A differential diagnosis between the true hermaphroditism (TH) and mixed gonadal dysgenesis (MGD) has important clinical implications for gender assignment and the decision for early gonadectomy; however, variable clinical and histological features frequently lead to the confusion of TH with MGD. A 17- month-old boy was presented with proximal hypospadias with chordee and right non-palpable testis in his scrotum. He also had right auricular anomaly including a separated tragus with skin tag. Left testis was well palpable in his left scrotum. Diagnostic right inguinal exploration showed Mullerian structures such as a gonad like an ovary and a fallopian tube with a uterus, which were removed. Repair of hypospadias and right auricular anomaly was also done. Following ultrasonography (USG) showed a normal looking testis in left scrotum. His chromosome was 45, XO/46, XY. We report a difficult case of mixed gonadal dysgenesis mimicking true hermaphroditism which combines ipsilateral congenital auricular anomaly.
Diagnosis, Differential ; Fallopian Tubes ; Female ; Gonadal Dysgenesis, Mixed* ; Gonads ; Humans ; Hypospadias ; Male ; Ovary ; Ovotesticular Disorders of Sex Development* ; Scrotum ; Skin ; Testis ; Ultrasonography ; Uterus

Foreign body ingestion is a frequent event in the pediatric age group, but the incidence of foreign bodies in the appendix is very low. The authors report a case of ingested foreign body trapped in the appendix in a 7-year-old girl. The foreign body was successfully treated by laparoscopic appendectomy under fluoroscopic guidance.
Appendectomy ; Appendix* ; Child ; Eating ; Female ; Foreign Bodies* ; Humans ; Incidence

Migration of a peritoneal catheter of a ventriculoperitoneal shunt into the scrotum is a rare complication. We treated a case of catheter migration in the scrotum. A 12-year old boy, who had had a ventriculoperitoneal shunt at the age of 4 months due to neonatal hydrocephalus, visited the outpatient clinic because of a right inguinal hernia. On physical examination, a firm mass was found in the left scrotum. Pelvic X-ray demonstrated a coiled catheter in the left scrotum. The catheter was successfully removed by exploring the left patent processus vaginalis after high ligation of the hernia sac. This case suggests a suction action of the patent processus vaginalis and the possibility of catheter migration long after shunt catheter insertion.
Ambulatory Care Facilities ; Catheters* ; Child ; Hernia ; Hernia, Inguinal ; Humans ; Hydrocephalus ; Ligation ; Male ; Physical Examination ; Scrotum* ; Suction ; Ventriculoperitoneal Shunt*

Adrenal cortical tumor is a rare disease both in adults and children. Most of these tumors are malignant and functional, especially in children, producing endocrine syndrome such as virilization, Cushing's syndrome, hyperaldosteronism or feminization. Recently, we experienced a case of adrenal cortical tumor in a 26 month-old boy who showed features of virilization. This case was diagnosed with typical hormonal findings and abdominal CT and confirmed by pathologic examinations. He was successfully treated with right adrenalectomy. We report a case of virilizing adrenal cortical tumor and review the literature.
Adrenalectomy ; Adult ; Child* ; Child, Preschool ; Cushing Syndrome ; Feminization ; Humans ; Hyperaldosteronism ; Male ; Rare Diseases ; Tomography, X-Ray Computed ; Virilism

Neurocristopathy is characterized as having a common origin in aberrant neural crest development. Congenital central hypoventilation syndrome (Ondine's curse) is characterized by marked depression of respiratory drive during sleep and normal ventilation while awake because of no response to both hypercapnea and hypoxia. The girl was full-term, weighing 3020 grams. The girl had poor respiratory effort at birth, but improved with oxygen supply and stimulation. abdominal distention and calcification were noted. During laparotomy transitional zone was found at distal jejunum; a jejunostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an apnea work-up, including brain sonography, echocardiogram, were normal. The girl died of sepsis at 37 days of age. para-aortic ganglioneuroblastoma was found on autopsy. We experienced a newborn with congenital central hypoventilation syndrome, Hirschsprung's disease and congenital ganglioneuroblastoma representative of neurocristopathy.
Anoxia ; Apnea ; Autopsy ; Brain ; Depression ; Female ; Ganglioneuroblastoma* ; Hirschsprung Disease* ; Humans ; Hypoventilation* ; Infant, Newborn* ; Jejunostomy ; Jejunum ; Laparotomy ; Neural Crest ; Oxygen ; Parturition ; Sepsis ; Ventilation

Idiopathic small bowel ulceration occurring beyond the duodenum is rare, and less than 5% of all the reported cases have occurred in children. In most of the cases, single ulcer of unknown cause is observed in jejunum or ileum. The diagnosis is difficult and usually made at the time of surgical exploration for the complications, such as perforation, hemorrhage or obstruction. We experienced a case of perforation of idiopathic ileal ulceration due to blunt abdominal trauma in a 11-year-old boy. The ileal segment including the lesion was resected and the pathologic findings were compatible with idiopathic small bowel ulceration. The clinical and pathological aspects are discussed, and the literatures were reviewed.
Child* ; Diagnosis ; Duodenum ; Hemorrhage ; Humans ; Ileum ; Jejunum ; Male ; Ulcer*

Congenital afibrinogenemia is a rare disorder that refers to a congenital lack of production of fibrinogen, a key component of the hemostatic system. Bleeding manifestations of congenital afibrinogenemia vary in severity from mild to catastrophic. This is a case report of splenic rupture occurred in an eight-year-old boy with congenital afibrinogenemia. A conservative treatment was carried out with perfusion of cryoprecipitate and purified virally inactivated fibrinogen concentrates and splenectomy was avoided.
Afibrinogenemia* ; Fibrinogen ; Hemorrhage ; Humans ; Male ; Perfusion ; Rupture* ; Spleen* ; Splenectomy ; Splenic Rupture

Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.
Adrenal Glands ; Adrenalectomy* ; Biopsy ; Drug Therapy ; Humans ; Liver* ; Magnetic Resonance Imaging ; Neoplasm Metastasis* ; Neuroblastoma* ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography, Prenatal

It has been widely accepted that complete surgical resection of hepatoblastoma is essential for long-term survival. But unfortunately less that 50% of hepatic tumors in children can be totally removed at the time of diagnosis. This report is to present the experience of successful resection of hepatoblastoma after concurrent radiotherapy with transarterial chemoinfusion in a child. We believe this modality of treatment enables complete resection of unresectable hepatoblastoma, which is resistant to the systemic chemotherapy.
Child ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Radiotherapy*

chemotherapy. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal wall (6-15mm in thickness) and subsequent small perforation of the posterior wall of the cecum with thick-walled localized abscess. She has recovered completely after aggressive medical management. We learned two lessons from our experience treating these patients:1) early diagnosis provided by a high index of suspicion and the use of ultra sonogram or CT scan is essential. And 2) although perforation is one of the surgical indications for the treatment of typhlitis, it is possible to manage the perforation nonoperatively in selected cases with localized abscess.The authors, over the last 6 months, have treated 2 patients with perforated typhlitis complicating acute lymphocytic leukemia (ALL) with good outcome. The first patient was a 13-year-old male who developed intermittent high fever, abdominal pain, abdominal distention and diarrhea during the course of maintenance chemotherapy. The peripheral leukocyte ranged from 230-470/mm3. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal and ascending colonic walls and subsequent ragged perforation of the posterior wall of the cecum. He survived after treatment by right hemicolectomy and aggressive supportive measures. The patient case was a 3 year-old female who developed intermittent high fever, right lower abdominal pain, a mass, and watery diarrhea during the course of maintenance.
Abdominal Pain ; Abscess ; Adolescent ; Cecum ; Child, Preschool ; Colon, Ascending ; Diarrhea ; Drug Therapy ; Early Diagnosis ; Female ; Fever ; Humans ; Leukocytes ; Maintenance Chemotherapy ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Tomography, X-Ray Computed ; Typhlitis*