Jay M. Pensler

Clinical and ultrastructural studies of Romberg's hemifacial atrophy

Rombergs disease is an uncommon and poorly understood condition manifested by progressive hemifacial atrophy of skin, soft tissue, and bone. In order to better define the natural history and anatomic variation of this disorder, we evaluated 41 patients by history, physical examination, and facial radiographs. Light microscopic studies were performed on tissue from 19 patients, and ultrastructural analysis was performed on specimens from 6 patients. The average age at inception of the disease was 8.8 years. Atrophy, within one or more trigeminal nerve dermatomes, progressed at a variable rate (mean period of active tissue dissolution = 8.9 ± 6 years). In 26 patients with skeletal involvement, the mean age of onset was 5.4 years, versus 15.4 years for 15 patients without skeletal involvement, a statistically significant difference (p < 0.01). However, there was no correlation between the severity of soft-tissue deformity and the age of onset. Electron microscopy demonstrated lymphocytic infiltrates in neurovascular bundles and abnormalities of vascular endothelium and basement membranes. We hypothesize that the pathogenesis of Rombergs disease involves chronic cell-mediated vascular injury and incomplete endothelial regeneration along branches of the trigeminal nerve (lymphocytic neurovasculitis).

The Calvarial Donor Site: An Anatomic Study in Cadavers

In a study of 200 fresh adult cadavers, calvarial thickness was measured at selected points. The variables of age, height, weight, sex, and race were subjected to multiple regression analysis to determine which were significant in the determination of skull thickness. The results indicate that weight, race, and sex are the most important variables. However, the magnitude of the effects of these variables is minimal when considered in relation to clinical requirements. The study provides the clinician with a reasonable basis to obtain preoperative estimation of the thickness of calvarial grafts in the adult at four commonly utilized points.

Skeletal Distraction of the Hypoplastic Mandible

Six patients underwent percutaneous pin placement, 17 intraoral corticotomies, and application of an Orthofix lengthening device to facilitate complex multiplanar distraction as mandated by the patients specific deformity. The patients underwent gradual bony and soft-tissue distraction at the rate of .25 mm four times a day. Average distraction was 18.3 + 5.5 mm (mean rt SD). After distraction, a Pday period of stabilization for each 1 mm of lengthening was used (36.4 ± 10.7 days) (M ± SD). All the patients had marked improvement in their occlusion postoperatively and significant amelioration of their preoperative respiratory and feeding difficulties. The patients all exhibited dramatic aesthetic improvement. lntraoral corticotomies performed in conjunction with gradual skeletal distraction appears to offer significant advantages over classical treatment for micrognathia in a very specific group of patients.

Orbital roof fractures in the pediatric population.

Twenty-three patients aged 3.3 ± 1.6 years (mean ± SD) presented between January of 1984 and September of 1987 with fronto-orbital trauma resulting in fractures of one (N = 20) or both (N = 3) orbital roofs. All patients had computed tomography (CT) with axial and coronal sections that revealed three fracture patterns of the orbital roof (nondisplaced, superiorly displaced, and infe-riorly displaced fractures). Orbital dystopia was exhibited in 35 percent (N = 8) of the patients. Exophthalmos was noted in 61 percent (N = 14) of the patients. Only 30 percent of the patients (N = 7) sustained associated maxillofacial fractures. Eight percent of fractures exhibited orbital encephaloceles. All patients lacked frontal sinus pneumatization. The majority of children with orbital roof fractures do not exhibit concomitant facial fractures. CT utilizing both axial and coronal sections is valuable in defining the extent and pattern of the fracture as well as in identifying associated neurologic injuries. Large, displaced orbital roof fractures, which occurred in 3 of 13 patients with displaced fractures in our series, should undergo early reduction to avoid late development of encephalocele.

Role of resorbable plates and screws in craniofacial surgery.

Twenty patients underwent craniomaxillofacial fixation procedures with the use of the LactoSorb (Walter Lorenz Surgical, Jacksonville, FL) resorbable plating system. The patients ranged in age from 4 months to 67 years. No significant problems from the use of this plating system were noted. The resorbable plating system offers several distinct advantages over traditional metallic systems.

The anatomy of Cupid's bow in normal and cleft lip.

This is a gross and microscopic examination of the cutaneous-vermiloion-mucosal region (Cupids bow) in infants with normal lip as compared to cleft lip. Morphologic observations of lips in situ were made with magnification. Specimens for histology were from the normal upper lip of infants (n = 3) and adults (n = 4), and from redundant tissue made available during primary repair of cleft lip (n = 13) and cleft lip revision (n = 4). Tissues were analyzed with hematoxylin and eosin, Gomori trichrome, and Fontana-Masson stains. Immunoperoxidase stains were used to emphasize vascular endothelium and epithelial cytokeratins. Our observations support the hypothesis that the anterior projection of the pars marginalis of the musculus orbicularis oris gives rise to the normal cutaneous-vermilion junction (“white skin roll” of Gillies-Millard). The band of vellus hairs, found atop the vermilion-cutaneous junction, also highlights the rim of Cupids bow. There was no abrupt alternation in vasculature, melanin, or cytokeratin content in the white roll. Proceeding caudally, the vermilion-mucosal region was characterized by progressively increasing epidermal thickness and size of rete ridges, decreasing melanin, more superficial capillaries, and an abrupt transition from keratinized to nonkeratinized squamous epithelium (“red line” of Noordhoff). In the cleft lip specimens, where white roll was absent, there was hypoplasia and disorientation of the underlying pars marginalis component of m. orbicularis oris. Vellus hair follicles were seen in the zone of presumptive white roll in clefts, just as in normal lip. Frequently, these pilar units contained disproportionately large sebaceous glands. In some cleft lip specimens, ectopic sebaceous glands were noted in the oral mucosa. We also observed a deficiency of vermilion width on the medial side of unilateral cleft lip, whereas the width of the vermilion at the crest of the bow was normal or slightly increased in the lateral cleft segment. A narrow vermilion band and exposed hypoplastic mucosa were characteristic of the prolabium in the bilateral complete cleft deformity. The findings of this study are pertinent to technical details for construction of the Cupids bow and vermilion in unilateral and bilateral cleft lip. (Plast. Reconstr. Surg. 92: 395, 1993.)sS

A comparison of speech results after the pharyngeal flap and the dynamic sphincteroplasty procedures.

Eighty-five patients underwent suzgery to reduce velopharyngeal incompetence with either a pharyngeal flap (n = 75) or a dynamic sphincteroplasty (n = 10) performed between April 1958 and August 1989, and were evaluated preoperatively and postoperatively by a plastic surgeon, speech pathologist, and otolaryngologist. Improvement in speech was noted in 75% (n = 56) of the patients with pharyngeal flaps and 70% (n = 7) of the patients with dynamic sphincteroplasties postoperatively. Thirty percent of the patients in both groups showed no improvement postoperatively in speech. Three patients (4%) who underwent pharyngeal flap procedures developed sleep apnea postoperatively. Persistent velopharyngeal incompetence may be treated effectively with either a pharyngeal flap or a dynamic sphincteroplasty. Either procedure appears to result in improved speech in most patients.

The efficacy of tongue resection in treatment of symptomatic macroglossia in the child.

Nine patients with symptomatic macroglossia, 3 with Beckwith-Wiedemann syndrome, 3 with Downs syndrome, and 3 with lymphatic malformation of the tongue, were evaluated for postoperative improvement following partial glossectomy. Symptoms evaluated were speech intelligibility, oral competence at rest, drooling, and normal deglutition. All patients with Downs syndrome and Beckwith-Wiedemann syndrome were improved postoperatively. All patients with Downs syndrome exhibited improved deglutition and reduced drooling. Only 1 patient (33%), however, achieved oral competence. Speech was improved postoperatively in 2 patients (67%) with Downs syndrome. Patients with lymphatic malformation of the tongue did not exhibit consistent long-term improvement postoperatively, suggesting that conservative management of macroglossia in this patient group may be indicated.

Levator repositioning and palatal lengthening for submucous clefts

Submucous clefts of the palate may present with velopharyngeal incompetence (VPI) or a history of recurrent otitis media. Many surgeons have favored a pharyngeal flap as primary treatment of the velopharyngeal incompetence associated with this disorder. The increasing number of case reports of sleep apnea and airway compromise associated with pharyngeal flaps prompted the use of levator muscle repositioning with palatal lengthening as initial therapy in 15 patients in an attempt to correct the pathologic anatomy while avoiding the postoperative sequelae. Patients were divided into two groups: group A (N = 8) had surgery before age 2 (11.8 ± 5.7 months), and group B (N = 7) had surgery after 2 years of age (64.3 ± 24.2 months). No patient in group A required a secondary operative procedure for velopharyngeal incompetence. Normal speech was obtained in 75 percent (N = 6), and slight velopharyngeal incompetence not requiring secondary correction was obtained in 25 percent (N = 2). Group B obtained less dramatic speech results: normal in 14 percent (N = 1), slight velopharyngeal incompetence in 58 percent (N = 4), and no improvement or severe velopharyngeal incompetence requiring a secondary procedure in 28 percent (N = 2). Patients with preoperative otologic disorders (N = 10) obtained significant improvement in 90 percent of cases (p = 0.002). Early surgical intervention in patients with abnormal speech prior to age 2 appears to result in normal speech in the majority of instances. Late repair with levator repositioning and palatal lengthening provided improved speech in 72 percent of patients.

The Superficial Musculoaponeurotic System in the Upper Lip: An Anatomic Study in Cadavers

Ten cadavers were employed to demonstrate the presence of the SMAS in the upper lip using macroscopic and microscopic techniques. The relationships and attachments of the SMAS to the dermis of the upper lip are described. In cadavers, medial traction on the SMAS in the upper lip in conjunction with superolateral traction on the SMAS in the cheek is found to decrease the depth of the nasolabial fold. Superior traction on the SMAS in the upper lip elevates the interlabial line, reestablishes the convexity of the lateral vermilion border, and partially increases concavity of the profile.