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Virendra Desai, David Gonda, Sheila L. Ryan, Valentina Briceño, Sandi K. Lam, Thomas G. Luerssen, Sohail H. Syed and Andrew Jea

complications Complications No. of Patients (%) p Value After Hrs (n = 276) Monday-Friday Daytime Hrs (n=434) Infection 10 (3.6) 9 (2.1) 0.238 CSF leakage 6 (2.2) 11 (2.5) 1 Early return to OR 13 (4.7) 14 (3.2) 0.321 Postop hemorrhage 4 (1.5) 3 (0.7) 0.44 Other 7 (2.5) 6 (1.4) 0.268 Death 3 (1.1) 0 (0) 0.058 Discussion The Weekend and After-Hours Effect on Complication Incidence in Pediatric Surgery and Neurosurgery Patients The correlation between performing emergency surgery

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Chiari I malformation and acute acquired comitant esotropia

Case report and review of the literature

Stephen J. Hentschel, Kimberly G. Yen and Frederick F. Lang

✓The authors describe a rare case of a Chiari I malformation presenting with acute acquired comitant esotropia (AACE) in a 5-year-old boy. A posterior fossa decompression with duraplasty and a C1–2 laminectomy were performed. There was an immediate postoperative improvement in the esotropia, which completely resolved by 7 months following surgery. The pertinent literature is discussed and reasons are presented for recommending posterior fossa decompression in certain patients, rather than strabismus surgery, as the initial treatment for esotropia. The authors suggest that in patients with AACE, even subtle symptoms and signs of Chiari I malformation should prompt imaging of the posterior fossa. Strong consideration should be given to performing posterior fossa decompression in patients with Chiari I malformation and AACE before strabismus surgery because the esotropia may completely resolve with decompression.

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Aaron J. Clark, Tene A. Cage, Derick Aranda, Andrew T. Parsa, Kurtis I. Auguste and Nalin Gupta

Object

Craniopharyngiomas are benign tumors but their close anatomical relationship with critical neurological, endocrine, and vascular structures makes gross-total resection (GTR) with minimal morbidity difficult to achieve. Currently, there is controversy regarding the extent, timing, and modality of treatment for pediatric craniopharyngioma.

Methods

The authors performed a systematic review of the published literature on pediatric craniopharyngioma to determine patterns of clinical practice and the reported outcomes of standard treatment strategies. This yielded 109 studies, which contained data describing extent of resection for a total of 531 patients. Differences in outcome were examined based upon extent of resection and choice of radiation treatment.

Results

Gross-total resection was associated with increased rates of new endocrine dysfunction (OR 5.4, p < 0.001), panhypopituitarism (OR 7.8, p = 0.006), and new neurological deficits (OR 9.9, p = 0.03) compared with biopsy procedures. Subtotal resection (STR) was not associated with an increased rate of new neurological deficits. Gross-total was associated with increased rates of diabetes insipidus (OR 7.7, p = 0.05) compared with the combination of STR and radiotherapy (RT). The addition of RT to STR was associated with increased rates of panhypopituitarism (OR 9.9, p = 0.01) but otherwise similar rates of morbidities.

Conclusions

Although subject to the limitations of a literature review, this report suggests that GTR is associated with increased rates of endocrinopathies compared with STR + RT, and this should be considered when planning goals of surgery.

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Arman Jahangiri, Annette M. Molinaro, Phiroz E. Tarapore, Lewis Blevins Jr., Kurtis I. Auguste, Nalin Gupta, Sandeep Kunwar and Manish K. Aghi

Object

Rathke cleft cysts (RCC) are benign sellar lesions most often found in adults, and more infrequently in children. They are generally asymptomatic but sometimes require surgical treatment through a transsphenoidal corridor. The purpose of this study was to compare adult versus pediatric cases of RCC.

Methods

The authors retrospectively reviewed presenting symptoms, MR imaging findings, laboratory study results, and pathological findings in 147 adult and 14 pediatric patients who underwent surgery for treatment of RCCs at the University of Californial at San Francisco between 1996 and 2008.

Results

In both the adult and pediatric groups, most patients were female (78% of adults, 79% of pediatric patients, p = 0.9). Headache was the most common symptom in both groups (reported by 50% of pediatric patients and 33% of adults, p = 0.2). Preoperative hypopituitarism occurred in 41% of adults and 45% of pediatric patients (p = 0.8). Growth delay, a uniquely pediatric finding, was a presenting sign in 29% of pediatric patients. Visual complaints were a presenting symptom in 16% of adult and 7% of pediatric patients (p = 0.4). There was no difference between median cyst size in adults versus pediatric patients (1.2 cm in both, p = 0.7). Temporary or permanent postoperative diabetes insipidus occurred in 12% of adults and 21% of pediatric patients (p = 0.4). Kaplan-Meier analysis revealed an 8% RCC recurrence rate at 2 years for each group (p = 0.5).

Conclusions

The incidence of RCCs is much lower in the pediatric population; however, symptoms, imaging findings, and outcomes are similar, suggesting that pediatric RCCs arise from growth of remnants of the embryonic Rathke pouch earlier in life than adult RCCs but do not differ in any other way. It is important to consider RCCs in the differential diagnosis when pediatric patients present with visual impairment, unexplained headache, or hypopituitarism including growth delay. Although the average RCC size was similar in our pediatric and adult patient groups, the smaller size of the pituitary gland in pediatric patients suggests an increased relative RCC size.

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Bernard Vallée, Gérard Besson, Jean Gaudin, Hervé Person, Jean-Marie Le Fur and Jacques Le Guyader

✓ The authors report the case of a 22-month-old girl who developed cervical pain, neck stiffness, and quadriparesis over 12 days. An epidural hematoma was removed, with complete recovery after 6 months. There was no history of trauma. A search of the literature revealed eight previous cases of spontaneous spinal epidural hematomas in children under the age of 10 years.

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Gay M. Guzinski, William J. Meyer and John D. Loeser

✓ Retrieval of free abdominal ventriculoperitoneal shunt catheters usually requires laparotomy. The authors describe successful removal of free peritoneal catheters by means of commonly available adult laparoscopic equipment in four pediatric patients aged 2 to 12 years.

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Fred Epstein and E. Larry McCleary

✓ This report summarizes the first author's experience with radical excision of intrinsic non-exophytic brain-stem gliomas in 34 pediatric patients. On retrospective analysis, these tumors may be classified into three subgroups: focal, diffuse, and cervicomedullary. A focal neoplasm is a circumscribed mass less than 2 cm in diameter and without associated edema. Tumors of a larger size or in which the “focal” neoplasm is associated with a large area of apparent edema are classified as diffuse. Cervicomedullary neoplasms occur at the junction of the medulla and spinal cord and involve both of the structures but do not extend rostrally into the pons.

A radical tumor excision was carried out in all patients, and the only mortality and morbidity occurred in children harboring diffuse gliomas. All of the diffuse gliomas were malignant (grade III or IV astrocytomas), whereas three of the four focal astrocytomas and all of the cervicomedullary tumors were grade II astrocytomas. No patient with a diffuse astrocytoma was benefitted by surgery, while two of the focal astrocytomas and all of the cervicomedullary neoplasms either became stable or improved postoperatively. It is concluded that, although surgery may be accomplished within the substance of the brain stem with low morbidity and mortality rates, it is not indicated for malignant astrocytomas as it has no impact on the biology of the neoplasm. Therefore, while primary radical excision is recommended for cervicomedullary neoplasms, which are often benign, the more traditional radiation therapy and/or chemotherapy remain appropriate for tumors above the medulla.

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Margaret D. Hourihan, Peter C. Gates and Victor L. McAllister

✓ The authors have reviewed 167 cases of subarachnoid hemorrhage (SAH) occurring in patients aged 20 years and younger in a 23-year period. The modes of presentation and etiology of SAH are similar in childhood and adolescence and in the adult population, but there was a different incidence of the specific pathology producing the bleeding in this series. Twenty-six percent of cases were due to bleeding arteriovenous malformations, 52% were due to ruptured aneurysms, and in 19% no cause was found. Aneurysms in this young age group differed in several important respects from those in the adult population: there was a male predominance, a higher incidence of internal carotid bifurcation aneurysms was seen, and multiple aneurysms were encountered less commonly.

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Robert P. Naftel, Chevis N. Shannon, Gavin T. Reed, Richard Martin, Jeffrey P. Blount, R. Shane Tubbs and John C. Wellons III

Object

The use of intraventricular endoscopy to achieve diagnosis or to resect accessible intraventricular or paraventricular tumors has been described in the literature in both adults and children. Traditionally, these techniques have not been used in patients with small ventricles due to the perceived risk of greater morbidity. The authors review their experience with the effectiveness and safety of endoscopic brain tumor management in children with small ventricles.

Methods

Between July 2002 and December 2009, 24 children with endoscopically managed brain tumors were identified. Radiological images were reviewed by a radiologist blinded to study goals and clinical setting. Patients were categorized into small-ventricle and ventriculomegaly groups based on frontal and occipital horn ratio. Surgical success was defined a priori and analyzed between groups. Trends were identified in selected subgroups, including complications related to pathological diagnosis and surgeon experience.

Results

Six children had small ventricles and 18 had ventriculomegaly. The ability to accomplish surgical goals was statistically equivalent in children with small ventricles and those with ventriculomegaly (83% vs 89%, respectively, p = 1.00). There were no complications in the small-ventricle cohort, but in the ventriculomegaly cohort there were 2 cases of postoperative hemorrhages and 1 case of infection. All hemorrhagic complications occurred in patients with high-grade tumor histopathological type and were early in the surgeon's endoscopic career.

Conclusions

Based on our experience, endoscopy should not be withheld in children with intraventricular tumors and small ventricles. Complications appear to be more dependent on tumor histopathological type and surgeon experience than ventricular size.

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Kyle M. Fargen, Richard C. E. Anderson, David H. Harter, Peter D. Angevine, Valerie C. Coon, Douglas L. Brockmeyer and David W. Pincus

Object

Although rarely encountered, pediatric patients with severe cervical spine deformities and instability may occasionally require occipitocervicothoracic instrumentation and fusion. This case series reports the experience of 4 pediatric centers in managing this condition. Occipitocervical fixation is the treatment of choice for craniocervical instability that is symptomatic or threatens neurological function. In children, the most common distal fixation level with modern techniques is C-2. Treated patients maintain a significant amount of neck motion due to the flexibility of the subaxial cervical spine. Distal fixation to the thoracic spine has been reported in adult case series. This procedure is to be avoided due to the morbidity of complete loss of head and neck motion. Unfortunately, in rare cases, the pathological condition or highly aberrant anatomy may require occipitocervical constructs to include the thoracic spine.

Methods

The authors identified 13 patients who underwent occipitocervicothoracic fixation. Demographic, radiological, and clinical data were gathered through retrospective review of patient records from 4 institutions.

Results

Patients ranged from 1 to 14 years of age. There were 7 girls and 6 boys. Diagnoses included Klippel-Feil, Larsen, Morquio, and VATER syndromes as well as postlaminectomy kyphosis and severe skeletal dysplasia. Four patients were neurologically intact and 9 had myelopathy. Five children were treated with preoperative traction prior to instrumentation; 5 underwent both anterior and posterior spinal reconstruction. Two patients underwent instrumentation beyond the thoracic spine. Allograft was used anteriorly, and autologous rib grafts were used in the majority for posterior arthrodesis. Follow-up ranged from 0 to 43 months. Computed tomography confirmed fusion in 9 patients; the remaining patients were lost to follow-up or had not undergone repeat imaging at the time of writing. Patients with myelopathy either improved or stabilized. One child had mild postoperative unilateral upper-extremity weakness, and a second child died due to a tracheostomy infection. All patients had severe movement restriction as expected.

Conclusions

Occipitocervicothoracic stabilization may be employed to stabilize and reconstruct complex pediatric spinal deformities. Neurological function can be maintained or improved. The long-term morbidity of loss of cervical motion remains to be elucidated.