Subaxial cervical juxtafacet cysts: single institution surgical experience and literature review

Objectives

Juxtafacet cysts (JFCs) of the subaxial cervical spine are rare causes of neurological deficits. Their imaging characteristics, relationship to segmental instability, and potential for inducing acute symptomatic deterioration have only been described in a few case reports and small case series. The objective of the current study was to review the surgical experience at our center and across the literature to better define these variables.

Methods

A single-institution, multisurgeon series of 12 consecutive patients (mean age 63.4 years, range 52–83 years) harboring 14 JFCs treated across 9 years was retrospectively reviewed. Clinical history, neurological status, preoperative imaging, operative findings, pathology, and postoperative outcomes were obtained from medical records. The mean follow up was 9.2?±?7.8 months. A literature review identified 35 studies with 89 previously reported cases of surgically treated subaxial cervical JFCs.

Results

Consistent with previously reported cases, most JFCs in our series involved the C7/T1 level. Nine patients reported axial neck pain, 12 patients had radicular symptoms, four patients had myelopathy, and one patient experienced rapid neurological decline attributable to cystic hemorrhage. Cyst expansion without hemorrhage caused subacute deterioration in one patient. All patients experienced sensory and/or motor improvement following surgical decompression. Preoperative axial neck pain improved in eight of nine patients (89 %). Seven out of 12 patients (58 %) underwent fusion either at the time of decompression (six patients) or at a delayed timepoint within the follow-up period (one patient). Prior history of cervical instrumentation, hypermobility on dynamic imaging, and other risk factors for segmental instability were more common in our series than in previous reports.

Conclusions

Our findings lead us to advocate for early decompression rather than prolonged conservative treatment, for pre- and postoperative dynamic imaging, and for fusion in selected cases as an initial surgical consideration.     

Synovial sarcoma of the larynx: case report and literature review

Synovial sarcoma is a rare mesenchymal malignancy which represents 8.5% of all soft tissue sarcomas and usually occurs in the lower extremities of young adults. Because the incidence in the head and neck region is very low, only 13 patients with endolaryngeal localisation have been reported so far. We present here a case of aryepiglottic synovial sarcoma. The tumour was completely resected, under suspension micro-laryngoscopy, using a CO2 laser. No recurrence was observed three years after the surgery. Complete surgical excision is the treatment of choice. The role of chemotherapy and radiotherapy is still debated. However, this tumour has a poor prognosis because of the occurrence of distant metastasis.     

Synovial sarcoma: a clinicopathological review

Summary We have reviewed 20 cases of synovial sarcoma. The main clinical and pathological features of these cases are outlined. Typically these tumours arise in the leg in young adults with a long history before presentation and diagnosis. Clinical and radiological findings are unhelpful in making an early diagnosis. The five year survival rate in this series was 43%. The best guide to prognosis was assessment of mitotic activity. A high index of suspicion is required if the diagnosis is not to be missed. Adequate wide excision of the tumour is the mainstay of treatment.

---

Résumé La révision de 20 cas de sarcome synovial a permis de dégager les principales caractéristiques cliniques et anatomopathologiques de cette affection. Dans les cas les plus typiques, ces tumeurs surviennent chez l'adulte jeune, au niveau des membres inférieurs. Elles évoluent longtemps avant d'entraîner une symptomatologie et de pouvoir être diagnostiquées. Les données cliniques et radiologiques ne permettent pas un diagnostic précoce. Dans cette série le taux de survie à 5 ans est de 43%. Le meilleur élément du pronostic est l'estimation de l'activité mitotique. La conclusion est qu'il faut être extrémement méfiant pour éviter de passer à côté du diagnostic. Une excision large de la tumeur, correctement effectuée, reste la base du traitement.

---

---

Reprint request to: S. A. Henderson     

Liver transplantation and neuroendocrine tumors: lessons from a single centre experience and from the literature review

Neuroendocrine tumor (NET) metastases represent at this moment the only accepted indication of liver transplantation (LT) for liver secondaries. Between 1984–2007, nine (1.1%) of 824 adult LTs were performed because of NET. There were five well differentiated functioning NETs (four carcinoids and one gastrinoma), three well differentiated non functioning NETs and one poorly differentiated NET. Indications for LT were an invalidating unresectable tumor (4×), and/or a diffuse tumor localization (3×) and/or a refractory hormonal syndrome (5×). Median post‐LT patient survival is 60.9 months (range 4.8–119). One‐, 3‐ and 5‐year actuarial survival rates are 88%, 77% and 33%; 1, 3 and 5 years disease free survival rates are 67%, 33% and 11%. Due to a more rigorous selection procedure, results improved since 2000; three out of five patients are alive disease‐free at 78, 84 and 96 months. Review of these series together with a review of the literature reveals that results of LT for this oncological condition can be improved using better selection criteria, adapted immunosuppression and neo‐ and adjuvant surgical as well as medical tretament. LT should be considered earlier in the therapeutic algorithm of selected NET patients as it is the only therapy that can offer a cure.     

Percutaneous nephrolithotomy in children: lessons learned in 5 years at a single institution

PURPOSE: We retrospectively compared morbidity and success rates in children who underwent percutaneous nephrolithotomy with 3 different sizes of instruments. MATERIALS AND METHODS: Percutaneous nephrolithotomy was performed using adult instruments via a 26Fr tract in 23 patients, using pediatric instruments via a 20Fr tract in 13 and using minimal access (14Fr) in 10. Percutaneous nephrolithotomy was performed using general anesthesia with parenteral antibiotic prophylaxis. Cystoscopy and retrograde pyelography were performed, and 5Fr to 6Fr soft ureteral catheters were placed during each procedure initially. Percutaneous access was established via fluoroscopic guidance with the patient in the prone position. Pneumatic, ultrasonic and holmium laser lithotriptors were used for in situ lithotripsy. A 14Fr nephrostomy catheter was placed at the end of the procedure in the 26Fr and 20Fr groups, and in the minimal access group the ureteral stent was left indwelling in cases of atraumatic procedure with no residual fragments. RESULTS: Mean patient age was 13.2, 5.9 and 6.3 years, respectively, in the 26Fr, 20Fr and minimal access groups (p=0.000). Sex distribution, previous surgery, stone size, surgery and fluoroscopy times, mean hemoglobin decrease and hospitalization time did not differ between the groups. However, blood transfusion rate was higher in groups 1 and 2. Stone-free rates were 69.5%, 80% and 90%, respectively, in groups 1 to 3. Overall, adjuvant shock wave lithotripsy treatment was needed in 3 children. CONCLUSIONS: Smaller instrument size to 20Fr did not significantly increase the operative time, and resulted in the same success rates as the adult sized devices. However, low blood transfusion rates were only reached in the minimal access percutaneous nephrolithotomy group.     

Supratentorial calcified pseudotumour: experience of a single institution and review of the literature

Background

Calcified pseudotumours (CPTs) are rarely found in the central nervous system (CNS). Information on supratentorial CPT is limited. We add our experience treating three supratentorial CPTs. Clinical presentation, pathological findings and clinical course are discussed, and a literature review is provided.

Method

Between September 2011 and December 2012, three patients (age range 33–60 years) were diagnosed with symptomatic supratentorial CPTs, for which they underwent resection. Their clinical, radiological, and surgical data were retrospectively retrieved from their medical records and reported.

Results

Symptoms at presentation included headache (n?=?1), blurred vision (n?=?1), and cognitive decline (n?=?1). One tumour was in the left frontal lobe very close to the convexity surface, and the other two were in the midline adjacent to the corpus callosum. Radiological features included a calcified mass on computerised tomography (n?=?3), a hypointense (n?=?2) or hyperintense (n?=?1) signal on T2 and a rim of enhancement with some non-homogenous lesional enhancement on T1-gadolinium (Gd) sequences on magnetic resonance imaging (n?=?2). Two patients underwent gross total resection and had an uneventful postoperative course. The third underwent partial removal due to the tumour’s adherence to adjacent vascular structures and suffered postoperative complications. On pathology, all lesions had low cellularity fibrovascular tissue mixed with lobules of a haematoxylinophilic, chondromyxoid-like matrix surrounded at its periphery by a variable layer of palisading spindle, epithelioid or multinucleated giant cells. All three CPTs had focal inflammatory infiltrates and variable degrees of metaplastic calcification and ossification.

Conclusions

Surgical removal is recommended for benign intracranial CPTs that are symptomatic, are associated with brain oedema, or show tumour growth. Stable and asymptomatic CPTs can be followed.     

Synovial sarcoma: a review of 33 cases

Thirty-three patients with synovial sarcoma were studied with respect to age, sex, site, clinical presentation, pathologic findings, and primary surgical treatment. Seventy percent of the patients were in the first three decades of life, and there was a 2:1 male predominance; the overall survival rate was 36%. There was a high local recurrence rate (64%) after simple local excision, and poor survival (27%). While wide local resection gave the best results (50% survival), it also carried a risk of high recurrence. Patients treated by amputation had a 42% survival rate. Thus, it can be concluded that adequate surgical treatment improved survival and reduced the recurrence rates. The lungs were the most common metastatic site, but lymph node metastasis occurred in 27% of the patients, often after tumor recurrence. Favorable prognostic factors included youth (patients younger than 20) and biphasic or epitheloid histologic types.     

Surgical management of symptomatic vertebral hemangiomas: a single institution experience and literature review

Vertebral hemangiomas (VHs), formed from a vascular proliferation in bone marrow spaces limited by bone trabeculae, are the most common benign tumors of the spine. While most VHs remain clinically quiescent and often only require surveillance, rarely they may cause symptoms. They may exhibit active behaviors, including rapid proliferation, extending beyond the vertebral body, and invading the paravertebral and/or epidural space with possible compression of the spinal cord and/or nerve roots (“aggressive” VHs). An extensive list of treatment modalities is currently available, but the role of techniques such as embolization, radiotherapy, and vertebroplasty as adjuvants to surgery has not yet been elucidated. There exists a need to succinctly summarize the treatments and associated outcomes to guide VH treatment plans.In this review article, a single institution's experience in the management of symptomatic VHs is summarized along with a review of the available literature on their clinical presentation and management options, followed by a proposal of a management algorithm.     

Pyloric atresia: 15-year review from a single institution

Background:

Pyloric atresia (PA) is a rare pathology. Calder presented the first pyloric atresia case in 1749 and Touroff, Sussman, Meltz, and their colleagues presented the first successful operation in 1940. PA has 3 types of anatomic variations: (1) type A, pyloric membrane or web; (2) type B, the pyloric channel is a solid cord; and (3) type C, in which there is a gap between the stomach and duodenum. Associated anomalies also have been described. Epidermolysis bullosa (EB) and intestinal anomalies occur most often with this condition.

Methods:

Charts of 16 cases of congenital PA, aged 1 to 30 days and admitted to our department between 1986 and 2001, were studied retrospectively in regard to sex, prenatal diagnosis, presence of polyhydramnios, time of admission, pathology, type of operation, associated anomalies, and mortality rate.

Results:

In the study group, the male to female ratio was 5:3, the mean birth weight was 2,312 g and the mean age of admission was 6.5 days. The distribution of the anatomic variations was type A in 9 (56.3%) and type B in 7 (43.7%) of cases. Associated anomalies were present in 7 cases (43.8%). Familial occurrence was a prominent feature of our series. Seven of 16 cases occurred in 3 families. We performed 9 web excisions together with Heineke Mikulicz (H-M) pyloroplasty, 5 atresia excisions and gastro-duodenostomy, and 2 H-M pyloroplasties alone. Stamm gastrostomy was supplemented in 3 cases. The overall mortality rate (n = 9) was 56.3%.

Conclusions:

Pyloric atresia can be managed succesfully if it is diagnosed early. In this group of patients, congenital anomalies or septicemia are the main causes of mortality.     

Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution

Background/Purpose

Surgical complications are common in survivors of congenital diaphragmatic hernia (CDH), but little is known about long-term incidence patterns and associated predictors.

Methods

A cohort of 99 CDH survivors was prospectively followed at a single-institution multidisciplinary clinic. Data were gathered regarding the adverse surgical outcomes of hernia recurrence, chest and spinal deformity, and operative small bowel obstruction (SBO), and then were retrospectively analyzed in relation to perinatal and perioperative markers of disease severity to determine significant predictors. Statistical methods used included univariate and multivariate regression analysis, hazard modeling, and Kaplan-Meier analysis.

Results

At a median cohort age of 4.7 (range, 0.2-10.6) years, 46% of patients with patch repairs and 10% of those with primary repairs had a hernia recurrence at a median time of 0.9 (range, 0.1-7.3) years after repair. Chest deformity was detected in 47%. Small bowel obstruction and scoliosis occurred in 13%. Recurrence and chest deformity were significantly more common with patch repair, liver herniation, age at neonatal extubation greater than 16 days, oxygen requirement at discharge, and prematurity. The strongest predictor of SBO was patch repair. Multivariate analysis showed that patch repair was independently predictive of recurrence and early chest deformity (odds ratios of 5.0 and 4.8, confidence intervals of 1-24 and 1-21, P < .05). Use of an absorbable patch was associated with the highest risk of surgical complications.

Conclusions

For long-term survivors of CDH, specific perinatal and operative variables, particularly patch repair, are associated with subsequent adverse surgical outcomes.     

Trigeminal neurinomas a series of 111 surgical cases from a single institution

Summary Neurinomas arising from the trigeminal nerve are rare (0.1–0.4% of intracranial tumours; 1–8% of all intracranial neurinomas).A series of 111 trigeminal neurinomas operated on at the Institute of Neurosurgery N. N. Burdenko of Moscow, Russia, during the period 1961–1994 is presented.Clinical features, diagnostic radiology, surgery and results of treatment are discussed.We distinguished four groups of trigeminal neurinomas:a) Posterior fossa tumours; b) Tumours of the Gasserian ganglion; c) Dumb-bell supra-subtentorial tumours: d) Neurinomas of the peripheral branches.The complex clinical symptomatology is related to the actual location of the lesion.Surgery of trigeminal neurinomas may be very difficult, particularly in cases of dump-bell supra-subtentorial lesions. In this series, 3 cases died postoperatively. Out of the 108 patients surviving surgery, long-term follow-up (min. 13 months, max. 33 years, average 13.5 years) was available in 98 cases. 84 patients (86.7%) showed good-to excellent results, with partial trigeminal deficit as the only surgical sequela.13 patients (11.7%) had a symptomatic recurrence following incomplete tumour removal. Second surgery, ranging from 1.4 to 9 years (average 3.8 years) following the first operation, was judged radical in 9 cases (69.2), however, the rate of complications of repeated surgery was higher than that occurring after the first operation.The advent of microsurgery, together with the introduction of the techniques of skull base surgery and of modern diagnostic imaging tools, have improved surgical results in terms of increased radicality and reduced complications.Dr. Aldo Spallone was visiting professor, coordinator of the Skull Base group at the Institute of Neurosurgery N. N. Burdenko, Moscow, during the period when the present study has been conducted (1993–1994).     

Solid organ transplantation in survivors of hematopoietic cell transplantation: a single institution case series and literature review

Beitinjaneh A, Burns LJ, Majhail NS. Solid organ transplantation in survivors of hematopoietic cell transplantation: a single institution case series and literature review.
Clin Transplant 2010: 24: E94–E102.
© 2009 John Wiley & Sons A/S. Abstract: For selected hematopoietic cell transplant (HCT) survivors with severe organ dysfunction, solid organ transplantation (SOT) may offer the best chance for better quality of life and extended survival. However, SOT following HCT has not been well described. We report our institutional experience of SOT in 12 HCT recipients and present a review of the published literature of this procedure in HCT survivors. Our experience with transplanted organs included kidney (n = 7), lung (n = 3), liver (n = 2) and heart (n = 1). Age at HCT ranged from 2 to 56 yr. Median time between HCT and SOT was 7.9 yr (range 1.2–15.9 yr). Among the 11 patients with post‐SOT follow‐up information, 10 had normal function of the transplanted solid organ at the time of last contact or death. Infections and secondary malignancy were the most common complications. Four other institutional experiences with kidney transplant, 21 case reports of liver transplant, 11 case reports of lung transplant and one cardiac transplant are also summarized. SOT is feasible for selected HCT survivors with organ failure and may be associated with favorable long‐term survival and graft function. More research is needed to further delineate the subset of survivors who will benefit the most from SOT with the least risk of complications.     

Laparoscopic splenectomy in children: experience in a single institution

PURPOSE: To assess the safety and effectiveness of laparoscopic splenectomy in children. MATERIALS AND METHODS: Hospital records of 63 patients who underwent laparoscopic splenectomy between 1998 and 2005 were reviewed retrospectively. In 16 patients concomitant cholecystectomy was performed. All procedures were performed by the same surgeon. The indications for splenectomy were hereditary spherocytosis (n = 35), idiopathic thrombocytopenic purpura (n = 22), autoimmune hemolytic anemia (n = 3), and other diseases (n = 3). Details of operative technique were reviewed and their implications on intraoperative complications are analyzed. The postoperative course and long-term results were assessed. RESULTS: There were 35 girls and 28 boys, whose average age was 11.3 years (range, 3.9-19.5 years). There were 7 conversions, mainly at the beginning of the series. A mild degree of intraoperative bleeding was observed in 23 (36.5%) cases. In two cases (3%) severe bleeding led to conversion. Postoperatively, 1 patient required blood transfusion and 1 patient had signs of mild general infection that was treated conservatively. There was no mortality in this series. The mean operation time was 134 minutes for splenectomy and 174 minutes for splenectomy and cholecystectomy. Operative time did not significantly diminish at the end of the 7-year study period. CONCLUSION: Laparoscopic splenectomy in children performed by an experienced team proved to be safe and effective with minimal side effects and should be recommended as a procedure of choice in children who require splenectomy.