The study design is retrospective. The aim is to describe our experience about the treatment of patients with neuromuscular scoliosis (NMS) using Cotrel–Dubousset instrumentation. Neuromuscular scoliosis are difficult deformities to treat. A careful assessment and an understanding of the primary disease and its prognosis are essential for planning treatment which is aimed at maximizing function. These patients may have pelvic obliquity, dislocation of the hip, limited balance or ability to sit, back pain, and, in some cases, a serious decrease in pulmonary function. Spinal deformity is difficult to control with a brace, and it may progress even after skeletal maturity has been reached. Surgery is the main stay of treatment for selected patients. The goals of surgery are to correct the deformity producing a balanced spine with a level pelvis and a solid spinal fusion to prevent or delay secondary respiratory complications. The instrumented spinal fusion (ISF) with second-generation instrumentation (e.g., Luque–Galveston and unit rod constructs), are until 1990s considered the gold standard surgical technique for neuromuscular scoliosis (NMS). Still in 2008 Tsirikos et al. said that “the Unit rod instrumentation is a common standard technique and the primary instrumentation system for the treatment of pediatric patients with cerebral palsy and neuromuscular scoliosis because it is simple to use, it is considerably less expensive than most other systems, and can achieve good deformity correction with a low loss of correction, as well as a low prevalence of associated complications and a low reoperation rate.” In spite of the Cotrel–Dubousset (CD) surgical technique, used since the beginning of the mid 1980s, being already considered the highest level achieved in correction of scoliosis by a posterior approach, Teli et al., in 2006, said that reports are lacking on the results of third-generation instrumentation for the treatment of NMS. Patients with neuromuscular disease and spinal deformity treated between 1984 and 2008 consecutively by the senior author (G.D.G.) with Cotrel–Dubousset instrumentation and minimum 36 months follow-up were reviewed, evaluating correction of coronal deformity, sagittal balance and pelvic obliquity, and rate of complications. 24 patients (Friedreich’s ataxia, 1; cerebral palsy, 14; muscular dystrophy, 2; polio, 2; syringomyelia, 3; spinal atrophy, 2) were included. According the evidence that the study period is too long (1984–2008) and that in more than 20 years many things changed in surgical strategy and techniques, all patients were divided in two groups: only hooks (8 patients) or hybrid construct (16 patients). Mean age was 18.1 years at surgery (range 11 years 7 months–max 31 years; in 17 cases the age at surgery time was between 10 and 20 years old; in 6 cases it was between 20 and 30 and only in 1 case was over 30 years old). Mean follow-up was 142 months (range 36–279). The most frequent patterns of scoliosis were thoracic (10 cases) and thoracolumbar (9 cases). In 8 cases we had hypokyphosis, in 6 normal kyphosis and in 9 hyperkyphosis. In 8 cases we had a normal lordosis, in 11 a hypolordosis and in 4 a hyperlordosis. In 1 case we had global T4–L4 kyphosis. In 8 cases there were also a thoracolumbar kyphosis (mean value 24°, min 20°–max 35°). The mean fusion area included 13 vertebrae (range 6–19); in 17 cases the upper end vertebra was over T4 and in 11 cases the lower end vertebra was over L4 or L5. In 7 cases the lower end vertebra was S1 to correct the pelvic obliquity. In 5 cases the severity of the deformity (mean Cobb’s angle 84.2°) imposed a preoperative halo traction treatment. There were 5 anteroposterior and 19 posterior-only procedures. In 10 cases, with low bone quality, the arthrodesis was performed using iliac grafting technique while in the other (14 cases) using autologous bone graft obtained in situ from vertebral arches and spinous processes (in all 7 cases with fusion extended until S1, it was augmented with calcium phosphate). The mean correction of coronal deformity and pelvic obliquity averaged, respectively, 57.2% (min 31.8%; max 84.8%) and 58.9% (mean value preoperative, 18.43°; mean value postoperative, 7.57°; mean value at last follow-up, 7.57°). The sagittal balance was always restored, reducing hypo or hyperkyphosis and hypo or hyperlordosis. Also in presence of a global kyphosis, we observed a very good restoration (preoperatory, 65°; postoperatory, 18° kyphosis and 30° lordosis, unmodified at last f.u.). The thoracolumbar kyphosis, when present (33.3% of our group) was always corrected to physiological values (mean 2°, min 0°–max 5°). The mean intraoperative blood lost were 2,100 cc (min 1,400, max 5,350). Major complications affected 8.3% of patients, and included 1 postoperative death and 1 deep infection. Minor complications affected none of patients. CD technique provides lasting correction of spinal deformity in patients with neuromuscular scoliosis, with a lower complications rate compared to reports on second-generation instrumented spinal fusion.
Neuromuscular scoliosis; Cotrel–Dubousset; Spinal fusion
Priority of neurological decompression was regarded as necessary for scoliosis patients associated with Chiari I malformation in order to decrease the risk of spinal cord injury from scoliosis surgery. We report a retrospective series of scoliosis associated with Chiari I malformation in 13 adolescent patients and explore the effectiveness and safety of posterior scoliosis correction without suboccipital decompression. One-stage posterior approach total vertebral column resection was performed in seven patients with scoliosis or kyphosis curve >90° (average 100.1° scoliotic and 97.1° kyphotic curves) or presented with apparent neurological deficits, whereas the other six patients underwent posterior pedicle screw instrumentation for correction of spinal deformity alone (average 77.3° scoliotic and 44.0° kyphotic curves). The apex of the scoliosis curve was located at T7–T12. Mean operating time and intraoperative hemorrhage was 463 min and 5,190 ml in patients undergoing total vertebral column resection, with average correction rate of scoliosis and kyphosis being 63.3 and 71.1%, respectively. Mean operating time and intraoperative hemorrhage in patients undergoing instrumentation alone was 246 min and 1,450 ml, with the average correction rate of scoliosis and kyphosis being 60.8 and 53.4%, respectively. The mean follow-up duration was 32.2 months. No iatrogenic neurological deterioration had been encountered during the operation procedure and follow-up. After vertebral column resection, neurological dysfunctions such as relaxation of anal sphincter or hypermyotonia that occurred in three patients preoperatively improved gradually. In summary, suboccipital decompression prior to correction of spine deformity may not always be necessary for adolescent patients with scoliosis associated with Chiari I malformation. Particularly in patients with a severe and rigid curve or with significant neurological deficits, posterior approach total vertebral column resection is likely a good option, which could not only result in satisfactory correction of deformity, but also decrease the risk of neurological injury secondary to surgical intervention by shortening spine and reducing the tension of spinal cord.
Scoliosis; Kyphosis; Chiari malformation; Adolescent; Corrective surgery
In line with recent advances in magnetic resonance imaging (MRI), cavernous hemangioma (cavernoma) can be detected efficiently and reliably in patients with intractable epilepsy that surgical resection is a common treatment for them. Because these lesions are usually small and may be located near the eloquent areas, stereotactically-guided resection should be considered. This study aims to report the surgical features and outcomes of 6 patients underwent the stereotactically-guided craniotomy for excision of cavernoma.
A total of six patients (1 male and 5 female, aged 10-42 years, mean age of 29 years) with cavernoma underwent stereotactically-guided craniotomy surgery in the Rasoul-e-Akram Hospital, affiliated to Tehran University of Medical Sciences (Tehran, Iran) for resection of cerebral cavernous hemangioma during September 2007 to May 2012. Clinical presentations were seizures, headache and hemorrhage. Two patients had a previous history of intracranial hemorrhage. Diagnosis was made using MRI and digital subtraction angiography. Locations of the lesions were parietal, frontal and temporoparietal. Size of lesion ranged from 0.7 to 3.1 cm (mean 1.7 cm). All those lesions were deep-seated or located near or within eloquent areas. Stereotactically-guided craniotomy was performed for excision of lesion. Clinical follow-up period ranged 5-48 months (mean 28 months). In all patients, complete surgical resection was achieved as demonstrated by postoperative image studies.
There was no associated morbidity or mortality. Histological diagnosis was consistent with cavernoma in all cases. Clinical follow-up revealed that all patients had complete recovery from preoperative symptoms and patients with seizures showed marked improvement.
In conclusion, stereotactic guided craniotomy in surgery of eloquent or deep-seated cerebral cavernous malformations represents a very accurate and safe approach.
Stereotactically-guided craniotomy, Cavernous hemangioma, Magnetic resonance imaging
The choice of anterior instrumentation in the treatment of lumbar scoliosis in adolescents and young adults is not a new topic for the authors. The first results achieved using the Dwyer surgical modality were reported by one of the authors followed by the results achieved using Zielke (VDS) instrumentation. Today, new techniques and new instrumentations have been developed that challenge the instrumentation choices. Here we describe how the new system of classification of scoliotic curves we developed has been used as a basis for treating idiopathic scoliosis in lumbar area in adolescents and young adults using an anterior approach.
A prospective study was carried out between 1998 and 2010 at two hospital centers on 33 adolescents and young adult with idiopathic lumbar scoliosis involving curves of three kinds, on whom surgical treatment was performed using a single solid rod. Topography of curves: our system of classification includes curves corresponding to the following three type of scoliosis: Type K I: double thoracic and lumbar curves (lumbar predominant) scoliosis (17 cases) mean age 16 years all female patients. Mean Cobb angle of lumbar curve 41°. Mean Cobb angle of thoracic curve 28°. The lumbar curve was left hand convex in 15 cases and right hand convex in 2 cases. Horizontal tilting of L4 mean value 22°. C7 offset mean value 3 cm. Type K IV A: unbalanced thoracolumbar scoliosis (13 cases) mean age 17 years, ten female patients and three male patients. Mean Cobb angle of thoracolumbar curve 39°. The thoracolumbar curve was left hand convex 4 times and right hand convex 9 times. Horizontal tilting of L4 mean value 18°. C 7 offset mean value 2.5 cm. Type K VI A: real lumbar (three cases). Age: 17, 15 and 13 years; all female patients. Cobb angle of the lumbar curve 66°, 29° and 70° (all LH convex). Horizontal tilting of L4: 40°, 20° and 46°. C 7 offset: 7 cm, 1 cm and 4 cm.
Surgical instrumentation: We used the EUROS AZUR anterior instrumentation for all the procedures. Cages have been used on five patients at the lower stages. Number of vertebrae instrumented: mean five vertebrae. The patients did not wear postoperative orthosis. Mean duration of procedure: 3 h 50 min. Mean blood loss: 350 cm3.
Type K I scoliosis (17 cases): Mean follow-up: 6 years. Correction of the lumbar curve Cobb angle: the mean angle has been corrected from 41° to 21°. Number of vertebrae instrumented: 4:6 times and 5:11 times. Correction of the upper thoracic curve Cobb angle: mean angle corrected from 28° to 19°. Correction of L4 horizontal tilting: mean residual was 7°. Correction of C 7 offset: mean 0.7 cm. Type K IV A scoliosis (13 cases): mean follow-up: 4 years. Correction of the lumbar curve Cobb angle: the mean angle has been corrected from 39° to 16°. Mean number of instrumented vertebrae: 5 (4:4 times, 5:6 times and 6:3 times.) Correction of L4 horizontal tilting: mean residual 5°. Correction of C 7 offset: mean 0.7 cm. Type K VI A scoliosis (three cases): mean follow-up: 7, 2 and 4 years; Correction of the lumbar curve Cobb angle: the angles have been corrected from 66° to 15°, from 29° to 11° and from 70° to 28°. Number of instrumented vertebrae: 5, 4 and 6. Correction of L4 horizontal tilting: residual tilting of 8°, 7° and 17°. Correction of C 7 offset: 1 cm, 0 cm and 1 cm.
There has been no report early or late septic or vascular or neurological complications. Instrumentation failure: there were three cases of screw breakage, all occurred on the lowest implant. Revision surgery was undertaken in both cases, only the last plate needed to be replaced and the rod could be kept without any other modification of the construct. In both cases, fusion has been achieved without any loss of correction. The mean loss of correction of the main curve was 2.5° for the three series.
Anterior instrumentation of lumbar idiopathic scoliosis gives highly satisfactory morphological and functional results, since the lumbar musculature is spared and the instrumentation placed at the apex of the curvature has selective effects. Despite our preference and that of other surgeons throughout the world for anterior instrumentation, we are still a minority in comparison with the users of posterior instrumentation. There are several reasons for this reticence, including surgeons’ training and ideas about pedicular screw fixation, but the main reason has been the lack of a sufficiently exact system of classification. Previous comparative studies between the anterior and posterior approaches have been biased by the use of an excessively restrictive mode of classification (lumbar/thoracolumbar) of the curves. Real lumbar scoliosis, unbalanced thoracolumbar scoliosis and thoracic and lumbar double curve (lumbar predominant) scoliosis should be properly defined before being compared.
Adolescent idiopathic scoliosis; Anterior instrumentation; Trunk balance; Sagittal balance; Shoulder balance; Curve’s classification
Cavernous hemangiomas represent the most common benign primary hepatic neoplasm, often being incidentally detected. Although the majority of hepatic hemangiomas remain asymptomatic, symptomatic hepatic hemangiomas can present with abdominal pain, hemorrhage, biliary compression, or a consumptive coagulopathy. The optimal surgical management of symptomatic hepatic hemangiomas remains controversial, with resection, enucleation, and both deceased donor and living donor liver transplantation having been reported.
We report the case of a patient found to have a unique syndrome of multiorgan cavernous hemangiomatosis involving the liver, lung, omentum, and spleen without cutaneous involvement. Sixteen years following her initial diagnosis, the patient suffered from intra-abdominal hemorrhage due to her giant cavernous hepatic hemangioma. Evidence of continued bleeding, in the setting of Kasabach-Merritt Syndrome and worsening abdominal compartment syndrome, prompted MELD exemption listing. The patient subsequently underwent emergent liver transplantation without complication.
Although cavernous hemangiomas represent the most common benign primary hepatic neoplasm, hepatic hemangioma rupture remains a rare presentation in these patients. Management at a center with expertise in liver transplantation is warranted for those patients presenting with worsening DIC or hemorrhage, given the potential for rapid clinical decompensation.
Hepatic hemangioma; Kasabach-Merritt syndrome; Liver transplantation
Early onset spinal deformities (EOSD) can be life-threatening in very young children. In the growing spine, surgical intervention is often unavoidable and should be carried out as soon as possible. A deformed section of the spine not only affects the development of the remaining healthy spine, but also that of the chest wall (which influences pulmonary function), the extremities and body balance. Posterior vertebral column resection (PVCR) represents an effective surgical solution to address such problems. However, reports in the literature concerning PVCR are mostly limited to its use in adolescents or adults. The purpose of this study was to illustrate our experience with PVCR in EOSD and to describe the surgical technique with respect to the unique anatomy of young children.
Materials and methods
Four children [mean age 3.7 (range 2.5–5.2) years] with severe spinal deformity underwent PVCR through a single approach. Multimodal intraoperative monitoring was used in all cases. Surgery included one stage posterior circumferential resection of one vertebral body along with the adjoining intervertebral discs and removal of all posterior elements. A transpedicular screw-rod system was used for correction and stabilisation. Fusion was strictly limited to the resection site, allowing for later conversion into a growing rod construct at the remaining spine, if necessary. Relevant data were extracted retrospectively from patient charts and long spine radiographs.
The mean operation time was 500 (range 463–541) min, with an estimated blood loss of 762 (range 600–1,050) ml. Mean follow-up time was 6.3 (range 3.5–12.4) years. After PVCR, the mean Cobb angle for scoliosis was reduced from 69° (range 50–99°) to 29° (5–44°) and the sagittal curvature (kyphosis) from 126° (87–151°) to 61° (47–75°). The mean correction of scoliosis was 57 % (18–92°) and of kyphosis, 51 % (44–62°). There were no spinal cord-related complications. In three patients, spinal instrumentation for growth guidance (fusion less growing rod technique) was applied. Two patients had complications: one patient had a complication of anesthesia, halo pin failure, and revision surgery with extension of the instrumentation cranially due to loss of correction; the second patient had a postoperative infection, which required plastic reconstructive measures.
PVCR appears to be an effective technique to treat severe EOSD. There are important differences in its use in young children when compared with older patients. In patients with EOSD, additional surgical procedures are often necessary during growth, and hence non-fusion instrumentation beyond the vertebral resection site is advantageous, as it permits spinal growth and the later addition of fusion.
Early onset spinal deformity; Early onset scoliosis; Posterior vertebral column resection; Growing rod instrumentation; Surgical technique; Multimodal intraoperative monitoring; Surgical complications; Paediatric spine surgery
Introduction. Spinal scoliosis and kyphosis in elderly people sometimes cause severe low back pain. Surgical methods such as osteotomy are useful for correcting the deformity. However, complications during and after surgery are associated with the osteotomy procedure. In particular, it is difficult to manage deformity correction surgery for patients with Parkinson's disease. Here, we present two cases of combined anterior and posterior surgery for deformity in patients with adult scoliosis and kyphosis due to Parkinson's disease. Case Presentation. Two 70-year-old women had spinal scoliosis and kyphosis due to Parkinson's disease. They had severe low back pain, and conservative treatment was not effective for the pain. Surgery was planned to correct the deformity in both patients. We performed combined posterior and anterior correction surgery. At first, posterior fusions were performed from T4 to the ilium using pedicle screws. Next, cages and autograft from the iliac crest were used in anterior lumbar surgery. The patients became symptom free after surgery. Bony fusion was observed 12 months after surgery. Conclusions. Combined posterior and anterior fusion surgery is effective for patients who show scoliosis and kyphosis deformity, and symptomatic low back pain due to Parkinson's disease.
Hepatic hemangiomas are congenital vascular malformations, considered the most common benign mesenchymal hepatic tumors, composed of masses of blood vessels that are atypical or irregular in arrangement and size. Hepatic hemangiomas can be divided into two major groups: capillary hemangiomas and cavernous hemangiomas These tumors most frequently affect females (80%) and adults in their fourth and fifth decades of life. Most cases are asymptomatic although a few patients may present with a wide variety of clinical symptoms, with spontaneous or traumatic rupture being the most severe complication. In cases of spontaneous rupture, clinical manifestations consist of sudden abdominal pain, and anemia secondary to a haemoperitoneum. Disseminated intravascular coagulopathy can also occur. Haemodynamic instability and signs of hypovolemic shock appear in about one third of cases. As the size of the hemangioma increases, so does the chance of rupture. Imaging studies used in the diagnosis of hepatic hemangiomas include ultrasonography, dynamic contrast-enchanced computed tomography scanning, magnetic resonance imaging, hepatic arteriography, digital subtraction angiography, and nuclear medicine studies. In most cases hepatic hemangiomas are asymptomatic and should be followed up by means of periodic radiological examination. Surgery should be restricted to specific situations. Absolute indications for surgery are spontaneous or traumatic rupture with hemoperitoneum, intratumoral bleeding and consumptive coagulopathy (Kassabach-Merrit syndrome). In a patient presenting with acute abdominal pain due to unknown abdominal disease, spontaneous rupture of a hepatic tumor such as a hemangioma should be considered as a rare differential diagnosis.
Hepatic hemangioma; Giant hepatic hemangioma; Liver tumor; Spontaneous rupture; Surgery
To report the first case of surgical treatment for severe kyphoscoliosis associated with respiratory disorder in a patient with congenital neuromuscular disease with uniform type 1 fibers (CNMDU1), including management of the possible onset of malignant hyperthermia (MH) in general anesthesia.
Summary of background data
CNMDU1 is rare among congenital neuromuscular diseases, and surgery for spinal deformity in CNMDU1 has not been described. Onset of MH in general anesthesia is a concern in this disease.
A 13-year-old female with motor retardation, suspected myopathy, and severe spinal deformity was followed at another pediatric hospital before referral to Meijo Hospital. Symptoms at the initial consultation were mild general muscular weakness and muscular atrophy. The rib hump was 60° and trunk balance was poor. The tendon reflex showed hyporeflexia, and blood tests were normal. Vital capacity was 0.69 L and forced expiratory volume percentage in 1 s was 75.5%, showing a restrictive and obstructive ventilatory defect. A plain radiograph showed severe kyphoscoliosis with thoracic scoliosis of 130° (T5–L1) and thoracic kyphosis of 110° (T2–T12) with almost no flexibility in bending or traction film.
After preoperative halo traction for 2 months, one-stage anterior and posterior correction and fusion from T2 to L3 was conducted. MH did not occur, but recovery of respiratory function required 8 days by intubation after surgery. Postoperatively, thoracic kyphosis improved to 25° and thoracic scoliosis was 66° (correction rate: 49%). Pathological results of an intraoperative muscle biopsy from the paraspinal muscles confirmed the diagnosis of CNMDU1. At 6 years after surgery, the patient has no problems in daily life and no respiratory difficulty.
Spinal deformity in CNMDU1 has a risk of severe progression, which makes early diagnosis by biopsy important. The surgery may be recommended before severe progression of spinal deformity and respiratory disorder. Perioperative MH is a concern, but can be managed by appropriate procedures.
Congenital neuromuscular disease with uniform type 1 fibers; Congenital neuromuscular disease; Severe kyphoscoliosis; Surgical correction; Malignant hyperthermia
Sotos syndrome is caused by a gene deletion with an autosomal dominant pattern of inheritance. Cerebral gigantism, hypotonia and joint hyperextensibility are characteristic features of this syndrome. A percentage of these patients develop progressive scoliosis early in life. In the literature, few studies on the evolution of scoliosis in Sotos syndrome have been published. We retrospectively evaluated eight patients diagnosed with Sotos syndrome and scoliosis treated at the Garrahan Children Hospital between 1988 and March 2009. Clinical charts and imaging studies were assessed. Eight patients (19%) presented with scoliosis and seven of them (87.5%) required surgical treatment. The mean follow-up was 9.5 years (range 3–18). Mean age at first consultation was 5.2 years (range 1.1–11.2). Mean Cobb angle for scoliosis at first consultation was 34.3° (range 20°–42°) and the mean Cobb angle for kyphosis was 45.6° (range 30°–90°). Mean age at surgery was 11.2 years (range 3.7–18.10). The surgical procedures performed were instrumented posterior arthrodesis, alone or combined with anterior arthrodesis, instrumented anterior arthrodesis, while one patient is currently in treatment with growing rods. Preoperative mean Cobb angle for scoliosis was 72.3° (range 54°–130°) and for kyphosis was 59.8° (range 30°–108°); postoperative mean Cobb angle for scoliosis was 45.5° (range 6°–90°) and for kyphosis was 40.2° (range 30°–80°). There were three early complications (pleural effusion in two cases and death due to sepsis in one) and two late complications (kyphosis above the instrumentation area and dislodgement of the proximal hooks). Incidence of scoliosis in Sotos syndrome is high and thus close monitoring of patients with Sotos syndrome during growth is important for early detection of this entity. Joint hyperextensibility and hypotonia that are characteristic of the syndrome should be considered at the moment of surgery to avoid short fusions.
Cerebral gigantism; Scoliosis deformity
Cavernous hemangiomas of the adrenal gland are rare. We report a case of a cavernous hemangioma of the adrenal gland presenting as an adrenal incidentaloma suspicious for adrenal cortical carcinoma (ACC).
PRESENTATION OF CASE
A 78 year old woman was admitted after a fall. Abdominal computed tomography revealed a large right adrenal lesion with features suspicious for adrenal cortical carcinoma (5.4 cm × 3.3 cm, unilateral, tumor calcifications, average Hounsfield units 55). The tumor was removed intact by a laparoscopic approach and pathology revealed a cavernous hemangioma of the adrenal gland.
Adrenal incidentalomas are found in up to 10% of patients undergoing abdominal imaging. Differential diagnosis includes both benign and malignant lesions. Guidelines for removal of adrenal incidentalomas recommend surgery based on functional status, size, and presence of concerning features on diagnostic imaging. Cavernous hemangiomas are rare, benign vascular malformations which can be challenging to distinguish pre-operatively from malignant lesions such as ACC.
Cavernous hemangiomas of the adrenal gland are exceedingly rare. These benign tumors have imaging features which may be suggestive of adrenal cortical carcinoma. The treatment of choice is surgical excision due the difficulty of excluding malignancy.
Adrenal; Cavernous hemangioma; Adrenal cortical carcinoma
The traditional surgical treatment of severe spinal deformities, both in adult and pediatric patients, consisted of a 360° approach. Posterior-based spinal osteotomy has recently been reported as a useful and safe technique in maximizing kyphosis and/or kyphoscoliosis correction. It obviates the deleterious effects of an anterior approach and can increase the magnitude of correction both in the coronal and sagittal plane. There are few reports in the literature focusing on the surgical treatment of severe spinal deformities in large pediatric-only series (age <16 years old) by means of a posterior-based spinal osteotomy, with no consistent results on the use of a single posterior-based thoracic pedicle subtraction osteotomy in the treatment of such challenging group of patients. The purpose of the present study was to review our operative experience with pediatric patients undergoing a single level PSO for the correction of thoracic kyphosis/kyphoscoliosis in the region of the spinal cord (T12 and cephalad), and determine the safety and efficacy of posterior thoracic pedicle subtraction osteotomy (PSO) in the treatment of severe pediatric deformities. A retrospective review was performed on 12 consecutive pediatric patients (6 F, 6 M) treated by means of a posterior thoracic PSO between 2002 and 2006 in a single Institution. Average age at surgery was 12.6 years (range, 9–16), whereas the deformity was due to a severe juvenile idiopathic scoliosis in seven cases (average preoperative main thoracic 113°; 90–135); an infantile idiopathic scoliosis in two cases (preoperative main thoracic of 95° and 105°, respectively); a post-laminectomy kypho-scoliosis of 95° (for a intra-medullar ependimoma); an angular kypho-scoliosis due to a spondylo-epiphisary dysplasia (already operated on four times); and a sharp congenital kypho-scoliosis (already operated on by means of a anterior–posterior in situ fusion). In all patients a pedicle screws instrumentation was used, under continuous intra-operative neuromonitoring (SSEP, NMEP, EMG). At an average follow-up of 2.4 years (range, 2–6) the main thoracic curve showed a mean correction of 61°, or a 62.3% (range, 55–70%), with an average thoracic kyphosis of 38.5° (range, 30°–45°), for an overall correction of 65% (range, 60–72%). Mean estimated intra-operative blood loss accounted 19.3 cc/kg (range, 7.7–27.27). In a single case (a post-laminectomy kypho-scoliosis) a complete loss of NMEP occurred, promptly assessed by loosening of the initial correction, with a final negative wake-up test. No permanent neurologic damage, or instrumentation related complications, were observed. According to our experience, posterior-based thoracic pedicle subtraction osteotomies represent a valuable tool in the surgical treatment of severe pediatric spinal deformities, even in revision cases. A dramatic correction of both the coronal and sagittal profile may be achieved. Mandatory the use of a pedicle screws-only instrumentation and a continuous intra-operative neuromonitoring to obviate catastrophic neurologic complications.
Thoracic pedicle subtraction osteotomy; Severe pediatric spinal deformities; Surgical treatment; Posterior-instrumented fusion only; Thoracic pedicle screws
Treatment of hemangioma/vascular tumors emphasize minimal invasion which require sophisticated facilities. This study reports the role of surgery in the management of symptomatic, and hemangioma which failed to respond to other modalities of treatment in resource-limited subregion.
A six-year (2004-2009) prospective study on the challenges and outcome of children referred for surgical management of hemangioma/vascular tumors was undertaken at the University of Benin Teaching Hospital, Nigeria.
Sixty-three children aged between a day and six years (median 5 years) comprising 38 males and 25 females (ratio 1.5:1) were managed. Upper limbs involvement, 18 (28.6%), and face/neck, 12 (19.1%), were most common and were present at birth in 27 (42.9%) babies, appeared between 2-3 weeks in 32 (50.8%), and after six months in 4 (6.3%). Cavernous hemangioma in 19 (30.2%) children, mixed cavernous/strawberry in 31 (49.2%) and strawberry in 13 (20.6), were the major types that ranged from spot-like to extensive huge lesions measuring 12×15 cm in diameter. Failure of 46 (65.1%) cases to respond to non operative treatment, ulceration in 3 (4.8%), infection in 5 (7.9%) and hemorrhage in 2 (3.2%) were indications for surgical intervention. Surgical options included complete excision and primary wound closure in 34 (54%) children, immediate skin graft after complete excision in 10 (15.9%), injection sclerotherapy in 2 (3.2%), serial ligation of feeder vessels in 2 (3.2%), and conservative treatment in 5 (7.9%). Excision and primary wound closure gave better outcome compared with others (P<0.0001). No mortality was recorded on 1-6 years follow-up but ugly scar, 8 (12.7%) and limb deformity, 3 (4.8%) were problems.
Surgical excision and primary wound closure gave good outcome which could be employed in complicated and hemangioma which failed to respond to other treatment in regions with limited resources.
Hemangioma; Cavernous Hemangioma; Strawberry Hemangiomas; Children
A variety of treatments has been described in the literature for the treatment of HV. We report the results of early surgical anterior instrumented fusion with partial preservation of the HV and posterior non-instrumented fusion in the treatment of progressive congenital scoliosis in children below the age of six. Between 1996 and 2006, 31 consecutive patients with 33 lateral HV and progressive scoliosis underwent short segment fusions. Mean age at surgery was 2 years and 10 months. Mean follow-up period was 6.1 years. The major scoliotic curve improved from 41° preoperatively to 17° on follow-up. Preoperative segmental Cobb angle averaging 39° was corrected to 15° after surgery, being 15° at the last follow-up (62% of improvement). Compensatory cranial and caudal curves corrected by 47 and 45%, respectively. The angle of segmental kyphosis averaged 16° before surgery, 11° after surgery, and 11° at follow-up. There were two wound infections requiring surgical debridment, one intraoperative fracture of the vertebral body and one case lost correction due to implant failure. All went on to stable bony union. There were no neurological complications. Early diagnosis and early and aggressive surgical treatment are mandatory for a successful treatment of congenital scoliosis and prevention of the development of secondary compensatory deformities. Anterior instrumentation is a safe and effective technique capable of transmitting a high amount of convex compression allowing short segment fusion, which is of great importance in the growing spine.
Hemivertebra; Congenital scoliosis; Fully segmented hemivertebra; Congenital spine deformity; Anterior instrumented fusion
Correction of adolescent idiopathic scoliosis (AIS) has been reported with various systems. All-screw constructs are currently the most popular, but they have been associated with a significant decrease in thoracic kyphosis, with a potential risk of junctional kyphosis, not observed with hybrid constructs in the literature. In addition, it is important to weigh potential advantages of pedicle screw fixation against risks specific to its use. Because hybrid constructs are associated with a lower risk of complications and better sagittal correction than all-screw constructs, at present we use lumbar pedicle screws combined with a new sublaminar connection to the spine (Universal Clamps) at thoracic levels. The purpose of this study was to determine the efficacy and safety of the Universal Clamp (UC) posteromedial translation technique for correction of AIS. Seventy-five consecutive patients underwent posterior spinal fusion and hybrid instrumentation for progressive AIS. Correction was performed at the thoracic level using posteromedial translation. At the lumbar level, correction was performed using in situ contouring and compression/distractions maneuvers. A minimum 2-year follow-up was required. Medical data and radiographs were prospectively analyzed and compared using a paired t test. The average age at surgery was 15 years and 4 months (±19 months). The average number of levels fused was 12 ± 1.6. The mean follow-up was 30 ± 5 months. The average preoperative Cobb angle of the major curve was 60° ± 20°. The immediate postoperative major curve correction averaged 66 ± 13%. The average loss of correction of the major curve between the early postoperative assessment and latest follow-up was 3.5° ± 1.4°. The mean Cincinnati correction index was 1.7 ± 0.8 postoperatively, and 1.57 ± 1 at last follow up. The mean rotation of the apical vertebra was corrected from 23.3° ± 9° preoperatively to 7.3° ± 5° at last follow up (69% improvement, P < 0.0001). In the sagittal plane, the mean thoracic kyphosis improved from 23.8° ± 14.2° preoperatively to 32.3° ± 7.3° at last follow up. For the 68 patients who had a normokyphotic or a hypokyphotic sagittal modifier, thoracic kyphosis increased from 20.5° ± 9.9° to 31.8° ± 7.4°, corresponding to a mean kyphosis correction of 55% at last follow up. No intraoperative complication occurred and none of the patients developed proximal junctional kyphosis during the follow up. The principal limitation of the UC technique was the rate of proximal posterior prominence (14.6%), leading us to recommend the use of conventional claws at the upper extremity of the construct. The technique was safe, and reduced operative time, radiation exposure, and blood loss. While achieving correction of deformity in the coronal and axial planes equivalent to the best reported results of all-screw or previous hybrid constructs, the UC hybrid technique appears to provide superior correction in the sagittal plane. The excellent outcome in all three planes was maintained at 2 year follow up.
Adolescent idiopathic scoliosis; Sagittal balance; Correction; Instrumentation
Outcomes for minimally invasive scoliosis correction surgery have been reported for mild adult scoliosis. Larger curves historically have been treated with open surgical procedures including facet resections or posterior column osteotomies, which have been associated with high-volume blood loss. Further, minimally invasive techniques have been largely reported in the setting of degenerative scoliosis.
We describe the effects of circumferential minimally invasive surgery (cMIS) for moderate to severe scoliosis in terms of (1) operative time and blood loss, (2) overall health and disease-specific patient-reported outcomes, (3) deformity correction and fusion rate, and (4) frequency and types of complications.
Between January 2007 and January 2012, we performed 50 cMIS adult idiopathic scoliosis corrections in patients with a Cobb angle of greater than 30° but less than 75° who did not have prior thoracolumbar fusion surgery; this series represented all patients we treated surgically during that time meeting those indications. Our general indications for this approach during that period were increasing back pain unresponsive to nonoperative therapy with cosmetic and radiographic worsening of curves. Surgical times and estimated blood loss were recorded. Functional clinical outcomes including VAS pain score, Oswestry Disability Index (ODI), and SF-36 were recorded preoperatively and postoperatively. Patients’ deformity correction was assessed on pre- and postoperative 36-inch (91-cm) standing films and fusion was assessed on CT scan. Minimum followup was 24 months (mean, 48 months; range, 24–77 months).
Mean blood loss was 613 mL for one-stage surgery and 763 mL for two-stage surgery. Mean operative time was 351 minutes for one-stage surgery and 482 minutes for two-stage surgery. At last followup, mean VAS and ODI scores decreased from 5.7 and 44 preoperatively to 2.9 and 22 (p < 0.001 and 0.03, respectively) and mean SF-36 score increased from 48 preoperatively to 74 (p = 0.026). Mean Cobb angle and sagittal vertical axis decreased from 42° and 51 mm preoperatively to 16° and 27 mm postoperatively (both p < 0.001). An 88% fusion rate was confirmed on CT scan. Perioperative complications occurred in 11 of the 50 patients (22%), with delayed complications needing further surgery in 10 more patients at last followup.
cMIS provides for good clinical and radiographic outcomes for moderate (30°–75°) adult idiopathic scoliosis. Patients undergoing cMIS should be carefully selected to avoid fixed, rigid deformities and a preoperative sagittal vertical axis of greater than 10 cm; surgeons should consider alternative techniques in those patients.
Level of Evidence
Level IV, therapeutic study. See Instructions for Authors for a complete description of levels of evidence.
A review of the current literature reveals no systematic analyses of the results of surgical correction of spinal deformity after pediatric organ transplantation. We therefore evaluated clinical and radiographic outcomes of spinal deformity correction after solid organ transplantation in childhood and adolescence. All 211 cases of heart, liver, and kidney transplantations performed in children in our country were reviewed. Six patients had undergone surgical correction of spinal deformity at a mean age 14.6 (range 12–17) years. Clinical data of the patients were evaluated. Radiographs of the whole spine were taken preoperatively, immediately after, at 2-year, and final follow-up visits. The Scoliosis Research Society (SRS) questionnaire was completed and a physical examination was performed at the final follow-up visit. The mean follow-up after spinal surgery was 4.9 years (range 2–7.6 years). Four patients developed scoliosis after organ transplantation without any evidence of scoliosis prior to organ transplantation. One patient with congenital scoliosis was operated on after kidney transplantation. One boy had osteoporosis and severe local kyphosis due to vertebral compression fractures. Four patients underwent anterior and posterior surgery, two posterior only. The mean preoperative Cobb angle of the thoracic curve was 54° (range 42–69°) in the patients with scoliosis. The postoperative values were 30° (26–38°) immediately after instrumentation and 39° (34–42°) at the final follow-up visit. The patient with vertebral compression fractures and progressive kyphosis had 70° curve before surgery, 23° immediately after the operation, and 60° at the final check up. The mean total score on the SRS questionnaire was 95.5 (range 90–101). There is a relatively high incidence (2.8%) of spinal deformities needing operative treatment after solid organ transplantation. Possible etiologies for spinal deformities are growth disturbance and muscle weakness due to the basic disease. The other important factors are related to immunosupressive medication, especially glucocorticoids needed after transplantation. Primary correction of these deformities was satisfactory, but during follow-up, a certain amount of recurrence of the curves was evident. Poor bone quality may explain some of the loss of correction.
Spinal deformity; Solid organ transplantation; Spinal surgery
In view of the limited data available on the conservative treatment of patients with congenital scoliosis (CS), early surgery is suggested in mild cases with formation failures. Patients with segmentation failures will not benefit from conservative treatment. The purpose of this review is to identify the mid- or long-term results of spinal fusion surgery in patients with congenital scoliosis.
Retrospective and prospective studies were included, reporting on the outcome of surgery in patients with congenital scoliosis. Studies concerning a small numbers of cases treated conservatively were included too. We analyzed mid-term (5 to 7 years) and long-term results (7 years or more), both as regards the maintenance of the correction of scoliosis and the safety of instrumentation, the early and late complications of surgery and their effect on quality of life.
A small number of studies of surgically treated patients were found, contained follow-up periods of 4-6 years that in the most cases, skeletal maturity was not yet reached, and few with follow-up of 36-44 years. The results of bracing in children with congenital scoliosis, mainly in cases with failure of formation, were also studied.
Spinal surgery in patients with congenital scoliosis is regarded in short as a safe procedure and should be performed. On the other hand, early and late complications are also described, concerning not only intraoperative and immediate postoperative problems, but also the safety and efficacy of the spinal instrumentation and the possibility of developing neurological disorders and the long-term effect these may have on both lung function and the quality of life of children.
Few cases indicate the long-term results of surgical techniques, in the natural progression of scoliosis. Similarly, few cases have been reported on the influence of conservative treatment.
In conclusion, patients with segmentation failures should be treated surgically early, according to the rate of deformity formation and certainly before the pubertal growth spurt to try to avoid cor- pulmonale, even though there is lack of evidence for that in the long-term. Furthermore, in patients with formation failures, further investigation is needed to document where a conservative approach would be necessary.
A retrospective study of 21 patients with idiopathic scoliosis who underwent endoscopic thoracoplasty was done. The objective of the study was to report and assess the morbidity and mid term outcomes of video-assisted thoracoplasty in idiopathic scoliosis. Patients with idiopathic scoliosis often present cosmetic complaints due to their rib deformity. This deformity may still exist after surgical correction of the main scoliotic curve. Endoscopic thoracoplasty has been reported as a safe method in limited cases of idiopathic scoliosis. Between 2002 and 2004, 21 patients underwent endoscopic anterior release and thoracoplasty for significant rib hump deformity associated with idiopathic scoliosis. Patients were operated on lateral position, with two endoscopic ports. Anterior release and rib resection were performed during the first stage, and instrumented posterior fusion was performed in a second stage. Patients were evaluated preoperatively, 1 week after surgery, 6 months after surgery and at their most recent follow-up with clinical and radiological measurement of the rib deformity. The mean age at surgery was 14.9 years old (range 13–17 years). The average Cobb’s angle of the main scoliotic curve was 70° (range 60°–85°). Average follow-up was 25 months (range 23–32 months). The mean number of resected ribs was five ribs (range 4–7) and the mean length of the resected rib was 4.2 cm (range 2.2–7 cm). Average operating time of endoscopic thoracoplasty (including anterior release) was 65 min (range 45–108 min). The mean preoperative height of rib hump deformity was 3.6 cm (range 2.5–5.5 cm). It was reduced to 1.5 cm at most recent follow-up. There was no significant thoracic pain necessitating medication postoperatively. No complications related to endoscopic anterior release and rib hump resection occurred in the series. Endoscopic thoracoplasty is a safe and reliable technique in idiopathic scoliosis. If indicated, the anterior release can be performed with video-assistance and the thoracoplasty can be performed on the same stage.
Scoliosis; Thoracoscopy; Thoracic deformity
Cavernous hemangiomas were first reported in 1929 by Globus and Doshay, and are defined as benign vascular structures developed between the neural tissues occurring in the central nervous system, consisting of a dilated vascular bed. Cavernous hemangiomas comprise nearly 5-12% of all spinal vascular malformations; however, existence in the epidural space without bone involvement is rare. Only 4% of all cavernous hemangiomas (0.22/1.000.000) are purely epidural cavernous hemangiomas. In this case report, we removed a hemorrhagic thoracic mass presenting with progressive neurological deficits in a 55-year-old male patient. We found this case to be appropriate for presentation due to the rare occurrence of this type of cavernous hemangioma.
Cavernous hemangioma; Extraosseous-epidural; Thoracic vertebrae; Hemangioma
Radiographic retrospective study of a consecutive series of 76 patients with adolescent idiopathic scoliosis (AIS) undergoing posterior only surgical correction and fusion.
To evaluate the sagittal profile changes in a population of adolescent idiopathic scoliosis after posterior only surgical correction.
Summary of background data
Although the relationship between pelvic indexes and sagittal profile is well known, little has been published about the sagittal profile changes after posterior surgery in adolescent idiopathic scoliosis.
Radiological data of 76 AIS patients were analyzed by an independent observer to compare pelvic indexes and spino-pelvic parameters before and at the last follow-up after surgical posterior correction. All patients underwent a posterior only surgical correction by using different anchor techniques (all screws or hybrid construct), but the same derotation correction maneuver (C-D technique). The collected data were analyzed, on AP and LL radiographic views of the entire spine in the upright position, from the same independent observer and using the same Impax software analysis. We collected for each patient on latero-lateral X-rays the following data: pelvic incidence (PI), pelvic tilt (PT), sacral slope (SS), lumbar lordosis (LL), thoracic kyphosis (TK), C7 plumb line (C7PL) and spino-sacral angle (SSA). All data were analyzed using a D’Agostino–Pearson normality test and the comparison between the groups was performed with a student’s t test.
The mean pelvic incidence (PI) of the cohort was 48.89° (±11.24), with a mean Cobb angle for the main curve of 60.13° (±13.6). The mean value of residual scoliosis after surgery was 28.18° (±13.22) with an average improvement of the curve in the frontal plane of 53.2 %. The amount of curve correction of the primary scoliosis curve was statistically significant (p < 0.0001). In the evaluation of the whole group after surgery, we observed an increasing amount of PT (average delta value 2.38°) with a statistical significance (p = 0.0034). If we compare the mean ideal PT value (11.09°) with the pre- and post-operative mean true PT values, we found statistical significance only for the post-operative difference (p = 0.0014). In the general assessment, C7PL seems to remain stable after surgery, and in particular it remains negative. In Lenke 1 group, there was a mean PI value of 50.54° (±11.45) which is higher than the one reported in the global assessment. Also in this subgroup, we observed a reduction in the mean SS values, with consequent increase in the PT values, as in the general assessment. The C7PL tends to move posteriorly after surgery and this difference is statistically significant. In Lenke 1 group we found a strong statistical significance between pre- and post-surgery data for the Cobb primary curve and for the C7PL, which continues to remain negative. The C7PL remains relatively stable only in the normokyphotic group, while it tends to move behind in the other three groups (Lenke 3, hyperkyphosis and hypokyphosis).
In our series of 76 adolescent affected by AIS, we reported mean PI values of 48.9° with a mean pre-operative PT of 11.51°. After surgery we observed an increase in the PT mean value, about three degrees higher than the ideal value, meaning that there was some compensatory mechanism. Patients affected by AIS showed a slight posterior imbalance and the intervention of scoliosis correction seems to cause a slight further posterior imbalance, especially in Lenke 1 type curves and in patients with hypokyphosis. The clinical significance of this slight imbalance must be carefully evaluated. Further studies are necessary to better establish which could be the best surgical strategy to obtain an optimal spinal sagittal balance.
Sagittal balance; Adolescent idiopathic scoliosis; Pelvic incidence; Sacral slope; C7 plumb line
The role of posterior correction and fusion in thoracolumbar and lumbar scoliosis as well as pedicle screw instrumentation in scoliosis surgery are matters of debate. Our hypothesis was that in lumbar and thoracolumbar scoliosis, segmental pedicle screw instrumentation is safe and enables a good frontal and sagittal plane correction with a fusion length comparable to anterior instrumentation. In a prospective clinical trial, 12 consecutive patients with idiopathic thoracolumbar or lumbar scolioses of between 40° and 60° Cobb angle underwent segmental pedicle screw instrumentation. Minimum follow-up was 4 years (range 48– 60 months). Fusion length was defined according to the rules for Zielke instrumentation, normally ranging between the end vertebrae of the major curve. Radiometric analysis included coronal and sagittal plane correction. Additionally, the accuracy of pedicle screw placement was measured by use of postoperative computed tomographic scans. Major curve correction averaged 64.6%, with a loss of correction of 3°. The tilt angle was corrected by 67.0%, the compensatory thoracic curve corrected spontaneously according to the flexibility on the preoperative bending films, and led to a satisfactory frontal balance in all cases. Average fusion length was the same as that of the major curve. Pathological thoracolumbar kyphosis was completely corrected in all but one case. One patient required surgical revision with extension of the fusion to the midthoracic spine due to a painful junctional kyphosis. Eighty-five of 104 screws were graded “within the pedicle”, 10 screws had penetrated laterally, 5 screws bilaterally and 4 screws medially. No neurological complications were noted. In conclusion, despite the limited number of patients, this study shows that segmental pedicle screw instrumentation is a safe and effective procedure in the surgical correction of both frontal and sagittal plane deformity in thoracolumbar and lumbar scoliosis of less than 60°, with a short fusion length, comparable to anterior fusion techniques, and minimal loss of correction.
Key words Idiopathic scoliosis; Surgery; Pedicle screw; Posterior instrumentation
Patient: Male, 38
Final Diagnosis: Cavernous hemangioma
Symptoms: Headache • parietal mass
Clinical Procedure: —
A rare disease
Bone hemangioma is a vascular hemartoma of bone structures. In general, this pathology is detected on incidentally investigated patients’ films. Bone hemangioma is most commonly seen in 4th decade of life and the male/female ratio is 1/1.5. The locations of these lesions are commonly long bones of the vertebral column and the skull.
Primary bone hemangiomas constitute less than 1% of all bone tumors, and they are seen at 0.2% in the calvarial region.
Cases with this pathology are seen on incidental radiological evaluations. We report the case of a 38-year-old man with localized headache and a palpable mass in the left parietal region, admitted and operated on after cranial CT and MRI. Pathology investigation revealed a cavernous hemangioma.
We suspected that in our case head trauma may have been the cause of cavernous hemangioma in the calvarial region, because cavernous hemangiomas are rarely located there. Localized headache and minor discomfort can be seen with this pathology. The best treatment for cavernous hemangiomas is the removal of the mass within the limits of safe surgery.
headache; trauma; cavernous hemangioma
Previous studies report an increase in thoracic kyphosis after anterior approaches and a flattening of sagittal contours following posterior approaches. Difficulties with measuring sagittal parameters on radiographs are avoided with reformatted sagittal CT reconstructions due to the superior endplate clarity afforded by this imaging modality.
A prospective study of 30 Lenke 1 adolescent idiopathic scoliosis (AIS) patients receiving selective thoracoscopic anterior spinal fusion (TASF) was performed. Participants had ethically approved low dose CT scans at minimum 24 months after surgery in addition to their standard care following surgery. The change in sagittal contours on supine CT was compared to standing radiographic measurements of the same patients and with previous studies. Inter-observer variability was assessed as well as whether hypokyphotic and normokyphotic patient groups responded differently to the thoracoscopic anterior approach.
Mean T5-12 kyphosis Cobb angle increased by 11.8 degrees and lumbar lordosis increased by 5.9 degrees on standing radiographs two years after surgery. By comparison, CT measurements of kyphosis and lordosis increased by 12.3 degrees and 7.0 degrees respectively. 95% confidence intervals for inter-observer variability of sagittal contour measurements on supine CT ranged between 5-8 degrees. TASF had a slightly greater corrective effect on patients who were hypokyphotic before surgery compared with those who were normokyphotic.
Restoration of sagittal profile is an important goal of scoliosis surgery, but reliable measurement with radiographs suffers from poor endplate clarity. TASF significantly improves thoracic kyphosis and lumbar lordosis while preserving proximal and distal junctional alignment in thoracic AIS patients. Supine CT allows greater endplate clarity for sagittal Cobb measurements and linear relationships were found between supine CT and standing radiographic measurements. In this study, improvements in sagittal kyphosis and lordosis following surgery were in agreement with prior anterior surgery studies, and add to the current evidence suggesting that anterior correction is more capable than posterior approaches of addressing the sagittal component of both the instrumented and adjacent non instrumented segments following surgical correction of progressive Lenke 1 idiopathic scoliosis.
Thoracoscopic anterior spinal fusion; Anterior spinal fusion; Adolescent idiopathic scoliosis; Sagittal profile; Computed tomography (CT); Thoracic kyphosis; Lumbar lordosis
Hepatic and splenic hemangiomas are common benign tumors that mainly affect female patients. Giant splenic hemangiomas are extremely rare, especially when correlated with multiple hepatic hemangiomas. Pathogenetic mechanisms between hemangiomas and oral contraceptives, as well as therapeutic approaches, are analyzed in this case report, in particular for the management of synchronous splenic and hepatic hemangiomas.
We report here a 42-year-old woman with a giant splenic hemangioma, multiple hepatic hemangiomas and a history of oral estrogen intake for many years. At first it was difficult to determine the organ from which the giant hemangioma originated. Angiography proved extremely helpful in tracing its origin in the spleen. Hematomas in the giant hemangioma posed a significant threat of rupture and catastrophic hemorrhage. We left the small hepatic hemangiomas in place, and removed the spleen along with the giant splenic hemangioma.
Diagnostic pitfalls in the determination of the origin of this giant hemangioma, attribution of its origin to the spleen angiographically, the unusual co-existence of the giant splenic hemangioma with multiple hepatic ones, and the potential threat of rupture of the giant hemangioma are some of the highlights of this case report. Estrogen administration represents a pathogenic factor that has been associated with hemangiomas in solid organs of the abdominal cavity. The therapeutic dilemma between resection and embolization of giant hemangiomas is another point of discussion in this case report. Splenectomy for the giant splenic hemangioma eliminates the risk of rupture and malignant degeneration, whereas observation for the small hepatic ones (<4 cm) was the preferable therapeutic strategy in our patient.