Pai syndrome: a report of a case and review of the literature

被引:22
作者
Al-Mazrou, KA
Al-Rekabi, A
Alorainy, IA
Al-Kharfi, T
Al-Serhani, AM
机构
[1] King Abdulaziz Univ Hosp, Dept Otolaryngol, Riyadh 11411, Saudi Arabia
[2] King Saud Univ, Coll Med, King Khalid Univ Hosp, Dept Histopathol, Riyadh 11461, Saudi Arabia
[3] King Saud Univ, Coll Med, King Khalid Univ Hosp, Dept Radiol, Riyadh 11461, Saudi Arabia
[4] King Saud Univ, Coll Med, King Khalid Univ Hosp, Dept Pediat, Riyadh 11461, Saudi Arabia
关键词
Pai syndrome; upper lip cleft; cutaneous polyps; intracranial lipoma;
D O I
10.1016/S0165-5876(01)00555-9
中图分类号
R76 [耳鼻咽喉科学];
学科分类号
100213 ;
摘要
Pai syndrome is a rare congenital disorder first described in 1987. The main clinical features of the syndrome include median cleft of the upper lip, intra-cranial lipoma, and cutaneous polyps. Only four cases have been described previously. This is the fifth who is a twin of Arabian descent to be reported. Full description of the clinico-pathological features and a review of the relevant medical literature is presented. To the best of our knowledge, this is the first case of Pai syndrome in a twin in the English literature. (C) 2001 Published by Elsevier Science Ireland Ltd.
引用
收藏
页码:149 / 153
页数:5
相关论文
共 18 条
[1]  
Chmielik M, 1993, Otolaryngol Pol, V47, P274
[2]  
Ducroz V, 1995, Ann Otolaryngol Chir Cervicofac, V112, P218
[3]   Nasal lipomas presenting as part of a syndromic diagnosis [J].
Hollis, LJ ;
Bailey, CM ;
Albert, DM ;
Hosni, A .
JOURNAL OF LARYNGOLOGY AND OTOLOGY, 1996, 110 (03) :269-271
[4]  
Kash F, 1996, INT J NEURORADIOL, V2, P109
[5]   Midline developmental anomalies with lipomas in the corpus callosum region [J].
Kieslich, M ;
Ehlers, S ;
Bollinger, M ;
Jacobi, G .
JOURNAL OF CHILD NEUROLOGY, 2000, 15 (02) :85-89
[6]   Median cleft of upper lip and pedunculated skin masses associated with de novo reciprocal translocation 46,X,t(X;16)(q28;q11.2) [J].
Masuno, M ;
Imaizumi, K ;
Fukushima, Y ;
Tanaka, Y ;
Ishii, T ;
Nakamura, M ;
Kuroki, Y .
JOURNAL OF MEDICAL GENETICS, 1997, 34 (11) :952-954
[7]   A case of Pai syndrome [J].
Mishima, K ;
Mori, Y ;
Minami, K ;
Sakuda, M ;
Sugahara, T .
PLASTIC AND RECONSTRUCTIVE SURGERY, 1999, 103 (01) :166-170
[8]   DEVELOPMENTAL NASAL ANOMALIES [J].
MORGAN, DW ;
EVANS, JNG .
JOURNAL OF LARYNGOLOGY AND OTOLOGY, 1990, 104 (05) :394-403
[9]   PEDUNCULATED CLUB-SHAPED SWELLING IN THE NOSTRIL [J].
OHSAKI, M ;
IWAHIRA, Y ;
MARUYAMA, Y .
PLASTIC AND RECONSTRUCTIVE SURGERY, 1992, 89 (01) :128-130
[10]   MEDIAN CLEFT OF THE UPPER LIP ASSOCIATED WITH LIPOMAS OF THE CENTRAL-NERVOUS-SYSTEM AND CUTANEOUS POLYPS [J].
PAI, GS ;
LEVKOFF, AH ;
LEITHISER, RE .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1987, 26 (04) :921-924