Congenital Palatal Fistula Associated with Submucous Cleft Palate.

Unlabelled: Although cleft lip and cleft palate are among the most common congenital malformations, the presence of an isolated congenital palatal fistula along with a submucous cleft is very rare. This appears as an oval-shaped, full-thickness fenestration in the palatal midline that does not fully extend anteriorly or posteriorly, accompanied by the findings of a submucous cleft. Because of the uncommon nature of this entity, there is controversy about its etiology, diagnosis, and management.

Achieving low cleft palate fistula rates: surgical results and techniques.

Objectives: To prospectively evaluate and reduce fistula rate after primary cleft palate repair in an academic setting.

Methods: After noting an institutional palate fistula rate of 35.8%, when a majority of palatoplasties were performed using the Furlow double-opposing Z-plasty, the decision was made to re-evaluate the surgical techniques used for palate repair.

Comparison of craniofacial phenotype in craniosynostotic rabbits treated with anti-Tgf-beta2 at suturectomy site.

Objective: Overexpression of transforming growth factor-beta 2 has been associated with craniosynostosis and resynostosis following surgery. We examined the effects of localized transforming growth factor-beta 2 inhibition on craniofacial phenotype in rabbits with craniosynostosis.

Design: Twenty-five New Zealand white rabbits with bilateral coronal craniosynostosis were divided into three treatment groups: (1) suturectomy control (n=8); (2) suturectomy with nonspecific, control immunoglobulin G antibody (n=6); and (3) suturectomy with anti-transforming growth factor-beta 2 antibody (n=11).

Verrucous lymphovascular malformation versus verrucous hemangioma: controversial nomenclature.

The International Society for the Study of Vascular Anomalies (ISSVA) divides congenital vascular anomalies into malformations and tumors and subclassified hemangiomas under tumors. However, evidence shows this accepted classification has not been widely employed. Particularly troublesome is the use of the term hemangioma, commonly used to describe a variety of vascular lesions (both malformations and tumors).

Biodegradation of Inion fast-absorbing biodegradable plates and screws.

Biodegradable plates and screws are recommended for use in surgery of the craniofacial skeleton of children. To be effective and not interfere with growth of the child's skull, the plates must biodegrade sufficiently to release the holding power of the plate and screw within 1 year. It is also essential that excessive foreign body reaction and cyst formation does not occur when the plates and screws biodegrade.

Functional outcomes of cleft lip surgery. Part II: Quantification of nasolabial movement.

Objective: To explore nasolabial movements in participants with repaired cleft lip and palate.

Design: A parallel, three-group, nonrandomized clinical trial.

Subjects: Group 1=31 participants with a cleft lip slated for revision surgery (revision), group 2=32 participants with a cleft lip who did not have surgery (nonrevision), and group 3=37 noncleft control participants.

Functional outcomes of cleft lip surgery. Part I: Study design and surgeon ratings of lip disability and need for lip revision.

Objective: Children with a cleft of the upper lip exhibit obvious facial disfigurement. Many require multiple lip surgeries for an optimal esthetic result. However, because the decision for lip revision is based on subjective clinical criteria, clinicians may disagree on whether these surgeries should be performed.

Benign fibrous histiocytoma with osteoclast-like giant cells in an infant.

Benign fibrous histiocytoma is a common soft tissue tumor that usually occurs in adults and is relatively rare in childhood. This report describes a 7-month-old Caucasian boy with an enlarging firm congenital nodule on his occipital scalp. Histologic analysis revealed a benign fibrous histiocytoma with osteoclast-like giant cells.

Anti-TGF-beta2 antibody therapy inhibits postoperative resynostosis in craniosynostotic rabbits.

Background: Postoperative resynostosis is a common clinical finding. It has been suggested that an overexpression of transforming growth factor (TGF)-beta2 may be related to craniosynostosis and may contribute to postoperative resynostosis. Interference with TGF-beta2 function with the use of neutralizing antibodies may inhibit resynostosis.

Postoperative anti-Tgf-beta2 antibody therapy improves intracranial volume and craniofacial growth in craniosynostotic rabbits.

Postoperative resynostosis and secondary craniofacial growth abnormalities are common sequelae after craniofacial surgery. It has been suggested that an overexpression of transforming growth factor-beta2 (Tgf-beta2) may be related to craniosynostosis and contribute to postoperative resynostosis. Interference with Tgf-beta2 function using neutralizing antibodies may inhibit resynostosis and improve postoperative craniofacial growth; the present study was designed to test this hypothesis.

Testing causal mechanisms of nonsyndromic craniosynostosis using path analysis of cranial contents in rabbits with uncorrected craniosynostosis.

Objective: Various causal mechanisms of familial nonsyndromic craniosynostosis have been presented. One hypothesis suggests that overproduction of bone at the suture is the primary origin of craniosynostosis, which affects brain and cranial growth secondarily through altered intracranial pressure (Primary Suture Fusion Model). Other hypotheses suggest that decreased cranial base growth or abnormal brain growth are the primary cause of craniosynostosis (Cranial Base, Brain Parenchyma Models, respectively).

No evidence for maternal-fetal microchimerism in infantile hemangioma: a molecular genetic investigation.

In this study, using the placental origin theory as a basis, we set out to explore whether hemangioma endothelial cells (HEC) were maternal in origin. We rigorously addressed this hypothesis using several molecular genetic techniques. Fluorescent in situ hybridization on surgical specimens of proliferating hemangiomas (n=8) demonstrated no XX-labeled HEC from resected tumors of male infants.

Intracranial volume changes in craniosynostotic rabbits: effects of age and surgical correction.

Background: The premature fusion of one or more cranial sutures, termed craniosynostosis, alters normal brain growth patterns and results in compensatory changes in the cranial vault. The authors previously reported that bilateral coronal suture fusion resulted in a reduction in intracranial volume in a rabbit model of nonsyndromic, familial coronal suture synostosis.

Methods: The current follow-up study involved collecting cross-sectional three-dimensional computed tomographic head scans from 142 rabbits (70 normal, 44 with uncorrected synostosis, and 28 synostosed rabbits with coronal suturectomy) at 0, 10, 25, 42, 84, and 126 days of age.

Age-related changes in lateral ventricle morphology in craniosynostotic rabbits using magnetic resonance imaging.

Background And Aims: Craniosynostosis occurs in 300-500 per 1,000,000 live births and results in secondary craniofacial, ocular, and intracranial anomalies. Neurologic problems associated with craniosynostosis include changes in intracranial morphology such as dilation of the cerebral ventricles, however, clinical studies are confounded by small sample sizes, heterogenous samples, and lack of age-matched controls. The present study was designed to assess age-related changes in the lateral ventricle volume of the brain in normal rabbits and rabbits with naturally-occurring coronal suture synostosis using serial magnetic resonance imaging.

Correction of unilateral coronal synostosis leads to resolution of mandibular asymmetry in rabbits.

Mandibular dysmorphology in unilateral coronal synostosis has been recognized clinically. In patients with unilateral coronal synostosis, the chin point deviates away from the affected side. To investigate whether this mandibular asymmetry resolves after correction of unilateral coronal synostosis, familial nonsyndromic rabbits were used.

Intracranial pressure changes in craniosynostotic rabbits.

Cranial vault and brain deformities in individuals with craniosynostosis are thought to result, in part, from changes in intracranial pressure, but clinical findings are still inconclusive. The present study describes intracranial pressure changes in a rabbit model with naturally occurring, uncorrected coronal suture synostosis. Longitudinal and cross-sectional intracranial pressure data were collected from 241 New Zealand White rabbits, divided into four groups: normal controls (n = 81); rabbits with delayed-onset coronal suture synostosis (n = 78); rabbits with early-onset unilateral coronal suture synostosis (n = 32); and rabbits with early-onset bilateral coronal suture synostosis (n = 50).

Rescue of coronal suture fusion using transforming growth factor-beta 3 (Tgf-beta 3) in rabbits with delayed-onset craniosynostosis.

Craniosynostosis results in cranial deformities and increased intracranial pressure, which pose extensive and recurrent surgical management problems. Developmental studies in rodents have shown that low levels of transforming growth factor-beta 3 (Tgf-beta 3) are associated with normal fusion of the interfrontal (IF) suture, and that Tgf-beta 3 prevents IF suture fusion in a dose-dependent fashion. The present study was designed to test the hypothesis that Tgf-beta 3 can also prevent or "rescue" fusing sutures in a rabbit model with familial craniosynostosis.

Early neuromotor behavior in craniosynostotic rabbits.

Objective: Clinical studies have shown both abnormal and normal mental and psychomotor development in patients with craniosynostosis. However, a number of confounding variables make study comparisons difficult. For these reasons, the present study describes early neuromotor development in an homogeneous rabbit model of craniosynostosis.

Cranial base changes following coronal suturectomy in craniosynostotic rabbits.

It has been suggested that surgical release of synostosed sutures may ameliorate various cranial base abnormalities in craniosynostotic patients. The present study was designed to test this hypothesis in a rabbit model with familial coronal suture synostosis (CSS). Data were collected from 56 New Zealand White rabbits: 32 unaffected controls, 11 with unoperated CSS, and 13 with CSS released by suturectomy performed at 25 days of age.

Mandibular form in a rabbit model of familial nonsyndromic coronal suture synostosis.

Nonsyndromic coronal suture synostosis produces predictable and well-documented morphologies of the cranial vault with anteroposterior growth restrictions and mediolateral compensatory growth. The potential effects of nonsyndromic coronal suture synostosis on mandibular form are not as clear, however. This study was designed to evaluate whether coronal suture synostosis is associated with alterations in mandibular form by using a familial rabbit model of coronal suture synostosis.

Coronal suturectomy does not cause acute postoperative displacement in the cranial bases of craniosynostotic rabbits.

Approximately 300 to 500 infants per 1,000,000 have prematurely fused cranial sutures (craniosynostosis). Craniosynostosis can result in increased intracranial pressure and craniofacial deformities, which often require extensive and costly craniofacial surgery. Because the neurocranium and basicranium are developmentally interrelated, understanding their influence on one another is important for surgical planning.

Ultrasound diagnosis of craniosynostosis.

Objective: To retrospectively study prenatal ultrasound images of patients with craniosynostosis to determine the extent to which prenatal diagnosis is possible.

Method: Prenatal ultrasound images of 19 patients with postnatally diagnosed metopic or coronal suture craniosynostosis were retrospectively reviewed. The 26 ultrasound examinations obtained were compared with normal images and tables of gestation.

Endocranial vascular patterns in a familial rabbit model of coronal suture synostosis.

Objective: The present study investigates the potential relationship between craniosynostosis and any changes in endocranial vasculature. The hypothesis that crania from rabbits with familial, nonsyndromic coronal suture synostosis and crania from rabbits with experimental immobilization of the coronal suture are associated with altered form of the middle meningeal vessels and dural venous sinuses is tested.

Design: Silicone rubber endocasts from 14 adult New Zealand white rabbits (Oryctolagus cuniculus) with familial nonsyndromic coronal suture synostosis (five with bilateral coronal suture synostosis and nine with unilateral coronal suture synostosis) were made to assess middle meningeal vessel and dural venous sinus form.

Shortening of mandibular linear distance with multivector distraction.

It was noted that when a multivector distractor is used, there is shortening of the linear distraction distance when the gonial angle is reduced and the transverse distance is increased. The purpose of this study was to measure the distance change at the mandibular corticotomy site when the gonial angle is reduced or the transverse distance is increased. The Synthes multivector distractor was used in this experiment.