Polythelia in a 13-year old girl.

We present a rare case of a 13-year old girl with a bilateral polythelia. We would like to draw attention to this particular mammary malformation.

Perineal healing and survival after anal cancer salvage surgery: 10-year experience with primary perineal reconstruction using the vertical rectus abdominis myocutaneous (VRAM) flap.

Salvage surgery of recurrent or persistent anal cancer following radiotherapy is often followed by perineal wound complications. We examined survival and perineal wound complications in anal cancer salvage surgery during a 10-year period with primary perineal reconstruction predominantly performed using vertical rectus abdominis myocutaneous (VRAM) flap. Between 1997 and 2006, 49 patients underwent anal cancer salvage surgery. Of these, 48 had primary reconstruction with VRAM. Overall survival was computed by the Kaplan-Meier method and mortality rate ratios (MRRs) by Cox regression. One patient (2%) died within 30 days postoperatively. Postoperative complications necessitated reoperation in eight (16%) patients. We found no major perineal wound infections. Major perineal wound breakdown occurred in the only patient in whom VRAM was not used. Five-year survival was 61% [95% confidence interval (CI) 43-75%]. Free resection margins (R0) were obtained in 78% of patients, with 5-year survival of 75% (95% CI 53-87%). Involved margins, microscopically only (R1) or macroscopically (R2), strongly predicted an adverse outcome [age-adjusted 2-year MRRs (95% CI) R1 vs. R0 = 4.1 (0.7-23.6), R2 vs. R0 = 10.9 (2.2-54.2)]. We conclude that anal cancer salvage surgery can yield long-time survival but obtaining free margins is critical. A low rate of perineal complications is achievable by primary perineal reconstruction using VRAM flap.

Growth hormone is a stimulating but not an essential factor in healing of colon. A study in GH-deficient dwarf rats.

BACKGROUND: Growth hormone (GH) has been implicated as an important factor in the healing and previous studies showed significant strength acceleration of experimental intestinal anastomoses. AIM: To study the healing of experimental colonic anastomoses in GH-deficient rats and to study the potential physiological effects of GH-substitution on healing parameters. CONCLUSION: Exogenous rhGH treatment started 7 days prior to surgery and continued until day 4 postoperatively accelerates the strength development of the experimental colonic anastomoses in dwarf rats indicating a potent role of growth hormone in colonic healing. However, GH is not essential in the healing process, since anastomotic healing in GH-deficient dwarf rats is like rats with normal pituitary function.

Surgery for mitral valve disease in the pediatric age group.

OBJECTIVES: We reviewed a 20-year experience with the surgical treatment of mitral valve disease in the pediatric age group at our institution with 2 objectives: to clarify the long-term results over the last 2 decades and to evaluate the recent advances in mitral valve operation in children. METHODS: Since December 1978, 56 patients have undergone a total of 36 mitral valve repairs and 30 mitral valve replacements. Associated cardiac anomalies were present in 46 patients (82%), and concurrent repair of associated lesions was performed in 37 patients (66%). The age of the patients ranged from 3 months to 15 years (mean, 3.6 years) at mitral valve repair, and ranged from 2 months to 16 years (mean, 5.7 years) at mitral valve replacement. Mean follow-up period was 92.0 months (range, 1-235 months). RESULTS: There were 2 hospital deaths and 2 late deaths in patients who underwent mitral valve repair. Reoperation was performed in 4 patients. Three of these patients underwent mitral valve replacement because of residual mitral incompetence. No hospital deaths occurred in patients who underwent mitral valve replacement. Two late deaths occurred after mitral valve replacement. Six patients had a total of 10 episodes of prosthetic valve thrombosis. Thrombolytic therapy with urokinase was successful in all episodes without serious complications. Five patients required reoperations 49 to 141 months (mean, 78.4 months) after the initial valve replacement for relative prosthetic valve obstruction as the result of somatic growth. A valve 2 or 3 sizes larger than the original prostheses was inserted without death. Actuarial survival and freedom from cardiac events at 10 years after the operation were 87.2% and 72.7% in children who underwent mitral valve repair, and 90.3% and 67.3% for those children who underwent mitral valve replacement. CONCLUSIONS: The current risk of mitral valve operation in the pediatric age group is low, and the long-term results are satisfactory, irrespective of severe deformation of the mitral valve apparatus and associated complex cardiac anomalies.

Entropy-controlled asymmetric synthesis. How differential activation entropy is induced in chiral tethered reactions.

[figure: see text] Kinetic measurements to determine effective molarities of intramolecular reactions using 2,4-pentanediol and related tethers showed that methyl groups on the tether accelerate the major diastereomeric process but decelerate the minor process. The efficient promotion of stereocontrol is suggested to be due to chiral perturbation of the reaction rate through the entropy term. The conformation of the encounter complex of the reagent and reactant moieties was deduced by stereochemical analysis of the intramolecular adducts.

Intrahepatic venovenous shunting to an accessory hepatic vein after Fontan type operation.

Because there are various types of systemic venous connections in patients with visceral heterotaxy, unanticipated abnormal systemic venous channels may pose additional problems after a Fontan type operation. We report a case of severe cyanosis caused by anomalous intrahepatic venovenous fistula to an accessory hepatic vein early after total cavopulmonary anastomosis.

Clinical and pathological features of accessory valve tissue.

BACKGROUND: Accessory valve tissue is a rare congenital cardiac malformation causing a variety of clinical features. We describe our experience with the surgical treatment of accessory valve tissue to clarify clinical and pathological features of this anomaly. METHODS: Between December 1990 and February 1999, 11 children underwent resection of accessory valve tissue together with repair of coexisting intracardiac malformations. Eight patients had accessory tricuspid valve, while 3 had accessory mitral valve tissue. Associated intracardiac malformations included tetralogy of Fallot in 6 patients, transposition of the great arteries in 3, coarctation complex in 1, and ventricular septal defect in 1. RESULTS: Accessory valve tissue was completely resected in all but 1 patient with accessory mitral valve and transposition of the great arteries. As total resection of the accessory valve tissue through the pulmonary artery was unsuccessful, partial resection and bidirectional Glenn shunt operation was performed instead of arterial switch operation in this patient. CONCLUSIONS: The clinical signs and symptoms of accessory valve tissue vary according to its location and coexisting cardiac malformations. Congenital heart surgeons should be familiar with the clinical and pathological features of accessory valve tissue, so as not to overlook it during operation.

Cor triatriatum, coarctation of the aorta, and ventricular septal defect: a rare, surgically correctable anomaly.

A case of cor triatriatum associated with coarctation of the aorta and ventricular septal defect, successfully treated surgically by a staged approach, is reported. This combination is a very uncommon but potentially correctable cause of congestive heart failure and death in infancy. Early accurate diagnosis aided by an awareness of this combination and prompt surgical correction should yield excellent long-term results.

Growth hormone treatment increases transmural colonic growth in GH-deficient dwarf rats.

Growth hormone (GH) has been implicated as an important factor in the growth regulation of several visceral organs including the gastrointestinal tract. Our aim was to study the effects of GH administration on colonic growth in dwarf rats with an isolated GH deficiency. Dwarf rats were treated with recombinant human growth hormone (rhGH; 2.0 mg/kg/day) for four weeks and compared with saline treated dwarf rats and rats with normal pituitary function. The colonic wall composition was measured by means of stereological techniques. RhGH treatment of the dwarf rats increased body weight by 80% and proximal and distal colon weight by 63% and 90%, when compared with placebo treated dwarf rats (P< 0.01). The weight of the proximal colonic mucosa increased by 83% (P< 0.01), submucosa by 78% (P< 0.05), and the muscularis propria by 51% (P< 0.001) in rhGH treated dwarf rats compared with dwarf controls. The weight of the distal colonic mucosa increased by 88% (P< 0.01), submucosa by 88% (P< 0.05) and the muscularis propria by 58% (P< 0.05) compared with dwarf controls. The growth of mucosa involved all mucosal layers, with a 73 and 92% increase in the proximal and distal colon luminal surface area respectively (P< 0.001, P< 0.01). The food consumption, expressed as g/day/100 g BW was 13% higher in dwarf rats receiving rhGH than in placebo treated rats (P< 0.05) and normal control rats (P< 0.05). When weights of the GI tract compartments are corrected for the increase in body weight the effects of GH treatment were small or non-significant. RhGH administration in GH deficient dwarf rats induces visceral growth with a pronounced increase in colonic luminal surface area and growth of all layers of the colonic wall. These findings confirm the important role of GH in the regulation of intestinal growth.

Oxy-function promoted hydroboration of conjugated dienes with controlled allylic borane rearrangement.

Hydroboration of conjugated dienes is promoted by the hydroxy and methoxy groups, which also control the rearrangement of the initially produced allylic boranes.

Risk factors influencing early and late mortality after total cavopulmonary connection.

OBJECTIVE: Among the later modifications of the Fontan type procedure, a significant alteration was introduced by de Leval and associates when they described the total cavopulmonary connection (TCPC). Although current results of TCPC have encouraged us to extend this procedure to high risk patients, risk factors influencing surgical outcome after TCPC have not been evaluated. We review our experiences with TCPC to identify which risk factors may have had a considerable impact on the outcome of patients undergoing TCPC and to clarify selection criteria of high-risk Fontan candidates for TCPC. PATIENTS AND METHODS: We retrospectively reviewed the medical and surgical records of all 76 patients who underwent TCPC between July 1988 and August 2000. A cross-sectional review of these patients was undertaken. RESULTS: There were eight early deaths and four late deaths. In a Fisher's exact test, the following variables were associated with an increased early mortality after TCPC: systemic ventricular morphology (right ventricle), ejection fraction of the systemic ventricle less than 60%, and prolonged cardiopulmonary bypass time (240 min or longer). The log-rank test demonstrated that heterotaxy syndrome, moderate to severe atrioventricular valve regurgitation, prolonged cardiopulmonary bypass time (240 min or longer), and prolonged aortic cross clamp time (70 min or longer) were associated with late mortality after TCPC. Six deaths occurred in eight (75%) patients who had six or more risk factors, whereas six deaths (9%) occurred in those who had five or fewer. CONCLUSIONS: Patients with complex cardiac anomalies who have six or more risk factors should be excluded from TCPC candidates.

Aspergillosis of the central nervous system causing subarachnoid hemorrhage from mycotic aneurysm of the basilar artery--case report.

The authors present an extremely rare case of aspergillosis of the central nervous system (CNS) causing subarachnoid hemorrhage (SAH). A 78-year-old female developed facial pain, progressive deterioration in left visual acuity, and left total ophthalmoplegia. Computed tomography demonstrated a heterogeneously enhanced mass extending from the sphenoid sinus to the left cavernous sinus and left orbit, and angiography showed luminal narrowing and irregularity of the left internal carotid artery at its siphon. Biopsy of the left orbital and sphenoid sinus mass resulted in the diagnosis of Aspergillus granuloma. Despite combined administration of amphotericin-B and 5-FC, she became comatose from brainstem infarction and finally, suddenly died. Postmortem examination revealed massive SAH due to a ruptured mycotic aneurysm of the basilar artery. Aspergillosis of the CNS is a growing problem with the wider use of immunosuppressants and antibiotics. To the authors' knowledge, however, only 13 cases of CNS aspergillosis causing SAH have been reported. The prognosis is absolutely bad, with all patients dying from rupture of major intracranial arteries such as the internal carotid artery and basilar artery. Early diagnosis and vigorous chemotherapy are important.

[Case report of acute central cervical cord injury with C7 laminar fracture and extradural hematoma (author's transl)].

A 57-year-old epileptic male with an acute central cervical cord injury was reported, who fell down from 2.5 meter height to a dry river bed presumably during a convulsive seizure. Upon physical examination at the time of admission, 4 hours post trauma, there were severe neck pain with limited neck motion, sensory level at C4 bilaterally, no motion at all in the upper extremities, and minimal motion in the lower extremities as well as apparent urinary retention. Plain cervical spine series showed moderate posterior osteophytes at C4--C5 and C5--C6, although the film of lateral view did not visualize a part of C7 well. After 4 days' clinical observation, a myelography was done at lateral cervical approach between C1 and C2, which showed a complete block at C3. Urgent laminectomy C3 through C7 showed a fracture of the C7 spinous process extending to the bilateral lamina and an extradural hematoma from C3 to C7 with thickness of 3 to 5 mm. Neither pial incision nor dorsal myelotomy was performed because of no remarkable swelling of the cord. Post-operative course was uneventful, the patient recovering first the motion in the lower extremities, urinary retention, and motion in the upper extremities. At 10 months' follow-up after the operation, he could take care of himself in dressing, eating and toileting, although he had had residual motor disturbance in his hands and fingers especially in fine finger movements. It was keenly felt important to visualize C7-T1 in roentgenograms of lateral cervical view in cases of acute cervical injury, for which swimmer view and/or tomogram might be necessary. Queckenstedt's test was criticized as a dangerous maneuver in such case but C1--C2 myelography is considered safer and more informative. Literature on the acute central cervical cord injury were reviewed.

[Secondary empty sella syndrome: report of three cases and review of the literature].

Three cases of the secondary empty sella syndrome were reported. Case 1. A 57-year-old female was admitted to our clinic because of recurrent visual disturbance. Fourteen years previously a pituitary adenoma was diagnosed and two courses of radiation therapy was given elsewhere, the total dosage being unknown. Pneumoencephalography revealed air collection in the sellar cavity. Exploratory craniotomy demonstrated an empty sella with downward migration of the optic nerve and chiasm. Case 2. A 40-year-old female was readmitted to our clinic for possible recurrent pituitary adenoma. Three years previously she underwent transsphenoidal surgery and postoperative radiation therapy with a tumor dose of 6,400 rad. She had headache and visual disturbance. Pneumoencephalography showed air extension into the sellar cavity. Case 3. A 50-year-old female was readmitted to our clinic because of general prostration and headache. Ten years previously she received transsphenoidal surgery and postoperative radiation therapy with a tumor dose of 6,650 rad for a pituitary adenoma. Endocrine evaluation revealed a hypopituitarism. Pneumoencephalography showed air extension into the sellar cavity. The etiology of secondary empty sella has not been clarified yet, although the primary empty sella has been considered to result from incomplete development of the diaphragma sellae. We consider that the most important factor should be present in the diaphragma itself. It is conceivable that the diaphragma sellae becomes weak due to 1) compression by the tumor, 2) mechanical injury during operation, 3) radiation effect. Then, these preceding factors might bring about herniation of the optic nerve and chiasm with the diaphragma sellae into the sellar cavity. Therefore, we propose that early diagnosis of pituitary tumor should be important, and that careful packing of the sellar floor at transsphenoidal surgery should be essential to prevent occurrence of the secondary empty sella syndrome.

[A case of hemangioma calcificans (author's transl)].

A 32-year-old male, who had had temporal lobe seizure for the past 10 years, was admitted to the neurosurgical institute of Tenri Hospital on March 10, 1981. Physical examination on admission revealed some memory disturbance, neuroasthenic tendency and a purplish nevus in the left foot. Plain x-ray series of the skull showed several nodular calcified lesions in the medial aspect of the left temporal lobe. Electroencephalography showed sporadic negative spikes and irregular slow waves dominant in the left anterior quadrant of the head. CT scan showed a high-density area in the left hippocampal gyrus with slight enhancement by contrast medium. Under stereotactic consideration, a left temporal osteoplastic craniotomy and total removal of the mass were performed on March 20, 1981. The histological examination proved it to be calcified cavernous hemangioma or hemangioma calcificans after Penfield and Ward (1948). Postoperative course was uneventful; even 4 weeks after surgery, electroencephalogram became normalized, and memory and anxiety scores became better. At present, in spite of decreased anticonvulsant, the patient has had no more seizure since surgery, and has returned to his business without any complaint. The authors have emphasized the necessity of active operation for hemangioma calcificans even for the one located in the depth of the dominant cerebral hemisphere. Stereotactic consideration can make the surgical risk minimize. CT scan especially with its contour plot of the images is helpful precisely to locate the lesion. For the nevus in the left foot, the biopsy was refused by the patient, which might have connection with the intracerebral cavernous angioma.

[Pineal metastatic tumor from lung cancer initially caused by neurological abnormalities of pineal body tumor].

A rare case is reported of pineal metastasis from lung cancer initially caused by neurological abnormalities of pineal tumor. A 70-year-old female suffering from headache and deterioration of consciousness for 1 week was admitted. She also had a tumor on both sides of her neck. On admission, neurological examination revealed disturbance of upward gaze, and CT scans showed hydrocephalus and pineal tumor. The tumor was seen as a slightly high density mass on non-contrast CT, and was homogeneously enhanced after administration of contrast material. Right V-P shunt and excision of the left neck tumor were performed at the same time. Pathological diagnosis of neck tumor was undifferentiated carcinoma metastasized to cervical lymph nodes. Extensive study was made, by bronchial fiberscope and biopsy, in order to find the origin of the malignancy and disclosed a small cell lung cancer of left lower lobe. The patient took radiation therapy for both the whole brain (60 Gy) and for the bilateral cervical regions (45 Gy). Two courses of chemotherapy using CDDP, ADR, VCR and CY were administered. Both the neck and the pineal tumors were markedly reduced in size at the termination of radiation therapy. However, she was readmitted 3 months later because of dyspnea. Chest X-P revealed enlargement of the left-lung tumor. She died on April 22, 1987. General autopsy disclosed invasive enlargement of left lung cancer, however, no remote metastasis was found. Examination of pineal region showed only necrotic pineal tissue, and no tumor cell was seen in either macroscopic or microscopic study.(ABSTRACT TRUNCATED AT 250 WORDS)

[A case of hypervascular meningioma: the effect of preoperative irradiation].

A 30-year-old, right handed male was admitted with headache and mild right-sided motor weakness of one year's duration. A CT scan revealed a mass over the left frontal convexity. A cerebral angiogram showed a huge, hypervascular tumor which was fed by bilateral external carotid and left internal carotid arteries. Because of its size and location, a surgical intervention was considered contraindicated at that time. Instead he was initially irradiated 52 Gy in total dose with 10MV x-rays (Lineac). A CT scan 4 months thereafter revealed remarkable decrease in the size of tumor and reduction of its mass effect. A CT scan 12 months later, however, revealed increase in low density area around mass, although the size of the tumor was much smaller than before. He was readmitted for surgery 14 months after irradiation. An angiogram visualized more reduction of tumor stain and the caliber of feeding arteries. The tumor was totally removed without resultant neurological deficits. A histological diagnosis of the tumor was meningotheliomatous meningioma with well developed vascular networks. Meningioma is usually not a radiosensitive tumor, but there are some reports of cases with hypervascular meningiomas which have been effectively treated with irradiation preoperatively. In the present case, because of a huge vascular tumor in the dominant hemisphere, irradiation was given initial and 14 months thereafter the tumor was totally removed without neurological deficits.

[Epithelial cysts of the central nervous system: report of two cases].

Two cases of epithelial cyst are reported. Case 1. A 68-year-old female visited our hospital with a complaint of decreased visual acuity, 0.04 in the left eye, in September 1986. Visual field examination showed bitemporal hemianopsia. CT scan demonstrated nonenhancing cystic lesion involving the suprasellar region. By a right frontotemporal craniotomy, the suprasellar cyst was explored. The wall of the cyst was partially removed to relieve pressure against both optic nerves and chiasma. Histologically, the cyst wall was lined with a single layer of non-ciliated cuboidal epithelium. Electron microscopic study revealed a number of microvilli, continuous basement membrane and desmosomes. Case 2. A 40-year-old female was admitted to our hospital because of sensory disturbance in the left hand for several years. She had an episode of convulsive seizure with a loss of consciousness. Neurological examination revealed hypesthesia of the left hand. CT scan showed non-enhancing right frontoparietal cystic lesion compressing the right lateral ventricle to the left. By a right frontoparietal craniotomy, the cystic lesion was explored. The cyst existed beneath an arachnoid membrane and contained CSF-like clear fluid. The cyst wall was resected, as far as possible, to obtain communication with normal subarachnoid CSF space. Histologically, by light and electron microscopic study, the cyst was considered to be an epithelial cyst. A correct diagnosis is difficult because other cystic lesions such as arachnoid cysts and craniopharyngioma are of similar low density and appear the same on CT scan. It is impossible to diagnose such an epithelial cyst by relying on neuroradiological study alone.(ABSTRACT TRUNCATED AT 250 WORDS)

[Two cases of the diencephalic syndrome were reported with hormonal studies (author's transl)].

Two cases of the diencephalic syndrome were reported with hormonal studies. The first case was initially admitted at her age of 4 years and 10 months old. Progressive visual disturbance and horizontal nystagmus had developed one year prior to the admission. Her weight gain had stopped since 10 months old. Partial removal of the hypothalamic pilocytic astrocytoma and 5,000 rad of lineac radiotherapy brought satisfactory effects on her previous symptoms. She was readmitted, however, at her age of 8 years and 10 monts old because of precocious puberty. Her breast development was in Tanner's grade 2, and her bone maturation was equivalent to one of 11 years old. Hormonal studies showed decreased urine 17-OHCS and 17-KS, elevated serum LH, E1 and E2. The CT scan disclosed some enhanced area in the vicinity of the optic chiasm, which had not changed for the last one year. The second case was admitted at his age of 10 years and 9 months old because of progressive marked emaciation of 6 months' duration. Parinaud's sign was only positive neurological finding. The CT verified enhanced areas in the pineal and chiasm. Cytological study of CSF gave the diagnosis of germinoma. Hormonal studies showed decreased urine 17-OHCS and 17-KS, decreased T3, T4, ACTH, and increased PRL as well as increased HGH. Local lineac irradiation by 5,500 rads was given, during which the enhanced area in CT disappeared and his weight gain started. Increased HGH became normalized but paradoxical response of HGH to GTT remained same.

[Three cases of arachnoid cysts associated with chronic subdural hematoma (author's transl)].

The association of arachnoid cyst of the middle cranial fossa and subdural hematoma has been emphasized previously but is not common knowledge. We present three cases of arachnoid cyst of the middle cranial fossa and associated chronic subdural hematoma. The importance of computed tomography for the diagnosis of arachnoid cyst associated with the hematoma is emphasized. Pathogenesis of arachnoid cyst and the hematoma remain uncertain. However histological findings suggested that subdural CSF collection might be important to develop hemorrhagic outer membrane of the chronic subdural hematoma.