A case of congenital orbital teratoma with marked unilateral proptosis was noted at birth, Patients with congenital orbital teratoma had a uniform clinical picture and were very similar in their morphological pattern, often polycystic and with varied amounts of solid tissue. Six months after birth, the orbital tissues were totally extirpated. Histological examination revealed components from all three germinal layers and no sign of malignancy. Within the first year, the condition of the patient has been satisfactory.
Humans ; Infant ; Orbital Neoplasms/*congenital/pathology ; Teratoma/*congenital ; Tomography, X-Ray Computed

Gastric Teratomas are exetremely rare in infancy and childhood. About 50 cases of gastric teratomas are reported in the world's literature. Most of them were male. Few were dignosed prior to surgery. The mos thelpful diagnostic aid was of teeth or bone in radiologic examinations. Treatment is surgical excision and all of them were benign. Recently, we experienced a case of gastric teratoma combined with congental syphilis which was confirmed by explo-lapa and biopsy in 2month old male infant, who had large intraabdominal mass, vomiting, respiratory distress and skin lesions. The literature on this subject was reviewed briefly.
Biopsy ; Humans ; Infant ; Male ; Skin ; Syphilis ; Syphilis, Congenital* ; Teratoma* ; Tooth ; Vomiting

PURPOSE: To assess the usefulness of MRI in preoperative diagnosis of congenital anorectal malformation MATERIALS AND METHODS: MR findings of 11 cases with surgically proved anorectal malformations were retrospectively reviewed and compared with operative findings, according to the level of atresia, the development of sphincter muscle, fistula and associated anomalies of other organs. RESULTS: Four of 11 cases were low type of anorectal atresia, 3 cases were intermediate type, and 3 cases were high type. There was one case of Currarino triad with low type of anorectal stenosis. MRI demonstrated the levels of atresia correctly in all cases and revealed fistulas in all high type of anomalies. Degrees of the development of the sphincter muscles were good in all cases of low types and fair in a case of intermediate type and an anorectal stenosis, whereas the development was poor in 2 cases of intermediate type and all 4 cases of high type. The associated anomalies in anorectal malformation were renal agenesis, congenital hip dysplasia and sacral defect with presacral teratoma in Currarino triad. CONCLUSION: MRI was a simple and useful study to confirm the level of atresia, fistula and associated anomalies in the diagnosis of the congenital anorectal malformation.
Constriction, Pathologic ; Diagnosis ; Fistula ; Hip Dislocation, Congenital ; Magnetic Resonance Imaging ; Muscles ; Retrospective Studies ; Teratoma

Teratomas represent 0.5% of all intracranial tumors. These benign tumors contain tissue representative of the three germinal layers. Most teratomas are midline tumors located predominantly in the sellar and pineal regions. The presence of a teratoma in the cavernous sinus is very rare. Congenital teratomas are also rare, especially those of a cystic nature. To our knowledge, this would be the first case report of a congenital, rapidly growing cystic teratoma within the cavernous sinus. A three-month-old boy presented with a past medical history of easy irritability and poor oral intake. A magnetic resonance image (MRI) scan of the head disclosed a large expanding cystic tumor filling the right cavernous sinus and extending into the pterygopalatine fossa through the foramen rotundum. These scans also demonstrated a small area of mixed signal intensity, the result of the different tissue types conforming to the tumor. Heterogeneous enhancement was seen after the infusion of contrast medium. However, this was a cystic tumor with a large cystic portion. Thus, a presumptive diagnosis of cystic glioma was made. With the use of a right frontotemporal approach, extradural dissection of the tumor was performed. The lesion entirely occupied the cavernous sinus, medially displacing the Gasserian ganglion and trigeminal branches (predominantly V1 and V2). The lesion was composed of different tissues, including fat, muscle and mature, brain-like tissue. The tumor was completely removed, and the pathological report confirmed the diagnosis of a mature teratoma. There was no evidence of recurrence. Despite the location of the lesion in the cavernous sinus, total removal can be achieved with the use of standard microsurgical techniques.
Cavernous Sinus/*pathology ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Paranasal Sinus Neoplasms/*congenital/*diagnosis/surgery ; Teratoma/*congenital/*diagnosis ; Tomography, X-Ray Computed

Epignathus is an extremely rare type of congenital teratoma arising in the oral cavity. Although it is a benign tumor, it is associated with high mortality and morbidity rates because of severe airway obstruction and other malformations. We present a case of epignathus affecting one fetus in a twin pregnancy. The tumor was associated with multiple congenital malformations including cleft palate, bifid tongue, bifid uvula, congenital heart defect, and bilateral inguinal hernias. The diagnostic value of three-dimensional ultrasonography and magnetic resonance imaging was explored with respect to antenatal counseling and peripartum management.
Airway Obstruction ; Cleft Palate ; Counseling ; Fetus* ; Heart Defects, Congenital ; Hernia, Inguinal ; Magnetic Resonance Imaging* ; Mortality ; Mouth ; Peripartum Period ; Pregnancy, Twin* ; Prenatal Diagnosis* ; Teratoma ; Tongue ; Ultrasonography* ; Uvula

Dysphasia related to oral anomaly is a common situation in oral and maxillofacial surgery. The etiology of oral anomalies causing dysphasia can be divided into congenital and acquired disease. Congenital diseases include teratoma or benign tumors and congenital defects such as cleft lip and palate. Benign tumors include cystic hygroma in the neck and hemangioma in the tongue. Certain syndromes with macroglossia and micrognathia are also related to difficulty in swallowing. The three common syndromes are Pierre-Robin syndrome, Beckwith-Widermann syndrome and ectodermal dysplasia. Taken together, these congenital diseases require a multi-discipline approach to obtain optimal results. Representative disease of acquired dysphasia is the oral cavity cancer. Cancer ablation results in tissue defect and decreased motor function. Free flap reconstruction is the choice of treatment following oral cavity caner operation; however, dysphasia after cancer operation is inevitable. In this review article, the full scopes of oral anomaly associated with dysphasia were classified and treatment was suggested.
Aphasia ; Cleft Lip ; Congenital Abnormalities ; Deglutition ; Ectodermal Dysplasia ; Free Tissue Flaps ; Hemangioma ; Lymphangioma, Cystic ; Macroglossia ; Micrognathism ; Mouth ; Mouth Neoplasms ; Neck ; Palate ; Pierre Robin Syndrome ; Surgery, Oral ; Teratoma ; Tongue

The development and evolution of fetal surgery and the recognition of the fetus as a patient came from two sources. First, were those obstetricians and perinatologists who detected life threatening anomalies before birth, and re-described a hidden mortality arising from death in utero. Ultrasonography, color Doppler ultrasound and ultrafast fetal magnetic resonance imaging have since enhanced the accuracy of prenatal evaluation. Second, were those pediatricians responsible for treating newborn infants with extremely serious problems, and that appeared untreatable, although, it was believed that they could have been treated at an earlier stage of development. After the natural history of several correctable lesions had been determined and the selection criteria for intervention developed, fetal surgery emerged as a means of improving the overall morbidity and mortality rates.
Cystic Adenomatoid Malformation of Lung, Congenital/surgery ; Fetal Diseases/*surgery ; Fetus/*surgery ; Hernia, Diaphragmatic/congenital/surgery ; Human ; Postoperative Care ; Preoperative Care ; Sacrococcygeal Region ; Spinal Neoplasms/embryology/surgery ; Teratoma/embryology/surgery

PURPOSE: Congenital pulmonary airway malformation (CPAM)—a rare developmental anomaly—affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. METHODS: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. RESULTS: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. CONCLUSION: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.
Birth Weight ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Female ; Gestational Age ; Humans ; Infant, Newborn ; Lung ; Medical Records ; Parturition ; Radiography ; Respiratory System ; Teratoma ; Thoracic Surgery, Video-Assisted ; Thoracoscopy ; Thorax ; Tomography, X-Ray Computed ; Ultrasonography ; Ultrasonography, Prenatal

Adrenal myelolipoma is an uncommon non-functioning tumor that is composed of variable amounts of mature adipose tissue and scattered islands of hematopoietic elements, including erythroid, myeloid, lymphoid series, and megakaryocytes. Adrenal myelolipoma should be differentiated from other fat-containing adrenal masses, such as teratoma, lipoma, and liposarcoma. This case report describes a 50-year-old adult who was raised as a male and developed giant adrenal myelolipomas that presented as symptomatic adrenal masses, and which were misdiagnosed as liposarcoma on radiologic examination. The patient had been raised as a male despite ambiguous genitalia, and a thorough investigation was never carried out because of his poor socioeconomic status. Physical examination showed profound short stature (<-3.0 standard deviation score), hyperpigmentation, and a micropenis without palpable gonads. Both a uterus and ovaries were evident in the pelvic cavity on abdominopelvic computed tomography. Adrenocorticotropic hormone stimulation tests confirmed adrenal insufficiency. Steroid replacement therapy was initiated before bilateral adrenalectomy and the histologic findings indicated myelolipoma. The patient's karyotype was 46, XX and mutation analysis of the CYP21A2 gene identified compound heterozygosity consisting of p.I173N and p.Q319*. The patient was treated with once-daily 0.5 mg dexamethasone and once-daily 0.1 mg fludrocortisone. Because the subject had been raised as a male, additional procedures such as an oophorohysterectomy are currently under consideration. We here describe an adrenal myelolipoma in this case that was complicated by a 21-hydroxylase deficiency. We conclude from our analysis that patients with congenital adrenal hyperplasia should be screened for incidental adrenal masses to avoid unnecessary surgical procedures.
Adipose Tissue ; Adrenal Glands ; Adrenal Hyperplasia, Congenital ; Adrenal Insufficiency ; Adrenalectomy ; Adrenocorticotropic Hormone ; Adult ; Dexamethasone ; Disorders of Sex Development ; Female ; Fludrocortisone ; Genital Diseases, Male ; Gonads ; Humans ; Hyperpigmentation ; Islands ; Karyotype ; Lipoma ; Liposarcoma ; Male ; Megakaryocytes ; Myelolipoma ; Ovary ; Penis ; Physical Examination ; Social Class ; Steroid 21-Hydroxylase ; Teratoma ; Uterus

Palpation of an abdominal mass in an infant or child presents a challenging problem in diagnosis and treatment. We reviewed the data on 166 patients under age 15 years who admitted to Ped. Dept. of PMC due to palpable abdominal mass in Jan. 1972-July 1977. The results are as follows 1. Of the 57 surgical cases, pathologically confirmed abdominal tumors were 39 cases. Of the 20 retroperitoneal tumors, Wilms tumors were 13 cases, neuroblastomas were 3 cases, polycystic kidney was 1 case, and retroperitoneal teratoma was 1 case. Of the 19 intraperitonel tumors, hepatomas were 3 cases, hepatoblastoma was 1 case, choledocal cysts were 3 cases, mesenteric and omental cysts were 4 cases, malignant lymphomas were 4 cases and ovarian cysts were 3 cases. Other surgical diseases were 2 cases of ascariasis and 2 cases of bezoar. 2. Medical cases were as follows : 35 cases of leukemia, 15 cases of infectious hepatitis, 14 cases of congenital syphilis 7 cases of liver cirrhosis 7 cases of lirerabscesses, 4 cases of miliary Tbc., 3 cases of congenital spherocytosis, 1 case of cryptococosis, I case of osteopetrosis and 1 case of erythroblastosis fetalis. 3. In age distribution, almost all cases(94%) of Wilms tumor and neuroblastoma were under age of 4 and half of medical cases in infancy were congenital syphilis. 4. Of the pathologically confirmed 39 abdominal tumors, 20 cases were retroperitoneal tumor and 19 cases were intraperitoneal tumor. Of the 20 retroperitonel tumor cases, 16 cases were renal origin, 3 cases were adrenal origin, and 1 case was teratoma. Of the 19 intraperitoneal tum orcases, 8 cases were hepatobiliary origin, 3 cases were ovary origin, 4 cases were omental and mesenteric origin and 4 cases were lymphatics origin. 5. Of the 39 abdominal tumor cases, 34 cases (87%) visited the hospital with the chief complaint of palpable abdominal mass. But, of the 94 medical cases, only 16 cases(17%) visited the hospital with the chief complaint of palpable abdominal mass. 6. In some cases presumptive diagnosis on the base of history taking, physical examination, chest X-ray, simple abdominal X-ray. Peripheral blood findings on admission were uncorrect. In 1 case of Wilms tumor, we suspected liver abscess on admission. In 1/3 case of intraperitoneal tumors, we suspected retroperitoneal tumor on admission. We suspected lymphoma on admission in 1 case of ascariasis and 1 case of bezoar. We misdiagnoed 1 case of miliary Tbc. As hepatoma, 1 case of liver cirrhosis as retroperitoneal tumor and 1 case of congenital syphilis as retroperitoneal tumor on admission. 7. In the treatment of malignant abdominal tumor, we tried all possible measures such as surgery, chemotherapy, and radiation therapy. But prognosis of all malignant abdominal tumors were very poor. Only 1 case of Wilms tumor and 1 case of hepatoblastoma were survived at the time of review.
Age Distribution ; Ascariasis ; Bezoars ; Carcinoma, Hepatocellular ; Child* ; Diagnosis ; Drug Therapy ; Erythroblastosis, Fetal ; Female ; Hepatitis A ; Hepatoblastoma ; Humans ; Infant* ; Infant, Newborn ; Leukemia ; Liver Abscess ; Liver Cirrhosis ; Lymphoma ; Neuroblastoma ; Osteopetrosis ; Ovarian Cysts ; Ovary ; Palpation ; Physical Examination ; Polycystic Kidney Diseases ; Prognosis ; Syphilis, Congenital ; Teratoma ; Thorax ; Wilms Tumor