Pediatric pituitary adenomas.

BACKGROUND: Pediatric pituitary adenomas are a rare medical entity that makes up a small portion of intracranial tumors in children and adolescents. Although benign, the majority of these lesions are secreting functional tumors with the potential for physiological sequela that can profoundly affect a child's development. FOCUS OF REVIEW: In this review, we discuss the medical and surgical management of these tumors with a focus on clinical presentation, diagnostic identification, surgical approach, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to treat these tumors. The management of pituitary tumors requires a multidisciplinary team of surgeons, endocrinologists, and neuroanesthesiologists as well as neurocritical care specialists to deliver comprehensive care.

Nasopharyngeal glial heterotopia with delayed postoperative meningitis.

A male infant, who underwent radical resection of a large glial heterotopia at the nasopharynx at 8 days, developed delayed postoperative bacterial meningitis at 9 months. Neuroradiological examination clearly demonstrated that meningitis had occurred because of the intracranial and extracranial connections, which were scarcely seen in the perioperative period. A transsphenoidal extension of hypothalamic hamartoma is possible because the connection started from the right optic nerve, running through the transsphenoidal canal in the sphenoid bone and terminating at the recurrent mass in the nasopharyngeal region.

A Case of Skull Base Plasmacytoma Presenting with Unilateral Cheek Paresthesia in Patient with Multiple Myeloma

Plasmacytoma is a monoclonal neoplastic proliferation of plasma cells derived from bone marrow. Plasmacytoma of the skull base is very rare. Recently, the authors experienced a case of a 50-year-old woman with a tumor like lesion originating from the right sphenoid bone. The patient presented with a 3-month history of right facial pain and paresthesia. She had been diagnosed and treated with multiple myeloma for more than 5 years, although she was in complete remission until recently. Imaging studies including contrast CT scan and MRI, showed a huge, enhanced and irregular shaped lesion in the sphenoid bone along with skull base destruction and intracranial invasion. The patient underwent transnasal endoscopic biopsy under local anesthesia. The pathology was consistent with a plasma cell tumor and it was confirmed as a solitary plasmacytoma of the bone of the skull base. We report this rare case along with a literature review.

Prescriptive proprioceptive insoles and dental orthotics change the frontal plane position of the atlas (C1), mastoid, malar, temporal, and sphenoid bones: a preliminary study.

The purpose of this series of case studies was to determine if the frontal plane position of the cranial bones and atlas could be altered using dental orthotics, prescriptive insoles, or both concurrently. The cranial radiographs of four patients were reviewed for the study. Three of the patients were diagnosed as having a temporomandibular joint (TMJ) dysfunction and a preclinical clubfoot deformity. The fourth patient was diagnosed as having a TMJ dysfunction, a preclinical clubfoot deformity and a Catetgory II sacral occipital subluxation, as designated in the chiropractic's Sacro Occipital Technique (SOT). Each patient had a series of four cranial radiographs taken using a modified orthogonal protocol. In two patients, improvement towards orthogonal was achieved when using both prescriptive dental orthotics and prescriptive insoles concurrently. Improvement towards orthogonal was less apparent when using only the prescriptive dental orthotic. No improvement or a negative frontal plane shift was noted when using only the prescriptive proprioceptive insoles. In the third patient, the frontal plane position of the cranial bones and atlas increased (away from orthogonal) when using the generic proprioceptive insoles alone or in combination with a prescriptive dental orthotic. In the fourth patient, the frontal plane position of the cranial bones improved using the dental orthotic. However, the proprioceptive insoles, when used alone or in combination with the dental orthotic, increased the frontal plane position of the cranial bones and atlas. This study demonstrates that changes in the frontal plane position of the cranial and atlas bones can occur when using proprioceptive insoles and/or dental orthotics.

Shortening the learning curve in endoscopic endonasal skull base surgery: a reproducible polymer tumor model for the trans-sphenoidal trans-tubercular approach to retro-infundibular tumors.

BACKGROUND: Endoscopic endonasal skull base surgery attracts an increasing number of young neurosurgeons. This recent technique requires specific technical skills for the approaches to non-pituitary tumors (expanded endoscopic endonasal surgery). Actual residents' busy schedules carry the risk of compromising their laboratory training by limiting significantly the dedicated time for dissections. OBJECTIVE: To enhance and shorten the learning curve in expanded endoscopic endonasal skull base surgery, we propose a reproducible model based on the implantation of a polymer via an intracranial route to provide a pathological retro-infundibular expansive lesion accessible to a virgin expanded endoscopic endonasal route, avoiding the ethically-debatable need to hundreds of pituitary cases in live patients before acquiring the desired skills. METHODS: A polymer-based tumor model was implanted in 6 embalmed human heads via a microsurgical right fronto-temporal approach through the carotido-oculomotor cistern to mimic a retro-infundibular tumor. The tumor's position was verified by CT-scan. An endoscopic endonasal trans-sphenoidal trans-tubercular trans-planum approach was then carried out on a virgin route under neuronavigation tracking. RESULTS: Dissection of the tumor model from displaced surrounding neurovascular structures reproduced live surgery's sensations and challenges. Post-implantation CT-scan allowed the pre-removal assessment of the tumor insertion, its relationships as well as naso-sphenoidal anatomy in preparation of the endoscopic approach. CONCLUSION: Training on easily reproducible retro-infundibular approaches in a context of pathological distorted anatomy provides a unique opportunity to avoid the need for repetitive live surgeries to acquire skills for this kind of rare tumors, and may shorten the learning curve for endoscopic endonasal surgery.

A prospective qualitative study on patients' perceptions of endoscopic endonasal transsphenoidal surgery.

BACKGROUND: Endoscopic transsphenoidal surgery has been shown to be a safe and effective treatment option for patients with pituitary tumours, but no study has explored patients' perceptions before and after this surgery. OBJECTIVE: The authors in this study aim to explore patients' perceptions on endoscopic transsphenoidal surgery. METHODS: Using qualitative research methodology, two semi-structured interviews were conducted with 30 participants who were adults aged > 18 undergoing endoscopic transsphenoidal surgery for the resection of a pituitary tumour between December 2008 and June 2011. The interviews were audiotaped and transcribed. The resulting data was analyzed using a modified thematic analysis. RESULTS: Seven overarching themes were identified: (1) Patients had a positive surgical experience; (2) patients were satisfied with the results of the procedure; (3) patients were initially surprised that neurosurgery could be performed endonasally; (4) patients expected a cure and to feel better after the surgery; (5) many patients feared that something might go wrong during the surgery; (6) patients were psychologically prepared for the surgery; (7) most patients reported receiving adequate pre-op and post-op information. CONCLUSIONS: This is the first qualitative study reporting on patients' perceptions before and after an endoscopic endonasal transsphenoidal pituitary surgery, which is increasingly used as a standard surgical approach for patients with pituitary tumours. Patients report a positive perception and general satisfaction with the endoscopic transsphenoidal surgical experience. However, there is still room for improvement in post-surgical care. Overall, patients' perceptions can help improve the delivery of comprehensive care to future patients undergoing pituitary tumour surgery.

Effect of excessive methionine on the development of the cranial growth plate in newborn rats.

OBJECTIVE: Methionine is an essential amino acid and pivotal for normal growth and development. However, previous animal studies have shown that excessive maternal intake of methionine causes growth restrictions, organ damages, and abnormal growth of the mandible in newborn animals. However, the effect of excessive methionine on the development of the cranial growth plate is unknown. This study investigated histological alterations of the cranial growth plate induced by high methionine administration in newborn rats. DESIGN: Twenty pregnant dams were divided into a control and an experimental group. The controls received a diet for rats and the experimental group was fed from the 18th gestational day with a special manufactured high methionine diet for rats. The high methionine diet was maintained until the end of the lactation phase (day 20). The offspring of both groups were killed at day 10 or 20 postnatally and their spheno-occipital synchondroses were collected for histological analysis. RESULTS: The weight of the high-dose methionine treated experimental group was considerably reduced in comparison to the control group at day 10 and 20 postnatally. The cartilaginous area of the growth plate and the height of the proliferative zone were markedly reduced at postnatal day 10 in the experimental group. CONCLUSIONS: In summary, the diet-induced hypermethioninemia in rat dams resulted in growth retardations and histomorphological changes of the spheno-occipital synchondrosis, an important craniofacial growth centre in newborns. This finding may elucidate facial dysmorphoses reported in patients suffering from hypermethioninemia.

Trans-lamina terminalis approach to third ventricle using supraorbital craniotomy: technique description and literature review for outcome comparison with anterior, lateral and trans-sphenoidal corridors.

BACKGROUND: The trans-lamina terminalis approach has been described to remove third ventricular tumors. Various surgical corridors for this approach include anterior (via bifrontal craniotomy), anterolateral (via supra-orbital craniotomy), lateral (via pterional craniotomy) and trans-sphenoidal corridors. Supra-orbital craniotomy offers a minimally invasive access for resection of third ventricular tumors. MATERIAL AND METHODS: The trans-lamina terminalis technique through a supra-orbital craniotomy is described. Also, a literature review of clinical outcome data was performed for the comparison of different surgical corridors (anterior, antero-lateral, lateral, and trans-sphenoidal). RESULTS: The operative steps and anatomic landmarks for supra-orbital craniotomy are discussed, along with 3 representative cases and respective outcomes. Gross total resection was achieved in 2 patients, and one patient required reoperation for recurrence. Based on the current literature, the clinical outcomes after supra-orbital craniotomy for trans-lamina terminalis approach are comparable to other surgical corridors. CONCLUSIONS: The supra-orbital craniotomy for trans-lamina terminalis approach is a valid surgical choice for third ventricular tumors. The major strengths of this approach include minimal brain retraction and direct end-on view; however, the long working distance is a major limitation. The clinical outcomes are comparable to other surgical corridors. Sound understanding of major strengths, limitations, and strategies for complication avoidance is necessary for its safe and effective application.

[Re-evaluation of using acupuncture needle as sphenoidal electrode in temporal lobe epilepsy].

In 1965, Feng of the Peking Union Hospital published an article entitled "Innovation in electroencephalography: the use of acupuncture needles as sphenoidal electrodes". It was a preliminary report, but surprisingly EEG records in the figures did not show definite spikes or sharp waves in the sphenoidal leads. In 1983, Feng and his colleagues reported a summary of 2,000 cases studied with acupuncture needle sphenoidal electrodes. This time, spikes or sharp waves were shown in EEG recordings. However, cases studied were several "paroxysmal disorders", including psychomotor seizure (155 cases), generalized seizure (765 cases), epileptic cephalgia (101 cases), syncope (104 cases), abdominal epilepsy (24 cases), encephalopathy (135 cases), brain tumor (32 cases), hemiplegia of unknown cause (43 cases), psychosis (34 cases), and others (607 cases). Therefore, there were many unknown cases and many cases that were not related to temporal lobe epilepsy. Surprisingly, the increase in detection by acupuncture needle electrode was higher for hemiplegia of unknown cause, brain tumors, and encephalopathies than for the temporal lobe epilepsy. Furthermore, the issue of no insulation of the acupuncture needle was not addressed. Therefore, we began in 1988 to reinvestigate the usefulness of uninsulated acupuncture needles as sphenoidal electrodes. We also compared the efficacy of anterior temporal electrodes (T1, T2) with those of acupuncture needle and EMG needle. Our results showed that when compared to the routine EEG recordings, acupuncture needle sphenoidal electrodes increased the yield of detecting anterior temporal spikes from 41% to 70%. Our data further showed that when compared to the EMG needle recordings, acupuncture needle recordings had the same detection rate, but the spike amplitude was slightly smaller (129 microv vs. 135 microv). Interestingly, we also found that anterior temporal surface electrode recordings were nearly as good as those of acupuncture needle and traditional insulated needle electrodes in the detection of anterior temporal spikes. Our data indicate that acupuncture needle sphenoidal electrode is as effective as the traditional insulated needle sphenoidal electrode in the detection of anterior temporal spikes. We agree with Feng that the use of acupuncture needle is easy, safe, and has minimal discomfort and complications. However, when the use of the acupuncture needle is not acceptable to patients or as in the pediatric group, anterior temporal electrode is an ideal alternative to acupuncture needle sphenoidal electrode.

Outcome of surgical intrasellar growth hormone tumor performed by a pituitary specialist surgeon in a developing country.

BACKGROUND: Acromegaly is an excessive GH secretion, which in most cases, is caused by a pituitary GH-secreting adenoma. Traditional treatment of acromegaly consists of surgery, drug therapy, and eventually radiotherapy. The aim of this retrospective study is to evaluate the results of transsphenoidal endoscopic surgery in a group of patients with intrasellar GH adenoma who were operated by a pituitary specialist surgeon. We shall then argue about the economical advantages, for the NHS of a developing country, between surgical and medical treatment. METHODS: We have analyzed data from 33 patients with intrasellar GH tumor who had been referred to the neuroendocrine department of the HGF, Brazil. The patients underwent a transsphenoidal endoscopic adenomectomy for acromegaly between 2000 and 2005. Their ages were between 20 and 67 years (mean, 44 years) at the moment of surgery. No cavernous sinus invasion was present. Follow-up was a median of 2 years (range, 12 months-6 years). RESULTS: All 33 patients had intrasellar adenoma, 84.84% of patients achieved remission by surgery. One patient was operated twice and reached hormonal normalization. Five patients still had the disease and refused a second surgery. A treatment with octreotide was started for these 5 patients and resulted in an adequate control of GH and IGF-1 levels. No patients had radiotherapy. CONCLUSION: Our patients, with intrasellar GH tumor, operated by a pituitary specialist neurosurgeon had remission rates approaching those obtained by most specialized neurosurgical centers worldwide. For equal results, our study shows that the surgical treatment is the best issue for the patient and for the NHS.

Fracture of the coronoid and pterygoid processes by firearms: case report.

A rare case of fracture of the coronoid and the pterygoid process caused by firearms is described. A 28-year-old male was hit by a bullet in the face, resulting in restricted mouth opening, difficulty in chewing and pain when opening the mouth. Clinical examination revealed a perforating wound in the right parotid region and a similar wound on the left side of the same region. A CT scan showed comminuted fracture of the left coronoid process and bilateral comminuted fracture of the pterygoid processes. Treatment was conservative, speech therapy was conducted and it was successful. Details of the clinical signs, radiology (3D-CT scan), treatment and follow-up are presented.

Ultrasonic bone cutting for surgically assisted rapid maxillary expansion (SARME) under local anaesthesia.

Ultrasonic bone-cutting surgery has been recently introduced as a feasible alternative to the conventional tools of cranio-maxillo-facial surgery, due to its technical characteristics of precision and safety. The device used is unique in that the cutting action occurs when the tool is employed on mineralized tissues, but stops on soft tissues. This technical note illustrates the use of Piezosurgery for all osteotomies of surgically assisted rapid maxillary expansion (SARME). The procedure, including pterygo-maxillary detachment, can be completed under local anaesthesia. Other advantages include minimal risk of jeopardizing critical anatomic structures (e.g. palatine artery), minimal intraoperative bleeding and postoperative swelling, and minimal thermal damage to bone surfaces. Narrow and rectilinear osteotomies can be easily performed with varying vibrating scalpels, at the cost of a longer operative time.

Fracture of the coronoid and pterygoid processes by firearms: case report

A rare case of fracture of the coronoid and the pterygoid process caused by firearms is described. A 28-year-old male was hit by a bullet in the face, resulting in restricted mouth opening, difficulty in chewing and pain when opening the mouth. Clinical examination revealed a perforating wound in the right parotid region and a similar wound on the left side of the same region. A CT scan showed comminuted fracture of the left coronoid process and bilateral comminuted fracture of the pterygoid processes. Treatment was conservative, speech therapy was conducted and it was successful. Details of the clinical signs, radiology (3D-CT scan), treatment and follow-up are presented.

Um caso raro de fratura dos processos coronóide e pterigóide por arma de fogo é descrito. Paciente do sexo masculino, 28 anos de idade, foi atingido por projétil de arma de fogo na face, resultando em limitação na abertura da boca, dificuldade para mastigar e dor ao abrir a boca. Exame clínico revelou ferimento perfurante em região parotídea direita e ferida similar do lado esquerdo na mesma região. Tomografia computadorizada (TC) mostrou fratura cominutiva do processo coronóide esquerdo e fratura cominutiva bilateral dos processo pterigóides. O tratamento foi conservador, através de fonoterapia, obtendo-se sucesso. Uma descrição detalhada das características clínicas, radiológicas (TC com reconstrução 3D), tratamento e proservação do caso é apresentada.

Pterygopalatine fossa infiltration through the greater palatine foramen: where to bend the needle.

OBJECTIVE: In the literature, there has been controversy regarding the length of the greater palatine canal. If the pterygopalatine fossa is infiltrated in an attempt to reduce bleeding during sinus surgery, this information is important to be able to place local anesthetic with maximal effect and the least likelihood of complications. STUDY DESIGN: The authors conducted a prospective cadaver-based study using high-definition computed tomography (CT) scans. METHODS: Twenty-two cadaver heads were CT scanned and the greater palatine canal length, pterygopalatine fossa height, and thickness of the soft tissue in the roof of the mouth were measured on parasagittal images using the CT scanner workstation. RESULTS: The mean length of the greater palatine canal was 18.5 mm (95% confidence interval [CI]=17.9-19.1) and the mean height of the pterygopalatine fossa was 21.6 mm (95% CI=20.7-22.5). The mean thickness of the soft tissue in the roof of the mouth overlying the foramen of the greater palatine canal was 6.9 mm (95% CI=6.2-7.6). CONCLUSION: To perform an effective infiltration of the pterygopalatine fossa, the needle should be bent at 25 mm from the tip at an angle of 45 degrees.

Hemodynamic responses to epinephrine-containing local anesthetic injection and to emergence from general anesthesia in transsphenoidal hypophysectomy patients.

Patients undergoing transsphenoidal pituitary surgery may experience hypertensive episodes during the intranasal injection of vasoconstrictor-supplemented local anesthetics or emergence from general anesthesia. The present research characterized the blood pressure responses during transsphenoidal surgery and tested the hypothesis that the underlying pituitary disease influences the incidence and magnitude of the blood pressure responses. The records of 100 patients were retrospectively reviewed. All had direct blood pressure measurements recorded using a computer-based anesthesia recording system. Mean age was 49 +/- 17 years (+/- SD) and 52% were male. Blood pressure increased by 60 +/- 37 mm Hg systolic and 23 +/- 22 mm Hg diastolic with intranasal injection and 42 +/- 24 mm Hg systolic and 23 +/- 16 mm Hg diastolic during emergence from general anesthesia. Systolic blood pressure increased by greater than 50% in 58% of patients following intranasal injection and in 33% of patients upon emergence from anesthesia. Blood pressure responses did not differ with respect to endocrinopathy type (Cushing's disease, acromegaly, or other pathology), gender, age, surgeon, history of prior transsphenoidal surgery, history of either hypertension or diabetes, or preoperative use of either beta-adrenergic or calcium channel-blocking drugs. There was poor correlation between the epinephrine dose injected (range 30-220 microg) and systolic blood pressure response (r = 0.24; r2 = 0.06; P = 0.031). Blood pressure increases were not associated with cardiac arrhythmias, persistent myocardial ischemia, or myocardial infarction. The authors conclude that in transsphenoidal hypophysectomy patients, large blood pressure increases are common with intranasal injection and upon awakening from general anesthesia. However, the authors were not able to find a variable that might enable the prediction of which patients are most likely to experience the most intense blood pressure elevations.

Ivory osteoma of the craniofacial skeleton.

The authors describe the clinical presentation, investigation, and surgical management of two cases of benign ivory osteoma of the craniofacial skeleton. In the first case, a bony mass located over the frontal region had become a cosmetic burden to the patient, and she requested removal with minimal morbidity. Accordingly, an endoscopic procedure, with minimal access incisions located behind the hairline, was devised. In the second case, the patient, a Jehovah's Witness, presented with a long history of slowly enlarging bony masses over the maxilla and in the pterygopalatine space. She finally requested surgical intervention when the symptoms of pain related to fifth nerve compression at the foramen ovale became intolerable. Key aspects of the preoperative and perioperative management strategies used to avoid blood transfusion are detailed, as is the surgical approach, which included a bicoronal scalp flap with temporary removal of the zygomatic arch and the coronoid process. Finally, the etiology, histology, and natural history of ivory osteomas are discussed.

Vascular complications of transsphenoidal surgery.

Vascular complication of transsphenoidal surgery can lead to mortality and serious morbidity. In a series of 3,061 transsphenoidal operations for pituitary disease, 24 such complications were encountered, seven of which were fatal. The anatomic substrate for such complications is discussed, along with technical aspects of surgery and other methods for the avoidance of vascular complications.

Effects of pulsed ultrasound on sphenoid bone temperature and the heart rate in guinea-pig foetuses.

Temperature increase induced by exposure to unscanned pulsed ultrasound at an intensity (I(SPTA)) 2.82 W/cm2 was measured in the brain adjacent to the sphenoid bone of foetal guinea-pigs in late gestation under in vitro and in vivo (in utero) conditions. After 120 s exposure a mean temperature increase of 2.6 degrees C was measured in vitro. Removal of the overlying parietal bones increased this value to 5.2 degrees C. Mean temperature increases at the sphenoid bone recorded in utero were 1.5 degrees C live and 2.0 degrees C post mortem. Measurement of foetal ECG showed that ultrasound-induced heating of the hypothalamic region did not significantly alter foetal heart rate.