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CC BY-NC-ND 4.0 · Indian J Plast Surg 2008; 41(02): 222-225
DOI: 10.4103/0970-0358.44939 
Original Article
Association of Plastic Surgeons of India

Roberts-SC syndrome, a rare syndrome and cleft palate repair

Murthy Jyotsna
Department of Plastic Surgery, Sri Ramachandra University, Porur, Chennai - 600 116, India
,
Dewan Madhu
Department of Plastic Surgery, Sri Ramachandra University, Porur, Chennai - 600 116, India
,
Hussain Altaf
Department of Plastic Surgery, Sri Ramachandra University, Porur, Chennai - 600 116, India
› Author Affiliations
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Publication History

Publication Date:
15 January 2020 (online)

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ABSTRACT

Roberts SC syndrome is a rare syndrome with only 17 previously recognized patients reported in medical literature. The syndrome is characterized by multiple malformations, particularly, symmetrical limb reduction, craniofacial anomalies such as bilateral cleft lip and palate, micrognathia, and severe growth and mental retardation. Our patient, a young child of five years having Roberts-SC, was successfully operated for cleft palate under general anesthesia. The main features of the syndrome and the technical problems of anesthesia and surgery are discussed in this report

KEY WORDS

Cleft palate - phocomelia - Roberts-SC syndrome
 
  • 1 Roberts JB. A Child with double cleft of lip and palate, protrusion of the intermaxillary portion of upper jaw and imperfect development of the bones of the four extremities. Ann Surg 1919;70:252-4.
  • 2 Hermann J., Optiz J M: The Sc phocomelia and the Roberts Syndrome: nostalgic aspect. Eur. J Paediatr. 125:117-134, 1977
  • 3 Zergollern L, Hitrec V. Four siblings with Robert′s syndrome. Clin Genet 1982;21:1-6.
  • 4 Parry DM, Mulvihill JJ, Tsai S, Kaiser-Kupfer MI, Cowan JM. SC phocomelia syndrome, premature centromere separation and congenital cranial nerve paralysis in two sisters, one with malignant melanoma. Am J Med Genet 1986;24:653-72.
  • 5 Jabs EW, Tuck-Muller CM, Cusano R, Rattner JB. Studies of mitotic and centromeric abnormalities in Roberts syndrome: Implications for a defect in the mitotic mechanism. Chromosome 1991;100:251-1.
  • 6 Stioui S, Privitera O, Brambati B, Zuliani G, Lalatta F, Simoni G. First-trimester prenatal diagnosis of Roberts syndrome. Prenatal Diag 1992;12:145-9.
  • 7 Hirschhorn K, Kaffe S. Prenatal diagnosis of Roberts syndrome. Prenatal Diagn 1992;12(11):976.
  • 8 Kaffe S, Rose JS, Godmilow L, Walker BA, Kerenyi T, Beratis N, et al. Prenatal diagnosis of renal anomalies. Am J Med Genet 1977;1:241-51.
  • 9 Sinha AK, Verma RS, Mani VJ. Clinical heterogeneity of skeletal dysplasia in Roberts syndrome: A review. Hum Hered 1995;45:241-2.