Management of isolated sagittal synostosis in the absence of scaphocephaly: a series of eight cases.

BACKGROUND: The diagnosis of isolated sagittal synostosis is usually made at birth, with a boat-shaped deformity of the skull (scaphocephaly). The Oxford Craniofacial Unit has successfully managed eight cases of isolated sagittal synostosis without scaphocephaly. To the authors' knowledge, there have been no reports of this in the literature. METHODS: The Oxford Craniofacial Unit database (set up in 2004) was searched. This identified 193 cases of isolated sagittal synostosis, eight of which did not have a scaphocephalic head shape (i.e., a cephalic index <76). RESULTS: Patients with isolated sagittal synostosis in the absence of scaphocephaly represent 4.1 percent of all isolated sagittal synostosis patients in the authors' database. Intracranial pressure was measured in six of the eight cases and found to be high in four of these (67 percent). CONCLUSIONS: It is unclear why the secondary features of sagittal synostosis did not develop in these cases. Possible explanations include fusion of the suture late after the majority of skull growth has taken place and a patent metopic suture allowing growth to occur anteriorly; other sutures appeared patent radiologically but may not have been functioning normally. Although difficult to recognize, patients with isolated sagittal synostosis in the absence of scaphocephaly are important to identify because they are at high risk of developing raised intracranial pressure. A new management algorithm for treatment of these cases is described that has been used successfully in the Oxford Craniofacial Unit.

A 10-year review of benign and malignant peripheral nerve sheath tumors in a single center: clinical and radiographic features can help to differentiate benign from malignant lesions.

BACKGROUND: Malignant peripheral nerve sheath tumors are rare, and their aggressive nature mandates treatment in specialist centers. In contrast, benign peripheral nerve sheath tumors are common and are treated by a variety of specialist surgeons, including plastic surgeons. The authors aimed to detect features in the clinical presentation of peripheral nerve sheath tumors that point toward a diagnosis of malignant peripheral nerve sheath tumor and therefore prompt referral to a specialist center. METHODS: All histologically diagnosed primary peripheral nerve sheath tumors from January of 1995 to December of 2004 were identified from histopathology records. Notes were reviewed and analyzed with regard to symptoms, signs, radiology, electrophysiology, surgery, and pathology. Statistical comparisons used Fisher's exact test and the Mann-Whitney test. RESULTS: During the study period, 32 cases of malignant peripheral nerve sheath tumor in 30 patients were treated. Factors in the clinical evaluation that significantly predicted the presence of malignant peripheral nerve sheath tumor included site, large size, depth in relation to the deep fascia, short duration of symptoms, and pain. Magnetic resonance imaging and computed tomography were sensitive and specific ways of confirming the clinical diagnosis. Interestingly, schwannomata were harder to distinguish from malignant peripheral nerve sheath tumors both clinically and radiologically. CONCLUSIONS: The authors have reviewed their institutional experience of peripheral nerve sheath tumors over a 10-year period. Their results will help to focus clinical and radiologic investigation of patients presenting with these tumors.

A paramedian cleft of the lower lip.

We report the unusual case of a baby with a paramedian cleft of the lower lip. In 1976, Tessier described a classification of craniofacial clefts based on personal observations, and he predicted the possibility of further clefts being discovered by labeling midline mandibular clefts "30." This case of a paramedian cleft of the lower lip would fit into what could be a new Tessier 28/29 cleft. In the future, clefts in new locations around the mouth may be seen, thus filling the gaps in Tessier's classification.

Acute transection of the left internal mammary artery remote from the anastomosis following coronary artery bypass surgery.

We report a case of spontaneous transection of the left internal mammary artery 4h after a patient underwent coronary artery bypass surgery. This complication has been reported in two patients following minimal access coronary artery bypass grafting, but to our knowledge, this is the first case of transection of the left internal mammary artery, following conventional myocardial revascularization via median sternotomy. Possible mechanisms leading to this rare, but potentially life-threatening complication are also discussed.