J Med Cases

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

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website https://jmc.elmerpub.com

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 1.** End-Organ Involvement and Perioperative Concerns in Patients With Teebi Hypertelorism Syndrome
System	Clinical features	Anesthetic concerns
CNS: central nervous system; IV: intravenous.
Airway and craniofacial	Characteristic facial features including midface hypoplasia, micrognathia, short and broad nasal bridge	Difficult 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/palate	Difficult 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
Cardiovascular	Congenital 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 dilatation	Maintenance of systemic venous return is critical in shunting lesions.
		Potential for paradoxical embolism with septal defects and intracardiac shunts
CNS	CNS 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 involvement	Limb anomalies including small hands/feet, interdigital webbing, clinodactyly	Vascular 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 tract	Cryptorchidism (undescended testes) and shawl scrotum in males	Preoperative assessment of renal function based on past history with imaging (ultrasound) as needed
	Bicornuate uterus in females
Gastrointestinal tract	Umbilical hernia and/or omphalocele	Preoperative imaging based on physical assessment and history
	Congenital diaphragmatic hernias or tracheoesophageal
	Absent gallbladder
	Anorectal malformations

↓ Table 1. End-Organ Involvement and Perioperative Concerns in Patients With Teebi Hypertelorism Syndrome

System

Clinical features

Anesthetic concerns

CNS: central nervous system; IV: intravenous.

Airway and craniofacial

Characteristic facial features including midface hypoplasia, micrognathia, short and broad nasal bridge

Difficult 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/palate

Difficult 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

Cardiovascular

Congenital 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 dilatation

Maintenance of systemic venous return is critical in shunting lesions.

Potential for paradoxical embolism with septal defects and intracardiac shunts

CNS

CNS 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 involvement

Limb anomalies including small hands/feet, interdigital webbing, clinodactyly

Vascular 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 tract

Cryptorchidism (undescended testes) and shawl scrotum in males

Preoperative assessment of renal function based on past history with imaging (ultrasound) as needed

Bicornuate uterus in females

Gastrointestinal tract

Umbilical hernia and/or omphalocele

Preoperative imaging based on physical assessment and history

Congenital diaphragmatic hernias or tracheoesophageal

Absent gallbladder

Anorectal malformations