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Malouf syndrome

Malouf syndrome (also known as "congestive cardiomyopathy-hypergonadotropic hypogonadism syndrome") is a congenital disorder that causes one or more of the following symptoms: mental retardation, ovarian dysgenesis, congestive cardiomyopathy, broad nasal base, blepharoptosis, and bone abnormalities, and occasionally marfanoid habitus (tall stature with long and thin limbs, little subcutaneous fat, arachnodactyly, joint hyperextensibility, narrow face, small chin, large testes, and hypotonia).

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Sudden infant death secondary to tonsillar impaction in the glottis: A case report
From Australian Journal of Oto-Laryngology, 7/1/01 by Malouf, John

We describe an unusual case of sudden and unexpected death in a child of nineteen months as a result of asphyxiation secondary to tonsillar impaction in the glottic aperture. The case illustrates a rare fatal complication of enlarged tonsils. We review the literature surrounding cases of intrinsic laryngeal obstruction.

Introduction

There is much in the literature regarding airway obstruction from foreign matter in both the paediatric and older age groups. The mortality appears high when this condition occurs at the extremes of age. In these age groups the diagnosis is not always obvious. The literature concerning airway obstruction from intrinsic structures is more sparse. A case of asphyxiation is described in which the lower pole of an enlarged but non-acutely inflamed tonsil obstructs the laryngeal inlet in an otherwise healthy 19-month-old child.

Case Report

A nineteen-month-old male child was found in his bed, prone, cyanotic and apnoeic. He had been seen sleeping approximately one hour previously. The parent attempted resuscitation and called the ambulance. The child could not be revived and life was pronounced extinct at the local hospital. The child had been well prior to the event. He had a history of breath holding episodes.

The body was free from signs of physical injury. The tonsils were noted to be "enlarged" particularly on the left side where it was markedly lobulated" (Fig. 1). Figure two shows the tonsil impacted in the laryngeal inlet. Small amounts of food material were present distal to the inlet. The lungs showed changes consistent with previous resuscitation. Other examination of the head and neck, limbs and body cavities was macroscopically and microscopically normal.

Histological analysis of the left tonsil revealed reactive changes only. It was noted again that the tonsil was enlarged in such a fashion (predominantly the lower pole) that it was almost pedunculated in configuration and it could be seen to occlude the laryngeal inlet with ease. The laryngeal inlet showed chronic inflammatory changes and the tongue showed focal congestion and mild inflammatory changes.

Routine viral serology was negative for TORCH, HSV, influenza A and B, Parainfluenza 1, 2, and 3, Adenovirus Resp. syncytial virus and Mycoplasma. CF testing was negative as was virology of lymph node biopsy. Cause of death was attributed to upper airway obstruction from the enlarged left-sided tonsil.

Discussion

This is an unusual cause of death. The tonsil was not found to be impacted in the laryngeal inlet, however it was demonstrated to easily obstruct the glottis as seen in figure two. The significance of the chronic inflammatory changes of the supraglottis is uncertain. If the obstruction were an acute event there would be no time for the changes of actue inflammation to develop. The pathologist also noted that there were food particles distal to the laryngeal inlet, possibly as a result of the resuscitation. He concluded that the tonsil had obstructed the glottis and caused death. Resuscitation may have dislodged it and allowed minute food particles to pass into the lower respiratory tract.

It is possible that the "breath holding" episodes the child exhibited previously were in fact intermittent and momentary obstruction of the glottis by the tonsil.

The fact that the tongue was inflamed also warrants further appraisal. Byard et al 1993 published a case of sudden infant death and acute posterior lingual inflammation. In this case death was attributed to "sudden unexpected death in an infant with acute posterior lingual inflammation". Here, the airway obstruction originated from an area of acute inflammation of the tongue base opposite, but not involving, the epiglottis. Indeed Byards case was consistent with a diagnosis of Sudden Infant Death Syndrome (SIDS) except for this inflammation of the tongue base. Interestingly, tongues of victims of SIDS have been shown to be larger than age matched controls (Siebert and Haas, 1991). Also, airways of SIDS victims may be narrower than vital peers (Tonkin and Beach, 1988).

Other cases of "intrinsic lesions" causing laryngeal obstruction include lingual thyroglossal duct cysts, Byard (1990) also kyphosis and arterial hypertension with the resultant compression of the trachea by the aorta. This latter case, reported by Wright and Alexander (1991), occurred in an elderly woman who developed respiratory distress, it is of interest that the obstruction disappeared with the induction of general anaesthesia because of the reduction in arterial blood pressure. Akhtar (1991) reported a case of a child with airway obstruction from a large mediastinal mass compressing the trachea and main bronchi.

The literature has many reports of foreign bodies in airways. Cases of intrinsic compression are less commonplace. This case is presented as a rare complication of tonsillar hypertrophy.

References

AKHTAR-T.M., RIDLEY-S. AND BEST-C.J. (1991) Unusual presentation of acute upper airway obstruction caused by an anterior mediastinal mass. Br-J-Anaesth. 1991 Nov; 67(5): 632-4

BYARD S.P., BOURNE A.J. AND SILVER M.M. (1990) The association of thyroglossal duct remnants with sudden death in infancy. Int. J. Pediatr. Otolaryngol. 20. 107-112

BYARD S.P. AND SILVER M.M. (1993) Sudden infant and acute posterior lingual inflammation. Int. J. Pediatr. Otolaryngol. 28. 78-- 81

SEIBERT JR. AND HASS J.E. (1991) Enlargement of the tongue in sudden infant death syndrome. Paedr. Pathol. 11. 813-826

TONKIN S. AND BEACH D. (1988) The vulnerability of the infant upper airway. In: Harper. R.M. and Hoffman H.J. (Eds.), Sudden Infant Death Syndrome. Risk Factors and Basic Mechanisms, PMA Publishing, New York. pp 417-422.

WILLINGER M., JAMES L.S. AND CATZ C. (1991) Defining the sudden infant death syndrome (SIDS); deliberations of an expert panel convened by the National Institute of Child Health and Human Development. Paediatr. Pathol. 11, 667-684.

JOHN MALOUF

Royal Brisbane Hospital

Queensland

John Malouf B.M., M.B.B.S., F.R.A.C.S.

The ENT Department,

The Royal Brisbane Hospital,

Queensland 4029

David Williams B.Sc.(Hons), M.B., Ch.B,M.Sc., Ph.D., F.R.C.PATH, D.M.J(Path), F.R.C.P.A.

Senior Specialist Forensic Pathologist,

The John Tonge Centre,

Brisbane

Rogan Coridge M.B.B.S., B.Sc., F.R.C.S. (ORLHNS)

Senior Registrar,

The ENT Department,

The Royal Brisbane Hospital,

Queensland 4029

Address for correspondence:

Dr John Malouf

66 Heussler Terrace

Brisbane

Queensland 4064

Telephone: (07) 33670665

Copyright Australian Society of Otolaryngology Head & Neck Surgery Ltd. Jul 2001
Provided by ProQuest Information and Learning Company. All rights Reserved

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