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Targeted disruption of Hoxd-10 affects mouse hindlimb development.

Targeted disruption of the Hoxd-10 gene, a 5' member of the mouse HoxD linkage group, produces mice with hindlimb-specific defects in gait and adduction. To determine the underlying causes of this locomotor defect, mutant mice were examined for skeletal, muscular and neural abnormalities. Mutant mice exhibit alterations in the vertebral column and in the bones of the hindlimb. Sacral vertebrae beginning at the level of S2 exhibit homeotic transformations to adopt the morphology of the next most anterior vertebra. In the hindlimb, there is an anterior shift in the position of the patella, an occasional production of an anterior sesamoid bone, and an outward rotation of the lower part of the leg, all of which contribute to the defects in locomotion. No major alterations in hindlimb musculature were observed, but defects in the nervous system were evident. There was a decrease in the number of spinal segments projecting nerve fibers through the sacral plexus to innervate the musculature of the hindlimb. Deletion of a hindlimb nerve was seen in some animals, and a shift was evident in the position of the lumbar lateral motor column. These observations suggest a role for the Hoxd-10 gene in establishing regional identity within the spinal cord and imply that patterning of the spinal cord may have intrinsic components and is not completely imposed by the surrounding mesoderm.

Animals↗

Fracture-dislocations of the sacrum. Report of three cases.

The pattern of fracture-dislocation of the upper part of the sacrum is demonstrated in three patients. The fracture line followed the segmental form of the sacrum and was usually caused by a posterior force against the pelvis which had been locked by hip flexion and knee extension. Fractures of the lumbar transverse processes also occurred, presumably from avulsion by the quadratus lumborum muscle. The damage to the sacral plexus found in all three cases recovered after several months. Radiographs of the injury are difficult to obtain in severely injured patients but oblique views of the sacrum help to determine the extent of the forward dislocation.

Adult↗

An unusual cause of sciatic pain.

We describe a 47-year-old woman with sciatic neuropathy caused by compression of the sacral plexus by posterior shift of the uterus.

Diagnosis, Differential↗

Recurrent genital herpes treatments and their impact on quality of life.

Herpes genitalis is one of the most common viral sexually transmitted diseases in the world, with an estimated seroprevalence in the US of greater than 20%. Two viruses of the same family cause herpes genitalis: herpes simplex virus 1 and 2. After the resolution of primary infection, the virus persists in the nerve roots of the sacral plexus, often causing recurrent (though generally less severe) outbreaks. These outbreaks, as well as the infectious potential to the patient's sexual partners, results in significant psychological stress on the patient, and has a tremendous negative impact on QOL. Current treatment modalities may result in a reduction in the number of outbreaks and viral shedding, but no cure exists. Although studies have clearly demonstrated the negative impact of recurrent genital herpes on QOL, an assessment scale specific to herpes was not developed until recently. Earlier studies indicated that patients did not perceive a significant benefit from episodic treatment with antivirals, but studies using the Recurrent Genital Herpes Quality of Life Questionnaire (RGHQoL) have now demonstrated that suppressive antiviral therapy improves quality of life in patients with frequent recurrences of genital herpes. However, not all patients with recurrent genital herpes need suppressive therapy, and proposed factors to consider include frequency of recurrence, physical and psychological distress caused by recurrences, and the potential for transmission to the patient's sexual partner. Newer therapeutic modalities, including the topical immune response modifier resiquimod and herpes vaccines, may eventually be shown to further decrease the psychological morbidity of recurrent genital herpes.

2-Aminopurine↗

MR imaging of spinal tumors in children with neurofibromatosis 1.

OBJECTIVE: The need for radiologic surveillance of spinal tumors in children with neurofibromatosis 1 is controversial and unresolved. We aimed to determine the incidence of spinal tumors in asymptomatic patients, describe the imaging findings, and evaluate the clinical usefulness of a prospective MR imaging surveillance program of the spine in children with neurofibromatosis 1. SUBJECTS AND METHODS: Of 62 children consecutively seen in a neurofibromatosis 1 clinic, 53 (85.5%) were recruited for MR imaging of the whole spine. All children satisfied the clinical criteria for diagnosis of neurofibromatosis 1. Imaging findings, clinical signs and symptoms, and subsequent clinical management were reviewed. RESULTS: The patients were 35 boys and 18 girls (age range, 11 months-18 years; mean age, 9.6 years), all of whom were asymptomatic, with no remarkable neurologic signs. Seven children (13.2%) had spinal neurofibromas: four had solitary neurofibromas (two dumbbell, one intradural, and one paraspinal tumor) and three had plexiform neurofibromas of the sacral plexus and thoracic and lumbar nerve sheaths. The incidences of scoliosis, localized cutaneous neurofibromas, and massive soft-tissue neurofibromas were 71.4%, 71.4%, and 28.6%, respectively, in the group with spinal neurofibromas (n = 7), and 30.4%, 39.1%, and 8.7%, respectively, in the group without spinal neurofibromas (n = 46). Patient clinical outcome was affected in only one patient (1.9%) in whom a solitary neurofibroma was resected. Follow-up imaging in 10 patients (mean period, 29 months) showed no evidence of tumor occurrence, progression, or recurrence. CONCLUSION: Although benign spinal neurofibromas are not uncommon in asymptomatic children with neurofibromatosis 1, the clinical usefulness of spine surveillance with MR imaging is limited in these children, making its effectiveness questionable.

Adolescent↗

[The level of the sciatic nerve division and its relations to the piriform muscle].

BACKGROUND: The sciatic nerve, as the terminal branch of the sacral plexus, leaves the pelvis through the greater sciatic foramen beneath the piriform muscle. Afterwards, it separates into the tibial and the common peroneal nerve, most frequently at the level of the upper angle of the popliteal fossa. Higher level of the sciatic nerve division is a relatively frequent phenomenom and it may be the cause of an incomplete block of the sciatic nerve during the popliteal block anesthesia. There is a possibility of different anatomic relations between the sciatic nerve or its terminal branches and the piriform muscle (piriformis syndrome). The aim of this research was to investigate the level of the sciatic nerve division and its relations to the piriform muscle. It was performed on 100 human fetuses (200 lower extremities) which were in various gestational periods and of various sex, using microdissection method. Characteristic cases were photographed. RESULTS: Sciatic nerve separated into the tibial and common peroneal nerve in popliteal fossa in 72.5% of the cases (bilaterally in the 66% of the cases). In the remainder of the cases the sciatic nerve division was high (27.5% of the cases) in the posteror femoral or in the gluteal region. Sciatic nerve left the pelvis through the infrapiriform foramen in 192 lower extremities (96% of the cases), while in 8 lower extremities (4% of the cases) the variable relations between sciatic nerve and piriform muscle were detected. The common peroneal nerve penetrated the piriform muscle and left the pelvis in 5 lower extremities (2.5% of the cases) and the tibial nerve in those cases left the pelvis through the infrapiriform foramen. In 3 lower extremities (1.5% of the cases) common peroneal nerve left the pelvis through suprapiriform, and the tibial nerve through the infrapiriform foramen. The high terminal division of sciatic nerve (detected in 1/3 of the cases), must be kept in mind during the performing of popliteal block anesthesia. CONCLUSION: Although very rare, anatomical abnormalities of common peroneal nerve in regard to piriform muscle are still possible.

Buttocks↗

Variations of nerves located in deep gluteal region.

The relation of the nerves with the piriformis muscle in the deep gluteal region examined in 50 buttock from 18 male and 7 male newborn cadavers. The nerves were found in usual position in 74% of sides, while one or more nerves perforated the piriformis in 16% and, unusual location of the nerves with intact piriformis was in 10% of sides. Abnormal located nerves are utilised in view of whether originating from dorsal or ventral part of the sacral plexus.

Buttocks↗

Major complications following exenteration in cases of pelvic malignancy: a 10-year experience.

AIM: To analyze the major complications after exenteration of gynecological and rectal malignancies. METHODS: Twenty-two patients with gynecological malignancy and 6 with rectal malignancy underwent pelvic exenteration (PE) between 1996 and 2005. PE was performed for primary malignancy in 71.4% of cases (vulvar cancer in 13, cancer rectal in 5, cervical cancer in 1 and Bartholin's gland cancer in 1 cases respectively and recurrent malignancy in 28.6% of cases (cervical cancer in 5, ovarian cancer in 1, uterine sarcoma in 1 and rectal cancer in 1 cases respectively). Posterior PE, total PE and anterior PE were most often performed. RESULTS: Major complications in the operative field involving the urinary tract infection or the wound dehiscence occurred in 12 patients (42.9%). Early complications included massive bleeding from the sacral plexus, adult respiratory distress syndrome (ARDS), thrombophlebitis, acute renal failure, urinary bladder dysfunction, ureter damage, re-operation and pulmonary embolus. Urinary incontinence was observed in 2 women as a late complication. In 1 patient a nephrostomy was performed in 1 patient due to extensive hydronephrosis and 1 patient had complications connected with the gastrointestinal tract. The mortality rate was 7%, of which inter-operative mortality accounted for 3.5%. Major complications often occurred in advanced primary vulvar cancer affecting those with recurrent malignancies. CONCLUSION: PE is more beneficial to patients with primary vulvar and rectal cancer than to those with recurrent cancer. Knowledge of the inherent complications and morbidity of PE is essential.

Acute Kidney Injury↗

[Postpartum footdrop].

We report on a rare peripartal neuropathy of the leg caused by prolonged difficult labour. Immediately after delivery two patients complained of unilateral footdrop and numbness in the leg. The footdrop was most probably due to compression of the lumbosacral trunk exposed to the foetal head. This trunk contains fibres from lumbar roots L4 and L5 and connects the lumbar with the sacral plexus. The outcome was favourable in both patients. If subsequent pregnancies occur, caesarean section may be indicated.

Adult↗

Lumbosacral nerve root avulsion: report of a case and review of the literature.

The 14th myelographically demonstrated case of lumbosacral nerve root avulsion is presented with a summary of the previously reported cases. In most cases lumbosacral nerve root avulsion is associated with pelvic fractures and sacroiliac dislocation, which cause a stretching force to be applied to the nerves of the lumbar and sacral plexuses, and in turn to the nerve roots intradurally. This force causes nerve root avulsion in the intradural course of the nerve root. The myelographic defect is a pseudomeningocele or diverticulum-like outpouching created by the tearing of the arachnoid covering of the nerve roots. Myelography clearly indicates nerve root avulsion and surgical exploration is not indicated.

Adult↗

[Percutaneous cervical cordotomy in the therapy of incidence pain].

BACKGROUND: Incident pain does not respond to opioid treatment and it is not easily relieved with other therapeutic strategies (local intrapleural or spinal analgesia, phenol blocks etc.). For this reason cervical percutaneous cordotomy at C(1)-C(2) interspace is the only effective antalgic therapy in patients whose life expectancy is more than three to six months. METHODS: This study is a rectrospective review of 22 patients with cancer and incident pain from brachial, lumbar-sacral plexus injury and gluteal ulcer. RESULTS: Cordotomy provided excellent contralateral side pain relief in 21 patients; pain relief was maintained up to death and to the moment of last observation in living patients. In one deaf patient it was impossible to carry out the procedure due to incomplete co-operation and pain returned after 48 hours. Ventilatory depression caused death in one patient. Other complications recorded included ataxia, headache, motor deficit, dysesthesia and orthostatic hypotension. CONCLUSIONS: The conclusion is drawn that percutaneous cordotomy should, in carefully selected cases, be considered the only technique to relieve incident pain.

Adult↗

Laparoscopic-assisted resection of a large retroperitoneal tumor.

BACKGROUND: Retroperitoneal tumors have traditionally been excised using standard open techniques. When preparing the manuscript we knew of only 1 report, in Japanese, describing the excision of 2 retroperitoneal neurofibromas using a minimally invasive technique. The feasibility of the technique was tested in the removal of a large retroperitoneal neurofibroma. METHODS: Laparoscopic-assisted resection of a large retroperitoneal tumor was offered to a 37-year-old female with a 12 x 10 x 9-cm neurofibroma located inferior to the left kidney. The patient was placed on a beanbag in a right lateral decubitus position. Three 5-mm ports were used, and the tumor was mobilized followed by placement of a hand port for completion of dissection and removal of the tumor. RESULTS: The tumor was successfully removed in about 3 hours, and blood loss was <100 cc. The patient developed urinary and bowel dysfunction probably secondary to the effect of the dissection and removal of the tumor on the nearby sacral plexus. CONCLUSIONS: We believe that laparoscopy is a feasible and appropriate technique available to general and laparoscopic surgeons in the management of retroperitoneal tumors, providing excellent visualization and adequate access for proper dissection through small incisions.

Adult↗

[Uterine avulsion by blunt abdomino-pelvic trauma].

OBJECTIVE: Our objective was to present a case of uterine avulsion due to blunt trauma. CLINICAL CASE: A 40-year-old female with diabetes mellitus and chronic renal failure was run over by a truck resulting in an unstable pelvic fracture. She was submitted to external pelvic fixation, but for homodynamic instability a diagnostic peritoneal lavage was done with resulting macroscopic blood. A laparotomy was done and a uterine avulsion was found in the union of the isthmus and corpus. A subtotal hysterectomy was performed and the pelvis was also packed due to damage of the sacral plexus. RESULTS: The patient survived the pelvic and genital injuries but died 14 days after the initial surgery due to renal failure. CONCLUSIONS: Uterine avulsion is an infrequent but potentially lethal lesion associated with complex pelvic fractures.

Abdominal Injuries↗

Pelvic retroperitoneal schwannoma presenting as a gynecologic mass: case report.

Schwannomas of the sacral plexus are retroperitoneal tumors, usually benign, that result from proliferation of perineural cells. They are rare, pelvic, well defined tumors that present infrequently as gynecologic masses. Misdiagnosis is not uncommon in these cases, since these masses are not often encountered in clinical practice and preoperative imaging methods can be only suggestive of the diagnosis. Furthermore, these masses are not often encountered in clinical practice. We report a case of a 28-year-old woman who presented with a pelvic mass and obscure clinical signs. Imaging methods suggested that this tumor was most probably an ovarian mass of embryonic origin. After dissection by laparotomy, the microscopic examination showed typical futures of a neurilemoma (ancient schwannoma). The goal of the operating intervention is to excise the tumor avoiding major trauma, thus cooperation between surgeons is indispensable. The patient has had no motor or sensory disturbances after surgery.

Adult↗

The role of pelvic exenteration for treatment of pelvic malignancy--a nine-year experience.

Pelvic exenteration offers the last chance for some women with gynecological and rectal malignancy. A series of 23 patients who underwent pelvic exenteration for local advanced gynecological and rectal malignancies between 1996 and 2004 were retrospectively reviewed. The exenteration was performed because of vulvar cancer in 14 patients and other pelvic malignancies in nine cases: rectal cancer in four cases, in three cases cervical cancer, in one case ovarian cancer and in one case uterine sarcoma. Nine patients developed major complications of the operative field involving the urinary tract or the wound. Early complications included massive bleeding from the sacral plexus in two cases (one patient died during surgery), acute respiratory distress syndrome (ARDS) in one case and thrombophlebitis in one case. Urinary incontinence was observed in two women as a late complication. Only one patient had a complication connected with the gastrointestinal tract. Twenty-two patients were followed-up. In the group of patients with vulvar cancer five women died after 4-29 months because of recurrence of disease. The nine surviving patients are still being followed-up and are without disease; survival time ranges from 6-74 months. In the group of patients with other malignancies four women died.

Aged↗

[Clinical observation on needle pricking for treatment of infertility due to varicocele after varicocelectomy].

OBJECTIVE: To observe clinical therapeutic effect of needle pricking therapy on infertility with varicocele after varicocelectomy. METHODS: One hundred and twenty-two cases were randomly divided into a treatment group of 62 cases and a control group of 60 cases. The treatment group were treated with needle pricking at sacral plexus nerve spots and lumbar plexus nerve spots, and the control group were treated with intramuscular injection of HCG. RESULTS: After treatment, the pregnancy rate of the patient's wife was 79.0% in the treatment group and 41.7% in the control group with a significant difference between the two groups (P < 0.01); the production hormones improved significantly in the both groups (P < 0.01); superoxide dismutase and Zn in the semen elevated significantly in the treatment group (P < 0.05). CONCLUSION: Needle pricking therapy can significantly improve and regulate endocrines in the patient of varicocele after varicocelectomy, and elevate quality of semen and sperm and the pregnancy rate of the patient's wife.

Biopsy↗

[Internal fixation for pelvic posterior ring lesions].

OBJECTIVE: To explore the choice for the internal fixation in treatment of pelvic posterior lesions. METHODS: From May 2000 to June 2005, the treatment was given to 40 patients (28 males, 12 females, aged 21-58 years) with pelvic posterior ring fracture and dislocation. Of the patients, 23 had a traffic accident, 11 had a crush injury and 6 had a fall. As for the state of an injury to the pelvic posterior ring, 22 patients had dislocation of the sacroiliac joint, 12 had a sacrum fracture dislocation, and 6 had an ala iliac fracture and dislocation of the sacroiliac joint. According to the Denis (1988) classification, fracture of the (sacral region I was found in 6 cases, fracture of the sacral) region II in 3 cases, and fracture of the sacral region III in 3 cases. As for the complication of the pelvic front ring fracture: separation of the symphysis pubis was found in 14 cases, fracture of the superior ramus and inferior ramus of the pubis on one side in 10 cases. The two-side superior ramus of public and inferior ramus of pubis in 8 cases, homopleural acetabular fracture on one side in 4 cases, acetabular fracture on one side and contralateral superior ramus and inferior ramus fracture of the pubis in 3 cases, and acetabular fracture on the opposite side in 1 case. As for the operation, 28 patients underwent the still-plate internal fixation of the sacroiliac joint from anterior at 24 h to 15 days after the injury, 2 underwent the screw internal fixation of the sacroiliac joint from posterior, and remaining 10 underwent the internal fixation by the Galveston Technique associated with the ISOLA system. The therapeutic results were analyzed. RESULTS: The follow-up of the 40 patients for 6 months to 3 years revealed that before operation 3 had a sacral plexus nerve injury, and after operation 1 patient developed perineum numbness and urinary incontinence, 1 developed claudication,3 developed posterior urethral fragmentation, and 2 developed urinary bladder rupture; however, they had a complete recovery after the reparative surgery. CONCLUSION: In treatment of the pelvic posterior ring lesions, an appropriate internal fixation can be chosen according to the type of the pelvic fracture, applicability of internal fixation, condition of the patient,equipment available, and the doctor's experience.

Adult↗

[Polyneuropathy due to "allergic vasculitis" possibly related to australia antigen. (author's transl)].

The authors describe a case of flaccid tetraplegia due to a polyneuropathy in a man aged 50 years, progressing rapidly to death from mesenteric infarction. As significant abnormalities, the hematologic examinations revealed a very high erythrocyte sedimentation rate, a marked leukocytosis and a positive Australia antigen in repeated controls. In spite of the clinical picture being that of a symmetrical polyneuropathy rather than that of mononeuritis multiplex, the diagnose of a collagenosis was suspected. Nevertheless steroid treatment was not given because the patient had a peptic ulcer. On histological investigation many focal infarctions were found in several internal organs but the brain was pratically normal. Instead the nerve trunks examined (the sciatic nerve and the lumbo-sacral plexus) showed gross changes in the vasa nervorum represented by abundant perivascular cuffing and marked evidence of axonal degeneration secondary to an ischaemic damage. In the kidneys there were definite signs of membranous glomerulitis. The pathological findings in this fatal case of diffuse peripheral involvement were not those of classical periarteritis nodosa, but showed the picture of so-called "allergic vasculitis". This type of blood vessels inflammation is now assumed to be part of an immune-complex disease, in which the Australia antigen could be one of the causative agents.

Hepatitis B Antigens↗