© Borgis - New Medicine 1/2001, s. 36-38
Zofia Dudkiewicz1, Maria Hortis-Dzierzbicka1, Ewa Sekula2, Wlodzimierz Piwowar1
Unique method of treatment of the Robin sequence
1Department of Paediatric and Adolescent Surgery, National Research Institute for Mother and Child, Warsaw, Poland, Centre for Craniofacial Disorders
Head: Associate Professor Zofia Dudkiewicz, M.D.
2Department of Infant Care National Research Institute for Mother and Child, Warsaw, Poland
Head: Professor A. Milanowski, M.D.
Summary
The authors present a unique protocol for the management of newborns and infants with Robin sequence, which has been employed at the National Institute for Mother and Child since the nineties. The target is to manage these children throughout early infancy and execute a one-stage palatoplasty and subperiostal release of the floor of the mouth (SRFM) when the patients are 3-4 months old. This allows for elimination of factors that result in respiratory insufficiency and feeding problems in these children. This therapeutic method is completely different from the commonly employed methods used in Robin sequence as an element of early combined surgical treatment.
The Robin sequence, consisting of micrognathia, cleft secondary palate, and upper airway obstruction is a congenital anomaly that appears either as an isolated Robin sequence or as a Robin sequence with concomitant defects and/or dysmorphic features, thus forming either a well-known or a hitherto unclassified syndrome. An extensive review of syndromic diagnoses associated with the Robin sequence has been done by Shprintzen (10, 11). Upper airway obstruction results in hypoxia, and while the infant is bottle-fed, he aspirates the formula and vomits, which in turn leads to aspiration pneumonia. Frequent incidents of hypoxia and feeding difficulties result in severe malnutrition and a failure to thrive. Prevention and treatment of upper airway obstructions constitute the basic management principle in children with this defect.
The term „Robin sequence ” is associated with recognition of the hypothesis that mandibular abnormalities may result in secondary anomalies that appear as a sequence of non-specific symptoms. These symptoms are non-specific since - as has been reported by Sadevitz after Cohen (7) - the etiology of developmental mandibular defects may be diversified. Among the causes of mandibular hypoplasia, Sadevitz includes:
1. A positional deformity of the mandible with a normal mandibular growth potential. Decreased volume of amniotic fluid in the uterus does not allow the foetal head to grow normally. The bending of the head provides a mechanical obstacle in mandibular growth. Other causes of intrauterine craniofacial compression include bigeminal or plural pregnancy, uterine abnormalities and tumours, or abnormal zygotic implantation. These factors may trigger deformities, while the growth potential of the mandible remains normal.
2. Intrinsic mandibular hypoplasia may be an oral manifestation of various syndromes, such as Stickler syndrome, which - according to data presented by Hermann and Opitz and confirmed by Shprintzen in 1988, occurs in one third of all children born with Robin sequence.
3. Neurologic or neuromuscular anomalies (Carey et al.), such as myotonic dystrophy and arthrogryposis. According to Smith, in the majority of patients arthrogryposis results in contractures secondary to neurological disturbances.
4. Diseases of the connective tissue, e.g. Larsen´s syndrome (multiple pterygium syndrome). In this syndrome, the position of the foetus is abnormal and functional disturbances result.
Regardless of the aetiology, in patients with Robin sequence the predominant problem lies in respiratory insufficiency, which is associated with the hypoplastic mandible (the tongue being displaced into the palatopharyngeal space), various forms of cleft palate, or a deformity within the palatopharyngeal ring. Such a configuration of anomalies is particularly life-threatening and, similar to gastro-oesophageal reflux, it results in respiratory and gastrointestinal disturbances and failure to thrive. Upper airway obstruction in neonates and infants with Robin sequence may lead to prolonged hypoxia, which can result in brain damage. The gravest problem is posed by the necessity of managing a child with Robin sequence throughout the neonatal period and infancy, when upper airway obstruction is the most pronounced. In recent years, the literature has included numerous reports concerning various management protocols. However, there is no uniform generally accepted method of management. Surgical correction of the cleft palate is performed in 1-2 year-old children, as in patients with other types of orofacial clefting (6).
In recent years, with the improvement of neonatal care, progress in respiratory assessment, respiratory therapy, surgical techniques and postoperative care, the effects of surgical management have contributed to decreased morbidity and mortality rates in these patients.
A unique management protocol for children with Pierre Robin sequence has been developed in the Institute for Mother and Child, Warsaw, Poland. This report presents therapeutic results achieved employing this protocol in children with isolated Robin sequence.
Material and methods
On the basis of the available literature and our experience in a team approach to treatment of Robin sequence, we are able to develop a causative model of conservative and surgical treatment for this defect. The principle underlying the conservative treatment protocol is to manage them successfully throughout early infancy, prepare such patients for an early surgical repair of the cleft palate, and provide appropriate postoperative care.
The material included 34 children with Robin sequence managed at the Centre for Craniofacial Disorders, Department of Paediatric and Adolescent Surgery, National Research Institute for Mother and Child, Warsaw, Poland, in the period 1992-1999. All newborns and infants less than 3 months old had previously been admitted to the Department of Infant Care of our institute, where they were diagnosed, treated and prepared for surgery on the following principles (8):
– The children were maintained in a position preventing upper airway obstruction (a prone, lateral recumbent or other position). In cases of severe respiratory disturbances they were always placed in a prone position with shoulders supported by rolls.
– Cardiac activity and blood oxygen saturation were continuously monitored (depending on indications, passive oxygen therapy or assisted respiration were employed).
– A nasogastric feeding tube was inserted and constituted the sole route for feeding.
– Diagnostic tests aiming at detection of infections were followed by appropriate treatment when necessary.
– Diagnostic studies were performed to disclose other congenital anomalies (consultations with a cardiologist, otolaryngologist, ophthalmologist, neurologist and geneticist).
– Diagnostic studies were carried out to detect gastro-oesophageal reflux (GER), such as 24-hour oesophageal pH-metry (a Marck III gastrograph with an antimony probe). Additionally, contrast X-ray and ultrasound examinations of the oesophagus were performed.
Infants and older children were admitted directly to the Department of Surgery, where the same diagnostic and therapeutic protocol was implemented while they were prepared for surgery. All the children experienced episodes of respiratory insufficiency, and resuscitation was necessary in the majority of the patients. When the power to decide rested with us, the surgery was performed on children that were 3 months old and whose body weight was above 3 kg.
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Piśmiennictwo
1. Argamaso RV: Glossopexy for upper airway obstruction in Robin sequence, CP Craniofac J 1992, 29:232-238. 2. Bull MJ, Givan DC, et al.: Improved outcome in Pierre Robin sequence: Effect of multidisciplinary evaluation and management, Pediatrics 1990, 86:294-301. 3. Caouette-Laberge L, Plamondon C, Larocque Y: Subperiosteal release of the floor of the mouth in Pierre Robin sequence: Experience with 12 cases, CP Craniofac J 1996, 33:468-472. 4. Delorme RP, Laroque Y, Caouette-Laberge L: Innovative surgical approach for the Pierre Robin Anomalad´: Subperiosteal Release of the Floor of the Mouth musculature, Plast Reconstr Surg 1989, 83:960-964. 5. Dudkiewicz Z, Sekula E, Nielepiec-Jalosinska A: Gastroesophageal reflux in the Pierre Robin sequence. Early surgical treatment, CP Craniofac J 2000, 37:205-208. 6. Lehman GS, Fishman IRA, Neiman GS: Treatment of cleft palate associated with Robin sequence: Appraisal of risk factors. CP Craniofac J 1995, 32:25-29. 7. Sadevitz V: Robin sequence: Changes in thinking leading to changes in patient care, CP Craniofac J 1992, 29:246-253. 8. Sekula E: Leczenie zachowawcze niemowlat z sekwencja Pierre Robin. Rozprawa na stopien doktora nauk medycznych, Warszawa 1998. 9. Sher AE: Mechanisms of airway obstruction in Robin sequence: Implications for treatment, CP Craniofac J 1992, 29:224-231. 10. Shprintzen RJ: Pierre Robin, micrognathia and airway obstruction: The dependency of treatment on accurate diagnosis. Intern Anesth Clin 1998, 26:64-71. 11. Shprintzen RJ:The implication of the diagnosis of Robin sequence, CP Craniofac J, 1992, 29:205-209. 12. Singer L, Sidoti EJ: Paediatric management of Robin sequence, CP Craniofac J 1992, 29:220-223.
