OMIM 601160 to pathological data reported in NLS. In the study, 5 unrelated families with 7 affected fetuses were included. Interestingly, we found striking clinicopathological similarities between OMIM 601160 and NLS, which may represent a variability of a single neurodegenerative disease with early prenatal onset. Molecular studies in multiplex families defined through detailed clinicopathological screening are needed to clarify the distinction, if any, between these two entities.     

Surgical Pathology of Epilepsy: A Review

This review discusses the neuropathological issues pertaining to temporal lobectomies and neocortical resections for medically refractory seizures of childhood. Most cases in our pediatric series are “lesional” and have CT/MRI abnormalities that contribute to a higher incidence of “dual pathology” lesions rather than pure mesial temporal sclerosis. Almost up to two thirds of our cases are represented by neuronal migration disorders and low grade gliomas. We have also encountered lesions that have both neoplastic and malformative foci, some of which behave more like tumors, while others defy precise classification as tumor or malformation. Our preliminary data indicate a role for cytomegalovirus in some cases of Rasmussen's encephalitis.     

Myeloproliferative Syndrome in Childhood

This report describes an atypical “myeloproliferative disorder” in a child that evolved over a 6-year period. The clinical and laboratory features resembled the adult type of chronic myelogenous leukemia, but a Philadelphia chromosome was lacking. Although the proper classification of this disorder remains uncertain, the case makes a good point for the use of diagnostic procedures such as the bone marrow core biopsy and cytogenetic studies to help unravel and define the characteristics of these unusual “myeloproliferative” disorders.     

Oropharyngeal fetus in fetu

Fetus in fetu is an extremely rare entity and refers to the growth of a parasitic twin within a more mature fetus due to its inclusion within cells of the blastocyst. The presence of a vertebral column is considered essential for the diagnosis of a fetus in fetu. The retroperitoneum is the most common location for a fetus in fetu and its location in the mouth is rare. We report a case of oropharyngeal mass detected on antenatal sonography with imaging features consistent with a fetus in fetu. Complete surgical removal of the mass was aided by imaging. Follow-up of these patients is recommended to detect the rare incidence of malignant degeneration.     

Asynchronous Pulmonary Hyperplasia Associated with Tracheal Atresia: Pathologic and Prenatal Sonographic Findings

We describe a case of pulmonary hyperplasia associated with tracheal atresia and a complete obstruction to the egress of pulmonary secretions. In classical pulmonary hyperplasia associated with cartilagenous laryngeal atresia and a persistent pharyngotracheal duct, the histologic appearance of the lungs is normal but exhibits “synchronous” hypermaturity. The histologic pattern in our case is much less mature, resembles CAM type III, and exhibits “asynchronous” development. We suggest that these histologic patterns be distinguished and that pulmonary hyperplasia is probably underrecognized and not nearly as rare as previously thought.     

Intraventricular twin fetuses in fetu

The authors report a rare case of multiple intracranial fetuses in fetu, fulfilling Willis' traditional criteria, which include an axial and appendicular skeleton with surrounding organized tissue. This case was ascertained from studies of a full-term female neonate who presented with ventriculomegaly. A CT scan showed intracranial calcifications that were suggestive of an axial skeleton. Her birth weight was 3.176 kg (50th-75th percentile), length was 52 cm (90th percentile), head circumference was 35 cm (50th-75th percentile), and Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. Prenatal ultrasonography studies performed at 12 weeks and 5 days, and 19 weeks and 6 days revealed normal findings. A 37-week prenatal ultrasonography study showed ventriculomegaly and obstructive hydrocephalus, with a possible intracranial teratoma. Cranial imaging at birth with ultrasonography, CT and MR imaging, and MR angiography demonstrated 2 complex intraventricular masses with cystic, solid, and bony elements. A craniotomy with resection of the masses was performed at 3 months of age. The infant survived and is now 12 months old with some developmental progress. Two axial skeletons, with accompanying rib cage and extremities, including well-formed feet and toes, were noted. Both anencephalic structures had skin with hair, fat, skeletal and smooth muscle, and bony structures with bone marrow and focal areas of calcification. Multiple viscera were present and included thymus, bowel, stomach, salivary gland, kidney, adrenal gland, lung, and presumed adnexal structures. A diagnosis of fetuses in fetu was rendered. Chromosomal studies of the child and tissue from the 2 fetuses in fetu showed normal female karyotypes. A single nucleotide polymorphism array analysis from the proband infant and tissue from the 2 identified fetuses in fetu appeared to be genetically identical. These results are consistent with a monozygotic twin embryonic origin of the fetus in fetu tissue, which is a mechanism that has been suggested in previous reports in which karyotypes, blood types, and limited genetic loci have been studied. This is the first report of a rare example of intracranial intraventricular twin fetuses in fetu for which a genome-wide single nucleotide polymorphism assay has confirmed their genetic identity.     

Expression of Early and Late Adenoviral Proteins in Fatal Adenovirus Bronchopneumonia

The localization and distribution of three adenoviral proteins, hexon, E1 A, and 55-kDaE1B, in 16 cases of fatal adenovirus bronchopneumonia in infants and children, are described. The proteins were immunohistochemically demonstrated in paraffin sections using monoclonal antibodies followed by the avidin-biotin-peroxidase method. The hexon antigen was present in inclusion-bearing bronchial, bronchiolar, and alveolar cells, mainly in the so-called rosette cells, as well as in necrotic debris in necrotizing areas. E1A antigen was also recognized in cells with nuclear inclusions where the reaction decorated the inclusion, nuclear chromatin, and cytoplasm but distributed mainly in alveolar cells and to a lesser extent in bronchial and bronchiolar cells. The 55-kDa E1B protein was extensively present in “activated,” reactive-appearing, nuclei of bronchial, bronchiolar, and alveolar epithelial cells and in the cytoplasm of rare cells having nuclear inclusions. These activated nuclei did not stain for the other two antigens. “Smudge” cells reacted poorly or not at all with any of the antibodies. The reactivity found produced a sort of complementary pattern between the hexon-positive, inclusion-containing cells and the 55-kDa E1B-positive, inclusion-noncontaining cells. The relationships of present findings and uirologic data are discussed.     

Fetus in fetu: a case report

A fetus in fetu is defined as a condition where a parasitic twin is found included within the body of its partner [8]. This is a report of a fetus in fetu treated successfully in a 4-mounth-old boy that fulfilled all the criteria of the disease. A brief review of the literature is also included.     

Nesidiodysplasia and Nesidioblastosis of Infancy: Structural and Functional Correlations with the Syndrome of Hyperinsulinemic Hypoglycemia

Subtotal pancreatectomy specimens of seven infants with persistent hyperinsulinemic hypoglycemia were studied; all showed the characteristic light microscopic picture of nesidioblastosis. Specimens were studied by electron and conventional light microscopy and by light microscopic immunohistochemistry for insulin, glucagon, somatostatin, and HPP (human pancreatic polypeptide); double staining and quantitative methods were also used. Findings were compared with those in age-matched controls.

In the hyperinsulinemic hypoglycemic infants, an increase in total endocrine cell volume was found; however, the typical features of nesidioblastosis were also found in the controls. In both groups, immunohistochemistry and electron microscopy suggested that some endocrine cells are capable of producing synchronously more than one hormone. Amphicrine (”composite” or “intermediate”) cells with exocrine and endocrine differentiation were found in three hypoglycemic infants.

Observations are discussed in relation to current concepts of embryo genesis of the gastroenteropancreative endocrine system. We conclude that nesidioblastosis, as defined anatomically cannot be considered as the morphologic basis of hyperinsulinemic hypoglycemia. The term “nesidiodysplasia” is suggested and includes increased, maldistributed, and malregulated or malprogrammed endocrine and amphicrine cells when associated with endocrine abnormality.     

Effect of helicobacter pylori eradication on sideropenic refractory anaemia in adolescent girls with Helicobacter pylori infection

We investigated the effect of Helicobacter pylori eradication on sideropenic refractory anaemia in adolescent girls with H. pylori-associated antral gastritis without evidence of haemorrhage or clinical symptoms other than sideropenic anaemia. We conducted an open therapeutic trial in 21 adolescent girls aged 15-17 y with sideropenic refractory anaemia, which was defined as iron-deficiency anaemia refractory to oral iron therapy for 3 mo. All subjects underwent gastroduodenal endoscopy. Thirteen patients with confirmed H. pylori infection were given a 2-wk course of triple therapy and 6 wk of oral ferrous sulfate. We compared the mean levels of haemoglobin and serum ferritin among the “initial sample” (the time when the sampling was done before treatment with oral iron), “before eradication” (the time prior to triple therapy for eradication after subjects had been given oral iron for 3 mo) and “after eradication” (the time when the follow-up endoscopy was performed) data in 11 subjects in whom H. pylori infection was eradicated. Haemoglobin levels (g/dl) were 8.6 ± 1.9, 8.6 ± 1.4 and 11.3 ± 2.3, respectively. Serum ferritin levels (μg/l) were 4.6 ± 2.4, 4.2 ± 2.3 and 17.5 ± 6.7, respectively. After eradication of H. pylori, mean levels of haemoglobin (p = 0.0002) and serum ferritin (p = 0.0002) showed a prominent increase between “before eradication” and “after eradication”. In conclusion, adolescent girls with sideropenic refractory anaemia should be evaluated for H. pylori infection. If H. pylori infection is coexistent, its eradication along with iron supplementation could correct the anaemia.