Voiding dysfunction in a patient with adolescent adrenoleukodystrophy

The details are reported of bladder dysfunction in a Japanese boy with adrenoleukodystrophy. He developed gait disturbance at the age of 15 years. Spastic paraparesis progressed from the legs to the hands and brain magnetic resonance imaging showed characteristic degenerative change. Detrusor hyperreflexia was found by a urodynamic study and detrusor-sphincter dyssynergia was also suspected.     

Temporary neurologic deterioration due to cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with adult-onset moyamoya disease

BACKGROUND: Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving CBF, but little is known about the change in CBF and its effect on neurologic status during the acute stage after revascularization. METHODS: 123I-IMP-SPECT was performed 1 and 7 days after STA-MCA anastomosis on 34 sides of 27 consecutive patients with adult-onset moyamoya disease (6 men, 21 women; 22-62 years old). The follow-up period ranged from 5 to 28 months (mean, 17.6 months). RESULTS: Thirteen patients (13 sides, 38.2%) suffered temporary neurologic deterioration due to hyperperfusion several days after surgery, which was sustained for several days (7.4 days in average). Postoperative magnetic resonance imaging/angiography showed the STA as a higher intensity signal than the preoperative finding without ischemic changes in all 13 patients. Postoperative SPECT revealed focal intense increase in CBF at the sites of anastomosis in all 13 patients. Eleven patients (32.4%) had transient focal neurologic deficit mimicking ischemic attack. Two patients (5.9%) had cerebral hyperperfusion syndrome associated with subarachnoid hemorrhage extending to the ipsilateral sylvian cistern. Symptoms were relieved by intensive blood pressure control, and no patients had permanent neurologic deficit or delayed neurologic deterioration during the follow-up period. CONCLUSIONS: Surgical revascularization including STA-MCA anastomosis is a safe and effective treatment for moyamoya disease, although temporary neurologic deterioration due to hyperperfusion could occur at a substantial rate. Routine CBF measurement is recommended for accurate diagnosis of postoperative hyperperfusion in moyamoya disease because its treatment is contradictory to that for ischemia.     

Intrahepatic biliary cysts in children with biliary atresia who have had a Kasai operation

Purpose

In patients with biliary atresia who had undergone a Kasai operation, treatment of intrahepatic biliary cysts (IBCs), particularly when complicated by cholangitis, is often difficult because the clinical implications and the course of IBCs are unclear. Thus, to determine the best treatment guideline, the morphology of IBCs, the clinical course, and the outcomes of such patients were evaluated.

Patients and Methods

A total of 44 patients with type III biliary atresia who underwent a Kasai operation from 1977 to 2005 were postoperatively examined for IBC by using ultrasonography and computed tomography. We classified the IBCs based on their number and shape.

Results

Intrahepatic biliary cysts developed in 12 of 54 patients. Three patients with solitary simple cysts and 1 patient with multiple simple cysts had no history of cholangitis. Two patients with multiple simple cysts had cholangitis at the time of IBC diagnosis and were treated with percutaneous transhepatic cholangiodrainage (PTCD). Patients with simple IBCs did not develop persistent cholangitis and their prognosis depended largely on their liver function; 3 of 6 patients remained healthy without cholangitis, whereas 3 patients required liver transplantation (LT) because of progressive liver failure or worsening hepatopulmonary syndrome, and not severe cholangitis. On the other hand, all 6 patients with multiple complicated IBCs had persistent cholangitis, eventually requiring LT. Even after bile flow to the intestine was reestablished after PTCD, both IBCs and cholangitis recurred. These patients required LT because of severe cholangitis.

Conclusions

Intrahepatic biliary cysts without cholangitis are not a source of infection and require no treatment. Simple IBCs with cholangitis can be controlled by antibiotics and/or PTCD. Patients with multiple complicated IBCs have a poor prognosis, requiring LT to control cholangitis. Although PTCD can control cholangitis in these patients as they wait for LT, PTCD does not alleviate it—LT is the final solution.     

Preoperative proximal splenic artery embolization: a safe and efficacious portal decompression technique that improves the outcome of live donor liver transplantation

Terminal liver cirrhosis is associated with marked severe portal hypertension, which increases the risk of intraoperative hemorrhage and graft hyper-perfusion, especially, in small-for-size graft. In cases with developed collateral vessels, we often face difficulties in perihepatic dissection with blood stanching against bleeding during recipient hepatectomy. For aseptic preoperative portal decompression, we established the proximal splenic artery embolization (PSAE) technique. Sixty adult living donor liver transplantation recipients with viral/alcoholic hepatic failure were divided into two groups; PSAE group (n = 30) and non-PSAE (n = 30). In the PSAE group, the splenic artery was embolized proximal to the splenic hilum 12-18 h before surgery. PSAE enabled shortening of operating time, reduced blood loss, led to less need for transfusion, and significantly reduced the post-transplant portal venous velocity and ascites. PSAE was not associated with complications, e.g., splenic infarction, abscess, or portal thrombosis. Six of the non-PSAE patients required additional surgical intervention to resolve postoperative hemorrhage and three patients required secondary PSAE for arterial-steal-syndrome. The hospital mortality rate of PSAE patients (3.3%) was significantly better than that of the PSAE group (13.3%, P < 0.05). Preoperative noninvasive PSAE makes more efficient use of portal decompression; thus, it can potentially contribute to improvement of outcome.     

Ectopic cystic thymoma associated with Raynaud's phenomenon.

Mediastinal cystic tumors are well-marginated round lesions that comprise 12% to 18% of all mediastinal masses. These lesions include a variety of diseases with overlapping radiologic appearances and variable prognoses. Pathological examinations are almost always required for differential diagnosis. We encountered a case of anterior mediastinal tumor discovered in the process of investigation of Raynaud's phenomenon. Taking into account the tumor location, a pericardial cyst was initially suspected. However, the tumor was surgically resected and histopathological examinations demonstrated thymus-like tissue in the cyst walls. Raynaud's phenomenon greatly improved after surgery. These findings suggested that cystic thymoma originated from ectopic thymic tissue and is accompanied by paraneoplastic syndrome.     

Seizure following superficial temporal-middle cerebral artery anastomosis in patients with moyamoya disease: possible contribution of postoperative cerebral hyperperfusion

Surgical revascularization for moyamoya disease prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). Seizure is known as a rare complication after revascularization for moyamoya disease, although its underlying mechanism is undetermined. We investigated the relationship between seizure attack and postoperative alteration in CBF in patients with moyamoya disease. CBF was routinely measured by N-isopropyl-p-[123I] iodpamphetamine (123I-IMP-SPECT) 1 and 7 days after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis on 64 sides of the 44 consecutive patients (male:female = 13:31, 2-62 years old) with moyamoya disease. Three patients (male:female = 1:2, 40-55 years old) suffered from seizure attack at 1 to 10 days after surgery. Two of the three patients manifested as seizures at 8-10 days after surgery and presented transient neurologic deteriolation before seizure. Postoperative SPECT revealed significant increase in CBF at the sites of the anastomosis in all three patients. Postoperative magnetic resonance imaging showed no ischemic changes, and magnetic resonance angiography showed the apparently patent STA-MCA anastomosis as thick high signal intensity sign in all three patients. The anatomical location and the temporal profile of hyperperfusion were completely in accordance with the focus of seizure attack. Intensive blood pressure control and the use of antiepileptic agents were instituted. The neurologic deficits were resolved and no seizure attack recurred in three patients. Seizure following STA-MCA anastomosis can be caused by hyperperfusion in patients with moyamoya disease. When seizure attacks occur, routine CBF measurement is recommended to differentiate hyperperfusion and ischemia, since the treatments for these conditions are contradictory.     

Laparoscopic repair of late-presenting Bochdalek hernia in 2 infants

Laparoscopic repair was performed on 2 infants with late-presenting Bochdalek hernia. Intraoperatively, the entire small intestine was herniated in 1 case and the stomach, small intestine, and part of the colon and spleen were herniated in the other case. Laparoscopic repair of Bochdalek hernia was successfully completed in both the cases. On the basis of our experience, 4 points seem important in laparoscopic surgery for Bochdalek hernia: (1) avoiding damage to the spleen while reducing organs back into the abdominal cavity; (2) ensuring visualization of diaphragmatic defect after reducing the spleen and intestinal tract; (3) ensuring sufficient width to suture the dorsal side of the diaphragm; and (4) identifying intestinal malrotation. We believe that the fourth point represents an advantage of a laparoscopic approach, which seems superior to the thoracoscopic approach and could represent a useful therapy for Bochdalek hernia in infants and older patients.     

Rapid revascularization after therapeutic parent artery occlusion for a large intracavernous carotid artery aneurysm

A 21-year-old woman presented with an unruptured large intracavernous aneurysm, which was spontaneously revascularized via unusual collateral pathways a short time after extracranial-intracranial bypass and surgical ligation of the proximal internal carotid artery. The patient had been treated for a large basilar trunk aneurysm with intraaneurysmal embolization using Guglielmi detachable coils, and an intracavernous carotid artery aneurysm treated conservatively. Two years later, the patient presented with right abducens nerve palsy, and was referred to our hospital. She had small nevi in the right forehead and eyelid. Cerebral angiography revealed enlargement of the intracavernous aneurysm. Superficial temporal artery-middle cerebral artery bypass followed by surgical carotid artery ligation were performed, and good patency of bypass and disappearance of the aneurysm were confirmed by intraoperative angiography. However, follow-up magnetic resonance angiography and cerebral angiography on the 20th postoperative day revealed revascularization of the internal carotid artery and the intracavernous carotid artery aneurysm via unusual collateral pathways. Subsequently, the recurrent aneurysm and the recanalized internal carotid artery were occluded by endovascular procedures. Histological examination of the nevus showed lack of properly organized vascular structures, and the diagnosis was angiodysplasia. The early development of unusual collateral pathway, and aneurysm formation at a young age might be related to the angiodysplasia. Revascularization is possible within a short time even in cases of intracavernous carotid artery aneurysm successfully treated with surgical ligation of the parent artery.     

Extent of sympathectomy affects postoperative compensatory sweating and satisfaction in patients with palmar hyperhidrosis

Purpose

Endoscopic thoracic sympathectomy (ETS) for the treatment of palmar hyperhidrosis is generally performed at one or two levels ranging between T2 and T4; however, compensatory sweating (CS) is an occasional bothersome side effect. The aim of our study was to evaluate the association between the extent of ETS and the degree of postoperative CS and palmar sweating, as well as patient satisfaction.

Methods

The participants represented a consecutive series of 76 patients who underwent bilateral ETS for palmar hyperhidrosis at level T2 and/or T3. Patients were interviewed by postal questionnaires to assess their self-reported degree of postoperative palmar sweating and CS and their outcome satisfaction. Of the 53 patients who replied to the postal questionnaire, 25 underwent bilateral ETS at one level (group A), and 27 underwent bilateral ETS at two levels (group B). One patient who underwent asymmetrical sympathectomy was excluded.

Results

The degree of postoperative palmar sweating was significantly lower in group B than in group A. The severity of CS was significantly higher in group B than in group A. The severity of CS was significantly inversely correlated with the degree of patient satisfaction. However, the degree of postoperative palmar sweating was not correlated with the degree of patient satisfaction.

Conclusions

Compared to ETS at two levels, single-level ETS of T2 or T3 reduces postoperative palmar sweating to a milder degree, and causes CS to a less severe degree. The severity of CS is inversely correlated with the degree of patient satisfaction.     

Inguinal hernia repair with the mesh plug method is safe after radical retropubic prostatectomy

Purpose

We evaluated the safety and efficiency of using the mesh plug method (MP) to repair inguinal hernias in patients with a history of radical retropubic prostatectomy (RRP). We also investigated how RRP influences the development of inguinal hernias and impacts their repair.

Methods

Among 488 adult male patients who underwent inguinal hernia repair during a recent 5-year period, 37 had a history of RRP. We compared the characteristics and surgical outcomes of the patients who had undergone RRP (post-RRP group) with those who had not (non-RRP group).

Results

All post-RRP hernias were treated by MP. The 37 post-RRP patients had a collective 41 hernias, 40 of which were of the indirect type. The side affected by the hernia did not differ significantly between the groups. We compared the short-term surgical outcomes of the indirect post-RRP hernias vs. the indirect non-RPP hernias without recurrence and incarceration. The operation times, postoperative hospital stay, and mobility rates did not differ significantly between the two groups. The blood loss was almost equal in both groups.

Conclusion

Inguinal hernia repair after RRP may be difficult because of inflammatory changes in the preperitoneal cavity, but the surgical outcomes of MP were equivalent in patients with or without a history of RPP in this study. MP is a safe and effective method for post-RPP hernia repair.