Two cases of fetus in fetu

Fetus in fetu (FIF) is a rare cause of abdominal mass in children. One of the malformed monozygotic diamniotic twins is located in the body of other twin. It is differentiated from teratoma by the presence of vertebral organization with limb buds and other organ systems. Diagnosis is based on radiologic findings. Surgical excision is the treatment of choice, leading to the complete removal of the mass. To our knowledge, less than 200 cases have been described in the literature. Herein, we report 2 cases of FIF, a newborn who was diagnosed antenatally and a three-and-half-year- old boy diagnosed with mediastinal FIF after admission for recurrent respiratory tract infections.     

Fetus-in-fetu presenting as cystic meconium peritonitis: diagnosis, pathology, and surgical management

Fetus-in-fetu (FIF), a rare congenital anomaly, is a fetus incorporating the well-differentiated tissue of its twin. The authors describe a newborn who presented with massive abdominal distension and severe respiratory distress. Abdominal x-rays showed multiple calcifications. The diagnosis of meconium pseudocyst was made. At emergency laparotomy an irregular fetiform mass was found in the retroperitoneum lying within a fluid-filled amniotic sac. It contained a vertebral column, 10 limblike structures, and cranial and caudal ends, supporting the diagnosis of fetus-in-fetu. This case highlights several important points. FIF often is overlooked in the differential diagnosis of a newborn abdominal mass and, as in this case, may be confused with meconuim pseudocyst. FIF should be differentiated from a teratoma because of the latter's malignant potential. Because this diagnosis is not made until pathological analysis, all parts of the mass should be removed to prevent malignant recurrence.     

Fetus in fetu--diagnostic criteria and differential diagnosis--a case report and literature review

Fetus-in-fetu (FIF) is a rare congenital condition in which a vertebrate fetus is incorporated within its twin. The authors report the case of a newborn boy with prenatal ultrasonographic diagnosis of intraabdominal mass, provoking compression of the left kidney. Plain abdominal radiography, ultrasonography, and computer tomography showed a cystic mass containing multiple calcifications. Pathologic examination showed an irregular fetiforme mass, weighing 8 g, attached to an amniotic sac by a rudimentary umbilical cord with 2 rudimentary limbs, vertebral bodies, encephalus, coroidal plexus, stomach, duodenum, bowel, adrenal glands, upper and lower respiratory tissue, spleen, lymphoid tissue, single cavity heart, and kidney. Molecular analysis using an informative genetic marker, for uniparental isodisomy of chromosomes 14 and 15 showed no genetic difference between the host infant and the fetiform mass. Serum alpha-fetoprotein was ≤ 16.000 UI/mL (reference values for age, 4 to 18.9904 UI/mL) before surgery and 8.364 UI/mL after surgery.     

Laparoscopic resection of solitary congenital liver cyst in a neonate

Solitary congenital liver cysts are a rare finding and present a challenge in the diagnosis. Routine prenatal ultrasounds confirmed the presence of an isolated cystic mass in the upper abdominal cavity of a fetus during the third trimester of gestation. Ultrasound examination performed after birth confirmed the presence of a floating cyst in the upper abdomen. Laparoscopy was performed on the 12th day to confirm the diagnosis using a 2.7 mm scope and instruments. Using a monopolar hooked cautery, the cyst was resected free from the liver surface without any complication. The postoperative course was uneventful. Histopathologic analysis demonstrated a true liver cyst with a mesothelial lining and hepatic residues. To our knowledge, this is the first case reported in literature where a solitary liver cyst was resected using minimal invasive techniques in a newborn.     

Spinal tuberculosis: a review

Spinal tuberculosis is a destructive form of tuberculosis. It accounts for approximately half of all cases of musculoskeletal tuberculosis. Spinal tuberculosis is more common in children and young adults. The incidence of spinal tuberculosis is increasing in developed nations. Genetic susceptibility to spinal tuberculosis has recently been demonstrated. Characteristically, there is destruction of the intervertebral disk space and the adjacent vertebral bodies, collapse of the spinal elements, and anterior wedging leading to kyphosis and gibbus formation. The thoracic region of vertebral column is most frequently affected. Formation of a 'cold' abscess around the lesion is another characteristic feature. The incidence of multi-level noncontiguous vertebral tuberculosis occurs more frequently than previously recognized. Common clinical manifestations include constitutional symptoms, back pain, spinal tenderness, paraplegia, and spinal deformities. For the diagnosis of spinal tuberculosis magnetic resonance imaging is more sensitive imaging technique than x-ray and more specific than computed tomography. Magnetic resonance imaging frequently demonstrates involvement of the vertebral bodies on either side of the disk, disk destruction, cold abscess, vertebral collapse, and presence of vertebral column deformities. Neuroimaging-guided needle biopsy from the affected site in the center of the vertebral body is the gold standard technique for early histopathological diagnosis. Antituberculous treatment remains the cornerstone of treatment. Surgery may be required in selected cases, e.g. large abscess formation, severe kyphosis, an evolving neurological deficit, or lack of response to medical treatment. With early diagnosis and early treatment, prognosis is generally good.     

Caution in antenatal intervention

We assessed 18 fetuses who harbored a urinary tract malformation that was diagnosed by antenatal sonography. The antenatal diagnosis corresponded to the postnatal diagnosis in 66 per cent of the cases. We review the course of 6 fetuses who had catheters placed percutaneously to drain dilated urinary tracts that were believed to be caused by posterior urethral valves (5) or an obstructed megaureter (1). Only 2 of these fetuses exhibited valves postnatally. No fetus had any recognized benefit from the antenatal intervention. We found that sonography may not readily differentiate fetuses with hydronephrosis with obstruction from those without obstruction. From this experience we conclude that intervention in pregnancies suspected of harboring a fetus with a malformed urinary tract should be done cautiously. Antenatal sonography is useful to identify the fetus with a dilated urinary tract. This identification permits perinatal specialists to be alerted so that preparations for reconstructive surgery in such cases can be made early postpartum.     

Fetus in fetu removal in a 47-year-old man.

A case of removal of a fetus in fetu in a 47-year-old man is reported. The patient had an upper abdominal mass since birth that had never caused any subjective symptoms. A preoperative computed tomographic scan was useful to confirm the diagnosis. The operative specimen consisted of a cystic mass about 20 cm in diameter, situated in the upper retroperitoneal space. The cyst was full of a yellowish fluid and hairs. A bony structure, about 10 cm in diameter, contained a vertebral axis connected to the ribs and was adherent to the cystic wall. To our knowledge this is the first reported case of fetus in fetu described in an adult man. The tumor, present for 47 years, did not grow or cause any complications and did not show any sign of malignancy.     

Fetus-In-Fetu or Well-Differentiated Teratoma- A Continued Controversy

Fetus in fetu is an uncommon anomaly in pediatric age group. There have been controversies regarding its differentiation from well differentiated teratoma. While presence of axial skeleton has been considered mandatory for diagnosis, there are many reports of fetus in fetu without any vertebral column. It has been suggested that the two entities form a spectrum of lesions depending upon the timing of embryological insult. The present case is another example of a lesion that fits in this spectrum of interesting lesion.     

Fetus-in-fetu: a case report with molecular analysis

This is the report of a case of fetus-in-fetu diagnosed in a 3-month-old boy and found to be located in the upper retroperitoneum. The entity was distinguished from teratoma by the presence of vertebral axis with limb buds. It corresponded to a diamniotic, monochorionic, monozygotic twin. Complete excision of the mass was performed. Radiological, histopathologic, and DNA fingerprinting studies performed on fetus-in-fetu specimen showed that the fetus was a monozygotic twin.     

Fetus-in-fetu: report of a case and a review of the literature

A 2-week-old girl was found to have an asymptomatic abdominal mass on routine examination. Surgical removal revealed the mass to have 4 limb buds situated relative to a palpable vertebral column. Karyotyping revealed the mass to be 46 XX. The mass was therefore diagnosed as a case of fetus-in-fetu. The literature on this rare entity is reviewed, and the diagnosis and pathogenesis of the disease are discussed.     

Abdominal pregnancy. Case report,review of the literature

Abdominal pregnancy (AP) is a rare form of ectopic pregnancy (EP). It is a medical emergency because of high maternal and fetal morbidity and mortality. Early diagnosis is essential. Surgery is indicated as soon as the diagnosis is made. We present a case with AP in the third trimester with malformed fetus and left ovarian tumour. The fetus has been extracted at 33 weeks gestational age; the placenta and the ovarian tumour have been removed five weeks later. Pathology showed corpus luteum with hemorrhage, serous cystadenoma, follicular cyst. The fetus died, the woman successfully recovered.     

The surgical pathology of notochordal remnants in adult intervertebral disks: a report of 3 cases

The notochord plays a critical role in organizing and directing vertebral development. In humans, most notochordal cells are eventually sequestered into the nucleus pulposus and disappear within the first decade of life. Although notochordal remnants and related lesions have been described in the axial skeleton of adults, their presence in intervertebral disks is rare. We describe herein 3 cases of incidental notochordal remnants identified in surgically removed adult intervertebral disks. Their histologic features were reminiscent of notochordal vestiges in the fetus. However, they raised the differential diagnosis of benign notochordal cell tumor and chordoma. Notochordal rests can be a source of diagnostic confusion and should be distinguished from notochordal neoplasms because they do not necessitate resection or other forms of therapy.     

Primary pancreatic neuroblastoma: an unusual tumor in infancy

Fetal imaging has dramatically impacted neonatal care by providing an advanced warning of many different congenital anomalies. The advancements and widespread use of fetal imaging has, however, increased the identification of various incidental findings that is creating new challenges for neonatal diagnosis and treatment. We report such a case where a fetal pancreatic neuroblastoma (NB) was incidentally detected by computed tomographic scan of the maternal abdomen. Primary pancreatic NB is a very uncommon childhood neoplasm that, to our knowledge, has never been previously reported in the English language presenting in either the prenatal or neonatal periods.A 21-year-old woman complaining of acute abdominal pain and carrying a 35 weeks' gestation fetus was referred for computed tomographic scan because of the concern of maternal appendicitis. That scan was inconclusive for appendicitis but did incidentally detect a fetal mass in the general region of the pancreas. At 36 weeks' gestation, the fetus developed signs of distress, which prompted a cesarean delivery. Neonatal workup confirmed the presence of an abdominal mass in the region of the pancreas, but precise anatomic localization was not possible. Also noted on neonatal workup were elevated urinary catecholamines consistent with a hormonal active tumor. These findings prompted an abdominal exploration of this neonate, which revealed a solid tumor contained in the distal pancreas. The mass was managed by an uncomplicated distal pancreatectomy. The neonate fully recovered, and histologic diagnosis revealed NB, whereas the postoperative urine catecholamines normalized. This case underscores the unintended clinical challenges created by widespread fetal imaging, while presenting the first prenatally diagnosed case in the English language medical literature and earliest treated patient with pancreatic NB.     

Spinal tuberculosis: A review

Abstract

Spinal tuberculosis is a destructive form of tuberculosis. It accounts for approximately half of all cases of musculoskeletal tuberculosis. Spinal tuberculosis is more common in children and young adults. The incidence of spinal tuberculosis is increasing in developed nations. Genetic susceptibility to spinal tuberculosis has recently been demonstrated. Characteristically, there is destruction of the intervertebral disk space and the adjacent vertebral bodies, collapse of the spinal elements, and anterior wedging leading to kyphosis and gibbus formation. The thoracic region of vertebral column is most frequently affected. Formation of a ‘cold’ abscess around the lesion is another characteristic feature. The incidence of multi-level noncontiguous vertebral tuberculosis occurs more frequently than previously recognized. Common clinical manifestations include constitutional symptoms, back pain, spinal tenderness, paraplegia, and spinal deformities. For the diagnosis of spinal tuberculosis magnetic resonance imaging is more sensitive imaging technique than x-ray and more specific than computed tomography. Magnetic resonance imaging frequently demonstrates involvement of the vertebral bodies on either side of the disk, disk destruction, cold abscess, vertebral collapse, and presence of vertebral column deformities. Neuroimaging-guided needle biopsy from the affected site in the center of the vertebral body is the gold standard technique for early histopathological diagnosis. Antituberculous treatment remains the cornerstone of treatment. Surgery may be required in selected cases, e.g. large abscess formation, severe kyphosis, an evolving neurological deficit, or lack of response to medical treatment. With early diagnosis and early treatment, prognosis is generally good.     

Extraosseous extension of vertebral hemangioma, a rare cause of spinal cord compression.

STUDY DESIGN: Case report. OBJECTIVE: To illustrate a rare cause of thoracic spinal cord compression, its diagnosis, and its management. SUMMARY OF BACKGROUND DATA: Asymptomatic vertebral hemangiomas are common, but extraosseous extension causing spinal cord compression with neurologic symptoms is rare, and few cases appear in the English-language literature. METHOD: A previously asymptomatic 63-year-old man sought medical attention for acute back pain and thoracic myelopathy of 6 week's duration. Magnetic resonance imaging confirmed the presence of a mass in the T10 vertebral body with paravertebral and intracanalicular extension contributing to cord compression. Decompression and reconstructive surgery were performed and radiotherapy administered after surgery. Preoperative angiography was not performed because of the patient's rapidly progressive neurologic deterioration and the consideration that the differential diagnosis of vertebral hemangioma was less likely. RESULTS: The diagnosis of benign capillary hemangioma was made histologically. Neurologic recovery was complete except for minor residual sensory changes in the legs. At follow-up 10 months after surgery the patient had returned to his usual active life and motor mower repairing business. CONCLUSION: Extraosseous extension of vertebral hemangiomas is a rare cause of thoracic spinal cord compression. As such, the available data are derived from reports based on series involving only a small number of cases, rather than on results of randomized controlled trials. Those causing progressive neurologic symptoms should be surgically decompressed, with the specific procedure determined by the extent and site of the lesion. Preoperative angiography is recommended, but embolization is not always necessary or even possible. Postoperative radiotherapy is recommended when tumor removal is subtotal.     

Retroperitoneal liposarcoma: a report of 2 cases and review of 163 cases in Japan

Two cases of retroperitoneal liposarcoma are reported. Case I was a 71-year-old-male presenting with a mass in the left abdomen. On X-ray examination, a large extrarenal tumor was found in the left retroperitoneal space. The tumor appeared to invade the psoas muscle and the aorta. Exploratory laparotomy was carried out. The histological diagnosis of the tumor was myxoid-type liposarcoma. He died of the disease. Case 2 was a 44-year-old-male who presented with a mass in the left abdomen. On X-ray examination, a large tumor was found in the left retroperitoneal space, and left kidney and the descending colon were markedly displaced by the tumor. Excisional surgery was carried out. The tumor was almost completely removed. The histological diagnosis was myxoid-type liposarcoma. A combination chemotherapy (actinomycin D, vincristine, cyclophosphamide) was started. but soon switched to radiation therapy because of liver dysfunction. Four months following the operation, there is no evidence of residual disease. One hundred sixty three cases of retroperitoneal liposarcoma reported in Japan are reviewed.     

Vertebral hemangioma with neurologic signs. Clinical presentation, results of a survey by the French Society of Neurosurgery

45 cases of vertebral hemangiomas with neurologic involvement are reported. This series corresponds to the french experience between 1969 and 1988 (series of the "Société Fran?aise de Neurochirurgie" (S.F.N.)). In this report the clinical presentation and the results of the radiological examination are detailed. Local vertebral pain was present in half of the cases. The neurological symptoms were related to spinal cord compression in 33/45 of the patients and to radicular suffering in the other cases. Evolution of the neurological symptoms appeared to be slow: the mean evolution time before diagnosis was of 10 months. Hemangioma involved the thoracic column in 73% of the cases. Neurologic symptoms was due to a diffuse narrowing of the spinal canal in 28.8% of the cases, to a local bony expansion in 60% of the cases. Hemangioma was found to involve the whole vertebra in 44.4% of the cases, the vertebral body alone in 24.4% of the cases and the posterior arch alone in 22.2% of the cases. An incomplete involvement of both vertebral body and posterior arch was found in 6.6% of the cases. 2.2% of the cases corresponded to pure epidural hemangioma. Preoperative diagnosis was established in 58% of the patients. In these cases, the diagnosis was based on standard radiography and CT datas. Results of the S.F.N. serie are compared with those of the main series of the literature.     

Adenocarcinoma of the Lower Esophagus After Placement of a Gastric Band

A 43-year-old man presented with severe back pain. He had a history of morbid obesity, for which an esophagogastric silicone band was placed 2 years before presentation. Magnetic resonance imaging of the vertebral column showed multiple osseous metastases. On the computerized tomography scan of the abdomen, a tumor of the lower esophagus just proximal to the esophagogastric band was seen, of which histological examination revealed an esophageal adenocarcinoma. Esophageal adenocarcinoma after bariatric surgery has been described previously in only a few cases. Although there is no evidence for a causal relationship with bariatric surgery, one should bear in mind that the incidence of esophageal adenocarcinoma is increased in patients with morbid obesity because of the higher incidence of gastroesophageal reflux disease. Also, the symptoms of adenocarcinoma might be masked after bariatric surgery.     

Congenital giant hydronephrosis in adults

Giant hydronephrosis is the term designating the presence of more than 1,000 ml. of fluid in the collecting system. Of 4 cases of congenital hydronephrosis found over a three-year period at the Ochsner Medical Institutions, 2 were associated with unsuspected carcinomas. Hydronephrosis may first be detected as a mass palpable in the abdomen and displacing the intestines on gastrointestinal films. Excretory urography, retrograde pyelography, and angiography confirm the diagnosis. The treatment of choice is nephrectomy.     

Abnormalities in the flow-volume loop in obstructive sleep apnoea sitting and supine.

We evaluated the effect of posture on the sensitivity and specificity of abnormalities in the flow-volume loop in 30 patients with suspected obstructive sleep apnoea. Flow-volume loops were judged as abnormal if the FEF50/FIF50 ratio was greater than 1 or if the sawtooth sign was judged to be present by at least two of three chest physicians. Detailed nocturnal recordings confirmed the presence of obstructive sleep apnoea in 17 of the 30 patients. Our results showed that both the sensitivity and the specificity of each of the flow-volume criteria for the diagnosis of obstructive sleep apnoea were higher when the loops were recorded in the supine than when they were recorded in the sitting position. The sensitivities were low, however, even with the supine posture--sawtoothing 41% and FEF50/FIF50 ratio greater than 1 47%. The highest sensitivity (71%) was obtained by considering a positive result as being the presence of either of the abnormalities in either the sitting or the supine posture. This sensitivity of the flow-volume loop was too low to recommend it as a routine screening test for the diagnosis of obstructive sleep apnoea but the presence of the sawtooth sign had a high specificity (92%) for the diagnosis of obstructive sleep apnoea. Furthermore, there was a greater fall in oxygen saturation in patients with apnoea who had sawtoothing than in those without sawtoothing. The presence of the sawtooth sign should increase the suspicion of sleep apnoea and suggest the need for further investigation. The effect of posture on the occurrence of abnormalities in the flow-volume loop suggests that position alters the configuration of the upper airway.