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.

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.

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.

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.

Cranial imaging in autosomal recessive osteopetrosis. Part II. Skull base and brain.

The authors reviewed cranial imaging studies (radiographs, computed tomographic scans, and magnetic resonance [MR] images) in 13 infants and children with the autosomal recessive form of osteopetrosis to characterize patterns of skull base, brain, and cranial nerve involvement at presentation and with progression of disease. Marked sclerosis and deposition of osteopetrotic bone was noted along the anterior (but not posterior) occipitomastoid suture (n = 8), at the basioccipital-exoccipital synchondrosis (n = 9), and along the sphenooccipital synchondrosis (n = 8). Endobones, presumably representing unresorbed primitive ossification centers, were seen in the sphenoidal body and basioccipital bone in 11 of the 13 patients. Marked cupping at the basioccipital-exoccipital synchondrosis was observed in three. Neurologic deficits included blindness (n = 11), conductive hearing loss (n = 11), and facial nerve palsies (n = 4). Delayed myelination was seen with MR imaging in two of five retarded infants, including one with a documented coexisting neuronal storage defect. Prominent extracerebral cerebrospinal fluid spaces were present over the frontal lobes in five of the eight developmentally normal patients, representing either subclinical parenchymal disease or a phenomenon related to discordant growth rates between skull and brain.