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Biomedical subjects

J B Mulliken

Publications and source records attributed to J B Mulliken.

At least 19 recordsLinked to original sources
PubMed1992

Isolation and characterization of human heparin.

Heparin was isolated from an unusually large human hemangioma that contained an elevated level of mast cells. Purification of multimilligram quantities of heparin from this tissue sample permitted a thorough examination of its structure and activity. Characterization of this human heparin included the following: one-dimensional and two-dimensional 1H-nuclear magnetic resonance spectral analysis; oligosaccharide mapping; saccharide compositional analysis; and in vitro assessment and anticoagulant activity. Oligosaccharide mapping and nuclear magnetic resonance spectroscopy showed that this human heparin is structurally similar to porcine intestinal mucosal heparin but distinctly different from bovine lung heparin. This human heparin also has substantially more in vitro anticoagulant activity than either of these pharmaceutical heparins.

Animals↗
PubMed1992

Interferon alfa-2a therapy for life-threatening hemangiomas of infancy.

BACKGROUND AND METHODS: Most hemangiomas are small, harmless birthmarks that appear soon after birth, proliferate for 8 to 18 months, and then slowly regress over the next 5 to 8 years, leaving normal or slightly blemished skin. In rare cases, hemangiomas can endanger vital structures, with a mortality of up to 60 percent. About a third of these life-threatening hemangiomas respond to treatment with corticosteroids, but for the others there is no safe and effective treatment. We evaluated the effects of daily subcutaneous injections of interferon alfa-2a (up to 3 million units per square meter of body-surface area) in 20 neonates and infants with life-threatening or vision-threatening hemangiomas that failed to respond to corticosteroid therapy. RESULTS: In 18 of the 20 patients the hemangiomas regressed by 50 percent or more after an average of 7.8 months of treatment (range, 2 to 13). One infant died of refractory proliferation of a lesion and consumptive coagulopathy. The condition of three other patients who had large hemangiomas associated with consumptive coagulopathies that were unresponsive to conventional therapies stabilized after seven days of treatment with interferon alfa-2a alone. Transient side effects of treatment with interferon alfa-2a included fever, neutropenia (one patient), and skin necrosis (one patient). No long-term toxicity has been observed after a mean follow-up of 16 months. CONCLUSIONS: Interferon alfa-2a appears to induce the early regression of life-threatening corticosteroid-resistant hemangiomas of infancy.

Disseminated Intravascular Coagulation↗
PubMed1992

The use of scapular and parascapular flaps for cheek reconstruction.

This is a retrospective review of our experience with microvascular transfer of scapular and parascapular flaps for the correction of lateral facial contour deficiencies. Twenty-eight patients with congenital (n = 8) and acquired (n = 20) defects were treated with 30 flaps; two patients had bilateral flaps. The etiology of the defects included hemifacial microsomia (n = 2), oblique facial cleft (n = 1), Romberg's hemifacial atrophy (n = 5), neoplasm (n = 4), irradiation (n = 8), trauma (n = 4), tumor excision (n = 4), facial lipodystrophy (n = 2), and silicone granuloma (n = 2). The follow-up evaluation was from 2 to 13 years, with an average of 6 years. Fabrication of a facial moulage was part of the preoperative planning for each patient. These were compound flaps, including skin, deepithelialized skin, fat, fascia, and bone, if necessary. All flaps were constructed with an intact skin paddle for postoperative monitoring. Based on dissections and anatomic findings at operation, several variations in the level of emergence of the circumflex scapular artery from the triangular space and its branching patterns were noted. All flaps survived; changes in the patients' weights were reflected in the flaps. Twelve patients required secondary procedures: excision of skin monitor islands, scar revisions, debulking, or flap resuspension to the malar region. Bone grafts or alloplastic implants were necessary in four patients in whom the malar eminence could not be adequately corrected by transfer of a flap. The deepithelialized scapular/parascapular flap is preferred for correction of large lateral facial defects.

Adipose Tissue↗
PubMed1992

Correction of the bilateral cleft lip nasal deformity: evolution of a surgical concept.

The origin of the nasal deformity of a bilateral complete cleft lip is both primary (deformation/malformation) and secondary (postoperative distortion). This is an interim report of a personal evolution from staged correction of the bilateral cleft nasal deformity to synchronous repair of the nose and the lip and premaxillary-maxillary clefts. The anatomic concept is that, because of the malpositioned alar cartilages, the columella only appears to be short in an infant with bilateral cleft lip. The technical stratagems to model the nose are: (1) alignment of the premaxilla and (2) anatomic placement of the alar cartilages with sculpturing of the overlying soft tissue.

Cleft Lip↗
PubMed1992

Frontal plagiocephaly: synostotic, compensational, or deformational.

Plagiocephaly is a term commonly used to describe congenital forehead asymmetry. Sixty patients with frontal plagiocephaly were evaluated retrospectively and separated into three types: synostotic (N = 24), compensational (N = 3), and deformational (N = 33). Categorization of frontal plagiocephaly as synostotic or deformational was reliably made by physical examination, focusing on the supraorbital rims, nasal root, ears, and malar eminences. Other anatomic parameters useful in the differential diagnosis included chin point, palpebral fissures, and facial height. This study documented that birth histories were similar for synostotic and deformational frontal plagiocephalic infants. However, other deformational anomalies were more common in deformational frontal plagiocephalic infants, whereas malformations had an equal incidence in deformational and synostotic frontal plagiocephalic infants. Torticollis was an associated finding in 64 percent of infants with deformational frontal plagiocephaly; almost all were ipsilateral. In contrast, head tilt, usually to the contralateral side, was noted in 14 percent of patients with synostotic frontal plagiocephaly. Female preponderance was noted in both synostotic (79 percent) and deformational (76 percent) frontal plagiocephaly. Left-sided involvement was seen in 73 percent of patients with deformational frontal plagiocephaly and in 46 percent of patients with synostotic frontal plagiocephaly. Premature pelvic descent, in the left occipital anterior position, may account for the high incidence of left-sided deformational plagiocephaly and ipsilateral torticollis.

Abnormalities, Multiple↗
PubMed1991

Craniofacial anomalies in twins.

Studies of twins provide insight into the relative contribution of genetic and environmental factors in the causality of structural anomalies. Thirty-five affected twin pairs were identified from a group of 1114 patients with congenital craniofacial deformities evaluated from 1972 to 1989. Forty-three of these 70 twins exhibited one or more craniofacial anomalies; these were analyzed for dysmorphic characteristics, zygosity, concordance, and family history. The anomalies were categorized into two groups: malformations and deformations. The malformations (n = 36) included hemifacial microsomia (n = 10), cleft lip and palate (n = 8), cleft palate (n = 4), rare facial cleft (n = 2), craniosynostosis (n = 2), Binder syndrome (n = 2), Treacher Collins syndrome (n = 2), craniopagus (n = 2), CHARGE association (n = 1), frontonasal dysplasia (n = 2), and constricted ears (n = 1). The deformations (n = 7) included plagiocephaly (n = 5), hemifacial hypoplasia (n = 1), and micrognathia (n = 1). Twenty-one monozygotic and 14 dizygotic twin pairs were identified. The concordance rate was 33 percent for monozygotic twins and 7 percent for dizygotic twins.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1991

Pyogenic granuloma (lobular capillary hemangioma): a clinicopathologic study of 178 cases.

Pyogenic granuloma (lobular capillary hemangioma) is a common acquired vascular lesion of the skin and mucous membranes in the pediatric age group. This is a retrospective analysis of 178 patients, 17 years of age and younger (mean age 6.7 yrs). Forty-two percent of the lesions occurred in the first five years of life; only 12% appeared in infants less than 1 year old. The male:female ratio was 3:2. Most patients (74.2%) had no history of trauma or predisposing dermatologic condition. The mean lesional size was 6.5 mm and the mean duration at diagnosis was 3.8 months. The granulomas were most commonly located in the head and neck area (62.4%), followed in order of decreasing frequency by trunk (19.7%), upper extremity (12.9%), and lower extremity (5.0%). The preponderance (88.2%) occurred on the skin, the remaining ones involved the mucous membranes of the oral cavity and conjunctivae. Histologic examination demonstrated normal numbers of mast cells, in contrast to increased mast cells characteristic of proliferative phase hemangiomas. Most lesions (n = 149) were treated by full-thickness skin excision and linear closure; there were no recurrences in this group. The recurrence rate in 23 lesions treated by shave (intradermal) excision and cautery or cautery alone was 43.5%.

Adolescent↗
PubMed1991

The O.M.E.N.S. classification of hemifacial microsomia.

The wide spectrum of anomalies associated with hemifacial microsomia (HFM) has made systematic and inclusive classification difficult. We propose a nosologic system in which each letter of the acronym O.M.E.N.S. indicates one of the five major manifestations of HFM. O for orbital distortion; M for mandibular hypoplasia; E for ear anomaly; N for nerve involvement; and S for soft tissue deficiency. The O.M.E.N.S. system is easily adapted for data storage, retrieval, and statistical analysis. A retrospective study of 154 patients with HFM classified according to the O.M.E.N.S. system confirmed the concept that the mandibular deformity is the cornerstone of the anomaly. Statistical analysis demonstrated a positive association between mandibular hypoplasia and the severity of orbital, auricular, neural, and soft tissue involvement. This study did not confirm a previously reported predominance of gender or sidedness. Analysis of statistical correlations failed to substantiate a Goldenhar variant as a syndromic entity. Our analysis showed that palatal deviation is probably caused by muscular hypoplasia and not by weakness of a particular cranial nerve.

Adult↗
PubMed1991

Neurosurgical complications in craniofacial surgery.

A retrospective review of the neurosurgical complications that occurred between 1980 and 1989 at The Children's Hospital in Boston is described. The results are compared with reports from other craniofacial centers. A total of 355 cases are analyzed, with an overall complication rate of 8.6%. Infection and cerebrospinal fluid leaks are the most common problems. This article discusses techniques incorporated into personal methods to reduce the incidence of these complications.

Adolescent↗
PubMed1991

Is there an association between hemangioma and syndromes with dysmorphic features?

Most vascular birthmarks can be categorized, based on clinical and cellular criteria, as either (1) a hemangioma, or (2) a malformation, or (3) a macular stain. Macular stains are commonly seen in newborns, and they consist of faint vascular stains of the glabella, eyelids, and nuchal region called "nevus flammeus," "stork bite," "salmon patch," etc. Unfortunately, the term "hemangioma" is frequently applied to all three types of cutaneous vascular lesions. Usually, these disparate vascular anomalies are listed in association with various malformative syndromes and are generically labeled "hemangioma." This study attempts to define accurately the specific vascular anomalies seen in children born with syndromes with dysmorphic features. This review of five standard textbooks of genetics showed that the majority of vascular anomalies reported in syndromic newborns are not hemangiomas. Rather, they are macular stains, and the vast majority of these fade with time. Congenital telangiectasias and other vascular malformations (capillary, lymphatic, venous, arterial, and combinations thereof) also occur in association with dysmorphic syndromes. In contrast, hemangioma, the most common neonatal tumor, is seen only incidentally with rare dysmorphic conditions. Specifically, hemangioma was found to occur only in association with midline (sternal, abdominal) clefting, right-sided aortic arch coarctation, and with a constellation of sacral and genitourinary defects.

Arteriovenous Malformations↗
PubMed1991

Surgical correction of the craniofacial anomalies in Apert syndrome.

The craniofacial anomalies in Apert syndrome are some of the most obvious manifestations of a systemic disorder directed by an abnormal genome (pleiotropy). Although our knowledge of etiopathogenesis is woefully inadequate, imaginative surgical techniques have evolved to correct the major functional and aesthetic problems in this syndrome. During infancy, the frontal (or nasofrontal) skeleton is modeled and advanced in order to protect the proptotic globes and to expand the anterior cranial fossa. This procedure also sets the stage for midfacial advancement, accomplished in either early or late childhood. The timing depends on indications, both physiologic and psychologic. Subcranial midfacial advancement, with a variety of technical alternatives, is a safe procedure with an acceptable aesthetic outcome. For some children with prominent hypertelorbitism, a facial bipartition or another variation on orbital translocation may be indicated. By adolescence, the extent of normal mandibular growth beyond the lagging midface becomes obvious. After completion of skeletal growth, these patients benefit from adjustments of the maxilla and the mandible and by procedures to refine the forehead, eyelids, nose, and chin. Until the mechanism of acrocephalosyndactyly I is understood, further technical improvements are needed before these patients have a truly "normal" face. More attention should be focused on correction of the entire cranial vault.

Acrocephalosyndactylia↗
PubMed1990

The healing of bone and cartilage.

Transplantation of bone should be preceded by careful assessment of the recipient site. The function of the transplanted bone as an interposition graft, as an onlay graft, or in restoration or construction of a missing part of the skeleton must be considered. Cortical bone provides superior mechanical strength and can be incorporated with plate fixation to span interposition defects. Membranous bone used as onlay grafts for augmentation of craniofacial skeletal contour has been shown to be superior to endochondral grafts in maintaining volume. The use of rigid fixation to secure onlay grafts may eliminate the differences in resorption seen with membranous versus endochondral bone. The vascularity and quality of soft tissue at the recipient site may necessitate the use of vascularized bone or composite free tissue transfer. The calvarium is the most popular donor site for bone grafts used in craniofacial skeletal procedures. This membranous bone undergoes less resorption and revascularizes faster than endochondral bone. Cranial bone has excellent mechanical strength due to its large cortical component. The calvarial donor site causes less discomfort to the patient compared with rib or iliac crest, and the scar is well hidden. Harvesting and shaping cranial bone require special expertise, and there is potential morbidity. In cartilage transplantation, the surgeon must take into account the properties of viscoelasticity, the intrinsic balanced system of forces, and immunologic privilege. Cartilage deformed by an external force will tend to return to its original shape unless the deformation is maintained for several months. Surgical carving produces changes in the balance of intrinsic tensile and expansile forces, causing distortion in cartilage shape. Distortion can be minimized by carving in balanced cross-section. Carved cartilage grafts should be used for special indications in rhinoplasty. Autogenous cartilage is the framework of choice in ear construction. Composite grafts incorporating cartilage have been used successfully in eyelid reconstruction. Fresh autogenous cartilage is preferable to preserved allogeneic sources, as the latter undergo eventual resorption because there are no viable chondrocytes to maintain the matrix.

Bone Regeneration↗
PubMed1990

Calvarial sutural abnormalities: metopic synostosis and coronal deformation--an anatomic, three-dimensional radiographic, and pathologic study.

The purposes of this study were (1) to evaluate the histologic differences between synostotic versus deformational suture abnormalities and (2) to correlate these histologic findings with anatomic and three-dimensional computed tomographic (CT) scans. We examined three infants with premature metopic synostosis; one infant also had microcephaly trisomy 13 and curious overriding of the coronal sutures. The three-dimensional CT scans demonstrated obliteration of the metopic suture inferiorly. Histologic sections of this suture showed complete bony stenosis. The same pattern was found in all three infants, including the two infants with trigonocephaly who did not have trisomy 13 or microcephaly. In the trisomy 13 infant, the overlapped inferior coronal suture was obliterated on CT examination. However, histologic sections in this region showed a merging of bone; there was no synostosis. In summary, three-dimensional CT re-formation correlated with metopic suture histology. "Stenotic" fusion existed in all infants with trigonocephaly, those with normal and abnormal karyotypes, with and without microcephaly. However, three-dimensional CT re-formation of the trisomic infant showed opacification of the coronal suture in the areas of greatest overlap, whereas histology revealed a curious bone remodeling pattern, possibly a precursor to "deformational" craniosynostosis.

Abnormalities, Multiple↗
PubMed1990

Clinical and ultrastructural studies of Romberg's hemifacial atrophy.

Romberg's 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 less than 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 Romberg's disease involves chronic cell-mediated vascular injury and incomplete endothelial regeneration along branches of the trigeminal nerve (lymphocytic neurovasculitis).

Adult↗