Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 17, Number 4, April 2026, pages 141-145


Perioperative Care of a Four-Year-Old Child With Teebi Hypertelorism Syndrome: A Rare Craniofacial Disorder

Table

Table 1. End-Organ Involvement and Perioperative Concerns in Patients With Teebi Hypertelorism Syndrome
 
SystemClinical featuresAnesthetic concerns
CNS: central nervous system; IV: intravenous.
Airway and craniofacialCharacteristic facial features including midface hypoplasia, micrognathia, short and broad nasal bridgeDifficult mask ventilation, as midface hypoplasia and broad nasal bridge may prevent an effective mask seal
Oropharyngeal involvement including a small oral cavity or cleft lip/palateDifficult endotracheal intubation due to micrognathia and small oral cavity, which may impair alignment of oral-pharyngeal-laryngeal axes
Risk of upper airway obstruction during sedation with a native airway or following recovery from anesthesia
CardiovascularCongenital heart disease (patent ductus arteriosus, atrial or ventricular septal defects)Preoperative echocardiography is recommended.
Valvular involvement (aortic stenosis)Potential for sensitivity to myocardial depressants
Root dilatationMaintenance of systemic venous return is critical in shunting lesions.
Potential for paradoxical embolism with septal defects and intracardiac shunts
CNSCNS malformations and structural lesions including ventriculomegaly, agenesis of the corpus callosum, spinal dysraphism (spina bifida, tethered cord)Intracranial pressure alterations may require consideration of the impact of anesthetic agents or intraoperative maneuvers that impact intracranial pressure.
Spina bifida/myelocele or tethered cord (as noted in case discussion)Cognitive impairment may impact cooperation and communication during perioperative care.
Functional involvement including developmental delay, intellectual disability
Musculoskeletal and extremity involvementLimb anomalies including small hands/feet, interdigital webbing, clinodactylyVascular access including placement of peripheral IV and arterial cannula may be difficult. Ultrasound guidance is recommended.
Attention to positioning is required with padding of pressure points and protection of neural repair sites (spina bifida repair) during long procedures.
Genito-urinary tractCryptorchidism (undescended testes) and shawl scrotum in malesPreoperative assessment of renal function based on past history with imaging (ultrasound) as needed
Bicornuate uterus in females
Gastrointestinal tractUmbilical hernia and/or omphalocelePreoperative imaging based on physical assessment and history
Congenital diaphragmatic hernias or tracheoesophageal
Absent gallbladder
Anorectal malformations