Scoliosis and diastematomyelia: four cases and a review of the literature

We report four cases of scoliosis associated with diatematomyelia observed between 1984 and 1998. The patients were four girls aged 10 to 12 years. Skin lesions were found on the midline in 3 cases and 2 had a neurological disorder. A myeloscan was performed in two cases, tomomyelography in one and MRI in one. Three patients were operated on with good outcome. The fourth child is under orthopedic treatment. We reviewed the literature on scoliosis with diastematomyelia.     

The Mitchell bunionectomy: a prospective study of 60 consecutive cases utilizing single K-wire fixation.

The authors prospectively evaluated 45 patients (60 feet) affected by hallux valgus and treated with a distal metatarsal osteotomy. The surgical procedure consisted of a modified Mitchell osteotomy, in which fixation was achieved with a Kirschner wire that was driven into the proximal osteotomy fragment and buttressed the distal one. Early weightbearing was allowed without a cast. Follow-up averaged 25 months. The mean American Orthopedic Foot and Ankle Society clinical hallux score increased from 44.6/100 preoperatively to 83.2/100. Radiographic evaluation showed that mean metatarsophalangeal and intermetatarsal angles decreased respectively from 31.7 degrees to 16.9 degrees, and from 15.4 degrees to 8.6 degrees. Short-term loss of correction occurred in three cases (4%). Six feet (10%) had unrelieved metatarsalgia that was related to excessive shortening of the first metatarsal and/or inappropriate orientation of the metatarsal head. Stabilization of the Mitchell osteotomy with a Kirschner wire proved safe and effective for the surgical correction of mild to moderate hallux valgus.     

Traumatic lumbosacral dislocation: four cases and review of literature

Diagnosis, physiopathology, and treatments of four patients with traumatic lumbosacral dislocations are described. This is a rare but severe lesion of the lumbosacral junction that usually occurs in patients with multiple trauma. It often is not thought of and the diagnosis may be missed. Evidence of lumbosacral dislocation should be examined and confirmed by computed tomography scans in patients with multiple fractures of transverse lumbar processes, asymmetric lumbosacral joints on frontal images, or slipping of L5 over S1 on lateral images. Treatment consists of reduction of the dislocated and fractured parts, lumbosacral arthrodesis, a posterolateral graft, and posterior instrumentation. Instrumentation may be short, extending from L5 to S1, or long, from L4 to S1, depending on the extension of the lesion. In some cases, reduction can be done intraoperatively, when the L4-S1 instrumentation is inserted, provided L5 transpedicle screws are pulled posteriorly. It usually is preferable to explore the vertebral canal to ensure that there is no disc lesion compressing the dura before proceeding with reduction. Compression of the dura could be avoided with a preoperative magnetic resonance imaging scan on which a lesion of the L5-S1 disc is sought. Additional interbody vertebral arthrodesis should be considered when the L5-S1 disc is affected severely. This lesion should be looked for preoperatively with a magnetic resonance imaging scan and intraoperatively by exploring the canal. This can be done at the time of the posterior surgery or during a second anterior surgical procedure.     

Laryngeal melanosis: Report of four cases and literature review

OBJECTIVE: Laryngeal melanosis is a rare condition defined by the presence of melanocytes within the laryngeal epithelial lining. Our aims were (1) to review our cases together with those in the literature, and (2) to determine whether melanocyte incidence is increased with exposure to irritant stimuli such as tobacco.METHODS: A retrospective study of all cases diagnosed with laryngeal melanosis in our hospital from January 1, 1990, to December 31, 1996, was accomplished. To determine the melanocyte incidence in the normal larynx as well as the influence of tobacco in development of laryngeal melanosis, 16 age-matched controls, 8 of whom were smokers and 8 of whom were not, were chosen, and a histochemical and immunohistochemical study was performed. The following antibodies were used: S-100 protein, CD1a, and HMB-45. A comparative study of the melanocyte incidence between patients with laryngeal melanosis and the controls was carried out. Also, a comparative study between smoking and nonsmoking patients was performed.RESULTS: Laryngeal melanosis was diagnosed in 4 patients at our hospital during this period of time. In the comparative study, the number of melanocytes in the 4 patients with laryngeal melanosis was higher than in the 8 smoking ( p < 0.01, Mann-Whitney U test) and 8 nonsmoking ( p < 0.01) controls, and there was a trend toward a higher number of melanocytes in the 8 smoking patients than in the 8 nonsmoking ( p = 0.064) controls.CONCLUSIONS: Laryngeal melanosis was more frequent in smoking men older than 50 years. Our observations underline the association of LM with larynx carcinoma and its relation to a stimulus such as tobacco. In fact, we have found activated melanocytes in our cases of laryngeal melanosis. They were identified by immunoreactivity for HMB-45. (Otolaryngol Head Neck Surg 1997;117:708-12.)     

Juvenile gigantomastia: presentation of four cases and review of the literature

Juvenile gigantomastia is a benign disorder of the breast in which one or both of the breasts undergo a massive increase in size during adolescence. The authors present a series of four cases of juvenile gigantomastia, advances in endocrine management, and the results of surgical therapy. Three patients were treated for initial management of juvenile gigantomastia and one patient was evaluated for a gestationally induced recurrence of juvenile gigantomastia. The three women who presented for initial management had a complete evaluation to rule out other etiologies of breast enlargement. Endocrine therapy was used in 2 patients, one successfully. A 17-year-old girl had unilateral hypertrophy treated with reduction surgery. She had no recurrence and did not require additional surgery. Two patients, ages 10 and 12 years, were treated at a young age with reduction mammaplasty, and both of these girls required secondary surgery for treatment. One patient underwent subtotal mastectomy with implant reconstruction but required two subsequent operations for removal of recurrent hypertrophic breast tissue. The second patient started a course of tamoxifen followed by reduction surgery. While on tamoxifen, the second postoperative result remained stable, and the contralateral breast, which had exhibited some minor hypertrophy, regressed in size. The fourth patient was a gravid 24-year-old who had been treated for juvenile gigantomastia at age 14, and presented with gestationally induced recurrent hypertrophy. The authors' experience has been that juvenile gigantomastia in young patients is prone to recurrence, and is in agreement with previous studies that subcutaneous mastectomy provides definitive treatment. However, tamoxifen may be a useful adjunct and may allow stable results when combined with reduction mammaplasty. If successful, the use of tamoxifen would eliminate the potential complications of breast prostheses. Lastly, the 17-year-old patient did not require secondary surgery, suggesting that older patients may be treated definitively with reduction surgery alone.     

Orbital roof growing fractures: a report of four cases and literature review

Trivial injuries among paediatric patients are usually underestimated. Such injuries may account for delayed problems such as growing fractures of skull vault. Growing fracture of the orbital roof is rarely reported. Four cases of paediatric cranial trauma with growing orbital roof fractures are presented. Three cases required only dural repair, while the fourth patient underwent both duraplasty and cranioplasty, due to a large orbital roof defect. At follow-up, all had satisfactory outcome. Trivial injuries among children should be evaluated with caution. The development of orbital symptomatology should ask for a complete radiological survey. The orbital roof growing fractures are potentially important cause of orbital problems. The dural repair alone, usually provides satisfactory outcome, while calvarial bone graft may be of help in cases with large bony defects. Early diagnosis and management of such cases may prevent permanent neurological deficits.     

Adenoacanthoma of the pancreas: report of four cases and literature review

Cases of 20 patients with adenoacenthoma of the pancreas with clinicopathologic data, including the four added, are reviewed. The clinical manifestations, sites of metastases, survival and gross pathology appear to be similar to the usual adenocarcinoma of the pancreas. Adenoacanthoma of the pancreas most probably represents squamous metaplasia of an adenocarcinoma or arises from an undifferentiated cell in the pancreatic duct system. The metastases are typically an admixture of both elements but in four cases, pure squamous or adenocarcinoma metastases were encountered. It is suggested that the pancreas should be included as a possible source in those patients with an unknown primary who have a metastasis consisting of either an admixture of squamous and glandular elements or a pure squamous type and in those instances in which a pure squamous and a pure adenocarcinoma are encountered in different metastases.     

Gallbladder torsion: report of four cases and review of the literature

Gallbladder torsion is a rare cause of acute acalculous cholecystitis. Preoperative diagnosis is difficult. The treatment of choice remains immediate cholecystectomy. We present four cases of gallbladder torsion and review the literature.     

Bulking agents: an analysis of 500 cases and review of the literature

Introduction and hypothesis

Stress urinary incontinence (SUI) is common, impacts women’s quality of life, and generates high costs. Physiotherapy is the first-line therapy, and if it fails, suburethral slings are the gold standard in SUI surgery. Bulking agents injected periurethrally might be a beneficial alternative, but there is a paucity of data on bulking therapy. The aim of this study was to prospectively analyze the efficacy and safety of bulking agents in the setting of a tertiary referral center.

Methods

In the last 13 years, 514 elderly women with SUI were treated by injection therapy with either collagen (Contigen®), hyaluronic acid (Zuidex®), ethylene vinyl alcohol (Tegress®), or polyacrylamide hydrogel (Bulkamid®). Subjective and objective outcome was recorded at the 12-month postoperative appointment using the King’s Health Questionnaire, visual analogue scale (VAS) describing their incontinence severity, standardized pad test, and urethral pressure profile.

Results

Demographic data were equally distributed in all four groups of agents used. Sixty-one patients were lost to follow-up (10.6 %). Statistically significant changes were found for maximum urethral closure pressure (MUCP), pad weight, and VAS before and after bulking for the four agents used. Pad test was negative in 73.2 % of patients after bulking therapy. Subjective assessment showed improvements in general health and role limitations. The overall complication rate was low for all agents.

Conclusions

This study shows improvement in incontinence after bulking therapy according to subjective and objective outcomes in an elderly population. In contrast to earlier reports, side effects due to injections were few and mild. We can advocate bulking therapy for treating SUI, as it is simple, safe, and shows both objective and subjective improvement and relief.     

Primary vertebral echinococcosis: four cases reports and review of literature

Primary vertebral echinococcosis has been considered to be a hypothetical and exceptional possibility. It affects bones in 1% of reported cases. Spine is involved in 45-50% of these. The disease preferentially affects the thoracic spine. It begins as a single primary cyst containing daughter cysts. We report four cases of primary vertebral echinococcosis, extending into the extradural space without any other primary site of infection. We conclude that primary vertebral echinococcosis, although rare should be considered in destructive lesions of the vertebrae and spine in regions that the disease is endemic. Advanced imaging studies should be performed to diagnose the disease. Early decompressive surgery of the spine, with chemotherapy, is the treatment of choice for these patients.     

Aplasia cutis congenita: report of four cases and literature review

Aplasia cutis congenita (ACC) is a defect of cutaneous development, most commonly on the scalp. The incidence is between 0.5–1/10,000 of newborns. It can be associated with other congenital syndromic anomalies. Different aetiological theories exist about ACC. The defects are probably not attributable to one single factor. Management of the less severe forms is relatively simple and treatment can be either conservative or operative. More severe cases involving the skull are associated with a 20–55% mortality rate, due to massive haemorrhage or infection. Four new cases are reported; three were treated conservatively, one patient had a severe form which was associated with other congenital anomalies and died 1 month after birth. This small series confirms the effectiveness of conservative therapy in the treatment of limited areas of ACC.     

A case of lung metastasis in myxoinflammatory fibroblastic sarcoma: analytical review of one hundred and thirty eight cases

Purpose

Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue tumour first identified at the end of the 1990s. This study presents our experience and literature reviews focusing on risk of recurrence.

Methods

Rizzoli Orthopaedic Institute database and literature were searched for patients with MIFS observed from 1997 to 2012. Data were analysed in a new database.

Results

Five patients underwent surgery at our institute, and 133 cases were retrieved from the literature. Not all clinicopathological data were available: 76/138 were men (55 %), median age was 45 [interquartile range (IQR) 34–56] years, median tumour size was three (IQR two to five) centimetres. Common sites of occurrence were hand (24 %), fingers (23 %) and foot (20 %). Pain was present at diagnosis in 14/82 patients (17 %), with a median duration of seven (IQR three to 12) months. Surgery was performed for a suspected benign tumour in 88 patients (74 %). Resection was incomplete in 45/71 cases (63 %); re-excision was performed in 32/45 (71 %). At a median follow-up of 26 months, 26/118 patients (22 %) developed recurrent disease; median time to recurrence was 15 months (IQR seven to 26). Actuarial relapse-free survival (RFS) at one, three and five years was 93 %, 72 % and 67 %, respectively. At univariate analysis, only symptom duration of six months or less was significantly associated with a worse RFS (p = 0.046). Metastatic disease to lymph nodes and/or lungs was observed in four patients (3 %).

Conclusions

Clinicopathological findings confirm the low-grade nature of MIFS. However, local recurrence occurs, and patients may be affected by aggressive forms with a potential for distant metastases. Follow-up is strongly advised.     

Radial nerve palsy in the newborn: a report of four cases and literature review

BACKGROUND: Radial nerve palsy in the neonate is a rare clinical entity but must be distinguished from the more common brachial plexus birth palsy which occurs during the perinatal period. Although longer term upper-limb function following brachial plexus birth palsy is highly variable depending on the extent of neurological involvement, sparse reports of neonates with radial nerve palsy have nearly universal spontaneous recovery with normal upper-limb function. METHODS: We report 4 cases of patients born with findings consistent with radial nerve palsy. RESULTS: All 4 cases of neonatal radial nerve palsy supported a common etiology of intrauterine compression and resulted in spontaneous recovery of radial nerve function. CONCLUSION: Neonatal radial nerve palsy should be suspected in newborns presenting with absent wrist and digital extension but intact deltoid, biceps, and triceps function with wrist and digital flexor function. The presence of ecchymosis and/or fat necrosis along the posterolateral brachium may support the notion that neonatal radial nerve palsy is caused by a compression injury during or before labor. Complete spontaneous recovery of radial nerve function may be anticipated if there is no associated infectious or constriction band pathology.     

Mucoceles of the sphenoidal sinus: a report of four cases and review of the literature

Mucoceles of the sphenoidal sinus represent less than 2% of paranasal sinus mucoceles. The purpose of this paper is to report our experience in patients with mucoceles of the sphenoidal sinus. Four patients with this localization were observed in our department from 1994 to 2005. Symptoms included headache, visual loss, and rhinorrhea. All patients underwent computed tomography (CT) and magnetic resonance imaging (MRI). Surgery was performed using an endonasal endoscopic procedure, in one case a navigational system was used. In light of these observations and a review of literature, the etiopathogenic characteristics, clinical aspects, and therapeutic indications of this localization are discussed.