DESCRIPTION A 2-week-old infant with unilateral cleft lip and palate (CLP) is brought to clinic by his parents. He was born full-term and the only associated abnormality is a small atrial septal defect. There is no family history of clefting.

Cleft Lip and Palate

Summary

Background The risk of malignant melanoma in congenital melanocytic naevi (CMN) is a matter of controversial and ongoing debate.



Objectives  The purpose of this systematic review is to provide a careful and detailed summary of the published data, including several recently published studies.



Methods  Articles on CMN (n = 1424) were retrieved from Medline, 1966–October 2005. Case reports and studies lacking relevant clinical information were excluded. Only systematic collections of cases were taken into consideration. Series with fewer than 20 patients or studies with a mean follow-up of <3 years were regarded as epidemiologically less significant.



Results  Fourteen articles were finally chosen for further analysis. The studies varied significantly with respect to study design (source of cases; retrospective vs. prospective analysis), age of patients, follow-up time, and naevus characteristics. The frequency of melanomas ranged between 0·05% and 10·7% and was significantly higher in smaller studies (P < 0·0001). In a total of 6571 patients with CMN who were followed for a mean of 3·4–23·7 years, 46 patients (0·7%) developed 49 melanomas. The mean age at diagnosis of melanoma was 15·5 years (median 7). By comparison with age-adjusted data from the Surveillance, Epidemiology and End Results database, we calculated that patients with CMN carry an approximately 465-fold increased relative risk of developing melanoma during childhood and adolescence. Primary melanomas arose inside the naevi in 33 of 49 cases (67%). In seven cases (14%), metastatic melanoma with unknown primary was encountered; in four cases (8%) the melanoma developed at an extracutaneous site. The risk of developing melanoma and the rate of fatal courses were by far highest in CMN ≥40 cm in diameter.



Conclusions  The overall risk of melanoma of 0·7% in all 14 studies was lower than expected. The higher incidence of melanomas in smaller studies indicates selection bias. The melanoma risk strongly depends on the size of CMN and is highest in those naevi traditionally designated as garment naevi. The median age of 7 years at diagnosis of melanoma points to a risk maximum in childhood and adolescence. Future studies on CMN should report: (i) diameter, percentage of body surface, and localization of the CMN; (ii) percentage of naevus area removed by excision or subject to dermabrasion or other superficial treatments; (iii) mean and median age at entry into the study; (iv) mean and median follow-up time; (v) details on each melanoma case; (vi) standardized morbidity ratio of melanoma; and (vii) percentage of neurocutaneous melanosis.

https://www.naevus-netzwerk.de/wp-content/uploads/Krengel_2006.pdf

Melanoma risk in congenital melanocytic naevi: a systematic review.

Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The syndrome is thought to represent an error in the morphogenesis of the embryonal neuroectoderm. We review 39 reported cases of neurocutaneous melanosis and propose revised criteria for diagnosis. Most patients with neurocutaneous melanosis presented in the first 2 years of life with neurologic manifestations of increased intracranial pressure, mass lesions, or spinal cord compression. Leptomeningeal melanoma was present in 62% of the cases, but even in the absence of melanoma, symptomatic neurocutaneous melanosis had an extremely poor prognosis. Useful diagnostic procedures include cerebrospinal fluid cytology and magnetic resonance imaging with gadolinium contrast. Patients may be aided by palliative measures such as shunt placement to reduce intracranial pressure. Dermatologists in their follow-up of patients with large or multiple congenital melanocytic nevi should be aware of this condition, to aid in prompt diagnosis and because the treatment of cutaneous lesions may be altered in the presence of symptomatic Nurocutaneous melanosis.

Neurocutaneous melanosis: Definition and review of the literature

Experience-dependent plasticity during development results in the emergence of highly adapted representations of the external world in the adult brain. Previous studies have convincingly shown that the primary auditory cortex (A1) of the rat possesses a postnatal period of sensory input-driven plasticity but its precise timing (onset, duration, end) has not been defined. In the present study, we examined the effects of pure-tone exposure on the auditory cortex of developing rat pups at different postnatal ages with a high temporal resolution. We found that pure-tone exposure resulted in profound, persistent alterations in sound representations in A1 only if the exposure occurred during a brief period extending from postnatal day 11 (P11) to P13. We also found that postnatal sound exposure in this epoch led to striking alterations in the cortical representation of sound intensity.

https://www.jneurosci.org/content/jneuro/27/1/180.full.pdf

Critical period window for spectral tuning defined in the primary auditory cortex (A1) in the rat.

The subject of the relationship between congenital nevocytic nevi and malignant melanomas has many controversial aspects. Data are insufficient to come to firm conclusions concerning how often melanomas supervene in such nevi and to determine the prognosis of those melanomas. What data are available indicate that malignant melanomas develop in a substantial percentage of large (giant) nevocytic nevi. This lends support to those who recommend surgical excision of such large lesions, when feasible. On the other hand, the information currently available concerning the association of malignant melanomas with medium- and small-sized congenital nevocytic nevi (i.e., those less than 20 cm in largest diameter) is scanty. Because of this lack of data on congenital nevocytic nevi, our current approach is to take into consideration the probability of cosmetic improvement by surgical removal in conjunction with education of the patient and/or family that malignant melanomas have been known to occur in congenital nevocytic nevi but are probably rare except in nevi of large size. Such information provided to the patient and/or family will often help in making the decision for removal or continued observation. A Congenital Nevocytic Nevus Registry has been established in the Oncology Section of the Skin and Cancer Unit at New York University Medical Center in the attempt to begin a long-term prospective study which might eventually provide some meaningful information concerning the natural history of such lesions, including the incidence of malignant melanomas.

Congenital nevocytic nevi and malignant melanomas.

Speech production and age at palatal repair were investigated in 80 cleft palate children. Children whose palates were repaired prior to the onset of speech production demonstrated significantly better speech than those whose palates were repaired between 12 and 27 months of age. The supposition that earlier palatal repair results in more normal speech development was, in fact, demonstrated in these cases. Rather than using chronologic age alone as the deciding factor in determining timing of initial palatal repair, the stage of each child's phonemic development should be considered if maximum speech potential is to be achieved and if speech development is to parallel normal noncleft peers. Determining this stage of development through early speech and language evaluations, beginning at 6 months of age, thus becomes an essential component in the habilitation of children with cleft palate. Continued research is needed to ensure against giving the obtainment of early speech normalcy disproportionate emphasis over craniofacial growth considerations. To this end, continued cooperative research between surgeons and speech pathologists is imperative in order to base these important decisions on substantiated findings.

John W. Curtin

Papers

Early cleft palate repair and speech outcome.

Incidence of malignancy in giant pigmented nevi.

Blunt trauma to the larynx and upper trachea: immediate treatment, complications and late reconstructive procedures.

Silicone pulleys and underlays in tendon surgery.

Osteochondroma of the mandibular condyle. Case report with histologic, radiographic and electromyographic observations.