Hallermann-Streiff syndrome: airway problems during anaesthesia.
AD Malde, SR Jagtap, SH Pantvaidya
Dept of Anaesthesiology, LTM Medical College, Sion, Bombay, Maharashtra.
A D Malde
Dept of Anaesthesiology, LTM Medical College, Sion, Bombay, Maharashtra.
In the patients with Hallermann-Streiff Syndrome, presence of mandibular hypoplasia and microstomia results in difficult intubation. The anaesthetic management of a three month old child with this syndrome for lensectomy is presented in this report. There was difficulty in maintaining the airway patency during induction which was solved with the help of a modified, improvised airway. Intubation was done after induction with a combination of inhalational and intravenous anaesthetic agents and muscle relaxant. Recognition of this syndrome should alert the physician to the possibility of difficulty in airway maintenance.
|How to cite this article:|
Malde A D, Jagtap S R, Pantvaidya S H. Hallermann-Streiff syndrome: airway problems during anaesthesia. J Postgrad Med 1994;40:216-8
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Malde A D, Jagtap S R, Pantvaidya S H. Hallermann-Streiff syndrome: airway problems during anaesthesia. J Postgrad Med [serial online] 1994 [cited 2022 Jun 27 ];40:216-8
Available from: https://www.jpgmonline.com/text.asp?1994/40/4/216/523
Hallerman Streiff syndrome or Francois Syndrome also known as oculomandibulodyscephaly with hypotrichosis was first described by Aubry in 1893. In 1948, Hallerman and in 1950 Streiff clarified the clinical features of this syndrome. Cardinal features are dyscephaly with bird facies, frontal or parietal bossing, dehiscence of sutures with open fontanelles, hypotrichosis of scalp, eyebrows and eyelashes, cutaneous atrophy of scalp and nose, mandibular hypoplasia, forward displacement of temporomandibular joints, high arched palate, small mouth, multiple dental anomalies and proportionate small stature. Ophthalmic features are microphthalmia, congenital cataracts, blue sclerae and nystagmus. The airway problems encountered during anaesthesia for lensectomy in an infant with this syndrome are described below and their management is highlighted.
Lensectomy was planned for this three-month-old female child weighing 1.75 kg. This was the first child of nonconsanguinous parents and was born by normal spontaneous delivery following eight months of uneventful pregnancy. Her birth weight was 1.5 kg. The physical examination revealed a small, frail infant with a height of 44 cm, head circumference of 32 cm and bird like facies. The skull was brachycephalic with frontal bossing. The cranial sutures were widely separated. Fontanelles were large. Skin over the scalp was atrophic and there was hypotrichosis of scalp, eyebrows and eyelashes. There was microphthalmia, bilateral cataracts, blue sclerae and nystagmus. The nose was thin and beak like with marked cutaneous atrophy. Mouth opening was small. She had hypoplastic mandible and high arched palate. Seven teeth, irregular in shape and implantation were present from birth [Figure:1]. Teeth were extracted prior to surgery as there were pus pockets around them and they were causing difficulty in feeding. Examination of respiratory and cardiovascular systems was unremarkable. Xray skull showed mandibular hypoplasia.
In the first instance, when the child was posted for lensectomy, certain difficulties were faced. The child was taken up after a preoperative starvation of four hours and premedication with intramuscular atropine (.02 mg). From the features of mandibular hypoplasia and microstomia, difficulty in intubation was anticipated. All accessories like various sizes of airways, laryngoscopes, endotracheal tubes, stylet, jet ventilation set and tracheostomy set were kept ready. A spontaneous inhalational induction was planned. Anaesthesia was induced with nitrous oxide, oxygen and halothane after preoxygenation using Jackson Rees circuit. There was difficulty in maintaining patent airway. The manoeuvres of opening the mouth and jaw retraction were not helpful. Nostrils were not allowing the passage of 6 PG infant feeding tube. The smallest airway (size 1 C, metallic) was found to be too large for the baby's small mouth. Because of its curvature, it could not be negotiated into position in the small oral cavity. During these attempts the child developed bradycardia. In presence of airway obstruction, artificial ventilation was not possible. A quick laryngoscopy was done. The glottic structures could not be visualised. However, oxygenation was carried out by oxygen insufflation directly over the poorly exposed larynx. The child responded to various resuscitative measures within a few seconds. Normal respiration, heart rate of 140 beats/minute and pink colour returned. The child was oxygenated till return of good cry. Surgery was deferred. The child was shifted to the intensive care unit. Subsequently, she developed upper respiratory tract infection which was treated with antibiotics.
A week later the child was rescheduled for surgery. A modified airway was prepared using a cut endotracheal tube attached to an endotracheal tube connector [Figure:2].
The length of the cut tube was 3 cm and internal diameter was 6 mm. After pre-oxygenation the child was induced with nitrous oxide, oxygen and halothane. The modified airway was used in such a way that the red rubber part was put in the mouth and wide part of the connector remained outside the mouth to which Jackson Ress circuit with mask was connected. This modified airway helped to keep the mouth open and to prevent the tongue from sticking to the palate. In addition, it provided a direct channel for the inhalational gases to reach the larynx. With this airway in place the child could be ventilated. Hence thiopentone (5 mg) followed by Suxamethonium (4 mg) were administered intravenously. On laryngoscopy only the tip of the epiglottis could be visualised. A 2.5 mm endotracheal tube with stylet was guided behind the epiglottis, which then slid in the trachea. After confirmation of the endotracheal placement of the tube, anaesthesia was maintained with nitrous oxide, oxygen and halothane. Muscle relaxation was obtained with vecuronium. Further intraoperative course was uneventful. Antibiotics were given, to prevent respiratory tract infection, postoperatively and the child was discharged on the fourth postoperative day without any complications.
In patients with Hallerman Streiff Syndrome securing an airway by intubation can pose a challenge,. Several features of this syndrome such as mandibular hypoplasia and microstomia can result in difficult intubation. Their natal teeth are brittle and may be easily broken or avulsed during laryngoscopy. The temporomandibular joint may be easily dislocated. At times, the temporomandibular joint is absent making the endotracheal placement of the tube by oral route impossible. Small nostrils, deviated nasal septum, high arched palate and anterior larynx preclude blind nasotracheal intubation. In this syndrome, the ascending ramus of the mandible is either underdeveloped or absent resulting in small mouth cavity. Further a normal sized tongue in a small mouth causes airway obstruction and in such a small infant, the smallest available airway becomes relatively large. Because of the typical curved shape of the metallic airway it was difficult to insert it in such a small oral cavity. Therefore, we prepared an airway, which was almost straight and hence could be negotiated in easily. With the assembly connected to a Jackson Rees circuit, it provided a direct channel up to the larynx for the inhalational gases.
The options available to circumvent the problems during difficult intubation are, awake intubation, intubation over a fibreoptic bronchoscope, retrograde intubalion and intubation under inhalational anaesthesia. In presence of anatomical abnormalities, awake intubation in a small infant such as this case, is almost impossible. Suitably sized fibreoptic bronchoscope is not available for this age group. For retrograde intubation also, child needs to be anaesthetised. Intubation under inhalational anaesthesia demands deeper plane of anaesthesia, whereas here we wanted to secure the airway by intubation as quickly as possible. Therefore, initially we used inhalational anaesthetic agent; since ventilation was possible with improvised airway in place, quick induction was achieved with intravenous anaesthetic agent and muscle relaxant. In the relaxed patient, we could secure the airway with an endotracheal tube.
The presence of natal teeth in this syndrome, do not permit effective feeding; hence these infants are undernourished and are prone to repeated respiratory tract infections.  Even minor infections cause much difficulty in breathing because of underdeveloped nasal structures. Fails and Schull have reported patients with respiratory difficulties requiring tracheostomy. Decrease in the thoracic compliance may be another factor in the development of pulmonary infections. Early deaths due to respiratory infections have been reported; therefore, pre and post-operative antibiotic cover is recommended.
It should be borne in mind that whenever infants with anatomical abnormalities like this are posted for surgery, there can be difficulty in airway maintenance and intubation. A modified airway such as this would go a long way in a safer management of the patient till intubation is achieved.
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