Cyclopia is rare congenital craniofacial anomaly, in which the eyes are fused together and located in a single orbit. It is consistently associated with severe holoprosencephaly, which is the failure of cleavage of the prosencephalon with a deficit in the midline facial development. chromosomal study revealed 47, X( ), +13 (Patau syndrome).
Holoprosencephaly ; Orbit ; Prosencephalon ; Trisomy*

We report a case of increased values of entropy parameters Response Entropy (RE) and State Entropy (SE) during intravenous general anesthesia in a sleepwalking patient. An ASA class II, 64-year-old woman with stress incontinence underwent mid-urethral sling surgery. Prior to surgery, the patient had been administered paroxetine, valproic acid and clonazepam for the treatment of sleepwalking disorder. After 10 min of target-controlled infusion of propofol and remifentanil, entropy values increased up to 94 (RE) and 88 (SE) for 10 min. The target effect-site concentrations of anesthetics increased from 4 to 7 microg/ml propofol and 4 ng/ml remifentanil, at which point values fell back to adequate anesthesia levels. Episodes of recall or of explicit memories did not occur during the anesthesia. In conclusion, sleepwalking patients with long-term use medications may need increment of anesthetic dose caused by the anesthetic drug metabolism activation or impairment or immaturity of inhibitory circuits in brain.
Anesthesia* ; Anesthesia, General ; Anesthetics ; Brain ; Clonazepam ; Entropy* ; Female ; Humans ; Metabolism ; Middle Aged ; Paroxetine ; Propofol* ; Somnambulism* ; Suburethral Slings ; Valproic Acid

We report a case of increased values of entropy parameters Response Entropy (RE) and State Entropy (SE) during intravenous general anesthesia in a sleepwalking patient. An ASA class II, 64-year-old woman with stress incontinence underwent mid-urethral sling surgery. Prior to surgery, the patient had been administered paroxetine, valproic acid and clonazepam for the treatment of sleepwalking disorder. After 10 min of target-controlled infusion of propofol and remifentanil, entropy values increased up to 94 (RE) and 88 (SE) for 10 min. The target effect-site concentrations of anesthetics increased from 4 to 7 microg/ml propofol and 4 ng/ml remifentanil, at which point values fell back to adequate anesthesia levels. Episodes of recall or of explicit memories did not occur during the anesthesia. In conclusion, sleepwalking patients with long-term use medications may need increment of anesthetic dose caused by the anesthetic drug metabolism activation or impairment or immaturity of inhibitory circuits in brain.
Anesthesia* ; Anesthesia, General ; Anesthetics ; Brain ; Clonazepam ; Entropy* ; Female ; Humans ; Metabolism ; Middle Aged ; Paroxetine ; Propofol* ; Somnambulism* ; Suburethral Slings ; Valproic Acid

Acute abdomen caused by abdominal tuberculosis is a rare manifestation, and includes bleeding of a gastric or ileal ulcer, obstruction of the small bowel by an adhesive band, perforation of the ileum, ileocolic intussusception and fistula, and mesenteric abscesses caused by necrotic lymph nodes. The clinical and radiologic features of these complicated tuberculosis may mimic other acute abdominal diseases. Although not definitive, careful evaluation of the radiologic findings of the bowel wall, mesenteric fat infiltration, and lymph node enlargement may provide useful diagnostic clues to the presence of acute abdomen due to tuberculosis.
Abdomen, Acute* ; Abscess ; Adhesives ; Fistula ; Hemorrhage ; Ileum ; Intussusception ; Lymph Nodes ; Tuberculosis* ; Tuberculosis, Gastrointestinal ; Ulcer

PURPOSE: To evaluate the CT findings of patients with surgically confirmed perforated appendicitis and to compare the characteristics between children and adults. METHOD AND MATERIALS: Patients in whom complicated appendicitis was clinically suspected underwent contrast enhanced CT scanning. The scans of 50 patients (19children and 31 adults) with surgically confirmed perforated appendicitis were analysed. Without knowledge of operative findings, we retrospectively analyzed the CT findings with regard to : 1) the detection of the appendiceal wall thickening; 2) the presence of appendicolith; 3) the size, features, and location of periappendical abscess; 4) mesenteric fat infiltration and lymphadenopathy; 5) wall thickening of the cecum and terminal ileum; and 6) ascites and free air. RESULTS: Appendiceal wall thickening was detected in seven children(37%) and 13 adults (42%) (p>0.05). Appendicolith was detected in 21 patients (42%) and was more frequent in children (13 cases, 68%) than in adults (8 cases, 26%). There were statistically significant differences between the two groups (p<0.05). The mean diameter of periappendiceal abscesses was 3.7+/-1.9 cm (3.2+/-1.9 cm in children and 4.0+/-1.8 cm in adults), with no stastistically significant differences between the two groups (p>0.05). Periappendiceal abscess with well-defined cyst was more frequent in children (17/19, 89%) than in adults (13/31,42%) (p<0.05). The most commonly involved site was the midabdomen and pelvis in children (9/19, 47%), and the right lower quadrant in adults (18/31, 58%), (p <.05). Mesenteric lymph nodes were commonly detected in children, and cecal wall thickening in adults. CONCLUSION: The CT findings of perforated appendicitis included appendiceal wall thickening, appendicolith, periappendiceal abscess, mesenteric fat infiltration and enlargement of mesentericlymph nodes, and thickening of the cecum wall Periappendiceal abscess with well-defined cyst in the midabdomen or pelvis was more frequent in children, as were appendicolith and enlargement of mesenteric lymph nodes.
Abscess ; Adult* ; Appendicitis* ; Ascites ; Cecum ; Child* ; Humans ; Ileum ; Lymph Nodes ; Lymphatic Diseases ; Pelvis ; Retrospective Studies ; Tomography, X-Ray Computed

BACKGROUND: Acoustic stimulation of the saccule can evoke the vestibulocolic reflex and the inhibitory potential can be measured in the ipsilateral sternocleidomastoideus as a vestibular evoked myogenic potential (VEMP). We investigated the saccular dysfunction in patients with Meniere's syndrome by using VEMP, and correlated the findings with those of other vestibular function tests. METHODS: Thirty-six patients, 29 with Meniere's disease and 7 with delayed endolymphatic hydrops, underwent interictal VEMP. Eight patients reported Tumarkins otolithic crisis. The patients also had a bithermal caloric test, testing of the subjective visual vertical (SVV), and brainstem auditory evoked potentials (BAEP) in addition to pure tone and speech audiometry. RESULTS: Abnormal VEMP was observed in 21 (58.3%) patients. All the patients with abnormal VEMP showed decreased amplitude or absence of VEMP in the lesion side. Three of them also exhibited delayed p13 or n23 latency. The VEMP was abnormal in four of the 13 patients with normal calorics, 13 of the 21 with normal SVV, and 11 of the 21 with normal BAEP. The abnormal VEMP was more common in patients with delayed endolymphatic hydrops (71.4%) and Tumarkins otolithic crisis (75.0%), but without statistical significance. CONCLUSIONS: VEMP detects saccular dysfunction in Meniere's syndrome. The abnormal VEMP in some patients with normal calorics, SVV or BAEP suggests differential involvement of the vestibular subsystem in Menieres syndrome especially in the early stage.
Acoustic Stimulation ; Audiometry, Speech ; Caloric Tests ; Endolymphatic Hydrops ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Meniere Disease* ; Otolithic Membrane ; Reflex ; Saccule and Utricle ; Vestibular Function Tests

Twenty-eight patients with 20 tibia fractures and 8 femur fractures were treated with external fixation. The average age at fracture was 10 years 10 months ranging from 5 years to 17 years 6 months. Of 28 fractures, 6 were closed fractures and 22 were open fractures. The average follow-up of these children was 23 months. Monofixators were used in 12 fractures and Ilizarov fixators in 16 fractures. The average time to healing of the fractures was 14.6 weeks(range, 6 to 44 weeks). Seven segmental bone defects(range, 2 to 17cm) were treated with the Ilizarov method of internal bone transport using the transport ring and bone grafting at the docking site. The average healing index for callus distraction was 25 days per centimeter. Seven patients had 10 major complications that necessitated additional operative procedures. There were 4 nonunions. Three patients had an infected nonunion, which was treated with the Ilizarov fixator and polymethyl-methacrylate antibiotic beads. One patient had a hypertrophic nonunion which was treated with plate fixation. Three patients who had an epiphyseal injury had shortening with angular deformity, which was treated by callus distraction. Three patients had a joint contracture, which was treated by the percutaneous tenotomy and Ilizarov fixator. The selection of the type of external fixator depends on the fracture pattern and the Ilizarov fixator is recommended for complicated fracture with severe comminution or segmental bone loss.
Bone Transplantation ; Bony Callus ; Child ; Congenital Abnormalities ; Contracture ; External Fixators ; Femur ; Follow-Up Studies ; Fractures, Bone ; Fractures, Closed ; Fractures, Open ; Humans ; Ilizarov Technique ; Joints ; Surgical Procedures, Operative ; Tenotomy ; Tibia

Interlocking and Ender nail fixations have been widely used for the surgical treatment of diaphyseal fractures of femur. Interlocking nail could give advantage of high stability to unstable, cominuted fractures. It requires, however, advanced technique and exposure to radiation harzard, often complicated by further comminution, displacement, breakage of drill bit or loosening of locking screws. Ender nail, on the contrary, is easier in procedure without reaming, thereby maintaining endosteum and requiring short operation time. It, however, cannot avoid complications of shortening, rotational deformity, migration of nails, etc. This study aimed at comparative evaluation of the clinical results of two different nailings(23 Interlocking and 19 Ender nails) performed from March 1987 to March 1993. The study materials were 42 femoral fractures out of 41 adult patients with the followed-up between one and seven years(average 3.5 years). The results are summarized as below: 1. Fracture occurred mostly in the mid-diaphysis(81%) and the Type III comminution(63%) was most common according to the Winquist-Hansen classifiaction. 2. The operative time averaged ninty five minutes in Interlocking nailing, while sixty minutes in Ender nailing. The blood loss was negligible in both techniques. 3. Fluoroscopic assessment after insertion of the nails revealed that Interlocking gave superior stability to Ender nail, while Ender nail produced earlier callus formation(7.5 weeks in average) than Interlocking nail(9.0 weeks in average, p < 0.05). It took both nails average eighteen weeks to obtain clinical union(p>0.05). 4. Interlocking nail was complicated by loosening of locking screws in two, angulation in one, breakage of drill bit in one cases. Ender nail showed protrusion in two, limb shortening in three, rotational deformity in three cases respectively. There was neither operative infection nor nonunion.
Adult ; Bony Callus ; Congenital Abnormalities ; Extremities ; Femoral Fractures ; Femur ; Humans ; Operative Time

We reviewed twenty-six tibial bone defects which had been treated by internal bone transport to evaluate the results and complications and of the Ilizarov method. The causes of defects were open fracture(13 cases) and infected non-union(13 cases) The mean bone defect was 8.2 cm, ranging from 2 cm to 20 cm, in open fracture and 6.5 cm, ranging from 2 cm to 17 cm, in infected nonunion. All cases attained bone union. The mean radiologic consolidation index was 1.3 month/cm. The mean external fixation time was 18.5 months, ranging from 4 months to 33 months. Complications were pin-tract infection(20 cases), equines contracture of ankle(4 cases), knee flexion contracture(1 case), axial deviation(5 cases), valgus deformity of ankle(1 case), leg length discrepancy(7 cases), stress fracture at corticotomy site(2 cases), delayed union(1 case), infection recurrence(1 case), soft tissue invagination(3 cases) and refracture(2 case). The Ilizarov method is a useful treatment for tibial bone defects. Bone grafting at the docking site is recommended in order to shorten the treatment time and to avoid refracture and nonunion.
Bone Transplantation ; Congenital Abnormalities ; Contracture ; Fractures, Open ; Fractures, Stress ; Ilizarov Technique ; Knee ; Leg

Gastroduodenal intussusception is a rare condition caused by the prolapse of a gastric tumor with subsequent invagination of a portion of the gastric wall into the duodenum. Various gastric lesions including adenoma, lipoma, leiomyoma, hamartoma, adenocarcinoma, gastrointestinal stromal tumor (GIST), and as in our case a Peutz-Jeghers polyp, can serve as a lead point of intussusception. Only three cases of gastroduodenal intussusception secondary to a Peutz-Jeghers polyp have been reported. We experienced a case of gastroduodenal intussusception in a 36-month-old boy who presented with intermittent nonbilious vomiting, abdominal pain, and anemia. An abdominal ultrasound and contrast enhanced CT scan showed gastrointestinal intussusception and a mass-like lesion in the second portion of duodenum. A laparotomy revealed a 7x8 cm sized mass at the gastric body, which was pathologically confirmed as a Peutz-Jeghers polyp.
Abdominal Pain ; Adenocarcinoma ; Adenoma ; Anemia ; Duodenum ; Gastrointestinal Stromal Tumors ; Hamartoma ; Intussusception ; Laparotomy ; Leiomyoma ; Lipoma ; Polyps ; Preschool Child ; Prolapse ; Stomach ; Vomiting