An International Journal of Anesthesiology, Pain Management, Intensive Care & Resuscitation

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Anaesthesia, Pain & Intensive Care

ISSN 1607-8322, ISSN (Online) 2220-5799

CLINIPICS – June 2015

Oro-facial-digital syndrome

Aparna Bagle-Girwalkar
Associate professor, Dept of anesthesiology, Dr D Y Patil Medical College, Pimpri,Pune, Maharashtra (India), Email: draparnabagle@gmail.com

ro-facial-digital syndrome(OFDS), autosomal recessive condition consists of a group of heterogeneous disorder characterized by abnormalities in oral cavity,face and digits.Associated phenotypic abnormalities that lead to delineation of 13 OFD subtypes withdifferent modes of inheritance.The features of different subtypes overlaps and some types are not yet well defined.¹ Classification system for OFDS continues to evolve as researchers found more affected individuals and learn more about this disease.^2,3

OFD type ІІ is characterized by frontal bossing,broad nasal bridge,midline cleft lip and palate,high arch palate,lobulated tongue,syndactly,polydactyl and duplication of first toe. Other systemic features include conductive deafness, congenital cardiac defects, choroidal coloboma and normal intelligence.Patient is 25 yr female with high arch palate,lobulated tongue,syndactly, polydactyl, normal hearing and intelligence and no cardiac and ophthalmic problem.

References:

Thomas S,Legendre M, Saunier S, Bessie’res B, Alby C,Bonnie’re M et al.TCTN3 mutation cause Mohr-Majeswki syndrome.Am J Hum Genet 2012:10(91):372-8?[PubMed][Free Full Text]
Oral-facial digital syndrome.Genetics home reference.ghr.nlm.nih-gov/condition/oral-facial digital syndrome.Reviewed Feb 2010, Published: Jan 17, 2013
Biswas A,Basu K,Chaterjee S,Ghosh JK,Sinha MK. Mohr-claussen oral-facial digital syndrome OFDS typeII.J Pak Med Assoc 2009:59:486-8[PubMed][Free Full Text]

Photograph 1: showing high arch palate and absent uvula

Photograph 2: showing lobulated tonge

Photograph 3: Showing syndactly of both foots

Photograph 4: Showing syndactly and polydactyl of both hands

CLINIPICS

Sliding hiatal hernia with GER The pictures of x-rays shown here were taken from a young 2 years old male child with severe dilatation of oesophagus and sliding hiatal hernia. He was severely malnourished with a weight of only 13 pounds. Massive reflux can be seen right up to proximal oesophagus. It requires special care to empty the contents of oesophagus and stomach at the time of induction of anesthesia to prevent aspiration pneumonitis.

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irst there was McSleepy^TM. Now it’s time to introduce the first intubation robot operated by remote control. This robotic system named The Kepler IntubationSystem (KIS), and developed by Dr. Thomas M. Hemmerling, McGill University Health Centre (MUHC) specialist and McGill University Professor of Anesthesia and his team, may facilitate the intubation procedure and reduce some complications associated with airway management.

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A neonate with distended abdomen

In surgery of the neonates and infants, one of the greatest factors in increased morbidity and mortality is hypothermia; especially in an unconscious, paralyzed patient under anesthesia. Appropriate measures must be undertaken to prevent hypothermia.

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CONGENITAL ABNORMALITIES

Fig. 1: Evisceration

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Oro-facial-digital syndrome

Oro-facial-digital syndrome(OFDS), autosomal recessive condition consists of a group of heterogeneous disorder characterized by abnormalities in oral cavity,face and digits.Associated phenotypic abnormalities that lead to delineation of 13 OFD subtypes withdifferent modes of inheritance.

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Endotracheal tube holder Tariq Hayat Khan, MCPS, FCPS Consultant Anesthesiologist & Pain Specialist, KRL Hospital, Islamabad (Pakistan)

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Congenital diaphragmatic hernia Prof. Naeem Khan Consultant & HoD, Pediatric Surgery, KRL Hospital, G-9/1, Islamabad (Pakistan)

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The Tec 6 desflurane vaporiser

Desflurane needs to be heated by a electrically powered heating element (H) to 39 °C, at which its SVP is approximately 1300 mmHg. The carrier gas flow is restricted by an orifice (O) so that the pressure of the carrier gas within the vaporiser is proportional to gas flow. This pressure is sensed by a differential pressure transducer (P), which adjusts a resistor (R1) so that the flow of desflurane out of the vaporising chamber is proportional to the carrier gas flow. This enables the output concentration to be made independent of carrier gas flow rate.

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NEONATAL MATTERS

Newborns, requiring surgery in prone position, should have pads under the chest and shoulders to allow free excursion of abdomen for assisted breathing; they require pulse oximetery probe, intravenous line, ECG leads, EtCO2 tube, ETT and head cover. (Professor Naeem Khan)

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A rotating bullet in the mediastinum Muhammad Faisal Khan, MCPS, FCPS*, Hameedullah, FCPS** * Senior Registrar, Department of Critical Care, King Khalid University, Riyadh (KSA) ** Aga Khan University, Karachi (Pakistan) Correspondence: Dr. Muhammad Faisal Khan,MCPS, FCPS, Senior Registrar, Department of Critical Care King Khalid University, Riyadh (KSA); e-mail: mfkdr.icu@gmail.com

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