- Email: [email protected]
Congenital and acquired deafness in clefting and craniofacial syndromes
199
the wounds associated with B. melaninogenicus. This technique has been verified by anaerobic cultures in nine patients critically ill with crepitant gangrene. When one considers the unreliability of anaerobic cultures, the importance of this test becomes clear. The authors have listed three other organisms that emit a red fluorescence: they are Corynebacterium minutissimum, streptomyces albus, and Corynebacterium acnes. These organisms are found in wounds distinctly different than those caused by B. melaninogenicus and have other characteristic features that are described for the reader’s edification.-A. B. Sokol
Corticosteroid How Effective?-A
Treatment
of
Cutaneous
Hemangiomas:
L. E. BartosheClin Pediatr 17:625-638,
Report of 24 Children.
sky, M. Bull. and M. Feingold. (August), 1978.
Twenty-four patients with cavernous or mixed hemangiomas received corticosteroid treatment for interference with important body functions (airway compromise, feeding problems, sight and hearing, voiding, stooling, or limb function), thrombocytopenia, or a lesion with cosmetic concern to the family (I3 patients). Oral prednisone (3 mg/kg daily) was the usual treatment. Five patients (21%) had no response, I2 (50%) had a possible response, five (21%) had a probable response, and two (8%) had a definite response as defined by the authors in the text. One patient experienced marked interference with growth following 5 mo of treatment due to thrombocytopenia. The authors conclude that only about 30% of patients can be expected to improve when treated with steroids before IO mo of age. Other studies quoted had better results but not as strict criteria to grade response.Randall W. Powell
HEAD AND NECK Early
Skeletal
Release
in the
Infant
With
Craniofaciaf
J. G. McCarthy. P. J. Coccaro. F. Epstein, and J. M. Converse. Plast Reconstr Surg 62:335-346, (September), 1978. Dysostosis.
Craniofacial dysostosis is defined as premature synostosis of the cranical sutures associated with facial deformities. The importance of the cranial base sutures have been overlooked in classical neurosurgical strip craniectomies with a resulting facial deformity being neither ameliorated nor improved. The role of the sphenozygomatic suture is illuminated in this article based upon IO surgical cases. Three surgical techniques are employed in craniofacial dysostosis. If both coronal sutures are synostosed, with associated facial deformity of brachycephaly, bulging of the pterion and mild exophthalmos, a cranial facial stripping of both coronal sutures, including the sphenozygomatic suture, is performed. If, in addition to the above, there is a severe degree of exophthalmos, the Tessier type of frontal bone advancement is recommended. In addition to this, the osteotomies are extended down encompassing the sphenozygomatic sutures. Finally, in the infant with unilateral coronal synostosis and plagiocephaly, an advancement of the frontal bone on the affected side is the procedure of choice. The sphenozygomatic suture on the affected side is also opened with rongeurs.-A. B. Sokol
Congenital and Acquired Deafness in Clefting and Craniofacial Syndromes.
(July),
L. Bergstrom.
Cleft Palate
J 15:254-261,
1978.
Because craniofacial defects have a high potential for involving adjacent sensory organs, the clinician needs to know that patients may benefit from early evaluation and that evaluations have high yield. Therefore, 284 patients were reviewed whose ages ranged from 2 days to 50 yt. All underwent otolaryngologic examinations, audiometry, where possible pure tone and speech testing and acoustic bridge testing. In addition, mastoid films were taken. The author reports a congenital hearing loss occurring in 87% of patients having ocular defects, 67% in those having microcephaly, and 60% in those having cranial nerve palsy and in 50% of those having central nervous system defects. Many of these losses were sensorineural. Micrognathia had a 70% association of hearing loss and all but two were conductive in nature. Where pinna (ear) defects occurred, 75% of those affected had congenital hearing loss and nearly r/r of the 75% were conductive in nature. Palatal, facial, vertebral, and skull anomalies and posterior choanal atresia were associated with congenital hearing loss in 65 to 100%. Defects of the heart had congenital hearing loss in 72% of the cases. 83% of patients having renal defects had congenital hearing loss. However, in the study, patients with microtia/atresia had only a 5% incidence of renal anomalies. The author, therefore, concludes and recommends otologic and audiologic consultation routinely in patients having clefting and cranial facial syndromes.-A. B. Sokol Conductive
Hearing Loss in Patients
With Velopharyngeal
J. C. Heller, G. W. Gens, C. B. Croft, and D. G. Moe. Cleft Palate J 15:246-253, (July), 1978.
Insufficiency.
The literature supports the finding that in normal children, 3%lO% have a hearing loss as compared to 50%-100% in cleft palate children and 40%80% of those children with velopharyngeal insufficiency and intact palates. The study is involved with a further definition of the latter group. Seventy-seven patients with velopharyngeal insufficiency (VPI) not due to overt cleft palate were involved. This sample included 28 males and 49 females ranging in age from 4 to I6 yr. The etiology were divided into 3 groups: 17 with submucous cleft palate, 17 with palatal paresis, and 43 with palatopharyngeal disproportion. Audiologic and otologic tests were given. Audiologic evaluation revealed that 40% had hearing losses. Of these, 74% were conductive, 16% sensorineural and 10% mixed. There was an improvement in sensitivity as the subjects became older, and this finding agreed with that reported in the literature. Otologic evaluation revealed 44% had abnormalities of the tympanic membrane and 39% had significant air-borne gaps. There were no significant differences in the etiologic subgroups. This study revealed that conductive hearing loss in VP1 subjects, more closely resembled those with cleft palates than the normal population.-A. B. Sokol Early Nonsurgical Closure of Postoperative h4. D. Berkmnn.
Plast
Reconstr
Surg
Palatal Fistulae.
62:537-541,
(Octo-
ber), 1978. The author ative palatal
reports on the nonsurgical closure of postoperfistulae that occurred in I1 patients. The
the wounds associated with B. melaninogenicus. This technique has been verified by anaerobic cultures in nine patients critically ill with crepitant gangrene. When one considers the unreliability of anaerobic cultures, the importance of this test becomes clear. The authors have listed three other organisms that emit a red fluorescence: they are Corynebacterium minutissimum, streptomyces albus, and Corynebacterium acnes. These organisms are found in wounds distinctly different than those caused by B. melaninogenicus and have other characteristic features that are described for the reader’s edification.-A. B. Sokol
Corticosteroid How Effective?-A
Treatment
of
Cutaneous
Hemangiomas:
L. E. BartosheClin Pediatr 17:625-638,
Report of 24 Children.
sky, M. Bull. and M. Feingold. (August), 1978.
Twenty-four patients with cavernous or mixed hemangiomas received corticosteroid treatment for interference with important body functions (airway compromise, feeding problems, sight and hearing, voiding, stooling, or limb function), thrombocytopenia, or a lesion with cosmetic concern to the family (I3 patients). Oral prednisone (3 mg/kg daily) was the usual treatment. Five patients (21%) had no response, I2 (50%) had a possible response, five (21%) had a probable response, and two (8%) had a definite response as defined by the authors in the text. One patient experienced marked interference with growth following 5 mo of treatment due to thrombocytopenia. The authors conclude that only about 30% of patients can be expected to improve when treated with steroids before IO mo of age. Other studies quoted had better results but not as strict criteria to grade response.Randall W. Powell
HEAD AND NECK Early
Skeletal
Release
in the
Infant
With
Craniofaciaf
J. G. McCarthy. P. J. Coccaro. F. Epstein, and J. M. Converse. Plast Reconstr Surg 62:335-346, (September), 1978. Dysostosis.
Craniofacial dysostosis is defined as premature synostosis of the cranical sutures associated with facial deformities. The importance of the cranial base sutures have been overlooked in classical neurosurgical strip craniectomies with a resulting facial deformity being neither ameliorated nor improved. The role of the sphenozygomatic suture is illuminated in this article based upon IO surgical cases. Three surgical techniques are employed in craniofacial dysostosis. If both coronal sutures are synostosed, with associated facial deformity of brachycephaly, bulging of the pterion and mild exophthalmos, a cranial facial stripping of both coronal sutures, including the sphenozygomatic suture, is performed. If, in addition to the above, there is a severe degree of exophthalmos, the Tessier type of frontal bone advancement is recommended. In addition to this, the osteotomies are extended down encompassing the sphenozygomatic sutures. Finally, in the infant with unilateral coronal synostosis and plagiocephaly, an advancement of the frontal bone on the affected side is the procedure of choice. The sphenozygomatic suture on the affected side is also opened with rongeurs.-A. B. Sokol
Congenital and Acquired Deafness in Clefting and Craniofacial Syndromes.
(July),
L. Bergstrom.
Cleft Palate
J 15:254-261,
1978.
Because craniofacial defects have a high potential for involving adjacent sensory organs, the clinician needs to know that patients may benefit from early evaluation and that evaluations have high yield. Therefore, 284 patients were reviewed whose ages ranged from 2 days to 50 yt. All underwent otolaryngologic examinations, audiometry, where possible pure tone and speech testing and acoustic bridge testing. In addition, mastoid films were taken. The author reports a congenital hearing loss occurring in 87% of patients having ocular defects, 67% in those having microcephaly, and 60% in those having cranial nerve palsy and in 50% of those having central nervous system defects. Many of these losses were sensorineural. Micrognathia had a 70% association of hearing loss and all but two were conductive in nature. Where pinna (ear) defects occurred, 75% of those affected had congenital hearing loss and nearly r/r of the 75% were conductive in nature. Palatal, facial, vertebral, and skull anomalies and posterior choanal atresia were associated with congenital hearing loss in 65 to 100%. Defects of the heart had congenital hearing loss in 72% of the cases. 83% of patients having renal defects had congenital hearing loss. However, in the study, patients with microtia/atresia had only a 5% incidence of renal anomalies. The author, therefore, concludes and recommends otologic and audiologic consultation routinely in patients having clefting and cranial facial syndromes.-A. B. Sokol Conductive
Hearing Loss in Patients
With Velopharyngeal
J. C. Heller, G. W. Gens, C. B. Croft, and D. G. Moe. Cleft Palate J 15:246-253, (July), 1978.
Insufficiency.
The literature supports the finding that in normal children, 3%lO% have a hearing loss as compared to 50%-100% in cleft palate children and 40%80% of those children with velopharyngeal insufficiency and intact palates. The study is involved with a further definition of the latter group. Seventy-seven patients with velopharyngeal insufficiency (VPI) not due to overt cleft palate were involved. This sample included 28 males and 49 females ranging in age from 4 to I6 yr. The etiology were divided into 3 groups: 17 with submucous cleft palate, 17 with palatal paresis, and 43 with palatopharyngeal disproportion. Audiologic and otologic tests were given. Audiologic evaluation revealed that 40% had hearing losses. Of these, 74% were conductive, 16% sensorineural and 10% mixed. There was an improvement in sensitivity as the subjects became older, and this finding agreed with that reported in the literature. Otologic evaluation revealed 44% had abnormalities of the tympanic membrane and 39% had significant air-borne gaps. There were no significant differences in the etiologic subgroups. This study revealed that conductive hearing loss in VP1 subjects, more closely resembled those with cleft palates than the normal population.-A. B. Sokol Early Nonsurgical Closure of Postoperative h4. D. Berkmnn.
Plast
Reconstr
Surg
Palatal Fistulae.
62:537-541,
(Octo-
ber), 1978. The author ative palatal
reports on the nonsurgical closure of postoperfistulae that occurred in I1 patients. The